# A rare Ewing-like small round cell tumor in prostate: a case report and literature review

**Authors:** Zhen Wang, Jian Ye, Junjie Hu, Nan Zhang, Yichu Yuan

PMC · DOI: 10.1007/s00432-023-05585-2 · Journal of Cancer Research and Clinical Oncology · 2024-03-01

## TL;DR

A rare Ewing-like tumor in the prostate was diagnosed in a 67-year-old man after initial misdiagnosis, highlighting the need for awareness of this aggressive cancer.

## Contribution

This case report highlights the diagnostic challenges and management of a rare Ewing-like small round cell tumor in the prostate.

## Key findings

- The patient was diagnosed with Ewing-like SRCT after transurethral surgery and genomic analysis.
- No FDA-approved drug specifically targets the identified gene mutations (RAF1, ARID1A, SMARCA4, BCL2L11).
- The patient was treated with Ewing-type regimens and has survived over 24 months.

## Abstract

Small round cell tumor (SRCT) is a group of malignancy with similar optical microscopic morphology. Despite its low incidence, SRCT has a high malignant degree and poor prognosis. Besides, atypical clinical symptoms make it difficult in preoperative diagnosis.

A 67-year-old man was presented to the outpatient service with dysuria and weak urine stream lasting for 3 months. After oral treatment with tamsulosin and finasteride for 2 months, the symptoms worsen. Transurethral prostate holmium laser enucleation was operated and postoperative pathology result revealed small blue round cell malignant tumor. Further immunohistochemistry and fluorescence in situ hybridization examination indicated Ewing-like SRCT. So a Da Vinci Robotic prostatectomy was performed further and whole-genome sequencing was conducted. Several gene mutations including RAF1, ARID1A, SMARCA4, and BCL2L11 were found but no FDA-approved drug could treat specifically. Then the patient received Ewing-type therapeutic regimens treatment and has been followed up to date (over 24 months).

Because of its non-elevated serum PSA level, prostate SRCT is often ignored as a possibility of malignant tumor and regarded as benign prostatic hyperplasia (BPH). The possibility of prostate SRCT need to be considered if dysuria symptoms could not alleviate significantly after a period of oral treatment.

## Linked entities

- **Genes:** RAF1 (Raf-1 proto-oncogene, serine/threonine kinase) [NCBI Gene 5894], ARID1A (AT-rich interaction domain 1A) [NCBI Gene 8289], SMARCA4 (SWI/SNF related BAF chromatin remodeling complex subunit ATPase 4) [NCBI Gene 6597], BCL2L11 (BCL2 like 11) [NCBI Gene 10018]
- **Diseases:** prostate cancer (MONDO:0005159), benign prostatic hyperplasia (MONDO:0010811)

## Full-text entities

- **Genes:** SMARCA4 (SWI/SNF related BAF chromatin remodeling complex subunit ATPase 4) [NCBI Gene 6597] {aka BAF190, BAF190A, BRG1, CSS4, MRD16, OTSC12}, ARID1A (AT-rich interaction domain 1A) [NCBI Gene 8289] {aka B120, BAF250, BAF250a, BM029, C1orf4, CSS2}, RAF1 (Raf-1 proto-oncogene, serine/threonine kinase) [NCBI Gene 5894] {aka CMD1NN, CRAF, NS5, Raf-1, c-Raf}, BCL2L11 (BCL2 like 11) [NCBI Gene 10018] {aka BAM, BIM, BOD}, PLAG1 (PLAG1 zinc finger) [NCBI Gene 5324] {aka PSA, SGPA, SRS4, ZNF912}
- **Diseases:** BPH (MESH:D011470), dysuria (MESH:D053159), malignancy (MESH:D009369), Ewing-like SRCT (MESH:D058405)
- **Chemicals:** finasteride (MESH:D018120), tamsulosin (MESH:D000077409), holmium (MESH:D006695)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

24 references — full list in the complete paper: https://tomesphere.com/paper/PMC10907407/full.md

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Source: https://tomesphere.com/paper/PMC10907407