# Ectopic Adrenocorticotropic Hormone Syndrome Secondary to Undifferentiated Pleomorphic Sarcoma of the Thigh: A Rare Paraneoplastic Manifestation

**Authors:** Gizem Guney, Ibrahim Yildiz

PMC · DOI: 10.7759/cureus.94397 · Cureus · 2025-10-12

## TL;DR

This paper reports the first case of a rare condition where a thigh tumor caused a hormone disorder typically linked to other types of cancer.

## Contribution

The first reported case of ectopic ACTH syndrome associated with undifferentiated pleomorphic sarcoma in adults.

## Key findings

- A 55-year-old woman was diagnosed with undifferentiated pleomorphic sarcoma of the thigh.
- The tumor was linked to ectopic ACTH syndrome, a rare paraneoplastic manifestation.
- This case expands the known associations of paraneoplastic syndromes with sarcomas.

## Abstract

Undifferentiated pleomorphic sarcoma (UPS) is a rare, high-grade soft tissue sarcoma. Ectopic adrenocorticotropic hormone (ACTH) syndrome is an uncommon paraneoplastic phenomenon and is most frequently associated with neuroendocrine tumors. Herein, we present a unique case of a 55-year-old woman diagnosed with UPS of the left thigh, complicated by paraneoplastic ectopic ACTH syndrome. To our knowledge, this is the first reported case of ectopic ACTH syndrome associated with UPS in the adult population.

## Linked entities

- **Proteins:** POMC (proopiomelanocortin)
- **Diseases:** undifferentiated pleomorphic sarcoma (MONDO:0002142), ectopic adrenocorticotropic hormone syndrome (MONDO:0018912)

## Full-text entities

- **Diseases:** UPS (MESH:D002277), soft tissue sarcoma (MESH:D012509), neuroendocrine tumors (MESH:D018358), Adrenocorticotropic Hormone Syndrome (MESH:C535668), Ectopic (MESH:C566852), Ectopic adrenocorticotropic hormone (ACTH) syndrome (MESH:D009384)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

1 figure with captions in the complete paper: https://tomesphere.com/paper/PMC12604604/full.md

## References

9 references — full list in the complete paper: https://tomesphere.com/paper/PMC12604604/full.md

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Source: https://tomesphere.com/paper/PMC12604604