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RARE TUMORS
ISSN 2036-3605 - eISSN 2036-3613Editor-in-Chief
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Prakash Adhikari, Department of ENT and Head and Neck Surgery, GMS Memorial Academy of ENT and Head and Neck Studies, TU Teaching Hospital, Kathmandu, Nepal
Rafael Álvarez-González, Graduate School of Biomedical Sciences, University of North Texas, Healt Science Center at Forth Worth, Forth Worth, Texas, USA Armando Bartolazzi, Cellular and Molecular Tumor Pathology Laboratory, Cancer
Center Karolinska, Karolinska Hospital, Stockholm, Sweden
Archit Bhatt, Department of Neurology and Ophtalmology, Clinical Center East Lansing, East Lansing, Michigan, USA
Kevin Camphausen, Radiation Oncology Branch, National Cancer Institute, Bethesda, Maryland, USA
Joseph R. Carver, Abramson Cancer Center, University of Pennsylvania, Pennsylvania, USA
William Cho, Department of Clinical Oncology, Queen Elizabeth Hospital, Hong Kong Domenico Coppola, Anatomic Pathology and Neuroendocrine Cancer Research
Divisions, Moffit Cancer Center and Research Institute, Tampa, Florida, USA Undurti N. Fams Das, UND Life Sciences, Shaker Heights, Ohio, USA
Ozsahin E. Mahmut, Department of Radiooncology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
Jimmy Thomas Efird, Centre of Health Vulnerable Populations, University of Carolina, Greensboro, Carolina, USA
Amany Elwakkad, National Research Center, Cairo, Egypt
Gulgun Engin, Department of Radiology, Faculty of Medicine, Istanbul University, Istanbul, Turkey
Guy Eslick, Harvard School of Public Health, MA, USA
Patricia D. Evilliers, Department of Anatomic Pathology, University of Alabama at Birmingham, Birmingham, USA
Luis E. Fayad, Department of Lymphoma & Myeloma, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
Faris Farassati, Department of Medicine, University of Kansas School of Medicine, Kansas City, Kansas, USA
Ali Gholamrezanezhad, Research Institute for Nuclear Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
Samy Lewiz Habib, Department of Medicine, Division of Nephrology, University of Texas Health Science Center, San Antonio, Texas, USA
Paul H. Hartel, Broaddus Hospital of Davis Health System, Elkins, West Virginia, USA Ghulam Sarwar Hashmi, Department of Oral and Maxillofacial Surgery, Z.A. Dental
College, Medical Colony, A.M.U. Aligarh, India
Joseph Herman, Department of Oncology, The Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins School of Medicine, Baltimore Maryland USA Kanya Honoki, Department of Orthopedic Surgery, Nara Medical University, Nara,
Japan
Charles Hsu, Division of Plastic and Reconstructive Surgery, Stanford University Medical Center, Palo Alto, CA, USA
Sergio Huerta, Dallas VA Medical Center Surgical Services, Dallas, Texas, USA Abdul Hussain, Minimal Access Unit, General Surgery Department, Princess Royal
University Hospital, London, UK
Rafael Jimenez, Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA
Bleddyn Jones, Gray Institute for Radiation Oncology and Biology, University of Oxford, Oxford, UK
Honoki Kanya, Department of Orthopedic Surgery, Nara Medical University, Nara, Japan
Babak Kateb, Intraoperative Surgical Planning Society, Los Angeles, CA, USA Sunali Khanna, Department of Oral Medicine & Radiology, Nair Hospital Dental
College, Mumbai, India
Boris Kirshtein, Department of Surgery, Soroka University Medical Center, Beer
Sheva, Israel
Antoniades Konstantinos, School of Dentistry, Aristotle University of Thessaloniki, Thessaloniki, Greece
Masafumi Koshiyama, Department of Ob/Gy at Otsu Red Cross Hospital, Otsu, Japan Sunil Krishnan, Radiation Oncology, Gastrointestinal Translational Research, M.D.
Anderson Cancer Center, Houston, TX, USA
Calin Lazar, Department of Plastic and Reconstructive Surgery, Rouen University Hospital, Rouen, France
Wei Li, Institute of Biomedical Engineering, School of Control Science and Engineering, Shandong University, Shandong, China
Simon Lo, Department of Radiation Medicine, The Ohio State University Arthur G. James Cancer Hospital, Columbus, OH, USA
Lorenzo Lo Muzio, Surgical Sciences Department, Faculty of Medicine, University of Foggia, Foggia, Italy
Paulette M. Fauceglia, Roswell Park Cancer Institute, Buffalo, New York, USA Sridhar Mani, Departments of Medicine and Molecular Genetics, Division of
Oncology, Albert Einstein School of Medicine, New York, USA Hiroyuki Matsubayashi, Shizuoka Cancer Center, Shizuoka-ken, Japan Toshihiro Matsuo, Department of Artificial Joints and Biomaterial, Hiroshima
University, Hiroshima, Japan
Axel Merseburger, Department of Urology, Eberhard-Karls-University, Tübingen, Germany
Oliver Micke, Department of Radiotherapy and Radiation Oncology, Franziskus Hospital, Bielefeld, Germany
Rene Mirimanoff, Service de Radio-Oncologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
Luca Morelli, Operative Unit of Patological Anatomy and Cytological Diagnostics, P.O. S. Chiara, Hospital of Trento, Trento, Italy
Charbel D. Moussallem, Orthpedic Surgery, Faculty of Medical Sciences, Lebanese University, Beirut, Lebanon
Hidenari Nagai, Division of Gastroenterology and Hepatology, Toho University Medical Center, Omori Hospital, Tokyo, Japan
Tan Dat Ngyuen, Department of Radiation Oncology, Institut Jean-Godinot, Reims, France
Athanasios Papatsoris, University of Athens, Athens, Greece
Nicholas A. Pavlidis, Department of Medical Oncology, School of Medicine, University of Ioannina, Ioannina, Greece
Yi Chu Pei, Department of Surgical Pathology, Changhua Christian Hospital, Changhua, Taiwan
Shahid Pervez, Department of Pathology and Microbiology, Agakhan University Hospital, Karachi, Pakistan
Camillo Porta, IRCCS San Matteo University Hospital Foundation, Pavia, Italy Fernando Quevedo, Division of Medical Oncology, Mayo Clinic, Rochester, MN, USA Malcolm Schinstine, Division of Pathology, Hilo Medical Center Laboratories, Hilo,
Hawaii, USA
Andrzej Semczuk, Lublin Medical University, Lublin, P oland
Khan Shah Alam, Department of Orthopaedics, All India Institute of Medical Sciences Ansari Nagar, New Delhi, India
Mark G. Shrime, Department of Otolaryngology/Head and Neck Surgery, Boston University Medical Center, Boston, Massachusetts, USA
Vernon Keith Sondak, Department of Cutaneous Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA
Giuseppe Spriano, Department of Otolaryngology/Head and Neck Surgery, National Cancer Institute Regina Elena, Rome, Italy
Keith Stubbs, University of Western Australia, Perth, Australia
Fabio Tavora, Department of Genitourinary Pathology, Armed Forces Institute of Pathology, Washington, DC, USA
Juliette Thariat, Department of Radiation Oncology, Anti Cancer Center Antoine-Lacassagne, University Nice Sophia-Antipolis, Nice, France
Takeshi Tomonaga, Department of Molecular Diagnosis, Graduate School of Medicine, Chiba University, Chiba Japan
Mark Gerard Trombetta, Department of Radiation Oncology, West Penn Allegheny Health System Allegheny General Hospital, PA, USA
Lyuba Varticovski, Laboratory of Human Carcinogenesis, Center for Cancer Research, National Cancer Institute, Bethesda, MD, USA
Salvador Villa I Freixa, Department of Radiation Oncology, Hospital Universitari Germans Trías, Badalona, Catalunya, Spain
Takuya Watanabe, Department of Internal Medicine and Gastroenterology, Medical Hospital, The Nippon Dental University of Life Dentistry at Nigata, Nigata, Japan Shigeru Yamada, Research Center for Charged Particle Therapy, National Institute of
Radiological Sciences, Chiba, Japan
Leandra Náira Zambelli Ramalho, Department of Pathology, Faculty of Medicine of Ribeirão Preto, University of São Paulo, Ribeirdo Preto, SP, Brazil
Metastatic pleomorphic sarcoma to left atrium
Ammar H. Hawasli, Rachael Cayce, Trung Luong, Evelyn Taiwo, Michael N. Feliciano, Sharon C. Reimold, John M. DiMaio,
Barbara B. Haley...1
Squamous cell carcinoma of the scrotum in a Nigerian: case report
Jerome E. Azike, N.O. Chukwujama, T.C. Oguike...4
Extraosseous osteosarcoma in Ibadan: case series over a 20-year period
Temitope O. Alonge, Henry A. Obamuyide, Gabriel Olabiyi Ogun...6
A multimodal approach to the treatment of bilateral choroidal metastases from thyroid carcinoma
Maria Grazia Fabrini, Federica Genovesi-Ebert, Franco Perrone, Mario De Liguoro, Clara Giovannetti, Fausto Bogazzi, Stanislao Rizzo,
Enio Martino, Luca Cionini...9
Pleomorphic sarcoma metastatic to the duodenum?
