SS18-SSX Translocation FISH Negative Mesenteric Synovial Sarcoma-Documentation of an Extremely Rare Entity
- PDF / 415,873 Bytes
- 5 Pages / 595.276 x 790.866 pts Page_size
- 6 Downloads / 173 Views
CASE REPORT
SS18-SSX Translocation FISH Negative Mesenteric Synovial Sarcoma-Documentation of an Extremely Rare Entity Rishi Philip Mathew 1 George Sunny 4
&
Teena Sleeba 1
&
T. S. Subi 2
&
Latha K. Abraham 3
&
Nirmal Ganesh Neelakandan 2
&
Accepted: 30 October 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Introduction First described in 1983, synovial sarcomas are a rare subtype of soft tissue sarcoma [1]. The reported incidence for soft tissue sarcomas are 2–3 cases per 100,000 people per year, of which only 6% turn out to be synovial sarcomas after histopathological examination [2]. The term synovial sarcoma is a misnomer as it only resembles synovial tissue at light microscopy and does not originate from the synovium. Although synovial sarcomas are most commonly (80%) seen in the deep soft tissues of the extremities around large joints and tendons, it can occur in any region of the body, and is most commonly seen in young adults with a male predominance. It is a highgrade tumor and 50% of the patients presents with distant metastases, most commonly to the lungs [3]. Approximately 5–10% of synovial sarcomas occur in the head and neck, mediastinum, esophagus, abdominal wall, and retroperitoneum. Other rare sites where the tumor has been documented in literature include the skin, blood vessels, nerves, mediastinum, pleural cavity, kidney, and the prostate [4]. Primary synovial sarcomas of the mesentery are extremely rare. To the best of our knowledge, only 7 cases of primary synovial sarcomas involving the mesentery have been reported till date. Our case was unique as the patient’s fluorescence in situ hybridization (FISH) test for SS18-SSx translocation,
widely considered the gold standard for diagnosing synovial sarcoma, was negative.
Case Report A 34-year-old female patient presented with complaints of abdomen pain and distention, early satiety, and constipation for 1 week. She was initially evaluated by ultrasound elsewhere (images not available), which showed a large abdominal solid-cystic mass. The uterus and ovaries were seen separately and were unremarkable. On local clinical examination, a large diffuse abdominal lump of approximate size 25 × 20 cm was palpable. No ascites was appreciable. Magnetic resonance imaging (MRI) of the abdomen (Fig. 1a–c) revealed a large 27 × 23 cm intrabdominal solid-cystic mass with a thick wall. The thick irregular wall of the lesion was mildly hyperintense to that of the muscle on T1- and T2-weighed images, and showed enhancement following administration of contrast. The possibility of a mesenteric origin for the mass was raised on MRI with the differentials being gastrointestinal stromal tumor (GIST) and a sarcoma. The patient was taken up for explorative laparotomy and possible excision. Intra-operatively, a large soft tissue mass was noted arising from the root of the mesentery, more towards
* Rishi Philip Mathew [email protected]
George Sunny [email protected]
Teena Sleeba [email protected]
1
Department
Data Loading...
