Primary Ewing sarcoma/primitive neuroectodermal tumor of the lung

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.

Primary thymic mediastinal seminoma with florid granulomatous reaction.

Primary mediastinal seminomas are rare tumors. Morphologically, they can be associated with secondary changes in the form of thymic remnants, cystic change, epithelioid granulomas, abundant fibrosis, and syncytiotrophoblast like cells. Ours was an interesting case where a 17‑year‑old adolescent male presented with anterior mediastinal mass. Fine needle aspiration and trucut biopsy of the mass favored tuberculosis; however, he did not respond to antituberculosis treatment regime. He was subjected to video‑assisted thoracoscopic surgery and the histopathological examination along with supportive immunohistochemistry of the mass revealed scattered seminoma cells which were being masked by extensive granulomatous reaction. The aim of this article is to report a case of primary mediastinal thymic seminoma in a young male associated with extensive granulomatous reaction masking the neoplastic population of cells leading to erroneous diagnosis on small biopsy.

Adult‑onset Still’s disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare manifestation with fatal outcome.

Adult‑onset Still’s disease (AOSD) is a rare inflammatory disorder of unknown etiology characterized by fever, evanescent pink salmon rash, arthritis, and multiorgan involvement. Here, we report an unusual manifestation of AOSD in a 40‑year‑old male who presented to our hospital with pyrexia of unknown origin and rash of 3 weeks duration. All his serological investigations and imaging studies were unremarkable. He was fulfilling clinical and laboratory criteria as per Yamaguchi for AOSD and was managed for the same. Our patient did not respond well to the treatment, had a downhill course, and succumbed to his illness. Autopsy confirmed myocarditis and florid bone marrow reactive hemophagocytosis as the cause of his death.

Primary primitive neuroectodermal tumor of kidney: A rare case report with diagnostic challenge.

Primary primitive neuroectodermal tumors (PNETs) of the kidney are quite rare and can be mistaken for a wide variety of other small round blue cell tumors which includes rhabdomyosarcoma, Wilm’s tumor, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma etc. Renal Ewings/PNET can occur in the age group from 4 to 61 years. Approximately, 90% of Ewing sarcoma (ES)/ PNET have a specifi c t(11;22) which results in a chimeric EWS-FLI-1 fusion protein. Immunohistochemical for the carboxy-terminus of FLI-1 is sensitive and highly specifi c for the diagnosis of ES/PNET. Herein, we have an interesting presentation in a 23-year-old male who came with neck pain and progressive quadriparesis and was diagnosed as a case of poorly differentiated malignant tumor with a differential of lymphoma versus metastatic renal cell carcinoma. The patient’s condition deteriorated fast and he had a rapid downhill course. The fi nal diagnosis of Ewings/PNET was confi rmed at autopsy.