Fabio R. Tavora, Allen P. Burke ...12
Complete small bowel obstruction caused by metastasis from primary nasopharyngeal carcinoma
Chi Pan Lau, Edwin Pun Hui, Anthony Tak-Cheung Chan ...16
Diagnosis of vulvar lesions by non-invasive optical analysis: a pilot study
Anne-Therese Vlastos, Igor Charvet, Ilaria Dellacasa, Federica Capanna, Marie-Françoise Pelte, Philippe Thueler,
Michel Saint-Ghislain, Christian Depeursinge, Paolo Meda...18
Congenital giant melanocytic nevi
Ghulam S. Hashmi, Syed S. Ahmed, Shahla Khan ...23
Intramedullary capillary hemangioma of the thoracic spine: case report and review of the literature
Rahul Kasukurthi, Wilson Z. Ray, Spiros L. Blackburn, Eriks A. Lusis, Paul Santiago...26
A case report of surgical debulking for a huge mass of elephantiasis neuromatosa
Manabu Hoshi, Makoto Ieguchi, Susumu Taguchi, Shinya Yamasaki...29
Value of centrifugated liquid-based cytology by Papanicolaou and May-Grünwald in oral epithelial cells
Hussain Gadelkarim Ahmed, Ali Mahmmoud Edris, Eneel Ahmed Mohmed, Mohammed Omer M. Hussein ...31
Retroperitoneal lipoma arising from the urinary bladder
Shingo Ukita, Masafumi Koshiyama, Megumi Ohnaka, Naoyuki Miyagawa, Yukio Yamanishi, Fumitomo Nishimura,
Michikazu Nagura, Tomoko Kim, Masaya Hirose, Tomoyuki Shirase, Hisato Kobayashi, Hiroshi Ozasa ...34
Primary breast lymphomas
Olivier Julen, Ilaria Dellacasa, Marie-Françoise Pelte, Bettina Borish, Christine Bouchardy, Federica Capanna, Georges Vlastos,
Jean-Bernard Dubuisson, Anne-Thérèse Vlastos ...36
Neuroblastoma occurring in a 38-year old Nigerian man: a rare finding
Martin A. Nzegwu, Aloy Aghaji...42
Primary lymphoepithelial carcinoma of the parotid gland in a North African woman
Soumaya Ben Abdelkrim, Amel Trabelsi, Faten Hammedi, Monia Omezzine, Soumaya Rammeh, Atef Ben Abdelkader,
Badreddine Sriha ...44
A review of the history, epidemiology and treatment of squamous cell carcinoma of the scrotum
Jerome E. Azike ...47
Assessment of cytological atypia, AgNOR and nuclear area in epithelial cells of normal oral mucosa exposed to toombak and smoking
Hussain Gadelkarim Ahmed, Abd-Elraheem Ali Babiker ...50
Desmoplastic small round cell tumor: impact of 18F-FDG PET induced treatment strategy in a patient with long-term outcome
Dorra Ben-Sellem, Kun-Lun Liu, Sébastien Cimarelli, André Constantinesco, Alessio Imperiale ...53
RARE TUMORS
2009; Volume 1
Epithelioid hemangioendothelioma of the temporal artery presenting as temporal arteritis: case report and literature review
Dina El Demellawy, Ahmed Nasr, Salem Alowami ...56
Retrorectal epidermoid cyst with unusually elevated serum SCC level, initially diagnosed as an ovarian tumor
Masaru Hayashi, Shigeki Tomita, Takahiro Fujimori, Hitoshi Nagata, Keiichi Kubota, Akiko Shoda, Kazumi Tada, Nobuaki Kosaka,
Ichio Fukasawa, Noriyuki Inaba ...59
Metastatic rectal cancer to the breast
Hsiao C. Li, Prapti Patel, Payal Kapur, Sergio Huerta ...63
Plexiform neurofibroma in the hepatic hilum associated with neurofibromatosis type 1: a case report
Sojun Hoshimoto, Zenichi Morise, Chinatsu Takeura, Masahiro Ikeda, Tadashi Kagawa, Yoshinao Tanahashi, Yasuhiro Okabe,
Yoshikazu Mizoguchi, Atsushi Sugioka ...66
Cystadenofibroma of the rete ovarii: a case report with review of literature
Manisha Ram, Abdel Abdulla, Khalil Razvi, Ivilina Pandeva, Awatif Al-Nafussi ...69
Angiomyomatous hamartoma: a rare case report with review of the literature
Manisha Ram, Nazar Alsanjari, Naseem Ansari...75
Malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation
Munir R. Tanas and Brian P. Rubin ...79
Malignant Triton tumor in the retroperitoneal space associated with neurofibromatosis type 1: a case study
Sojun Hoshimoto, Zenichi Morise, Chinatsu Takeura, Masahiro Ikeda,Tadashi Kagawa, Yoshinao Tanahashi, Yasuhiro Okabe,
Yoshikazu Mizoguchi, Atsushi Sugioka ...82
A case of primary renal angiosarcoma
Kazuhiko Yoshida, Fumio Ito, Hayakazu Nakazawa, Yoshiko Maeda, Hikaru Tomoe, Motohiko Aiba...85
A legacy of tinnitus: multiple head and neck paragangliomas
Tricia M.M. Tan, Emma C.I. Hatfield, Rajesh V. Thakker, Eamonn R. Maher, Karim Meeran, Niamh M. Martin, Jeremy J. Turner...88
Soft tissue mixed tumor of the hand
Hiroshi Shimosawa, Michiro Susa, Takayuki Honma, Eiichi Hiraishi, Hiroshi Sakihara ...90
Primary sarcoma of the liver and transplantation: a case study and literature review
Benjamin Bismuth, Hélène Castel, Emmanuel Boleslawski, David Buob, Marc Lambert, Nicole Declerck,Valérie Canva,
Eli-Serge Zafrani, Philippe Mathurin, François-René Pruvot, Sébastien Dharancy ...93
Pitfalls in neuroendocrine tumor diagnosis
Emilio Bajetta, Marco Platania ...96
Hepatic angiosarcoma five years following spontaneous intraperitoneal bleed of a hepatic mass
Jessica L. Cioffi-Pretti, Alexandra N. Kalof, George Ebert, Laurence E. McCahill ...98
Colonic cancer in adolescents. A report of three cases
M.A.C. Odike, A.E. Dongo, E.F. Alufohai, A.I. Odike...102
Tumors and tumor-like lesions of the heart valves
Shi-Min Yuan, Hua Jing, Jacob Lavee ...105
Treatment outcome of maxillary sinus cancer
Hye Sung Won, Sang Hoon Chun, Bum-soo Kim, So Ryoung Chung, Ie Ryung Yoo, Chan-Kwon Jung, Yeon-Sil Kim,
Dong-il Sun, Min Sik Kim, Jin-Hyoung Kang ...110
Invasive neuroendocrine tumor of the kidney: a case report
Ephrem O. Olweny, Michael H. Hsieh, Jill C. Buckley, Jack W. McAninch...115
Bilateral angiosarcoma of the breast in a fourteen-year-old child
