Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS)

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade pleomorphic neoplasm with no identifiable line(s) of differentiation using currently available diagnostic techniques. Therefore, it is essentially a diagnosis of exclusion, which requires generous tissue sampling, adequate contextually interpreted immunohistochemistry, and relevant molecular studies. UPS is a common soft tissue sarcoma (historically one of the entities referred to as malignant fibrous histiocytoma (MFH)), which can develop in various organs, but lung involvement is usually due to metastasis. Primary Pulmonary UPS (PPUPS) is exceptionally rare and here we present a 66-year-old man who presented with anemia and weight loss, found to have a 17 cm right lung mass with invasion to the chest wall and diaphragm. Extensive sampling and immunohistochemistry studies failed to reveal any line of differentiation. Upon exclusion of a possible extrapulmonary origin, a diagnosis of PPUPS was rendered. In addition, we reviewed all 84 previously reported cases of PPUPS/PPMFH in the literature since 1979 and summarized the clinical information.

Round cell liposarcoma of scrotum with indolent course in young adult.

Myxoid/Round cell liposarcoma accounts for one-half of all liposarcomas and occurs as the second most common subtype. Both myxoid and round cell types share clinical, histological features and are accepted to represent a spectrum of lesions ranging from pure myxoid to near completely round cell liposarcoma. Round cell liposarcoma is highly metastatic and is classified as high grade and poorly differentiated myxoid sarcoma. Typical non-round cell myxoid liposarcoma is less metastatic and has more favorable prognosis. Karyotypic study of myxoid and round cell liposarcomas reveal a characteristic reciprocal translocation t (12;16)(q13;p11) resulting in the fusion of CHOP gene with TLS gene in more than 90% of cases. Most masses within the scrotal sac arise from the testis proper, and less likely from the extratesticular tissue. Liposarcoma represents approximately 20% of malignant extratesticular neoplasms, with the well differentiated subtype being the most common. Myxoid/round cell liposarcoma and round cell liposarcoma are rarely encounter in extratesticular soft tissue. We reported a rare case of round cell liposarcoma (high grade myxoid liposarcoma) of extratesticular tissue. To our knowledge, this is the first case of a large size (> 5cm) round cell liposarcoma arising from soft tissue within the scrotal sac of young adult with indolent course. Simple excision or enucleation are inadequate therapies and wide excision of the hemiscrotum including inguinal soft tissue and nodes is recommended. The role of retroperitoneal lymph node dissection and adjuvant chemotherapy remains controversial.