# Xanthogranulomatous adrenalitis: A rare and difficult differential diagnosis of adrenal gland tumors

**Authors:** Wolfgang Saeger, Andreas M. Luebke, S. T. Mekoula, Jörg-Michael Pahnke

PMC · DOI: 10.1007/s00292-024-01312-x · Pathologie (Heidelberg, Germany) · 2024-03-12

## TL;DR

A 29-year-old woman with a fever and an adrenal tumor was diagnosed with xanthogranulomatous adrenalitis, a rare condition that is difficult to distinguish from tumors.

## Contribution

The paper presents a rare case of xanthogranulomatous adrenalitis and discusses its differential diagnosis.

## Key findings

- The tumor was composed of densely packed large histiocyte-like cells positive for Vimentin, CD68, and CD163.
- The diagnosis was confirmed as xanthogranulomatous adrenalitis after excluding other similar conditions.
- The case highlights the diagnostic challenges in distinguishing this condition from adrenal tumors.

## Abstract

Ein radiologisch festgestellter Tumor einer 29-jährigen Frau mit Fieber um 39 °C wurde unter dem Verdacht einer Cholezystitis oder eines Leberabszesses operiert, dabei ein solider Tumor in der Nebennierenloge gefunden und reseziert. Die Schnellschnittbefundung ergab keine klare Diagnose bezüglich der Dignität und der Zuordnung. Histologisch zeigte sich, dass der Tumor aus dicht gelagerten großen histiozytenartigen Zellen mit Expression von Vimentin, CD68 und CD163 sowie Negativität für Keratin, Langerin und SMA aufgebaut ist. Wir diagnostizierten eine xanthogranulomatöse Adrenalitis und diskutierten die Differentialdiagnosen (Langerhans-Zellhistiozytose, Rosai-Dorfman-Krankheit, Malakoplakie, Erdheim-Chester-Krankheit).

## Linked entities

- **Proteins:** PRELID1 (PRELI domain containing 1), CD68 (CD68 molecule), CD163 (CD163 molecule), keratin (keratin, type I cytoskeletal 19), SMN1 (survival of motor neuron 1, telomeric)

## Full-text entities

- **Genes:** CD207 (CD207 molecule) [NCBI Gene 50489] {aka CLEC4K}, CD68 (CD68 molecule) [NCBI Gene 968] {aka GP110, LAMP4, SCARD1}, CD163 (CD163 molecule) [NCBI Gene 9332] {aka M130, MM130, SCARI1}, VIM (vimentin) [NCBI Gene 7431], SMN1 (survival of motor neuron 1, telomeric) [NCBI Gene 6606] {aka BCD541, GEMIN1, SMA, SMA1, SMA2, SMA3}
- **Diseases:** liver abscess (MESH:D008100), cholecystitis (MESH:D002764), Langerhans cell histiocytosis (MESH:D006646), tumor (MESH:D009369), Erdheim-Chester disease (MESH:D031249), malakoplakia (MESH:D008287), Rosai-Dorfman disease (MESH:D015618), Xanthogranulomatous adrenalitis (MESH:C536763), adrenal gland tumors (MESH:D000310), fever (MESH:D005334)
- **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/PMC11045564/full.md

## References

8 references — full list in the complete paper: https://tomesphere.com/paper/PMC11045564/full.md

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Source: https://tomesphere.com/paper/PMC11045564