Albertus N. van Geel, Michael A. den Bakker...117
Primary extrauterine endometrial stromal sarcoma: response to hormone therapy
The managament of rare nasal mass-nasal dermoid sinus cysts: open rhinoplasty
Emel Cadalli Tatar, Ömer Tarik Selçuk, Güleser Saylam, Ali Özdek, Hakan Korkmaz ...121
Lipid-rich histology in a basal-type immuno-profile breast carcinoma: a clinicopathological histochemical and immuno histo chemical analysis of a case
Serena Russo, Diana Coppola, Paola Vinaccia, Antonella Siciliano, Francesca Baldassarre, Giovanni Battista, Giuseppe Pisani...124
Synchronous malignant B-cell lymphoma and gastric tubular adenocarcinoma associated with paraneoplastic cutaneous vasculitis: hypereosinophilic syndrome with mixed cryoglobulinemia is an important sign of paraneoplastic syndrome
Kazuhisa Nozawa, Hiroshi Kaneko, Tomoyasu Itoh, Yoko Katsura, Masaaki Noguchi, Fujihiko Suzuki, Yoshinari Takasaki,
Hideoki Ogawa, Kenji Takamori, Iwao Sekigawa...128
Hemangiopericytomas of the spine: case report and review of the literature
Chad D. Cole Meic H. Schmidt...132
Cervical intramedullary schwannoma: a case report and review of the literature
Jardel Mendonça Nicácio, José Carlos Rodrigues Jr, Marcos Henrique Lima Galles, Igor Vilela Faquini,
Clemente Augusto de Brito Pereira, Mario Ganau...137
Primary mediastinal giant cell tumor
Judd Goldberg, Shameen Azizad, Jela Bandovic, Arfa Khan...141
Benign multicystic mesothelioma: a case report of three sisters
Eve M. Bernstein, Alison Tate, Dan Arin Silasi, Thomas Rutherford...143
Congenital infantile digital fibromatosis: a case report and review of the literature
Valérie Failla, Odile Wauters, Nazli Nikkels-Tassoudji, Alain Carlier, Josette André, Arjen F. Nikkels...146
Giant mesenteric cystic lymphangioma presenting with abdominal pain and masquerading as a gynecologic malignancy
John Maa, Christianne Wa, Adnan Jaigirdir, Soo-Jin Cho, Carlos U. Corvera ...148
Malignant peritoneal mesothelioma. Is there a new treatment?
Kakil Ibrahim Rasul, David J. Kerr ...150
Intracystic papillary carcinoma of the breast in a 21-year old premenopausal Nigerian woman: a case report
Ivy N. Umanah, Akpan S. Okpongette ...155
Diagnostic confusion resulting from CD56 expression by cutaneous myeloid sarcoma
Thanh Ho, Franklin Sedarat, Nagesh Rao, Sheeja T. Pullarkat...156
The epidemiology of malignant giant cell tumors of bone: an analysis of data from the Surveillance, Epidemiology and End Results Program (1975-2004)
Jennifer L. Beebe-Dimmer, Karynsa Cetin, Jon P. Fryzek, Scott M. Schuetze, Kendra Schwartz ...159
Temsirolimus in the treatment of renal cell carcinoma associated with Xp11.2 translocation/TFE gene fusion proteins: a case report and review of literature
Jigarkumar Parikh, Teresa Coleman...164
Metastasizing pleomorphic adenoma presenting as an asymptomatic kidney tumor twenty-nine years after parotidectomy – urological viewpoint and overview of the literature to date
Jan Ebbing, Carolin Blind, Harald Stein, Kurt Miller, Christoph Loddenkemper ...167
A case of primary mucosa-associated lymphoid tissue lymphoma of the prostate
Noriko Koga, Masanori Noguchi, Fukuko Moriya, Kouichi Ohshima, Nobuyuki Yoshitake, Kei Matsuoka, Jan Ebbing, Carolin Blind,
Harald Stein, Kurt Miller, Christoph Loddenkemper...169
Re: Koga et al. A case of primary mucosa-associated lymphoid tissue lymphoma of the prostate
Damien C. Weber...171
A clinical and molecular project on gonadoblastoma needs international collaboration
Nicolas Kalfa, Olivier Maillet, Charles Sultan...172
Primary NK/T cell lymphoma nasal type of the stomach with skin involvement: a case report
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Metastatic pleomorphic
sarcoma to left atrium
Ammar H. Hawasli,1Rachael Cayce,2 Trung Luong,2
Evelyn Taiwo,1 Michael N. Feliciano3,
Sharon C. Reimold,2,4John M. DiMaio,5 Barbara B. Haley1,2
Departments of 1Hematology-Oncology, 2Internal Medicine, 3Pathology,
4Cardiology, and 5Cardiothoracic Surgery, University of Texas Southwestern Medical Center, Dallas, TX, USA
Abstract
Although several thousand patients are diagnosed with sarcoma annually in the United States, metastases to the heart are very uncommon. In this case report, an overall low frequency cancer presents masquerading with common cardiac symptomology. This case illustrates the importance for detailed diagnos-tic cardiac evaluations and heightened suspi-cion by physicians to consider metastatic dis-ease to the heart in cancer patients with car-diovascular complications. Also discussed is a review of surgical and chemotherapeutic options for this problem.
Introduction
A 53-year-old Caucasian gentleman present-ed with progressive lower back pain, urinary and bowel incontinence, and lower extremity swelling over a one month period. He had been diagnosed six months prior at an outside institution with a high-grade pleomorphic sar-coma with focal myxoid and epithelioid ele-ments of the duodenum. Small bowel resection was performed because of bowel obstruction. Perioperatively, the patient suffered a myocar-dial infarction (MI) due to focal narrowing of the left anterior descending artery and a drug-eluting stent was placed during cardiac catheterization. Post-operative transthoracic echocardiogram (TTE) was interpreted as mild mitral valve thickening, with flail chordae tendineae and an estimated ejection fraction of 35-40%. After discharge, the patient was lost to follow-up.
