Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY, 14642, USA.
Diagn Pathol. 2021 Apr 23;16(1):35. doi: 10.1186/s13000-021-01096-1.
Small cell neuroendocrine carcinoma of the prostate (SCNECP) is a rare, aggressive subtype of prostate carcinoma. Most SCNECP arise from conventional prostate adenocarcinoma (CPAC) treated with androgen deprivation therapy (ADT).
We identified four cases of CPAC treated with ADT, which evolved to SCNECP with liver metastasis. The average interval between the diagnosis of CPAC and SCNECP was 102 months (range: 12 to 168). Histologically, the tumors showed nests of cells with high nuclear:cytoplasmic ratios, granular chromatin, and frequent mitoses. All cases were synaptophysin, chromogranin, and AE1/AE3 positive, with a Ki-67 labeling index ≥70%. NKX3.1 was negative in all but one case and TTF-1 was positive in half. Weak ERG positivity by IHC was seen in one case which also demonstrated the TMPRSS2-ERG gene rearrangement; all other cases were negative for ERG by IHC. Serum prostate specific antigen (PSA) levels were normal to near-normal in all. The median interval between the diagnosis of SCNECP and death was 3.25 months (range: 0.75 to 26).
Our case series highlights the importance of considering a prostate primary, even in the setting of normal PSA levels and loss of prostate markers, when diagnosing neuroendocrine carcinoma in the liver. Further, we emphasize the significance of diagnosing SCNECP that metastasizes to the liver, as it portends a particularly dismal prognosis.
前列腺小细胞神经内分泌癌(SCNECP)是一种罕见的、侵袭性强的前列腺癌亚型。大多数 SCNECP 源自接受雄激素剥夺治疗(ADT)的常规前列腺腺癌(CPAC)。
我们发现了 4 例接受 ADT 治疗的 CPAC 病例,这些病例发展为伴有肝转移的 SCNECP。CPAC 诊断与 SCNECP 诊断之间的平均间隔时间为 102 个月(范围:12 至 168 个月)。组织学上,肿瘤显示细胞巢,细胞核与细胞质比值高、颗粒状染色质和频繁有丝分裂。所有病例均突触素、嗜铬粒蛋白和 AE1/AE3 阳性,Ki-67 标记指数≥70%。除 1 例外,所有病例均为 NKX3.1 阴性,TTF-1 阳性占一半。1 例病例免疫组化显示 ERG 弱阳性,同时显示 TMPRSS2-ERG 基因重排;其余病例免疫组化 ERG 均为阴性。所有病例的血清前列腺特异性抗原(PSA)水平均正常或接近正常。SCNECP 诊断与死亡之间的中位间隔时间为 3.25 个月(范围:0.75 至 26 个月)。
我们的病例系列强调了在诊断肝脏神经内分泌癌时,即使 PSA 水平正常且前列腺标志物丢失,也要考虑前列腺原发灶的重要性。此外,我们强调诊断转移性至肝脏的 SCNECP 的重要性,因为它预示着预后特别差。