Generally, parapharyngeal space (PPS) tumours are less than 1% of all head and neck tumours . Most PPS tumours are benign (70-80%). The malignant ones are fewer. The post - styloid compartment lesions tend to be of neurogenic origin whereas the prestyloid ones arise from the minor salivary glands in the lateral pharyngeal wall or extensions of tumours of the deep lobe of the parotid . Among the benign tumours of the PPS, the pleomorphicadenoma is the commonest . We present a case of a left parapharyngeal mass which caused a cerebrovascular accident (CVA) and was excised transorally with resolution of the CVA. We believe that the left parapharyngeal tumour in our case was compressing on the ipsilateral carotid sheath and therefore by inference on the walls of the internal carotid artery thus limiting the blood supply to the left half of the brain. This led to the left brainstem infarct with the resultant right hemiparesis. The occluded vessel recanalized after the tumour was excised with resolution of
Abstract: Obstructive sleep apnea syndrome (OSAS) is always caused by anatomic abnormalities, including nasal cavity, pharynx, and neuromuscular dysfunctions, leading to airway narrowing. OSAS associated with a mass in the aerodigestive tract is rare. In the present study, we report OSAS caused by 9 cases of preoperative uncom- mon tumors in the aerodigestive tract. Two tumors in the parapharyngeal space were pleomorphicadenoma, one oropharyngeal tumor was mucoepidermoid carcinoma, one tumor in the right tonsil was schwannoma, and five tumors were non-Hodgkin’s lymphoma (NHL). Of the five NHL cases, one in the nasopharynx was diffuse large B-cell lymphoma, two were mantle cell lymphoma, one was chronic lymphocytic leukemia/small lymphocytic lymphoma, and one was NHL. Tumors in the aerodigestive tract should be considered in the differential diagnosis of OSAS upon exacerbation of snoring or sudden gasping. Further examinations should be performed, including a routine workup (computed tomography (CT) and magnetic resonance imaging) and positron emission tomography/CT.
These cases have challenged the classical theory which proposes that PA surgical manipulations seed tumor cells and allow them to permeate blood vessels through which they spread and metastasize. As a result of the aforementioned evidence, new theories have risen. Czader et al. propose that MPA and CEPA are di ﬀ erent stages along a common biological pathway in malignant mixed-tumors spectrum. They hypothesize that metastasis capability of MPA more likely occurs secondary to accumulation of genetic mutations . The case reports done by Czader et al. and Fujimura et al. show that MPA can potentially progress overtime to a more malignant phenotype [4, 5].
The incidence of parapharyngeal space (PPS) tumors is low. Worldwide, only 0.5% of head and neck tumors originate in the PPS, 80% of which are benign . Of the primary tumors of PPS, 50% originate in the salivary glands and represent mostly pleomorphic adenomas from the deep lobe of the oblongata, 30% are neuro- genic tumors, typically from IX-XII schwannoma of the brain and sympathetic nerves, and 20% originate in soft tissue ; they include angio- mas, lipomas, teratomas, rhabdomyomas, and fibromas. An organized hematoma occurring in the PPS is extremely rare, and only one case has been reported thus far . This type of tumor is difficult to detect in the early clinical stage because of its unique anatomical loca- tion . The determination of the specific mechanism of PPS hematoma in the case reported in 2009 was not clear. The patient denied trauma, hemorrhage and blood system disease, the medical history and preoperative laboratory examinations did not indicate any definitive abnormality. The final diagnosis of
Fine needle aspiration (FNA) of the neck mass was consistent with poorly differentiated metastatic carci- noma, likely of squamous origin. Computerized tomo- graphy (CT) with contrast revealed a 3.0 × 3.0 × 1.7 cm mass within the left parapharyngeal space contiguous with the deep lobe of parotid, located between the carotid sheath and medial pterygoid muscle; a separate sharply circumscribed homogenous left level II. A lesion measur- ing 2.7 × 2.4 × 3.8 cm was also noted. Positron emitted tomography (PET) revealed hypermetabolic activity within both masses with no other lesions (Figure 1).