Case report
On presentation to our facility, physical examination revealed normal lower extremity
strength, focal tenderness over the sacrum, pitting edema in the lower extremities and a murmur of mitral regurgitation with basilar crackles and dullness. Computed tomography (CT) showed a wedge-shaped splenic infarct and magnetic resonance imaging (MRI) of the spine demonstrated osseous metastatic dis-ease involving the vertebral bodies, with epidural and neuroforaminal tumor extension in the sacrum. A TTE was performed in antici-pation of chemotherapy with adriamycin and revealed a large vegetation attached to the anterior leaflet of the mitral valve with pro-lapse into the left atrium and ventricle (Figure 1A, arrow) and hypoechoic regions within the cardiac tissue (Figure 1A, arrowhead). The patient was taken to the operating room to remove the vegetation, biopsy the hypoechoic mass and replace the mitral valve. A 9 cm veg-etation was excised from the anterior mitral valve leaflet (Figure 1B, left and middle). Intraoperatively, the patient was noted to have diffuse disease throughout the atrial septum and mitral valve (Figure 1B, right) and the pro-cedure was terminated. Post-operative cardiac MRI showed a lobulated cardiac mass infiltrat-ing the atrial septum, the posterior aortic wall and the posterior aspect of the anterior mitral valve annulus. In the axial plane, the mass measured at least 5.3 x 2.2 cm and extended to the superior portion of the left atrium near the entry of the right superior pulmonary vein
(Figure 1C; mass indicated with arrowheads). Histology of the atrial mass revealed a high-grade pleomorphic sarcoma, metastatic to heart. Low power frozen sections of the atrial wall showed hypercellularity (Figure 2A). Medium and high power frozen (Figure 2B and C) and permanent (Figure 2D) sections showed tumor giant cells (indicated with arrowheads) among spindled and epithelioid
Rare Tumors 2009; volume 1:e1
Correspondence: Dr. Barbara B. Haley, The Harold C. Simmons Comprehensive Cancer Center, University of Texas Southwestern Medical Center at Dallas, Dallas, TX, USA E-mail: [email protected] Key words: sarcoma, cardiac metastases, tumor, cancer.
Received for publication: 1 May 2009. Accepted for publication: 5 May 2009.
Acknowledgement: we would like to thank Dr. John Bagwell and Leah Gaither for their assis-tance.
This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0)
©Copyright A.H. Hawasli et al., 2009 Rare Tumors 2009; 1:e1
doi:10.4081/rt.2009.e1
Figure 1. Echocardiography (A) performed before excision of vegitation (B) and post-operative cardiac MRI (C).
tumor cells. The atrial mass was immunohisto-chemically positive for vimentin, confirming mesenchymal differentiation (Figure 2E). Immunohistochemical stains for other mark-ers, including CD117, AE1/AE3, desmin, myo-genin, caldesmon, S100, HMB-34, calretinin, CD31 and CD34 were negative (Figure 2F).
A diagnosis of metastatic cardiac sarcoma was established and treatment with gemc-itabine and docetaxel was initiated. Adria-mycin-based therapy was not considered sec-ondary to the patient’s reduced cardiac func-tion (37% ejecfunc-tion fracfunc-tion). Five weeks after initiating chemotherapy, the patient was admitted for worsening lower extremity edema but denied shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, chest pains, palpitations, weight gain, or symptoms of tran-sient ischemic attacks. Electrocardiogram revealed atrial flutter at 119 beats per minute with 2:1 block and the old anterolateral infarct. The patient declined cardioconversion and was treated with amiodarone. At the time of prepa-ration of this report, the patient was tolerating palliative gemcitabine and docetaxel chemo -therapy.
Discussion
Soft-tissue sarcomas are a diverse mix of cancers with an incidence of 10,390 new cases diagnosed annually in the United States.1
Despite aggressive treatment with surgery, radiation and chemotherapy, outcomes remain suboptimal as 3,680 (35%) soft-tissue sarcoma patients die annually.1 Death secondary to
metastases is common and sites of metastases vary depending on the type of soft-tissue sarco-ma with most types showing a predilection for the lungs. Metastatic involvement of the heart by soft-tissue sarcoma has been reported in the literature but most cases of cardiac involvement occur in the setting of widespread metastases.2 Our patient is among the first
reported cases of an epithelioid high-grade pleomorphic sarcoma metastatic to the heart in absence of known widespread metastases.
Familiarity with the unique characteristics of the soft-tissue sarcomas will enable a physi-cian to identify expected patterns of metastat-ic spread. One should be suspmetastat-icious of cardiac metastases in a soft-tissue sarcoma patient who suddenly develops unexplained congestive heart failure, new cardiac murmurs, arrhyth-mias or embolic phenomena. For such patients, metastatic cardiac disease should be included in the differential diagnosis and fur-ther evaluation with electrocardiograms, echocardiograms, and cardiac MRI should be made. Accurate imaging of cardiac cancer is very important in the multimodal medical and surgical management of metastatic cardiac
sarcoma.3 In this patient, cardiac metastasis
was only incidentally discovered during pre-chemotherapy cardiac evaluation. However, several aspects of this patient’s clinical course suggested the presence of this diagnosis. The patient’s initial myocardial infarction prompt-ed cardiac catheterization, which showprompt-ed luminal narrowing assumed to be secondary to atherosclerotic disease. Yet, on repeat studies, the patient had no evidence of significant ath-erosclerotic disease. The patient’s infarct like-ly resulted from an embolic event originating from the large mitral valve vegetation or from extrinsic compression of the artery by tumor. The splenic lesions noted on CT scans likely represent distant emboli with infarction. Finally, review of the outside hospital TTE abnormalities noted after the patient’s periop-erative MI indicated that he had cardiac metas-tases at initial presentation. A high degree of suspicion for cardiac involvement by sarcoma may have hastened a correct diagnosis.
Conclusions
Management of metastatic sarcoma to the heart remains a difficult task. Some reports discuss surgical resection of cardiac metastat-ic lesions with variable success,4,5while others
report the use of cardiac transplantation.6 In
this case, surgical excision was attempted, but the extensive infiltrating nature of the tumor prohibited complete debridement leaving sys-temic therapy as the only viable option. There are several generally accepted systemic thera-pies for metastatic sarcoma with most regi-mens including doxorubicin as a single agent7
or in combination with other drugs. The most widely employed combination chemotherapy regimens are doxorubicin/dacarbazine,8,9
dox-orubicin/ifoxfamide/mesna10,11
mesna/doxoru-bicin/ifosfamide/dacarbazine,12ifosf amide/epi
-r u bicin/mesna,13and gemcitabine/docetaxel.14-16
However, the use of doxorubicin was felt to be contraindicated due to the depressed cardiac function in this patient. If a sarcoma expresses CD117, imatinib would be a viable treatment option due to the drug’s oral administration, favorable toxicity profile, and anti-tumor activ-ity on some subsets of sarcoma, particularly gastrointestinal stromal tumors.17-19Expression
of this marker is felt to be favorable due to this relationship. Thus far, reports do not associate imatinib with induction of cardiac failure.20
Unfortunately our patient’s cardiac tumor did not express CD117 and the drug was not a treatment option. Since metastatic sarcoma to the heart is a rare occurrence, no prospective or randomized trials exist to help guide treat-ment. In fact, there are no data to show whether systemic treatment of unresectable cardiac sarcomas significantly improves dura-tion or quality of life. In the setting of conges-tive or restricconges-tive heart failure, many of the first-line chemotherapy options are not feasi-ble for fear of therapy worsening cardiac func-tion. In our case, gemcitabine/docetaxel chemotherapy was felt to be an active regimen with controllable toxicity and without potential devastating cardiac side effects. Tolerance to the treatment regimen thus far has been acceptable and the patient is continuing this chemotherapy.
References
1. Jemal A, Siegel R, Ward E, et al. Cancer statistics, 2008. CA Cancer J Clin 2008;58: 71-96.
2. Catton C. The management of malignant cardiac tumors: clinical considerations. Semin Diagn Pathol 2008;25:69-75. 3. Yuan SM, Shinfeld A, Lavee J, et al. Imag
in g morphology of cardiac tumours. Car d
-Case Report
iol J 2009;16:26-35.
4. Harting MT, Messner GN, Gregoric ID, Frazier OH. Sarcoma metastatic to the right ventricle: surgical intervention fol-lowed by prolonged survival. Tex Heart Inst J 2004;31:93-5.
