Malignant Small Blue Cell Tumors Research Articles

Opaque Hemithorax in a Young Girl: A Rare Case of Askin’s Tumour

Askin’s tumor is a primitive neuroectodermal tumor primarily affecting the thoracopulmonary region. It typically manifests in children and adolescents, with a notable female preponderance. Histologically, it presents as a malignant small blue cell tumor. Clinical presentation often includes respiratory symptoms such as pain, dyspnoea, and weight loss. Prognosis is generally unfavorable with a median survival of around 8 months and an overall survival rate of approximately 60% at 5 years. This report highlights the case of a 13-year-old female diagnosed with Askin’s tumor. This report highlights the case of a 13-year-old female presented with a 2-month history of back pain, right-sided chest pain, exertional breathlessness, and loss of appetite. Examination revealed dyspnoea, pallor, and bilateral cervical lymphadenopathy. Radiological and histopathological investigations confirmed the diagnosis of Askin’s tumor with malignant pleural effusion and distant metastasis. This case underscores the importance of considering Askin’s tumor in the differential diagnosis of thoracic masses in pediatric patients. Early recognition and a multidisciplinary approach are crucial for optimizing patient outcomes.

Desmoplastic small round cell tumor of the central nervous system: report of two cases and review of the literature

Desmoplastic small round cell tumor (DSRCT) is a malignant tumor often involving the abdominal and/or pelvic peritoneum. Only one fully documented example has arisen in the central nervous system (CNS). Herein, we describe two additional examples, fulfilling the morphologic, immunohistochemical, and molecular criteria (EWS/WT1 translocation) of DSRCT. Both arose in the cerebellopontine angle (CPA) and underwent spinal dissemination. Patient 1, a 37-year-old male, underwent a subtotal resection, and 2 years later died of recurrent disease with spinal dissemination. Patient 2, a 39-year-old man, presented with cerebellar and CPA lesions as well as spinal leptomeningeal deposits. After 27 months of adjuvant therapy, he is alive with progressive disease. In conclusion, CNS DSRCT follows a similar aggressive course as do peritoneal examples. Although rare, DSRCT warrants consideration in the differential diagnosis of "malignant small blue cell tumors" of the CNS.