The lower pole of the parotid gland is the commonest location but deep lobe tumors can present as a parapharyngeal mass. Occasional to the tumor sites is the involvement of the accessory parotid. Pleomorphic adenomas usually are slow growing painless masses. Small tumors typically form smooth, mobile, firm lumps but larger tumors tend to become bossellated and may attenuate the over lying skin or mucosa. Some cases may reach a grotesque size. Multifocal, recurrent tumors may show some fixation to the underlying structures. PAs are seen in solitary, synchronous or metachronous paradigms. Association with other tumors, particularly Warthin tumor, in the ipsilateral or contralateral gland was also reported [2,3,8-13].
Although, the submandibular approach was found suffi- cient for the excision of both the submandibular and par- apharyngeal tumor in the current case, this surgical approach is considered a shortcoming, as some authors have expressed concern about limited exposure, need for finger dissection, and poor vascular control at the skull base with this approach . Injury to nerves and vessels as well as bleeding from the base of the skull has also been reported to be more common with blunt dissection than with sharp dissection. It is our opinion that the approach should be individualized based on the size and extent of tumor, and the knowledge, skill and experience of the operative surgeon.
Another differential diagnosis that should be considered is mucinous carcinoma. Papanico- laou staining alone might not be able to distinguish this case of MPMC from mucinous carcino- ma. However, Giemsa staining was helpful in that metachroma- sia observed on Giemsa staining is strong evidence of stromal mucin, which is different from epithelial mucin. In mucinous car- cinoma, mucin is secreted from carcinoma cells, i.e. epithelial cells, and the mucin does not show metachromasia on Giemsa staining because it is not stromal mucin. In our case, metachroma- sia on Giemsa staining was very clear and apparent; thus, the possibility of mucinous carcino- ma could be ruled out.
Rhabdomyosarcoma (RMS) is a malignant neoplasm of primitive mesenchyme exhibiting skeletal muscle differentiation. Intraoral Rhabdomyosarcomas are rare. We report here a case of pleomorphic Rhabdomyosarcoma of oral cavity in a 10-year-old child. The patient was diagnosed clinically and radiographically and planned for surgery. The clinical specimen was sent for histopathological studies . Definitive diagnosis is made by microscopic analysis and other auxiliary techniques such as immunohistochemistry, electron microscopy, cytogenetic analysis and molecular biology. Clinical, histological and immunohistochemical aspects suggests the diagnosis of rhabdomyosarcoma. Although rare, Rhabdomyosarcoma should be included in the differential diagnosis of intra-oral lesions, especially in children. This case illustrates clinical, histopathological aspects and diagnostic difficulties of Rhabdomyosarcoma. Taken together, we conclude that in children, any fast growing swelling should be carefully examined with a high degree of suspicion of this entity.Timely diagnosis and multidisciplinary treatment approach may improve the patient's survival.
Case presentation: We extensively analyze a giant submandibular mixed tumor of 25-year evolution in a 57-year- old Caucasian woman. Deoxyribonucleic acid ploidy was evaluated in different superficial and deep areas using flow cytometry analysis and correlated with pathological and immunohistochemical characteristics. Superficial areas exhibited a typical histological pleomorphicadenoma pattern and were deoxyribonucleic acid diploid. Deep samples showed deoxyribonucleic acid aneuploidy, atypical histological benign features and expression of markers involved at an early-stage of malignant transformation, such as tumor protein 53 and antigen Ki67.
Background. Potential epigenetic biomarkers for malignant transformation to Carcinoma ex- PleomorphicAdenoma (Ca ex PSA) have been sought previously with and without specific comparison to the benign variant, Pleomorphic Salivary Adenoma (PSA). Previous analysis has been limited by a non-quantitative approach. We sought to demonstrate quantitative promoter methylation across a panel of tumour suppressor genes in both Ca ex PSA and PSA.