5. Kono T, Amano J, Sakaguchi M, Kitahara H. Successful resection of cardiac metastatic liposarcoma extending into the SVC, right atrium, and right ventricle. J Card Surg 2005;20:364-5.
6. Goldstein DJ, Oz MC, Rose EA, et al. Experience with heart transplantation for cardiac tumors. J Heart Lung Transplant 1995;14:382-6.
7. Tierney J Fea. Adjuvant chemotherapy for localised resectable soft-tissue sarcoma of adults: meta-analysis of individual data. Sarcoma Meta-analysis Collaboration. Lancet 1997;350:1647-54.
8. Antman K, Crowley J, Balcerzak SP, et al. An intergroup phase III randomized study of doxorubicin and dacarbazine with or without ifosfamide and mesna in advanced soft tissue and bone sarcomas. J Clin Oncol 1993;11:1276-85.
9. Zalupski M, Metch B, Balcerzak S, et al. Phase III comparison of doxorubicin and dacarbazine given by bolus versus infu-sion in patients with soft-tissue sarcomas:
a Southwest Oncology Group study. J Natl Cancer Inst 1991;83:926-32.
10. Edmonson JH, Ryan LM, Blum RH, et al. Randomized comparison of doxorubicin alone versus ifosfamide plus doxorubicin or mitomycin, doxorubicin, and cisplatin against advanced soft tissue sarcomas. J Clin Oncol 1993;11:1269-75.
11. Grobmyer SR, Maki RG, Demetri GD, et al. Neo-adjuvant chemotherapy for primary high-grade extremity soft tissue sarcoma. Ann Oncol 2004;15:1667-72.
12. Elias A, Ryan L, Sulkes A, et al. Response to mesna, doxorubicin, ifosfamide, and dacar-bazine in 108 patients with metastatic or unresectable sarcoma and no prior chemotherapy. J Clin Oncol 1989;7:1208-16. 13. Frustaci S, Gherlinzoni F, De Paoli A, et al. Adjuvant chemotherapy for adult soft tis-sue sarcomas of the extremities and gir-dles: results of the Italian randomized cooperative trial. J Clin Oncol 2001;19: 1238-47.
14. Hensley ML, Maki R, Venkatraman E, et al. Gemcitabine and docetaxel in patients with unresectable leiomyosarcoma: results of a phase II trial. J Clin Oncol 2002;20: 2824-31.
15. Leu KM, Ostruszka LJ, Shewach D, et al. Laboratory and clinical evidence of
synergis-tic cytotoxicity of sequential treatment with gemcitabine followed by docetaxel in the treatment of sarcoma. J Clin Oncol 2004;22: 1706-12.
16. Maki RG. Gemcitabine and docetaxel in metastatic sarcoma: past, present, and future. Oncologist 2007;12:999-1006. 17. Verweij J, Casali PG, Zalcberg J, et al.
Progression-free survival in gastrointesti-nal stromal tumours with high-dose ima-tinib: randomised trial. Lancet 2004;364: 1127-34.
18. Blanke CD, Demetri GD, von Mehren M, et al. Long-term results from a randomized phase II trial of standard- versus higher-dose imatinib mesylate for patients with unresectable or metastatic gastrointesti-nal stromal tumors expressing KIT. J Clin Oncol 2008;26:620-5.
19. Demetri GD, von Mehren M, Blanke CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med 2002;347:472-80. 20. Verweij J, Casali PG, Kotasek D, et al.
Imatinib does not induce cardiac left ven-tricular failure in gastrointestinal stromal tumours patients: analysis of EORTC-ISG-AGITG study 62005. Eur J Cancer 2007;43: 974-8.
Squamous cell carcinoma
of the scrotum in a Nigerian:
case report
Jerome E. Azike,1,2N.O. Chukwujama,1,2 T.C. Oguike1,2
1Department of Surgery, College of Medicine and Health Sciences, Imo State University, Orlu, Imo State, Nigeria; 2Imo State University Teaching Hospital, Orlu, Imo State, Nigeria
Abstract
Squamous cell carcinoma of the scrotum is rare and to the best of our knowledge has never been reported from Nigeria. We report on a case thought to be occupation-related in a 42-year old Nigerian taxi driver who had previ-ously been an automobile mechanic and later a long-haul truck driver. He presented with a stage D disease and only palliation was feasi-ble.
Case report
A 42-year old taxi driver who had been a car mechanic for four years 25 years earlier pre-sented in 2007 with a ten-year history of a small pruritic painless nodule on his ventral scrotal surface which had ulcerated but failed to heal. He infrequently washed his work-out-fit while an auto-mechanic and admitted to its frequent soiling, sometimes up to his under-wear, with used engine oil while at work. He took a bath at the close of work each day; how-ever, his personal hygiene outside the work-place was good.
On examination he was pale, had a foul-smelling, grotesque, fungating, ulcero-prolifer-ative mass with rolled edges which involved the whole scrotum and proximal third of the penile shaft, extending 5 cm to the right thigh antero-medially and 3 cm of the anal verge.
The scrotum was fixed to the pubis. There were bilateral inguinal lymphadenopathy involving both the vertical and horizontal chains. He looked otherwise well nourished and was ambulant. He was also rather depressed emotionally.
Ultrasonography revealed a hepatomegaly and para-aortic lymphadenopathy, the largest 2.8 cm in diameter. His general condition was optimized and biopsy confirmed the diagnosis. He had palliative scrotectomy with bilateral orchidectomy and subsequently cisplatinum-based combination chemotherapy, after he had been counseled and informed consent
obtained. Histopathological review showed scrotal squamous cell carcinoma with testicu-lar involvement (Figure 1). He succumbed to the disease after seven months.
Discussion
The scrotum is a seven-layer pouch which invests the testes, testicular adnexae, and dis-tal spermatic cord. The scrodis-tal lymphatics drain into the corresponding superficial inguinal nodes. Anastomoses exist between the lymphatics of the contralateral network across the median raphe. Tumors have been reported that arise out of virtually any of the components of the scrotal wall.1
Squamous cell carcinoma of the scrotum is now exceedingly rare. It was the first malig-nancy linked to occupational exposure.1-2
Previously, it most commonly resulted from exposure to environmental carcinogens such as chimney soot, tars, paraffin, and some petroleum products. Currently most cases result from poor hygiene and chronic inflam-mation.3
Used engine oils have elevated polycyclic aromatic hydrocarbons which tend to be greater for petrol engines than for diesel engines. Prolonged and repeated contact with such oils can cause skin and scrotal cancer. Car mechan-ics are at potential risk from used engine oil.4
Best occupational health practices are invaluable at the workplace for those at risk to
minimize exposure. Prognosis correlates with extent of nodal involvement with virtually no survivors if iliac nodes are involved.3,5Ability to
achieve a negative margin at the time of initial surgery is an important prognostic factor as adjunctive therapy such as radiotherapy or chemotherapy or both has not proven useful.1
In conclusion, the index patient presented very late when even surgery, chemotherapy and radiotherapy could not possibly offer any mean-ingful improvement in terms of outcome. We believe this case will raise the awareness in our environment of the existence of this dis-ease and improve the index of suspicion among practicing surgeons and oncologists.
Rare Tumors 2009; volume 1:e2
Correspondence: Jerome E. Azike, MBBS, FWACS, Department of Surgery, College of Medicine & Health Sciences, Imo State University, Orlu, Nigeria.
E-mail: [email protected]
Key words: cancer of the scrotum, occupational cancer, automobile mechanic, used engine oil. Received for publication: 2 May 2009. Accepted for publication: 8 May 2009.
