Malignant ovarian sex cord tumor with annular tubules in a patient with Peutz-Jeghers syndrome: A case report
Malignant ovarian sex cord tumor with annular tubules in a patient with Peutz-Jeghers syndrome: A case report
复制标题
DOI：
10.1038/modpathol.3880079
复制
发表时间：
2000-04-01
期刊：
影响因子：
7.5
通讯作者：
Krigman, HR
中科院分区：
文献类型：
--
作者：
Lele, SM;Sawh, RN;Krigman, HR
大多数卵巢环状小管性索肿瘤（SCTAT）是散发性的良性肿瘤。在患有Peutz-Jeghers综合征（PJS）的患者中，卵巢SCTAT通常是偶然发现的。迄今为止，SCTAT的恶性行为仅在零星病例中报道。我们报告了一例双侧恶性SCTAT病例，患者为一名患有PJS的47岁女性，最初在宫颈阴道细胞学检查中被诊断为腺癌。宫颈阴道液和腹腔液细胞学标本的特征是肿瘤细胞的假乳头状簇和三维管状，细胞质稀少，核质比高。手术切除标本的检查显示双侧卵巢实体瘤，由简单和复杂的环形小管组成，透明核心，典型的SCTAT。肿瘤栓子存在于输卵管淋巴血管间隙和左右盆腔淋巴结中。流式细胞术显示肿瘤细胞呈二倍体表型。该病例是第一例双侧恶性SCTAT发生于PJS患者，表现为非典型宫颈阴道涂片。
The majority of ovarian sex cord tumors with annular tubules (SCTAT) are benign neoplasms that arise sporadically. in patients who have Peutz-Jeghers syndrome (PJS), ovarian SCTAT is often an incidental finding, Malignant behavior in SCTAT has heretofore been reported only in sporadic cases. We report a case of bilateral, malignant SCTAT developing in a 47-year-old woman who had PJS, originally diagnosed as adenocarcinoma on cervicovaginal cytology. Cervicovaginal and peritoneal fluid cytologic preparations were characterized by pseudopapillary clusters and three-dimensional tubes of tumor cells with scanty cytoplasm and high nuclear: cytoplasmic ratio. Examination of surgical resection specimens revealed bilateral, solid ovarian tumors composed of simple and complex annular tubules with hyaline cores, typical of SCTAT. Tumor emboli were present within salpingeal lymphovascular spaces and in both right and left pelvic lymph nodes. Flow cytometry of tumor cells demonstrated a diploid phenotype. This case represents the first documented example of bilateral, malignant SCTAT arising in a patient who had PJS, presenting with an atypical cervicovaginal smear.