Clinical and radiographic presentation of pelvic sarcoma in children

Introduction: Pelvic sarcomas may present with vague symptoms. The aim of this study was to examine the characteristics and clinical presentations of different types of pelvic sarcoma in children. Methods: This is a retrospective cohort study of patients up to 21 years of age with the diagnosis of pelvic sarcoma between January 2000 and June 2013. Data on demographics, tumor type and location, and clinical presentation were collected from the medical records. Results: A total of 59 patients [37 males (62.7%) and 22 females (37.3%)] were examined in this study. Mean age at presentation was 11.3 ± 5 years (range 0.8–21 years). Thirty-six patients had Ewing sarcoma (61%), 9 osteosarcoma (6.8%), 4 undifferentiated sarcoma (6.8%), 2 (3.4%) rhabdomyosarcoma, 2 synovial cell sarcoma, and one (1.7%) of each fibrosarcoma, dermatofibrosarcoma, fibromyxoid sarcoma, chondrosarcoma, chordoma, and epithelioid sarcoma. Pain at presentation was reported in 41 patients, 13 mass, 8 limping, and 5 neurologic symptoms. Most of the bony tumors were painful (77%), while most of the soft tissue tumors were painless (70%). Nine patients presented with constitutional symptoms. Most patients presented within 4–12 months from symptoms beginning. Twenty-one patients (35.6%) presented with metastases (14 Ewing sarcoma, 6 osteosarcoma, and 1 synovial cell sarcoma). Pelvic radiographs showed lytic lesion in 11 patients, 4 sclerotic lesions, 6 mixed lesion, 6 had only soft tissue mass, 1 radiograph showed osteopenia, and 2 radiographs were reported normal. Conclusion: Ewing sarcoma was the most common pelvic sarcoma tumor in children. In most cases, pelvic sarcoma in children presented with pain mimicking other benign conditions. Some patients presented with metastatic disease with no prognostic clinical or radiographical signs or symptoms. Pelvic sarcoma should be considered a differential diagnosis as part of children work up.

Synovial Cell Sarcoma of the Kidney: A Case Report

Background: Primary renal sarcomas are rare neoplasm that accounts about 1% of malignant renal tumors. Prevelance of primary renal Synovial cell sarcoma is rare and comprise 1-3% of all malignant renal neoplasm. Synovial cell sarcoma overlaps with multiple spindle cell neoplasms affecting the kidney, this need immunohistochemical panel to can diagnose it. This paper reports a case of Renal Synovial cell sarcoma. We report a case of 32 year old female presented with presented by upper pole left kidney swelling. Computarized tomography (CT) revealed a heterogeneous, well marginated soft tissue mass 8x7 cm arising in the upper pole of left kidney with solid necrotic components and heterogeneous enhancement. Left radical nephrectomy was done. Methods: The kidney was excised and gross examination revealed that upper pole of the kidney was replaced completely by grayish tan firm mass with cystic areas measuring 6x6x3 cm in diameter and shows areas of hemorrhage and necrosis. Microscopic evaluation and immunohistochemistry study were performed. Results: The mass was Renal Synovial cell sarcoma. Conclusion: Although Renal Synovial cell sarcoma is rarely diagnosed in kidney but it should be considered in the differential diagnosis of spindle cell tumors affecting the kidney and must be excluded by immunohistochemical studies as it has poor prognosis in the kidney

Superior vena cava obstruction as manifestation of synovial cell sarcoma: a case report and review of literature

Synovial cell sarcomas are one of the most common soft tissue tumors affecting adolescents and young adults. The common location of these tumors is often extremities. Primary involvement of mediastinum is a very rare presentation of this tumor, only few cases have been reported so far. We are reporting the case of a 27-year-old male patient who presented to us with a history of dyspnoea and chest discomfort of one week duration along with features of SVC obstruction. Chest is a frequently involved site of metastasis and lymphomas but rarely involved in synovial sarcoma. The learning point from this case is that rare causes like synovial sarcoma of mediastinum should always be included in differential diagnosis of mediastinal mass and SVC obstruction. We are reporting this case in view of its rarity. Though uncommon this case report emphasizes that synovial cell sarcoma should also be considered in differential diagnosis of mediastinal mass and early recognition with HPE, IHC and prompt institution of treatment can be lifesaving.