This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0)
©Copyright J.E. Azike et al., 2009 Rare Tumors 2009; 1:e2 doi:10.4081/rt.2009.e2
Figure 1. a, b, c, d. Histology of the various sections of the tumor; (a) Normal skin and contiguous areas the well differentiated squamous cell carcinoma invading the surround-ing deeper tissue; (b) High magnification of the malignant squamous cells; (c) Remnants of the testicular tubules being invaded by the tumor; (d) The malignant squamous cells invading the deeper testes.
References
1. Rowland RG, Herman JR. Tumors and Infectious Diseases of the Testis, Epididymis, and Scrotum. In Gillenwater JY, Grayhack JT, Howards SS and Mitchell
ME, eds. Adult and Pediatric Urology, Phila delphia: Lippincott, Williams and Wilkins; 2002.
2. Waldron HA. A brief history of scrotal can-cer. Br J Ind Med 1983;40:390-401. 3. Presti JC Jr. Genital Tumors. In Tanagho EA,
McAnich JW, editors. Smith’s General Urology. New York: Mc Graw Hill 2008:375-87.
4. Wyre Borough Council, Lancashire U.K., Health and Safety Factsheet – No. 12 (internet), 2002. Available from: http:// www.wyrebc.gov.uk (Cited 5th July, 2008) 5. Ray B, Whitmore WF Jr. Experience with Carcinoma of the Scrotum. J Urol 1977; 117:741-5.
Extraosseous osteosarcoma
in Ibadan: case series over
a 20-year period
Temitope O. Alonge,1Henry
A. Obamuyide,2Gabriel Olabiyi Ogun3 1Consultant Orthopedic Surgeon/Senior Lecturer, Department of Orthopedics and Trauma, University College Hospital, and College of Medicine, University of Ibadan, Ibadan, Nigeria;
2Resident in Surgery, Department of Surgery, University College Hospital, Ibadan, Nigeria;
3Consultant Pathologist/Lecturer, Department of Pathology, College of Medicine, and University College Hospital, Ibadan, Nigeria
Abstract
Extraosseous osteosarcoma (EOO) is a rare form of sarcoma. There have been few reports of cases and outcome from an African population. Out of 112 cases of sarcomas seen at the UCH, Ibadan between 1986-2005, 5 were EOO. All presented late on account of initial excision without histology and outcomes were poor. EOO occurs in the black population of Sub-Saharan Africa. The outlook for these patients is still bleak.
Introduction
Extraosseous osteosarcoma (EOO) is a malignant mesenchymal neoplasm that pro-duces osteoid, bone or chondroid material and is located in the soft tissue without attachment to the skeleton as determined by imaging modalities or inspection during surgical oper-ative procedure.1EOO is a rare type of tumor
making up only 1% of all soft tissue sarcomas2,3
and 4% of all osteosarcomas.4
Wilson reported the first case of EOO in 19415 and since then fewer than 300 cases
have been reported in the English Language literature.6The tumor is most common in
mid-dle-aged and elderly patients. A previous study showed that EOO is a doxorubicin-resistant lesion with a poor prognosis and a form of soft tissue sarcoma that should be viewed by clini-cians as clinically and therapeutically distinct from osseous osteosarcoma.6
The single most important criteria for diag-nosis of this tumor is the presence of tic osteoid and bone; sometimes with neoplas-tic cartilage.1,5The histological variants of EOO
include osteoblastic, chondroblastic,
fibroblas-tic, osteoclastic or giant cell type, telangiectat-ic, small cell or well differentiated forms. The immunohistochemical profile includes the expression of osteocalcin and osteonectin which are specific for osseous osteosarcomas, but a study by Fanburg-Smith et al.7showed
that osteocalcin is highly sensitive for EOO neoplastic cells.1 Closely related differentials
include calcified hematoma, myositis ossifi-cans, synovial sarcoma, epithelial sarcoma, liposarcoma or malignant fibrous histiocytoma.
Methods
All sarcomas recorded at our institution between January 1986 and December 2005 were compared and extraosseous variety iden-tified. Their charts were selected and reviewed for relevant data. Out of 112 cases of osteosar-coma recorded in our hospital between January 1986 and December 2005, 5 cases were extraosseous. This series presents the 5 cases of EOO recorded at our institution dur-ing the 20-year period from 1986-2005 and this includes one patient reported previously by Ogundiran et al.8
Case Series
Case
#1
A 16-year-old male student who presented with a 4-year history of progressive left ankle swelling and pain. He had excision of a mass in his left ankle at a private facility without histological evaluation a year prior to presen-tation at the University College Hospital (UCH), Ibadan. On presentation at the UCH, examination revealed a 10x7 cm mass over the left lateral malleolus that was tender, differen-tially warm, with both cystic and solid areas. No significant popliteal or inguinal lym-phadenopathy was noted. Laboratory studies were essentially normal.
He had a local excision of the mass and the operative finding was a 10x7 cm multilocued brownish soft tissue mass over the left lat-eral malleolus covered with a fibrous capsule with a pedicle arising from the ankle joint. There was associated erosion of the adjoining bone and the histology report revealed soft tis-sue osteosarcoma. He was lost to follow-up but represented two years later with recurrence.
Case
#2
A 70-year-old man who presented with a 1-year history of a painful scalp swelling asso-ciated with bleeding. A similar swelling had recently grown on the right temporal region prior to presentation at the UCH. Incisional
biopsy and histology of the initial mass at the source of referral revealed an extraskeletal osteosarcoma.
Examination revealed a 15x7 cm left fron-toparietal mass with overlying scar. He also had a smaller temporal mass. Both were tender and hemorrhagic. Skull X-ray showed an extensive soft tissue swelling extending from the left frontal to the left parietal region. There was an associated increase in the density of the underlying subcutaneous tissue but no cal-cifications. Routine laboratory studies were within normal limits. He had radiotherapy but later represented with facial recurrence after two months.
Case
#3
A 21-year-old man with a 1-year history of a painful left upper back swelling and 4 month history of progressive weakness of both legs which progressed to paraplegia. He had a biopsy of the mass at a private facility five months before presentation at the UCH but no histological evaluation of the mass was car-ried out. Examination at the UCH revealed a left periscapular fluctuant mass with overlying hypertrophic scar which was also attached to underlying structures. He also had a T5 para-plegia. Chest X-ray showed a left paravertebral soft tissue mass lesion with evidence of destruction of the 5th rib and left sided
pul-monary effusion. Urinary Bence Jones protein was negative, the full blood count was normal but the ESR was elevated. Two pleural aspi-rates were hemorrhagic and CT myelography showed an isolated left sided isodense lesion
Rare Tumors 2009; volume 1:e3
Correspondence: Temitope O. Alonge, Consultant Orthopedic Surgeon/Senior Lecturer, Department of Orthopedics and Trauma, University College Hospital and College of Medicine, University of Ibadan, Ibadan, Nigeria E-mail: [email protected]
Key words: extraosseous osteogenic sarcomas, Sub-Saharan Africa, histology.
Received for publication: 3 May 2009. Accepted for publication: 5 May 2009.
Contributions: TOA conceived, designed, super-vised and helped to draft the manuscript and approved the final version; HAO participated in the design, gathered the data, drafted the manu-script and approved the final copy; GOO reviewed the manuscript for critical content and approved the final copy.
This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0)
©Copyright T.O. Alonge et al., 2009 Rare Tumors 2009; 1:e3
in the spinal cord with extensive erosion of the ribs as well as infiltration into the left pleural cavity.
Incisional biopsy of the left chest wall lesion revealed osteogenic sarcoma. Chemotherapy was to be administered but the patient could not afford it and he was subsequently lost to follow-up.
