关键词: Ewing’s sarcoma Pathological examination Prostate Small round cell tumor Whole-genome sequencing

Mesh : Male Humans Aged Prostate Dysuria / surgery In Situ Hybridization, Fluorescence Sarcoma / surgery Prostatic Hyperplasia / surgery Laser Therapy DNA Helicases Nuclear Proteins Transcription Factors

来  源:   DOI:10.1007/s00432-023-05585-2   PDF(Pubmed)

Abstract:
BACKGROUND: 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.
METHODS: 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).
CONCLUSIONS: 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.
摘要:
背景:小圆细胞肿瘤(SRCT)是一组具有相似光学显微形态的恶性肿瘤。尽管发病率低,SRCT恶性程度高,预后差。此外,不典型的临床症状使术前诊断困难。
方法:一名67岁男子出现排尿困难,尿流微弱,持续3个月。口服坦索罗辛和非那雄胺治疗2个月后,症状恶化。经尿道前列腺钬激光剥除术,术后病理提示小蓝圆细胞恶性肿瘤。进一步免疫组织化学和荧光原位杂交检查提示尤文样SRCT。因此,进一步进行了达芬奇机器人前列腺切除术,并进行了全基因组测序。几种基因突变包括RAF1,ARID1A,发现了SMARCA4和BCL2L11,但没有FDA批准的药物可以特异性治疗。然后,患者接受了尤因类型的治疗方案治疗,并进行了最新随访(超过24个月)。
结论:由于其血清PSA水平未升高,前列腺SRCT作为恶性肿瘤的可能性常被忽略,被视为良性前列腺增生(BPH)。如果口服治疗一段时间后排尿困难症状不能明显缓解,则需要考虑前列腺SRCT的可能性。
公众号