Case Report Classical biphasic pulmonary blastoma in a young woman: case report and review of literature

Case Report

Classical biphasic pulmonary blastoma in a young

woman: case report and review of literature

Maojiang Yang, Bing Li, Chuan Zhang, Sushant Kumar Das, Hanfeng Yang, Xiaoxue Xu

Department of Pain Management, Affiliated Hospital of North Sichuan Medical College, Nanchong, China

Received October 1, 2019; Accepted November 25, 2019; Epub December 1, 2019; Published December 15, 2019

Abstract: Pulmonary blastoma (PB) is a very rare malignant lung tumor, consisting of immature epithelium and/ or mesenchymal tissue. The two tissue components are derived from the same precursor cells. In this study, we present a case of a 29-year-old woman with classical biphasic pulmonary blastoma, who underwent right middle

lobe resection and subsequent treatment. Due to the low incidence rate and the reclassification of PB, no standard

treatment is available currently. In our case, the patient received radiotherapy and chemotherapy and is doing well at 6 months’ follow up. In retrospect, we review the pathology, clinical manifestations, imaging features and treat-ment of this disease.

Keywords: Classic biphasic pulmonary blastoma, metastasis, treatment, lung

Introduction

Primary pulmonary diphasic differentiation tumor is a very rare malignant tumor. Pulmona- ry blastoma (PB), comprising approximately 0.25%-0.5% of all lung malignancies, is consid-ered to consist of immature epithelium and/or mesenchymal tissue, similar in morphology to fetal lung tissue [1-4]. Since its first descrip-tion it was called “lung embryoma” [5, 6]. Furthermore, there is no standardized treat-ment for this disease. The prognosis of classic biphasic pulmonary blastoma is poor and the overall 5-year survival rate is around 15% [7, 8]. Herein, we describe a 29-year-old woman with classic biphasic pulmonary blastoma (CBPB), who received comprehensive treatment in our unit.

Case report

A 29-year-old woman presented with cough and phlegm accompanied by blood in sputum for three months. On chest X-ray examination at local hospital an abnormal shadow was detect-ed on the right lung field. After 2 weeks of anti-inflammatory treatment, the condition did not alleviate. She was then transferred to our hos-pital for treatment. Contrast enhanced chest CT was performed which revealed a 5.1×4.1×

4.1 cm nidus in the middle lobe of right lung (Figure 1). The boundary of the lesion was clear and smooth, the enhancement was uneven, and many small patches of necrosis could be seen in it. In addition, several large vessels were seen passing through the margin and inside the lesion. Tumor marker examination revealed gastrin-releasing peptide precursor was 116.30 pg/ml, and cytokeratin 19 frag-ment was 5.88 pg/m. A CT-guided percutane-ous needle biopsy of the lung tumor was per-formed. Results showed that the epithelial cells had adenoid and squamous cell distribution. Immunohistochemical staining showed ER (-), PR (-), TTF-1 (+), P40 (+), and CDX2 (+), mainly composed of adenocarcinoma. Some of the tumor cells expressed P40, suggesting the pos-sibility of adenosquamous carcinoma (Figure 2).

hetero-morphic spindle cells were seen. Immunohi- stochemical staining showed CK (epithelial component +), TTF-1 (adenoid region +), CgA (-), P40 (squamous differentiation component +), vimentin (spindle cell +), desmin (-), CD34 (par-tial spindle cell +), Ki67 (epithelial region 60%, spindle cell region 30%), SMA (-), and beta-catenin (+) (Figure 3). Tumors had dual differen-tiation, most of which were low-grade fetal ade-nocarcinomas, and a few (about 3%) were immature mesenchymal cells, which is consis-tent with pulmonary blastoma. Subsequently, she received radiotherapy and nedaplatin as