Case
#4
A 20-year-old man who presented with an 11-month history of progressive soft tissue swelling of the right thigh associated with pain. The soft tissue mass was excised initial-ly at a private facility but no histological evalu-ation of the specimen was carried out. Examination at the UCH revealed a massively swollen right thigh with multiple areas of ulceration. Biopsy and histology at the UCH revealed extraosseous osteogenic sarcoma. The patient was given a hemostatic dose of radiotherapy but was subsequently lost to fol-low-up.
Case
#5
A 40-year-old housewife who presented with a 20-month history of painful left breast lump which had been excised previously at another hospital without histological evaluation, but recurred eight months before presentation at UCH. Examination revealed a globular left breast mass which was warm, multinodular, fixed to the pectoralis fascia and which meas-ured 20x18 cm. There was associated ipsilater-al axillary lymph node enlargement. Other sys-tems were essentially normal.
A clinical diagnosis of locally advanced can-cer of the left breast was made. A core needle biopsy revealed an osteogenic sarcoma which was confirmed by the histology of the mastec-tomy specimen. She was scheduled for post-operative radiotherapy but defaulted and died six months post-mastectomy.
Discussion
The exact cause of EOO is unknown and it is presumed to be mainly an idiopathic condition. Unlike osseous osteosarcoma (OOO), it has not been documented in siblings or in associa-tion with hereditary retinoblastoma but risk factors associated with the development of this tumor include previous exposure to radiation, such as X-rays and radioactive thorium dioxide (Thorothrast).9Other associated factors
sug-gested in literature in the development of EOO include trauma, the assessment and evalua-tion of which is difficult and controversial.10
Some cases have been associated with intra-muscular injection11 while some EOO have
been reported to follow myositis ossificans.12,13
A common mode of presentation is as a swelling of insidious onset with associated pain in one-third of patients but often the tumor grows to a large size before the patient seeks medical advice.14 It may ulcerate with
growth, but that usually occurs after biopsy or attempts at excision and the tumor usually occurs in middle-aged and elderly patients.15
The anatomic location of EOO is usually the muscles of the thigh, which are the most com-monly affected; the large muscles around the pelvic and shoulder girdles and the retroperi-toneum, though rare locations like the thyroid gland, penis, mediastinum and the kidney are occasionally encountered.1,12,15,17,18
At the Memorial Sloan-Kettering cancer center, a review of 48 cases of EOO during 1950-1983 showed a median age at diagnosis of 51 years (range, 6-80 years).14 The most
common primary sites were the thighs and buttocks (54.2%) with preponderance in patients aged 50 years and above and slightly more common in males (58%) than in females.12In the report by Chung and Enzinger,
EOO occurs principally as a soft tissue mass involving an extremity with a predilection for the thighs (lower extremity 46.6%; upper extremity 20.5%) and the retroperitoneum in 17%. In most cases, the tumor was deep seated and firmly attached to the fascia, but occasion-ally they are freely movable and confined to the subcutis or dermis. The duration of symptoms prior to presentation ranges from two weeks to 25 years (median six months). Prior trauma to the site was observed in 12.5% and irradiation to the affected site in 5.7% of cases.12
Although osseous osteosarcoma (OOO) occurs predominantly in the first two decades of life, EOO are rarely encountered under 40 years of age.15However, in this case series, 3 of
the 5 patients were aged 21 years and under in keeping with other cases which have been doc-umented in the pediatric age group.16
Imaging modalities by either plain conven-tional radiograph, CT scan or MRI of the soft tis-sue is essential to rule out any continuity of tumor with bone. If adjacent bone shows radio-logical changes of involvement by the tumors, it is most likely to be originating from the bone rather than from the soft tissue. A soft tissue mass with spotty to massive calcification with-out adjacent bone involvement is one of the clas-sic radiographic appearances of this tumor.15
EOO is a difficult disease to treat and the optimum therapy has not been fully deter-mined due to the relative rarity of these tumors. Advances in the care of these patients will require disease-specific clinical trials.6A
wide local excision, with at least 5cm margin of normal tissues should be the treatment of choice.15 The local recurrence-free survival
rate observed in a recent study6suggests that
patients with extremity EOO can be treated with limb salvage operative procedures.
However, if these are not possible due to the anatomic location of the tumor, amputation is recommended.19
In a review of 48 cases of EOO and 39 trials of chemotherapeutic agents reported by Sordillo et al. they showed that no patient had a major response.14The average 5-year survival
rate in 5 previous studies ranges from 15-25%2,9and the response to multimodality
ther-apy is not as good as for OOO.14Tumor size (<5
cm vs. ≥5 cm) was the major predictor of patient survival.13
Of note, the challenges of managing malig-nancies and other chronic illnesses in a devel-oping economy like ours is exemplified by these cases. The issues of late presentation, non-submission of biopsy specimen for histo-logical assessment, non-affordability of treat-ment modality and default from follow-up raise critical issues. These include late pres-entation, lack of health or social insurance making the patient bear the full cost of their treatment and probably also the ignorance of the initial managing physician regarding the need for histological assessment of all biop-sies or their unwillingness to insist on this (as a measure to reduce costs and increase their competitiveness). These factors may all be related to cost of treatment, and patients, therefore, have a significantly long delay before presentation and would usually present in a private facility with a view to reducing the cost of treatment.
Conclusions
In conclusion, the outlook for patients with EOO is grave and the majority of patients with this tumor succumb to metastatic disease within a period of three years after the initial diagnosis.1 Extraosseous osteosarcoma occur
in the populations of Sub-Saharan Africa and the poor prognosis for EOO in our environment is further compounded by the factors earlier highlighted. Since recurrence occurs mostly within two years after surgery, adjuvant chemotherapy and radiotherapy have been found to be beneficial.15
References
1. Weiss SW, Goldblum J. Osseous soft tissue tumours in Enzingers and Weiss: Soft Tissue Tumours 4th ed. Pg 1389-1417,
Mosby St Loius, 2001.
2. Allan CJ, Soule EH. Osteogenic sarcoma of the soft tissue: a clinicopathologic study of 26 cases and review of the literature. Cancer 1971;27:1121-33.
3. McCarter MD, Lewis JJ, Antonescu CR, et
Case Report
al. Extraskeletal osteosarcoma: analysis of outcome of a rare neoplasm. Sarcoma 2000; 4:119–23.
4. Van Rojswik CSP, Lieng TG, Kroon HM, et al. Retroperitoneal extraosseous osteo s a r -c oma. J Clin Path 2002;54:77-8.
5. Wilson H. Extraskeletal ossifying tumours. Ann Surg 1941;113:95-112.
6. Ahmad SA, Patel SR, Ballo MT, et al. Extraosseous osteosarcoma. Response to treatment and long term outcome. J Clin Oncol 2002;20:521-7.
7. Fanburg-Smith JC, Bratthauer GL, Miettinen M. Osteocalcin and osteonectin immunore-activity in extraskeletal osteosa r coma: a study of 28 cases. Hum Pathol 1999;30:32. 8. Ogundiran TO, Ademola SA, Oluwatosin
OM, et al. Primary osteogenic sarcoma of the breast. World J Surg Oncol 2006;4:90.
9. Lee WR, Laurie J, Townsend A. Fine struc-ture of radiation-induced osteogenic sar-coma. Cancer 1975;76:1414-5.
10. Fine G, Stout AP. Osteogenic sarcoma of the extraskeletal soft tissue. Cancer 1956; 9:1027-43.
11. Lee JH, Griffith WJ, Bottomley RH. Extrao -s -s e ou-s o-steogenic -sarcoma following an intramuscular injection. Cancer 1977;40: 3097-101.
