Synovial Sarcoma In The Anterior Abdominal Wall Of A Young Nigerian Female: Case Report.

摘要

Synovial sarcoma is a malignant mesenchymal neoplasm so named after its microscopic resemblance to normal synovium. Large joints of the extremities are the most common site and, rarely, the tumor may be found in the anterior abdominal wall. We report a case of synovial sarcoma in the right upper quadrant of the anterior abdominal wall of a 21-year-old Nigerian female. The patient underwent surgical excision with clear margins and the histological and immunohistochemical studies confirmed a monophasic synovial sarcoma. Funding BC is supported by the Earthwide Surgical Foundation. Histological and immunological examinations were provided by the Brigham and Women’s Hospital Department of Pathology (Boston, MA). Acknowledgements We would like to thank the pathologists of the Brigham and Women’s Hospital for their continued support of this collaboration. Introduction Synovial sarcoma is a malignant mesenchymal neoplasm with dual epithelial and mesenchymal spindle cell morphology. It commonly occurs in the individuals in their second to fifth decades of life[1-4]. The first two cases of the tumor arising in the abdominal wall were reported by Pack and Ariel in 1950[5]. A literature search showed that up to 2005, a total of 44 cases were reported in English literature with about 30 cases reported between 1978 and 2005. We report the first case of histologically confirmed anterior abdominal wall sarcoma in a Nigerian patient. Case Report A 21-year-old previously healthy Nigerian female presented for medical care in a mission hospital in Onicha-Ngwa, a suburban part of Abia State, Nigeria. She noticed a non-tender, swelling in the right upper quadrant of her abdomen two years prior to presentation with gradual increase in size. She reported no gastrointestinal symptoms or weight loss. The mass was located within the muscular layer of the right upper quadrant of the anterior abdominal wall and measured 15 by 6 centimeters (fig. 1). It was ovoid in shape and firm but with a soft center. The overlying skin was mobile. No additional masses were found on physical examination and imaging. She was healthy looking and had no jaundice or signs of anemia.Preoperative investigations were limited to complete blood count (within normal limits), urinalysis (within normal limits) and non-fasting blood glucose (87milligrams/deciliter). An abdominal ultrasound revealed a mixed echogenic mass arising from the anterior abdominal wall. Intra-operatively, the encapsulated mass was firm and appeared to arise from the oblique muscles or, possibly, the rectus muscle. There was hypervascularity of the parietal peritoneum but the tumor did not grossly penetrate into the abdomen. The aponeuroses and muscle groups bordering the mass appeared grossly normal. The tumor was completely excised with a 2cm surgical margin, which included portions of the rectus sheath and the parietal peritoneum. The overlying subcutaneous tissue and skin were spared and used for wound closure. The defect was reconstructed with polypropylene mesh which was covered with the overlying skin and subcutaneous tissue (figs. 2). The bisected tumor showed multi-cystic blood-filled areas and some pale solid portions (fig. 3). The histo-pathological report demonstrated a monophasic (fibroblast-like spindle cell) synovial sarcoma (figs. 4 and 5) with a negative margin. Immunohistochemical stain showed a strong positive reaction to monoclonal antibody to TLE1 (fig. 6). The tumor was also positive to EMA and CD34 (fig.6). Following surgery, the patient had an uneventful course. At three-month follow-up there is no sign of recurrence, the wound is well-healed, and she is preparing for radiation therapy (fig. 7).