Baburao Archana, Maurya Poonam, Chakenahalli Amulya, Narayanswamy Huliraj
Department of Pulmonary Medicine, Rajarajeswari Medical College and Hospital, Bangalore.
Department of medical Oncology, Kempegowda Institute of Medical Sciences, Bangalore.
Monaldi Arch Chest Dis. 2021 Nov 16;92(2). doi: 10.4081/monaldi.2021.1874.
Desmoplastic small round cell tumor (DSRCT) is a rare, extremely aggressive and malignant tumor predominantly affects young adolescent males and typically presents as a large intra-abdominal mass. However, tumor arising from other body sites are also reported in the literature. Histology and immunohistochemistry play an important role in the diagnosis and differentiating this rare tumor from other round cell tumors. A multidisciplinary approach consisting of a combination of surgery, chemotherapy and radiation therapy is the treatment of choice as there is no standard therapy. We report a case of DSRCT of pleura presenting as bilateral pleural effusion in a young adolescent male who was treated with both surgery and chemotherapy. However, the patient succumbed to illness after one year of diagnosis.
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见、极具侵袭性的恶性肿瘤，主要影响青少年男性，通常表现为腹腔内的巨大肿块。然而，文献中也报道了起源于身体其他部位的肿瘤。组织学和免疫组织化学在诊断以及将这种罕见肿瘤与其他圆形细胞肿瘤鉴别方面发挥着重要作用。由于没有标准治疗方法，多学科方法（包括手术、化疗和放疗的联合）是首选的治疗方式。我们报告了一例青少年男性胸膜促结缔组织增生性小圆细胞肿瘤，表现为双侧胸腔积液，接受了手术和化疗。然而，患者在确诊一年后病逝。
