Primary Ewing sarcoma/primitive neuroectodermal tumor of the lung

Abstract

We present the autopsy findings and differential diagnosis in a 42year old male who presented with fever and rapidly progressive respiratory symptoms like breathlessness, nonproductive cough and right sided chest pain. Initial imaging workup done at our hospital revealed a large unilateral tumor with tracheal shift. While being evaluated patient developed facial puffiness, tachypnea suggestive of superior vena cava obstruction. Antemortem biopsy of lung mass was attempted twice and that suggested malignant lesion. Unfortunately, the individual had a rapid downhill course following admission. Post mortem examination was conducted that on opening the thoracic cavity revealed total replacement of right lung tissue by a necrotic growth which was deeply adherent to the rib cage. The contralateral lung as well as all other visceral organs were unremarkable grossly. Histopathology confirmed primary Ewing sarcoma of the lung. We hereby, report a rare case of primary lung Ewing sarcoma diagnosed at autopsy.

A huge Ewing's sarcoma of the rib presenting with superior vena cava syndrome and dysphagia

“…The common symptoms of Ewing's sarcoma of the rib are chest pain, fever and malaise, and the most important clinical findings are a palpable mass and pleural effusion 7 . Devika et al 8 reported a male patient who developed facial puffiness and tachypnea which suggested superior vena cava obstruction. However, Ewing's sarcoma of rib presenting with superior vena cava syndrome (dizziness) and dysphagia has not yet been reported.…”

Primary EWS/PNET of the lung with TP53 germline and SKT11 somatic mutation: A case report and review of the literature

“…PPES is rare, and the clinical symptoms of cough, phlegm, chest pain, hemoptysis or fatigue, etc are nonspecific. The disease has been found to occur at any age, mostly in children and adolescents, and peaks at the age of 20–30 years old, without any difference in gender 5–7 …”

“…IHC showed characteristic expression of neural differentiation, with high expression of CD99, NSE, FLI‐1 and Syn as relatively specific indicators for diagnosis. Such type of fusion gene is often used to diagnose pathological states, quantitative polymerase chain reaction (qPCR), EWS FLI1 and FLI1 EWS quantitative expression, to reveal the EWSR1 rearrangement by FISH 3,7,11,12 …”

“…The disease has been found to occur at any age, mostly in children and adolescents, and peaks at the age of 20-30 years old, without any difference in gender. [5][6][7] CT characterization of PPES is still limited 8-10 and the masses are usually described as being of different size, single or multiple, with clear boundaries and possible lobulation. A mass in a single lung is more common, F I G U R E 1 CT thorax.…”

“…Such type of fusion gene is often used to diagnose pathological states, quantitative polymerase chain reaction (qPCR), EWS FLI1 and FLI1 EWS quantitative expression, to reveal the EWSR1 rearrangement by FISH. 3,7,11,12 There is currently no standard treatment for PPES. Treatment includes surgery, chemotherapy and radiation.…”