# Rare primary pleomorphic rhabdomyosarcoma of the thyroid gland with lung and adrenal metastases: a case report and literature review

**Authors:** Rami Altabbouche, Jacek Musiał, Anna Jałocha-Kaczka, Łukasz Dziki, Kasper Maryńczak, Krzysztof Kaczka

PMC · DOI: 10.3389/fonc.2025.1756395 · Frontiers in Oncology · 2026-01-20

## TL;DR

This case report describes a rare thyroid cancer that spread to the lungs and adrenal gland, treated with a personalized combination of surgery, chemotherapy, and radiotherapy.

## Contribution

The first reported case of primary thyroid pleomorphic rhabdomyosarcoma with distal metastases and partial response to treatment.

## Key findings

- The patient showed partial regression of metastatic lesions after four cycles of chemotherapy.
- Histopathology confirmed the aggressive nature of the tumor with strong positivity for skeletal muscle markers.
- Metastases were identified in the lungs and left adrenal gland via imaging.

## Abstract

Primary thyroid pleomorphic rhabdomyosarcoma (PRMS) is an extremely rare and aggressive neoplasm, with only five cases reported in the literature to date. We present the sixth reported case of primary thyroid PRMS and the first case demonstrating distal metastases to the lungs and left adrenal gland in a 71-year-old female. The patient underwent total thyroidectomy with lymphadenectomy, with histopathological evaluation confirming PRMS and demonstrating a highly aggressive biological behavior. The immunophenotypic profile showed strong immunopositivity for skeletal muscle markers, including desmin and vimentin. Further computerized tomography imaging identified a metastasized lesion in the left adrenal gland and a diffuse lung nodular dissemination. The patient received a personalized treatment plan which combined both systemic chemotherapy and radiotherapy. After four cycles of chemotherapy, the patient achieved partial regression of metastatic lesions. The highly aggressive nature of PRMS emphasizes the importance of having individualized and multidisciplinary treatment approaches to such rare cases.

## Linked entities

- **Proteins:** LOC101066771 (desmin-like), PRELID1 (PRELI domain containing 1)
- **Diseases:** pleomorphic rhabdomyosarcoma (MONDO:0004233), thyroid cancer (MONDO:0002108)

## Full-text entities

- **Genes:** DES (desmin) [NCBI Gene 1674] {aka CDCD3, CSM1, CSM2, LGMD1D, LGMD1E, LGMD2R}, VIM (vimentin) [NCBI Gene 7431]
- **Diseases:** PRMS (MESH:D012208), neoplasm (MESH:D009369), metastases (MESH:D009362), thyroid gland (MESH:D013966)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12865711/full.md

## References

18 references — full list in the complete paper: https://tomesphere.com/paper/PMC12865711/full.md

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Source: https://tomesphere.com/paper/PMC12865711