Mesenteric leiomyosarcoma masquerading as generalized Weakness: A rare case report

Karan Sood; Pratiksha Tyagi; Amol Dongre

doi:10.31893/multiscience.2025470

Keywords:

Mesenteric leiomyosarcoma

smooth muscle tumors

spindle cell neoplasms

retroperitoneal sarcoma

chemotherapy

radical surgery

Abstract

Mesenteric leiomyosarcoma (LMS) is a rare malignancy arising from smooth muscle cells, often presenting with nonspecific symptoms, making early diagnosis challenging. While leiomyosarcomas are known to develop in retroperitoneal or pelvic regions, their occurrence in the mesentery is exceptionally uncommon. This case report discusses the presentation, diagnosis, and management of mesenteric root leiomyosarcoma in a 40-year-old female, highlighting the complexities associated with this rare disease and reviewing relevant literature. A 40-year-old female presented with generalized weakness for five years and lower abdominal pain for one year. Physical examination revealed pallor, and blood tests showed anemia. Imaging studies revealed a large lobulated mass in the abdomen, which was surgically resected. Histopathological analysis confirmed the diagnosis of a high-grade spindle cell tumor, specifically mesenteric leiomyosarcoma, with negative CD117 staining and positive smooth muscle actin (SMA) staining. The patient underwent completion radical surgery with resection and anastomosis of the small intestine and was treated with adjuvant chemotherapy (six cycles of Adriamycin). The patient demonstrated excellent clinical response and remained disease-free for 10 years following surgery and chemotherapy. Mesenteric leiomyosarcoma is a rare subtype of soft tissue sarcoma that is often asymptomatic until advanced stages. The diagnosis is often delayed due to its nonspecific presentation, such as abdominal pain, weakness, or anemia, as seen in this case. Histopathologically, LMS is characterized by spindle cells arranged in fascicles, displaying nuclear pleomorphism, high mitotic activity, and areas of necrosis. Immunohistochemistry is crucial for diagnosis, distinguishing LMS from other spindle cell tumors, such as gastrointestinal stromal tumors (GIST), which express CD117 and DOG1. In contrast, LMS stains positive for smooth muscle markers like desmin, caldesmon, and SMA. Genetic mutations, such as loss of tumor suppressors TP53 and RB1, along with dysregulation of the PI3K/AKT/mTOR pathway, play a critical role in the pathogenesis of LMS. Surgical resection remains the mainstay of treatment, with adjuvant chemotherapy showing modest efficacy in preventing recurrence. However, high-grade tumors and large tumor sizes pose significant risks for recurrence and metastasis. Mesenteric leiomyosarcoma is a rare but aggressive neoplasm with a poor prognosis if not treated aggressively. Early recognition, radical surgical resection, and adjuvant chemotherapy are essential for long-term survival. This case emphasizes the importance of a multidisciplinary approach, individualized treatment plans, and regular follow-up for early detection of recurrence. Hormonal therapies and novel agents targeting genetic abnormalities may hold promise for future treatments in select cases.
References
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11. Singer S, Nielsen TO, Demetri GD. "Molecular insights into the pathogenesis and treatment of soft tissue sarcomas." Journal of Clinical Oncology, 2008; 26(20): 3154-3160. DOI: 10.1200/JCO.2007.15.3416.
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14. George, S., et al., Phase 2 trial of aromatase inhibition with letrozole in patients with uterine leiomyosarcomas expressing estrogen and/or progesterone receptors. Cancer, 2014. 120(5): p. 738-43.
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How to cite

Sood, K., Tyagi, P., & Dongre, A. (2024). Mesenteric leiomyosarcoma masquerading as generalized Weakness: A rare case report. Multidisciplinary Science Journal, (| Accepted Articles). https://doi.org/10.31893/multiscience.2025470

[1] Kluger, Y. and O. Ben-Ishay, Leiomyosarcoma of the Mesenteric Root: A Strategic Location of a Rare Tumor. Case Rep Oncol, 2015. 8(2): p. 359-62.

[2] Raut, C.P., and P.W. Pisters, Retroperitoneal sarcomas: Combined-modality treatment approaches. J Surg Oncol, 2006. 94(1): p. 81-7.

[3] Rajendran, S., et al., The diagnosis and treatment of asymptomatic mesenteric cyst. BMJ Case Rep, 2014. 2014.

[4] Ferrari, A., et al., Soft tissue sarcoma across the age spectrum: a population-based study from the Surveillance Epidemiology and End Results database. Pediatr Blood Cancer, 2011. 57(6): p. 943-9.

[5] Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J. "Gastrointestinal stromal tumors and leiomyosarcomas in the mesentery." Human Pathology, 2009; 40(9): 1344-1353. DOI: 10.1016/j.humpath.2009.02.008.

[6] Choi H, Macapinlac HA, Burgess MA, Patel SR. "Pathologic and imaging features of mesenteric leiomyosarcoma." Radiologic Clinics of North America, 2015; 53(5): 1071-1082. DOI: 10.1016/j.rcl.2015.05.006.

[7] Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. WHO Classification of Tumours of Soft Tissue and Bone. 4th edition. Lyon: International Agency for Research on Cancer (IARC), 2013.

[8] Stojadinovic A, Leung DH, Allen P, et al. "Primary visceral leiomyosarcoma: A clinical and molecular analysis of a rare tumor." Cancer, 2002; 94(6): 1469-1478. DOI: 10.1002/cncr.10355.

[9] Helman LJ, Meltzer P. "Molecular and genetic aspects of sarcomas." New England Journal of Medicine, 2006; 355(10): 1058-1070. DOI: 10.1056/NEJMra053241.

[10] Ahn S, Kang YN, Lee KH, et al. "Histopathologic and molecular features of mesenteric leiomyosarcomas." Modern Pathology, 2014; 27(2): 151-159. DOI: 10.1038/modpathol.2013.158.

[11] Singer S, Nielsen TO, Demetri GD. "Molecular insights into the pathogenesis and treatment of soft tissue sarcomas." Journal of Clinical Oncology, 2008; 26(20): 3154-3160. DOI: 10.1200/JCO.2007.15.3416.

[12] Fleet, M., et al., Duodenal leiomyosarcoma presenting with iron deficiency anemia. J Pediatr Surg, 1994. 29(12): p. 1601-3.

[13] McGrath, P.C., et al., Gastrointestinal sarcomas. Analysis of prognostic factors. Ann Surg, 1987. 206(6): p. 706-10.

[14] George, S., et al., Phase 2 trial of aromatase inhibition with letrozole in patients with uterine leiomyosarcomas expressing estrogen and/or progesterone receptors. Cancer, 2014. 120(5): p. 738-43.

[15] O'Cearbhaill, R., et al., Treatment of advanced uterine leiomyosarcoma with aromatase inhibitors. Gynecol Oncol, 2010. 116(3): p. 424-9.

[16] Thanopoulou, E., et al., Treatment of hormone positive uterine leiomyosarcoma with aromatase inhibitors. Clin Sarcoma Res, 2014. 4:p. 5.

[17] Uchida, T., et al., The effectiveness of medroxyprogesterone in the treatment of multiple metastasizing leiomyosarcomas: report of a case. Surg Today, 1996. 26(2): p. 138-41.

[18] Demetri, G.D., et al., Efficacy and safety of trabectedin in patients with advanced or metastatic liposarcoma or leiomyosarcoma after failure of prior anthracyclines and ifosfamide: results of a randomized phase II study of two different schedules. J Clin Oncol, 2009. 27(25): p. 4188-96.

Abstract

References

How to cite