12. Chung EB, Enzinger F. Extraskeletal osteo -genic sarcoma. Cancer 1987;60:1132-42. 13. Bane BL, Evans HL, Ro JY, et al. Extras k e l
-e tal sarcoma: a clinicopathologic r-evi-ew of 26 cases. Cancer 1990;65:2762-70. 14. Sordillo PP, Hadju SL, Magill GB, et al. Ext
-r a osseous osteogenic sa-rcoma: a -review of 48 patients. Cancer 1983;51:727-34. 15. Lin SY, Chen WM, Wu HH, et al. Extrao s
-s e ou-s o-steogenic -sarcoma. J Chin Med Assoc 2005;68:542-5.
16. Humphrey GM, Brown I, Squire R, et al. Extraosseous osteogenic sarcoma-A rare pediatric malignancy: Case report and review of the literature. J Pediatr Surg 1999; 34:1025-8.
17. Hertel V, Basten O. Extraosseous osteosarcoma of the thyroid gland. Laryngo r h i n o o -t ol ogie 2006;85:913-6.
18. Dudhat S, Desai S, Borges A, et al. Retro -peritoneal extraosseous osteosarcoma. A case report and review of literature. Indian J Cancer 1999;36:186-9.
19. Lewis RJ, Loiz MJ, Beazley RM. Extra -osseous osteogenic sarcoma: case report and approach to therapy. Ann Surg 1974:40: 597-600.
A multimodal approach to the
treatment of bilateral choroidal
metastases from thyroid
carcinoma
Maria Grazia Fabrini,1 Federica Genovesi-Ebert,2
Franco Perrone,3Mario De Liguoro,1 Clara Giovannetti,4Fausto Bogazzi,4 Stanislao Rizzo,2Enio Martino,4 Luca Cionini1
1Divisions of Radiotherapy, 2Ophthalmic Surgery, 3Health Physics and
4Endocrinology and Metabolism of Pisa University Hospital, Italy
Abstract
A 58-year old man, affected by metastatic thyroid carcinoma, experienced a progressive bilateral visual impairment. Ophthalmic exam-ination revealed the presence of a choroidal mass with an associated exudative retinal detachment in both eyes. Twelve years before, a diagnosis of metastatic thyroid carcinoma had been established and the patient had been subject to several therapeutic procedures.
In May 2007, he received a radiotherapy treatment to the left eye with an episcleral plaque and bilateral bulbar injection of beva-cizumab. The patient had a rapid and stable visual acuity recovery. Twenty months after treatment, the lesion treated with radiotherapy was still stable whereas the contra-lateral lesion had evolved and determined a vitreal hemorrhage.
Introduction
Choroidal metastases from thyroid cancer are uncommon findings.1They often occur in
advanced stages, and generally several years after the initial diagnosis. Main related symp-toms can include pain and impairment of visu-al ability. Eye metastases may cause blindness with a rapid degradation of the patient’s quali-ty of life.2,3
This paper reports a case of bilateral choroidal metastases in a patient in satisfacto-ry general condition, with a long histosatisfacto-ry of metastatic thyroid carcinoma initially respond-ing to radio-metabolic therapy.
A multimodal approach was adopted to treat the ocular lesions, in order to restore his visu-al acuity. After a follow-up of 18 months, the patient’s visual ability was still good, although multiple metastases developed in other sites. A
vitreal hemorrhage occurred 20 months after treatment in the right eye, while the lesion treated with brachytherapy remained unal-tered.
Case Report
In October 1996, a 58-year old male patient in satisfactory general condition and under medical therapy for arterial hypertension, was treated with total thyroidectomy, parathy-roidectomy, bilateral cervical lymphadenecto-my and also with resection of a single ischio-pubic recurrence. Histo-pathological examina-tion of the thyroid gland and of the bone metastasis revealed a thyroid carcinoma (G3), confirmed by positive thyroglobulin immunos-taining, with solid and papillary areas of oxyfil cell-type. No diffusion to neck lymph nodes was found. Disease was scored as T3N0M1, IVC stage following the American Joint Cancer Committee grouping.
After surgery, the patient received a radio-metabolic treatment with 131-I. During the fol-low-up, the thyroglobulin hematic levels were periodically checked.
From 1997 to 2007, a diffusion of multiple metastases was observed; the largest recur-rences were treated by surgical resections. In order to limit the development of recurrences, other systemic 131-I radio-metabolic therapies were administered (Figure 1).
In May 2007, the patient presented a pro-gressive visual loss in both eyes; he had a past history of retinal venous thrombosis in his left eye, with a residual best corrected visual acu-ity of 20/40.
Ophthalmologic examination, performed in May 2007, showed that ocular adnexa, extra-ocular movements and pupillary reactions were normal in both eyes and no exophthal-mus was found. No pupillary defects were evi-denced and the Best Corrected Visual Acuity was 20/70 in the left eye (LE) and 20/25 in the right eye (RE).
Slit lamp examination of the anterior seg-ment revealed only a light nuclear sclerosis. Intra-ocular pressure was 15 mm of Hg in both eyes.
Ophthalmoscopic examination detected a solid lesion localized in the posterior pole closely to the macula in the RE and a similar lesion in the posterior pole of the LE, where hypertrophic areas of the retinal pigment epithelium related to the previous venous branch occlusion were also evident (Figure 2). B-scan ultrasound examination confirmed the presence of a slightly elevated, solid choroidal mass in both eyes: in RE it was 1.25 mm in thickness, with an associated serous retinal detachment, in LE it measured 3.3 mm in height and 4.8 mm in maximum base
diam-eter, with an associated serous retinal detach-ment.
Standardized A-scan echography showed in both lesions an irregular structure with a sharp initial spike and moderate-high internal reflectivity.
Fundus fluorescein angiography evidenced an area of irregular fluorescence with a mild leakage in correspondence of the supero-tem-poral arcade in the RE. A block of the fluores-cence due to the retinal pigment epithelium was noted in the posterior pole of the LE, in correspondence of the choroidal mass. The ischemic maculopathy, secondary to the previ-ous venprevi-ous thrombosis, appeared as an hypo-fluorescent lesion with late staining.
Indocyanine angiography showed the hyper-fluorescence of the lesion in the RE, especially in the intermediate phases, with wash out of the dye in the late phases. In the LE, indocya-nine angiography revealed an atrophic area of retinal pigment epithelium and of choriocapil-laris in correspondence of the fovea, while the mass-lesion showed an irregular staining of the dye in the intermediate and late phases (Figure 2).
Optical Coherence Tomography showed a massive detachement of the neuro-epithelium in the RE (Figure 3); a hypertrophic hyper-reflective area of the retinal pigment
epitheli-Rare Tumors 2009; volume 1:e4
Correspondence: Franco Perrone,
U.O. Fisica Sanitaria, Azienda Ospedaliero-Universitaria Pisana, via Roma 67, 56125 Pisa, Italy E-mail: [email protected]
Key words: choroidal metastases, thyroid carcino-ma, radiotherapy, anti-VEGF factors.
Acknowledgments: the authors are grateful to the Pisa section of the “Lega Italiana per la Lotta Contro i Tumori” for supporting the present pub-lication.
Contributions: radiotherapy treatment and fol-low-up: MGF, MDL; ophthalmologic folfol-low-up: FGE; radiotherapy treatment planning, data analysis and dose evaluation: FP; brachytherapy: MGF, SR, FGE; endocrinological follow-up: CG, FB, EM; text editing: CG, MDL, MGF, FGE, FB, FP; text reviewing: EM, LC.
Conflict of interest: the authors reported no poten-tial conflict of interests.
Received for publication: 20 May 2009. Revision received: 28 May 2009. Accepted for publication: 2 June 2009. This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0)
©Copyright M.G. Fabrini et al., 2009 Rare Tumors 2009; 1:e4
