# Unmasking the uncommon: retroperitoneal Leiomyosarcoma case report

**Authors:** Boujguenna Imane, Mohammed Essaid Ramraoui, Fatima Boukis, Faisal ElMouhafid

PMC · DOI: 10.1093/omcr/omaf002 · Oxford Medical Case Reports · 2025-03-28

## TL;DR

This paper presents a rare case of retroperitoneal leiomyosarcoma in a 63-year-old woman, highlighting the challenges in diagnosis and the importance of surgery and follow-up.

## Contribution

The novelty lies in the detailed case report of a rare retroperitoneal leiomyosarcoma with specific immunohistochemical findings and treatment outcome.

## Key findings

- The tumor was confirmed as leiomyosarcoma through histopathology and immunohistochemistry.
- Complete surgical resection with negative margins was achieved, and the patient remains recurrence-free six months post-surgery.
- The case emphasizes the difficulty in diagnosing retroperitoneal leiomyosarcoma before histopathology.

## Abstract

Leiomyosarcoma is a rare subtype of soft tissue sarcoma originating from smooth muscle cells. The clinical presentation varies based on the tumor’s location. We report the case of a 63-year-old woman with no significant medical history, who presented with persistent epigastric pain. A suspected lymphadenopathy was found on a CT scan. Following surgical excision, histopathology and immunohistochemistry confirmed the diagnosis of leiomyosarcoma, negative for CD117 and Dog1, but positive for H-Caldesmon. Retroperitoneal leiomyosarcoma is aggressive and rare, making diagnosis difficult prior to histopathology. Complete surgical resection with negative margins is the gold standard of treatment, though it can be challenging to achieve. A multidisciplinary approach is crucial to improve survival and quality of life. The patient is under regular follow-up and remains free of recurrence six months post-operatively.

## Linked entities

- **Proteins:** KIT (KIT proto-oncogene, receptor tyrosine kinase), ANO1 (anoctamin 1)
- **Diseases:** leiomyosarcoma (MONDO:0005058), soft tissue sarcoma (MONDO:0018078)

## Full-text entities

- **Genes:** ANO1 (anoctamin 1) [NCBI Gene 55107] {aka DOG1, INDMS, MYMY7, ORAOV2, TAOS2, TMEM16A}, KIT (KIT proto-oncogene, receptor tyrosine kinase) [NCBI Gene 3815] {aka C-Kit, CD117, MASTC, PBT, SCFR}
- **Diseases:** tumor (MESH:D009369), Leiomyosarcoma (MESH:D007890), epigastric pain (MESH:D010146), lymphadenopathy (MESH:D008206), soft tissue sarcoma (MESH:D012509)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

6 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11952906/full.md

## References

10 references — full list in the complete paper: https://tomesphere.com/paper/PMC11952906/full.md

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Source: https://tomesphere.com/paper/PMC11952906