Department of Pathology, School of Medicine, Koc University, Istanbul, Turkey.
Department of Gynecology and Obstetrics, School of Medicine, Koc University, Istanbul, Turkey.
Indian J Pathol Microbiol. 2023 Apr-Jun;66(2):392-395. doi: 10.4103/ijpm.ijpm_360_21.
Malignant peritoneal mesothelioma (MPM) is an exceptionally rare tumor type. Although some somatic/germline genetic alterations including BAP1 loss have been identified in some cases, the molecular properties of MPMs are remained poorly understood. In recent years, anaplastic lymphoma kinase (ALK) gene rearrangement was revealed in a subset of (3.4%) MPMs. Low-grade serous carcinomas (LGSCs) are a rare subtype of ovarian carcinoma and have some morphologic and immunophenotypic overlapping features with MPMs and this may cause misdiagnosis in daily practice. Here, we report a case of 18-year-old women with STRN-ALK-rearranged MPM and no previous exposure to asbestos. This case was presented with bilateral pelvic masses and histologically was displaying pure papillary morphology with mild-to-moderate nuclear atypia, psammoma bodies, and diffuse PAX8 expression as LGSCs. With the detection of ALK alteration in some of the MPMs, a targeted treatment option has emerged for these unusual tumor types.
恶性腹膜间皮瘤(MPM)是一种极其罕见的肿瘤类型。虽然在一些病例中已经确定了一些体细胞/种系遗传改变,包括 BAP1 缺失,但 MPM 的分子特性仍知之甚少。近年来,在一小部分(3.4%)MPM 中发现了间变性淋巴瘤激酶(ALK)基因重排。低度浆液性癌(LGSCs)是卵巢癌的一种罕见亚型,与 MPM 具有一些形态学和免疫表型重叠特征,这可能导致日常实践中的误诊。在这里,我们报告了一例 18 岁女性,患有 STRN-ALK 重排的 MPM,且无石棉暴露史。该病例表现为双侧盆腔肿块,组织学上表现为纯乳头状形态,伴有轻至中度核异型性、砂粒体和弥漫性 PAX8 表达,类似于 LGSCs。随着一些 MPM 中 ALK 改变的检测,为这些不常见的肿瘤类型出现了一种靶向治疗选择。