Primary pulmonary Ewing’s sarcoma: rare cause of massive hemothorax in a young girl-case report

Abstract

Background Ewing’s sarcoma is a common malignant bone tumor in children and young adults. Rarely, extra-skeletal soft tissues and visceral organs can also be the site of origin of Ewing’s sarcoma. Primary pulmonary Ewing’s sarcoma is an extremely rare malignancy. Case presentation We report an unusual case of primary pulmonary Ewing’s sarcoma in a 15-year-old girl who initially presented with massive hemothorax. By histopathology evaluation of surgical biopsy specimens, the diagnosis of extraosseous Ewing’s sarcoma was confirmed by both light microscopy and immunohistochemistry. Emergency, open surgery was performed by thoracic surgery at an early stage. After 3 cycles of chemotherapy, the patient was found to be stable at follow-up examination. No more hydrothorax or other symptoms. Conclusions We have described an extremely rare case of primary pulmonary Ewing’s sarcoma with massive hemothorax. The patient underwent surgical resection and postoperative chemotherapy, no sign of recurrence to date as an outcome.

Application of Anlotinib Combined With Neoadjuvant Chemotherapy in Primary EWS/PNET of Lung: A Case Report

“…CT characterization of Ewing’s sarcoma in the lung is still limited, and is usually described as ( 7 , 11 – 13 ). 1st, The masses are of different sizes, being single or multiple, with clear boundaries and possible lobulation.…”

“…Ewing’s sarcoma is characterized by strong invasiveness, rapid metastases and poor prognosis. The 5-year survival rate is only 25% ( 7 ). Current treatments include surgery, chemotherapy and radiation.…”

Ewing Sarcoma of the Lung: Imaging of a Rare Tumor

“…First described by James Ewing et al [2], the Ewing sarcoma family of tumors encompasses a group of malignancies that include Ewing sarcoma of the bone, extraosseous Ewing sarcoma, peripheral primitive neuroectodermal tumor (PNET), and thoracopulmonary PNET, also known as Askin tumor [3]. PPES is rare, with only a handful of cases reported so far (Table 1) [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. Imaging plays an important role in the evaluation of this rare clinical entity.…”

A primary Ewing's sarcoma of pleura: Case report and literature review

“…18-Fluorodeoxyglucose positron emission tomography showed that Ewing sarcoma of the lung often showed large volume, smooth edge, and high fluorodeoxyglucose uptake [ 4 ]. In addition, it can also be used as a tool for initial staging, efficacy evaluation, and recurrence detection and help to exclude the possibility of secondary extrapulmonary Ewing sarcoma [ 6 ].…”

“…Immunohistochemical staining showed diffuse membranous CD99 positive cells ( Fig. 5 ), which is of diagnostic significance for Ewing's sarcoma and primitive neuroectodermal tumor [ 4 ], followed by a FISH image of specimens confirmed the rearrangement of the 22q12 (EWSR1 gene), a potent and reliable auxiliary technology for the EES diagnosis [ 5 ]. The whole-body bone scan and abdominal ultrasound examination showed no abnormal lesions.…”