Disorders of the Pleura |

Primary Pleural Synovial Sarcoma: A Diagnostic Challenge FREE TO VIEW

Prachi Kate, MBBS; Swapnil Karnik, MBBS; Bhushan Khedkar, MBBS; Amit Singh, MBBS; Nita Munshi, MBBS
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Ruby Hall Clinic, Pune, India

Chest. 2013;144(4_MeetingAbstracts):501A. doi:10.1378/chest.1705367
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SESSION TITLE: Pleural Global Case Reports

SESSION TYPE: Global Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Primary pleural sarcomas comprise <1% of all primary lung malignancies. Main challenges with diagnosis of pleural sarcoma is its differentiation from mesothelioma (which is more common & resembles it morphologically) & Tuberculosis (specially in TB prevalent countries like India).

CASE PRESENTATION: A 24 year old 3rd trimester primigravida with history of gestational hypertension presented with intermittent fever, dry cough, non-exertional shortness of breath worsening for 1 week. Physical examination revealed afebrile female in no acute distress with reduced air entry on left side of chest. CBC showed neutrophilia & microcytic, hypochromic anemia with anisocytosis. Chemistry revealed LDH 872 U/L,; Albumin 2.6 g/dl & CA125 335 U/ml. Ultrasound (US) thorax showed well defined heterogenous lesion with few cystic areas in left lower zone measuring 9.2 cm X 10 cm. CT showed left hemithorax mass with mediastinal shift & thin layer of pleural effusion. Emergency C-section was done to deliver the baby at 7 months. After one week CT guided biopsy of the chest mass was done. Biopsy showed sheets of spindle cells with elongated hyperchromatic nuclei & clear vacuolated cytoplasm over a background of myxoid change. Differential diagnosis of synovial sarcoma & inflammatory myofibroblastic tumour were considered. Immunohistochemistry (IHC) was focal positive for cytokeratin, positive for EMA and faint positive for CD99. Surgical resection of the tumor was done followed by chemotherapy. Breast feeding was prohibited though lactation was not affected. After 1 year follow-up, patient does not have any recurrence. The baby is healthy. A 42 years old male engineer with no past medical history presented with recurrent massive left pleural effusion. He was started with empiric anti-tubercular treatment & was referred to us for further management. Physical examination revealed decreased air entry on left side. CBC and chemistry was normal. CT scan of thorax showed massive left sided pleural effusion with left pleural mass and midline shift. Therapeutic thoracocentesis was done followed by CT guided pleural biopsy. Biopsy results were similar to previous case. IHC showed positive EMA , positive CD99, strongly positive BCL2 and focal positive calretinin. Diagnosis of synovial sarcoma was established and pleuropneumonectomy was done followed bychemotherapy. Patient did could not complete the chemotherapy and expired 5 months later.

DISCUSSION: The origin of synovial sarcoma is unknown & the name is derived because of its resembelance to synovial cells. Diagnosis is a challenge because of resembling appearance and rarity. Diagosis is usually made on histology, immunohistochemistry with cytogenetic testing, FISH or RT-PCR. EMA, CD 99, BCL2, calretinin are usually positive and synaptophysin and HBME are characteristically negative.

CONCLUSIONS: Synovial sarcomas are rare tumors & diagnosis is a challenge. Proper diagnosis must be established before definitive treatment is planned in order to avoid improper treatment.

Reference #1: Synovial sarcoma prognostic factors and patterns of failure.Paulino AC.Am J Clin Oncol. 2004;27(2):122.

DISCLOSURE: The following authors have nothing to disclose: Prachi Kate, Swapnil Karnik, Bhushan Khedkar, Amit Singh, Nita Munshi

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