chemotherapy and is doing well at 6 months’ followup. Discussion

PB is an uncommon primary lung malignancy with a fre-quency of only 0.25-0.5% of primary lung malignancies [1- 4]. It was first described by Barrett in 1945, and then Barnard named it as “embryo-ma”. Later it was renamed as “pulmonary blastomalater” [5, 6, 9]. In the past, PB was clas-sified into three subgroups: (1) classic biphasic pulmonary blastoma (CBPC), character-ized by histopathologic hetero-geneity, i.e. intermixing of epi-thelial and mesenchymal ma- lignant cells, (2) well-differen- tiated fetal adenocarcinoma (WDFA), characterized by a monophasic epithelial tumor, and (3) pleuro-pulmonary blas-toma (PPB), characterized by a monophasic mesenchymal tumor. According to the 2015 WHO classification, PB is con-sidered as a type of sarcoma-toid carcinoma and is defined as a biphasic tumor consisting of fetal adenocarcinoma (typi-cally low-grade) and primi- tive mesenchymal stroma [10]. Actual cases of PB may be less than those previously reported because many earlier reports on PB have included fetal ade-nocarcinoma and PPB [11]. Figure 1. Typical CT (computed tomography) images of our patient. Contrast

enhanced chest CT was performed which revealed a 5.1×4.1×4.1 cm nidus in the middle lobe of right lung. (A) Lung window, (B) Mediastinal window, (C, D) Coronal and sagittal CT.

Figure 2. The results of CT-guided puncture. (A) Epithelial cells had adenoid and squamous cell distribution, (B) Immunohistochemical staining showed ER (-), PR (-), TTF-1 (+), P40 (+), and CDX2 (+), mainly composed of adeno-carcinoma. Some of the tumor cells expressed P40.

while others are often found by accident [15-17]. In our patient, cough and phlegm accom-panied by blood in sputum were the major symptoms.

Currently, there is no specific serum marker for pulmonary blastomas [18]. Serum levels of tumor markers, AFP, NSE, and CEA, were increased in our patient as reported in the previous reports. Especially, increased AFP positive tumor cells have been seen in most pulmonary blastoma cases [19, 20]. In this case study, we found abnormal increases in gastrin-releasing peptide precursors and cyto-keratin 19 fragments. On imaging, lesions often present as solitary masses in the periph-eral pulmonary zone with clear margins. Contrast enhanced CT scans often show a het-erogeneous enhancing soft tissue mass with smooth or lobulated margins and probable areas of necrosis [21]. In this patient, in addi-tion to the above findings, several large blood vessels were seen passing through the lesion and surrounding the lesion.

Due to the biphasic differentiation of tumors, general fine needle aspiration biopsy often can-not make a definite diagnosis due to lack of materials. The diagnosis depends on postop-erative pathologic analysis [11, 15]. Immuno- histochemical analysis is of great significance in the diagnosis of this disease, especially com-bining epithelial markers with mesenchymal markers [22-24]. Similar to previous literature, we found CK (epithelial component +), TTF-1 (adenoid region +), P40 (squamous differentia-tion component +), vimentin (spindle cell +), CD34 (partial spindle cell +), Ki67 (epithelial

therapy has not been well consolidated because it is still rare, though radiotherapy and chemotherapy regimens are progressing rapid-ly [25]. The prognosis of biphasic pulmonary blastoma is extremely poor as distant metasta-sis is common. As reported, the 5-year survival rate and 10-year survival rate of BPB are 16%-25% and 8% respectively [13]. The reported median survival is 26 months in patients under-going lobectomy or lobectomy, and the median survival is 9 months in patients undergoing pneumonectomy [26]. In our case, the patient underwent a right middle lobe resection and was treated with nedaplatin. After a follow-up of six months, the patient’s condition was stable and no recurrence or distant metastasis was observed.

Conclusions

Classical biphasic pulmonary blastoma is rare. Surgical resection is the main method for its treatment. In addition, radiotherapy and che-motherapy have also been reported, but so far, no consensus has been reached.

Acknowledgements

We owe thanks to the patient and her family for their willingness to provide information. The authors thank all of the staffs who contributed in this paper.

Disclosure of conflict of interest

None.

Address correspondence to: Xiaoxue Xu, Depart-

ment of Pain Management, Affiliated Hospital of

Figure 3. Postoperative pathologic results. A. Tumors show classical bipha-sic differentiation. B. Immunohistochemical staining showed CK (epithelial component +), TTF-1 (adenoid region +), CgA (-), P40 (squamous differen-tiation component +), vimentin (spindle cell +), desmin (-), CD34 (partial spindle cell +), Ki67 (epithelial region 60%, spindle cell region 30%), SMA (-), and beta-catenin (+).

North Sichuan Medical College, Nanchong, China. E-mail: nclittlesnownc@163.com

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