Recurrent Leiomyosarcoma of the Small Bowel: A Case Series

Abstract

Background/Aim: Leiomyosarcoma is an extremely rare, small bowel neoplasm (2% of all gastrointestinal tumours). Early diagnosis is challenging due to the slow growth of the cancer. The biological behaviour of this group of tumours is aggressive, and the first-line treatment is surgical resection. Patients and Methods: This is a report of 4 cases of small bowel leiomyosarcoma that were treated in the last ten years at Hospital San Martino: one involving the jejunum and three involving the ileum (age range=69-86 years). Three patients underwent surgical resection and one was treated with chemotherapy. Results: All patients who were eligible for surgery underwent radical resection with R0 margins. Mean overall survival was 33 months (range=8-84 months). Conclusion: Specific guidelines for small bowel leiomyosarcoma do not currently exist and these rare cases should be discussed in a multidisciplinary context. The first treatment approach is surgery, and in some cases, multivisceral resection may be needed to obtain free margins, even in recurrent cases.

Current landscape of primary small bowel leiomyosarcoma: cases report and a decade of insights

“…Upon postoperative re-examination of the images, a minute presence of free gas within the abdominal cavity was observed (Figure 1D). To the best of our knowledge, the intestinal perforation observed in this particular case represents the fourth reported instance since the World Health Organization's refinement of LMS (7,18,28). In the second case, the patient initially received treatment for lung cancer, and his laboratory tests indicate relatively normal results.…”

“…The final diagnosis of LMS of the duodenum may have been fortuitous. The Given the rarity of small bowel LMS, the absence of specific guidelines necessitates that surgical excision with negative margins (R0) remains the primary treatment objective (7). LMSs infrequently exhibit lymph node metastases, thus advanced lymph node dissection is typically unnecessary during these procedures (10).…”

“…In our case, an MRI was not performed due to the emergency department procedure, however, the CECT in combination with laboratory indicators may have a potential diagnostic value of LMSs. Given the rarity of small bowel LMS, the absence of specific guidelines necessitates that surgical excision with negative margins (R0) remains the primary treatment objective ( 7 ). LMSs infrequently exhibit lymph node metastases, thus advanced lymph node dissection is typically unnecessary during these procedures ( 10 ).…”