# Malignant high-grade unclassifiable spindle cell sarcoma in the hand: A diagnostic and therapeutic challenge

**Authors:** Victor Hugo Garzón-Ortega, Kenzo Alejandro Fukumoto-Inukai, Carlos A. Morales-Morales, Yusef Jiménez-Murat

PMC · DOI: 10.1016/j.jpra.2025.08.001 · JPRAS Open · 2025-08-09

## TL;DR

A rare case of high-grade spindle cell sarcoma in the hand is presented, emphasizing the need for accurate diagnosis and careful surgical treatment to preserve function.

## Contribution

This case demonstrates the successful complete resection of a complex spindle cell sarcoma while preserving hand function.

## Key findings

- Histopathological analysis confirmed high-grade spindle cell sarcoma despite initial biopsy suggesting dermatofibroma.
- The patient remained recurrence-free at 10 months with preserved hand function after surgical resection.
- Immunohistochemistry showed positivity for CD99 and CD56, with multiple other markers negative.

## Abstract

Introduction: Spindle cell sarcoma is a rare malignancy characterized by spindle-shaped cells, often mimicking other tumors and posing diagnostic challenges due to its nonspecific clinical features. This case highlights the importance of a multidisciplinary approach and demonstrates the feasibility of complete tumor resection with functional preservation, even in large and complex tumors.

Case Presentation: A 51-year-old woman with no significant medical history presented with a large, exophytic mass on her left hand, causing notable functional impairment in daily activities. Physical examination revealed a rubbery mass, measuring 15 × 8 cm. Imaging studies, including radiography, computed tomography, and magnetic resonance imaging, were performed. Given the impact of grasp functionality on daily life, surgical resection was scheduled. The excised tissue was subsequently submitted for histopathological evaluation.

Results: Imaging demonstrated a vascularized mass without bone involvement. Initial biopsy suggested multicentric cellular dermatofibroma. However, histopathological examination of the excised specimen revealed findings consistent with high-grade spindle cell sarcoma. Immunohistochemistry showed positivity for CD99 and CD56, with negative markers for S-100, factor XIII, TLE-1, CD34, SOX10, H3K27ME, SS18 and actin. At the 10-month follow-up, she remained recurrence-free with preserved hand function.

Conclusions: A meticulous surgical approach is vital for the management of the balance of complete tumor excision and preservation of hand function. A thorough histopathological examination is necessary for diagnosis. Collaboration across disciplines is key to improving treatment outcomes.

## Linked entities

- **Proteins:** CD99 (CD99 molecule (Xg blood group)), NCAM1 (neural cell adhesion molecule 1), S100A1 (S100 calcium binding protein A1), TLE1 (TLE family member 1, transcriptional corepressor), CD34 (CD34 molecule), SOX10 (SRY-box transcription factor 10), SS18 (SS18 subunit of BAF chromatin remodeling complex), ACTIN (hypothetical protein)
- **Diseases:** spindle cell sarcoma (MONDO:0002927)

## Full-text entities

- **Genes:** NCAM1 (neural cell adhesion molecule 1) [NCBI Gene 4684] {aka CD56, MSK39, NCAM}, CD99 (CD99 molecule (Xg blood group)) [NCBI Gene 4267] {aka HBA71, MIC2, MIC2X, MIC2Y, MSK5X}, S100A1 (S100 calcium binding protein A1) [NCBI Gene 6271] {aka S100, S100-alpha, S100A}, SS18 (SS18 subunit of BAF chromatin remodeling complex) [NCBI Gene 6760] {aka SMARCL1, SSXT, SYT}, CD34 (CD34 molecule) [NCBI Gene 947], SOX10 (SRY-box transcription factor 10) [NCBI Gene 6663] {aka DOM, PCWH, SOX-10, WS2E, WS4, WS4C}, F13A1 (coagulation factor XIII A chain) [NCBI Gene 2162] {aka F13A}, TLE1 (TLE family member 1, transcriptional corepressor) [NCBI Gene 7088] {aka ESG, ESG1, GRG1, TLE-1}
- **Diseases:** Spindle cell sarcoma (MESH:D012509), functional impairment (MESH:D003072), malignancy (MESH:D009369), dermatofibroma (MESH:D018219)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12605922/full.md

## References

8 references — full list in the complete paper: https://tomesphere.com/paper/PMC12605922/full.md

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Source: https://tomesphere.com/paper/PMC12605922