Department of Pathology, The Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou, Zhejiang, China.
Department of Pathology, Yuyao People's Hospital of Zhejiang Province, Yuyao, Zhejiang, China.
Histol Histopathol. 2020 Mar;35(3):257-268. doi: 10.14670/HH-18-156. Epub 2019 Sep 3.
Solid pseudopapillary neoplasms (SPN) of the pancreas are a rare and low-grade malignant entity with a female predominance. However, it also occurs in males, but the rarity and lack of concern makes its clinicopathological features unclarified.
The morphological, immunohistochemical, prognostic features and CTNNB1 exon 3 mutation status of SPN were compared semi-quantitively between 9 male and 21 female patients.
SPN in males grew in a distinctive solid pattern, with abundant fibrotic stroma and clear cells. Collagen tended to be the main component of tumor stroma in males, while hyaluronan composed a considerable proportion in females. A much stronger expression of androgen receptor (AR) was found in males, and CD56 and/or synaptophysin (Syn) was expressed frequently in both genders. All patients survived. One male patient had post-operational liver nodules and accepted interventional therapy without biopsy. Mutations of CTNNB1 exon 3 were observed in all cases, distributed at codon 32, 33 and 37 in both genders, as well as 34, 41 and 62 in females.
SPN in males presented with significantly different morphological features from that in females, which might be helpful in differential diagnosis, especially when with extensive positivity for CD56 and/or Syn. The stronger expression of AR in males might be a clue to explore the underlying mechanism of the gender difference.
胰腺实性假乳头状瘤(SPN)是一种罕见的低度恶性肿瘤,以女性多见。但它也发生在男性中,只是由于罕见且关注度低,其临床病理特征尚不明确。
对 9 例男性和 21 例女性患者的 SPN 的形态学、免疫组织化学、预后特征和 CTNNB1 外显子 3 突变状态进行半定量比较。
男性 SPN 呈独特的实性生长模式,富含纤维性基质和透明细胞。男性肿瘤基质中胶原倾向于成为主要成分,而女性中透明质酸占相当大的比例。男性雄激素受体(AR)表达更强,两性均常表达 CD56 和/或突触素(Syn)。所有患者均存活。1 例男性患者术后肝脏出现结节,接受介入治疗但未行活检。所有病例均观察到 CTNNB1 外显子 3 的突变,分布在两性的 32、33 和 37 密码子,以及女性的 34、41 和 62 密码子。
男性 SPN 的形态特征与女性明显不同,有助于鉴别诊断,尤其是 CD56 和/或 Syn 广泛阳性时。男性中 AR 的表达更强可能是探索性别差异潜在机制的线索。