# Primary retroperitoneal müllerian adenocarcinoma: a case report

**Authors:** Xiaohui Feng, Ping Zhang, Feng Gao

PMC · DOI: 10.3389/fonc.2024.1364008 · Frontiers in Oncology · 2024-06-27

## TL;DR

A rare case of primary retroperitoneal müllerian adenocarcinoma is reported in a 45-year-old woman with metastases and confirmed through biopsy and immunohistochemistry.

## Contribution

This case report adds to the limited literature on primary retroperitoneal müllerian adenocarcinoma, highlighting its diagnostic features.

## Key findings

- The tumor was confirmed as primary retroperitoneal müllerian adenocarcinoma through biopsy and immunohistochemical analysis.
- Immunohistochemistry showed positive keratin7 and PAX8, with negative markers for other common tumors.
- The case highlights the importance of considering PRMA in retroperitoneal mass diagnoses.

## Abstract

A 45-year-old woman presented with right hip pain for a month. Imaging results revealed that the left peritoneal mass was accompanied by metastases of the right sciatic branch, lung, and retroperitoneal lymph nodes. A biopsy of the left peritoneal mass was performed. The pathological morphology demonstrated clear cell adenocarcinoma. Immunohistochemical staining revealed a positive expression of keratin7 and PAX8 and a negative expression of keratin20, GCDFP-15, ER, PR, WT1, CDX2, villin, TTF-1, napsin-A, vimentin, calretinin, and GATA3. Finally, the diagnosis of primary retroperitoneal müllerian adenocarcinoma (PRMA) was confirmed. PRMA is a very rare type of primary retroperitoneal tumor. PRMA should be considered for the retroperitoneal mass.

## Linked entities

- **Proteins:** PAX8 (paired box 8), KRT20 (keratin 20), PIP (prolactin induced protein), EREG (epiregulin), PGR (progesterone receptor), WT1 (WT1 transcription factor), CDX2 (caudal type homeobox 2), adv (advillin), TTF1 (transcription termination factor 1), Napsa (napsin A aspartic peptidase), PRELID1 (PRELI domain containing 1), CALB2 (calbindin 2), GATA3 (GATA binding protein 3)

## Full-text entities

- **Genes:** CDX2 (caudal type homeobox 2) [NCBI Gene 1045] {aka CDX-3, CDX2/AS, CDX3}, PIP (prolactin induced protein) [NCBI Gene 5304] {aka BRST-2, GCDFP-15, GCDFP15, GPIP4}, EREG (epiregulin) [NCBI Gene 2069] {aka EPR, ER, Ep}, PGR (progesterone receptor) [NCBI Gene 5241] {aka NR3C3, PR}, GATA3 (GATA binding protein 3) [NCBI Gene 2625] {aka HDR, HDRS}, VIM (vimentin) [NCBI Gene 7431], KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, WT1 (WT1 transcription factor) [NCBI Gene 7490] {aka AWT1, GUD, NPHS4, WAGR, WIT-2, WT-1}, TTF1 (transcription termination factor 1) [NCBI Gene 7270] {aka TTF-1, TTF-I}, NAPSA (napsin A aspartic peptidase) [NCBI Gene 9476] {aka KAP, Kdap, NAP1, NAPA, NR1H2-AS1, SNAPA}, PAX8 (paired box 8) [NCBI Gene 7849] {aka PAX-8}, CALB2 (calbindin 2) [NCBI Gene 794] {aka CAB29, CAL2, CR}, KRT7 (keratin 7) [NCBI Gene 3855] {aka CK7, K2C7, K7, SCL}
- **Diseases:** clear cell adenocarcinoma (MESH:D018262), peritoneal mass (MESH:D010532), PRMA (MESH:D000230), hip pain (MESH:D010146), metastases (MESH:D009362), retroperitoneal mass (MESH:C536030), retroperitoneal tumor (MESH:D012186)
- **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/PMC11236549/full.md

## References

9 references — full list in the complete paper: https://tomesphere.com/paper/PMC11236549/full.md

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Source: https://tomesphere.com/paper/PMC11236549