Multiple Intra-abdominal Leiomyosarcoma in a Single Patient
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CASE REPORT
Multiple Intra-abdominal Leiomyosarcoma in a Single Patient Rajshekhar C. Jaka 1,2
&
N. Chandra Mouli 1 & T. Ravi Kumar 1 & Akshay V. Gokak 1
Received: 25 November 2019 / Accepted: 27 January 2020 # Indian Association of Surgical Oncology 2020
Leiomyosarcomas (LMS) are malignant lesions that develop from the smooth muscles of blood vessels, visceral organs and uterus. It is the predominant sarcoma originating from the large blood vessels. Leiomyosarcoma originating from small bowel or intra-abdominal blood vessels is very rare. We report a unique and rare case of multiple synchronous leiomyosarcomas originating from blood vessels of mesentery, omentum and bowel.
Case Report Forty-one-year-old male patient was presented to the emergency department with gross distension of abdomen, bilious vomiting, melena and anaemia. On examination, he had a huge mass in the abdomen with a poor general condition. Computerised tomography (CT) showed a large mass in the upper abdomen with multiple small tumours. Image-guided biopsy of the large mass showed spindle cell tumour. There was no distant metastasis. In view of acute intestinal obstruction, he underwent surgery with probable preoperative diagnosis of gastrointestinal stromal tumour (GIST). The large mass was 30 cm in size in the upper mesentery involving the proximal jejunum. It was removed intact along with jejunum. Multiple small independent tumours ranging in size from 1– 5 cm, present over the omentum, small and large bowel, mesentery, peritoneum and urinary bladder, were removed (Fig. 1). At initial look, we were not sure of removing all tumours but we held our nerves and persisted with patience as surgery was the only curative option. All tumours were well-encapsulated, distinct and not muscle invasive. There
* Rajshekhar C. Jaka [email protected] 1
BR Life SSNMC Super Specialty Hospital, Rajarajeshwari Nagar, Bengaluru, Karnataka state, India
2
Dr Jaka’s Clinic, Bengaluru, Karnataka state, India
was no sign of capsule rupture to suspect peritoneal sarcomatosis. All visible tumours were meticulously removed from the abdomen (cytoreductive surgery including omentectomy, peritonectomy). The total number of tumours removed was more than 340. Final biopsy reported it as intermediate grade leiomyosarcoma on histological features and immunohistochemistry (positive for SMA, caldesmon and vimentin; negative for CD 117, DOG-1, CD34), with high Ki67 (50%) and mitosis of 22–25/50HPF. Mutation analysis for c-KIT and PDGFR was negative. Postoperative recovery was speedy and uneventful. After discussing in the tumour board meeting, he received 6 cycles of Adriamycin and ifosfamide adjuvant chemotherapy. He is doing well on 6 months of follow-up.
Discussion Leiomyosarcomas account for 11% of all soft tissue sarcomas (STS) [1]. We had never seen so many independent synchronous tumours in a single patient and neither is it reported in the literature, and we were able to successfully remove all the tumours. The possible origin is from the muscle layer of smaller
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