The most important reason we focus on PA is that there is malignant transformation possibil- ity in tumors with long-term evolution, recur- rence, advanced age of patients or location in a minor salivary gland [6, 9], which ranges from 1.9%-23.3% . Malignant changes include three different types: carcinoma ex-pleomor- phic adenoma (CXPA, also called malignant mixed tumor), carcinosarcoma, and metasta- sizing PA, and the last twos are relatively rare . The classic clinical history of CXPA is a slow-growing mass for many years, which expe- rienced a sudden fast growth phase. Like other malignant tumors, CXPA could spread through direct extension, distance metastasis through Figure 3. The macroscopical images of excisional mass. A. The mass mea-
As mentioned above, conflicting data exist in literature concerning the presence of ERs and PRs. PLCIS is known to be immunohistochemi- cally positive for hormone receptors in addition to a high proliferation rate and HER2/neu over expression/ amplification . While around 80% cases of IPLC are positive for ER in most series, positivity for PR varies from 67% to 90% and Her2neu receptor varies from 53% to 81% in different case series . In the present case the IPLC was triple negative. It is unlikely that a prominent apocrine morphology may have con- tributed to the triple negative hormonal profile as most PLCs have apocrine features. It would be interesting to note if cytogenetics in such a case is different from the more common hor- mone positive variants of PLC.
Herein, the negative prognostic factors that indicated an aggressive tumor biology as evidenced by its unusual spread to the spleen were the advanced T stage (pT4a) and the invasiveness. In fact, the primary tumor infiltrated the skin surface and the next muscle tissue, while no vas- cular or perineural invasion was described. Initially, latero- cervical lymph nodes were not involved, but 1 year later the tumor relapsed to the supraclavicular lymph nodes. Furthermore, histologic examination of the primary and secondary lesions revealed a poorly differentiated epithelial neoplasm that was immunohistochemically positive for p63. Moreover, Ki67 labeling index was high (50%).
the central nervous system (CNS), optic nerve glioma (pilocytic astrocytoma) is the most com- mon. Other histological types of gliomas (e.g., diffuse astrocytoma and glioblastoma) can be seen, and other locations in the CNS can also be involved in this disease . Only a few cases of PXA have been described in individuals with NF1 [2-8]. Cases of cerebellar PXA are rare, and those cases associated with NF1 (PXA-NF1) are even rarer, with only 2 cases having been reported to date [6, 9]. We present herein a third case of PXA-NF1 with unusual clinicoradio- logical features. Molecular analysis of this tumor was also performed.
chemistry. Previous cases were reported that some immunohistochemical markers are used to remarkable distinguish PA from BSCC, such as cytokeratin and Ber-EP4 [17-21]. As report- ed, the expression of cytokeratin 19 is positive in PA [18, 19, 21] and is negative to BSCC [18, 20, 21]. In addition, positive immunohisto- chemical staining of Ber-EP4 provides strong evidence to identify PA from BSCC [17-19]. In a word, a definite diagnosis among extragingival PA and BSCC is of significance. Apart from BSCC, differential diagnosis for extragingival PA should consider a variety of mucosal and submucosal lesions of the oral cavity, such as peripheral ossifying fibroma, peripheral giant cell granuloma, odontogenic gingival epithelial hamartoma, other peripheral hyperplastic swellings superficial to the alveolar ridge and so on .
Irradiation of head and neck is the most presumable causative factor associated with the development of two pleomorphic adenomas in the parotid gland . In 1983, it was reported an increased incidence of salivary gland tumors after the atomic bomb explosions in Japan and the exposure effects were indicated to be both cumulative and dose dependent .
Pleomorphicadenoma (PA) is a common tumor occurred in salivary glands. The occurrence of PA located in breast is extremely rare and PAB was mostly found in the mammary subareola  of postmenopausal female . PA has a characteristic mixture of epithelial components and myoepithelial components embedded in myxochondroid matrix . To our knowledge, only 77 cases of PAB [4-7] have been reported in the literature since the first reported case of PAB by French scholar Lecène in 1906 . Indeed, the breast is a rare location of the PA and the rarity causes the confusion and diffi- culty in pathological diagnosis among other breast neoplasms, such as mucinous carcino- ma  and metaplastic carcinoma . Herein, we reported two cases of PAB and reviewed rel- evant literature briefly in order to avoid misdiag- nosis in this rare location.