Primary Pulmonary Synovial Sarcoma: A Case Report and Review of Current Diagnostic and Therapeutic Standards

Abstract

A 30-year-old female presented with hemoptysis, chest pain, and a rapidly enlarging pleural-based mass, and was found to have primary synovial sarcoma of the lung. Primary pulmonary sarcomas comprise <1% of all primary lung malignancies. They present clinically in young adults with cough, chest pain, shortness of breath, or hemoptysis, with a mass on x-ray and computerized tomography scan. Diagnosis is made by histology and immunohistochemistry. Histologic diagnosis has recently been supplemented by cytogenetic analysis, which offers important prognostic information. The mainstay of treatment remains complete surgical excision. Prognosis is poor, with an overall 5-year survival rate of 50%. The Oncologist 2004;9:339-342 The Oncologist 2004;9:339-342 www.TheOncologist.com Correspondence: Sheri Dennison, M.D., Department of Internal Medicine, Madigan Army Medical Center, Tacoma, Washington 98431-0001, USA. Telephone: 253-967-7523; Fax: 253-967-4013; e-mail: sheri.dennison@nw.amedd.army.mil Received August 4, 2003; accepted for publication December 16, 2003. ©AlphaMed Press 1083-7159/2004 CASEA 30-year-old Filipino-American female presented to the emergency department with hemoptysis and left-sided chest pain. The patient had experienced a mild cough and left-sided chest pain for 3 months. She awoke the morning of admission with severe chest pain and several episodes of hemoptysis, each with approximately 30 ml of bright red blood.Her past medical history was unremarkable. She had no previous surgeries. She did not smoke or drink alcohol. She had no family history of cancer. She had spent the previous year in Korea; she had a negative purified protein derivative 6 months prior to admission. On a review of systems, she was in excellent health and had no symptoms other than the cough and chest pain. The physical exam was remarkable only for The Oncologist ® Cancer Medicine: Case Discussion LEARNING OBJECTIVESAfter completing this course, the reader will be able to:1. Describe primary pulmonary synovial sarcoma as a rare tumor that usually arises in young adults as a large pleuralbased intrathoracic mass.2. Explain that the diagnosis is made by histology, immunohistochemical studies, and, more recently, by identification of the SYT-SSX1 or SYT-SSX2 chromosomal translocations.3. List factors that portend a worse prognosis, including inability to achieve a complete resection, larger tumor size, male sex, older age, high-grade tumor with necrosis, and the SYT-SSX1 variant.Access and take the CME test online and receive 1 hour of AMA PRA category 1 credit at CME.TheOncologist.com CME CMEThis material is protected by U.S.

Molecular Detection of SYT-SSX Fusion Gene Transcripts Currently Represents the Most Specific and Sensitive Tool for Diagnosing Intrathoracic Synovial Sarcoma

“…This tumor is not derived from "synovium", but from immature mesenchymal elements. Synovial sarcoma accounts for approximately 8-14%of soft tissue sarcomas (1,2 …”

A giant pleural poorly differentiated synovial sarcoma (PDSS) in a 64-year-old woman

“…The 5-year survival rates range from 50% to 75% (1,4,6,10). Favored prognostic indicators are: young age <20 years, Her-2 expression, complete resection, response to chemotherapy.…”

“…They might differentiate into mesenchymal or epithelial structures. They account for 5-14% of all the soft tissue sarcomas (3,4). In 1996, Gaertner et al reported the first five cases of SS in the pleural cavity (5).…”

Sarcoma sinovial anorretal: relato de caso

“…A confirmação diagnóstica é dada por estudo histopatológico e imunohistoquímico com vincristina (mais específica para spindle cells) e citoqueratina (mais específica para células epiteliais). O estudo citogenético pode revelar translocação cromossômica característica, denominada SYT-SSX, envolvendo os cromossomas X e 18 2,5,6,[8][9][10][11][12] . O tratamento é primariamente cirúrgico [1][2][3][4][5][6][7][8][9][10][11][12] , sendo a amputação abdomino-perineal do reto a cirurgia de escolha [1][2][3][4] , por tratar-se de uma lesão de alto índice mitótico, com altos índices de recidiva local (28 a 36%) 8 e de metástases à distância, presentes em 25% dos casos no momento do diagnóstico 9 .…”

“…O tratamento é primariamente cirúrgico [1][2][3][4][5][6][7][8][9][10][11][12] , sendo a amputação abdomino-perineal do reto a cirurgia de escolha [1][2][3][4] , por tratar-se de uma lesão de alto índice mitótico, com altos índices de recidiva local (28 a 36%) 8 e de metástases à distância, presentes em 25% dos casos no momento do diagnóstico 9 . A quimioterapia com doxarrubicina, dacarbazina ou ifosfamida ainda apresenta controvérsias, sendo utilizada com intuito de prevenir ou surpreender metástases à distância 2,[5][6][7][8]10,12 . A …”