Department of Cytology and Gynecological Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Obstetrics and Gynecology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Ann Diagn Pathol. 2021 Jun;52:151721. doi: 10.1016/j.anndiagpath.2021.151721. Epub 2021 Feb 10.
Juvenile granulosa cell tumor(JGCT) is an uncommon ovarian sex-cord stromal tumor, with diverse clinical, radiological and histopathologic features. The present study describes the clinicopathological and histomorphological spectrum of JGCTs, and highlights the key differentiating features from its mimics.
A retrospective analysis of all cases reported as JGCTs during 2011-19 (8 years) was performed with detailed evaluation of clinical, histopathologic data and follow-up details.
Of a total 115 GCTs reported during the study period, 15(13%) were reported as JGCTs. The mean age at presentation was 17 years. Abdominal pain and distension were the most common clinical presentations. Five patients were pre-menarchal with 3 exhibiting precocious puberty. Majority of tumors were unilateral(left>right), solid-cystic, ranging in size from 4 to 20 cm. Microscopically, macrofollicular architecture was most frequent (n = 12;80%). The tumor cells depicted variable nuclear pleomorphism, small distinct nucleoli and moderate-abundant pale eosinophilic-clear/vacuolated cytoplasm. Mitotic activity ranged from 1 to 10/10HPFs. Uncommon histopathologic features included microcystic and tubulo-cystic architecture, myxoid degeneration, bizarre tumor giant cells, hob-nailing of the tumor cells, intracytoplasmic hyaline globules, multifocal calcification and thick hyalinized blood vessels. Majority(n = 12;80%) presented in stage I. Surgical treatment included unilateral salpingo-oophorectomy without any adjuvant chemotherapy, bilateral salpingo-oophorectomy (BSO) and total abdominal hysterectomy with BSO with adjuvant BEP chemotherapy (Bleomycin, etoposide, cisplatin).
JGCT is a rare ovarian tumor affecting young women and children with diverse histopathologic features. Despite an aggressive histopathology, these tumors have a good outcome, when diagnosed at an early stage.
幼年型颗粒细胞瘤（JGCT）是一种罕见的卵巢性索间质肿瘤，具有不同的临床、放射影像学和组织病理学特征。本研究描述了 JGCT 的临床病理和组织形态学谱，并强调了与模仿者区分的关键特征。
对 2011-19 年（8 年）期间报告的所有 JGCT 病例进行回顾性分析，详细评估临床、组织病理学数据和随访细节。
在所报告的 115 例 GCT 中，有 15 例（13%）报告为 JGCT。患者的平均发病年龄为 17 岁。腹痛和腹胀是最常见的临床表现。有 5 例患者为月经初潮前，其中 3 例表现为性早熟。大多数肿瘤为单侧（左侧>右侧），实性-囊性，大小从 4 到 20cm 不等。镜下，最常见的是巨滤泡状结构（n=12；80%）。肿瘤细胞表现出不同程度的核多形性、小而明显的核仁以及中等量的淡嗜酸性透明/空泡状细胞质。有丝分裂活性范围为 1 至 10/10HPFs。罕见的组织病理学特征包括微囊性和管状囊性结构、黏液样变性、奇异的肿瘤巨细胞、肿瘤细胞的钉状突起、细胞内透明小体、多灶性钙化和厚玻璃样血管。大多数患者（n=12；80%）处于 I 期。治疗包括单侧附件切除术，不进行任何辅助化疗，双侧附件切除术（BSO）和全子宫切除术加 BSO 并辅以 BEP 化疗（博来霉素、依托泊苷、顺铂）。
JGCT 是一种罕见的卵巢肿瘤，影响年轻女性和儿童，具有不同的组织病理学特征。尽管组织病理学表现具有侵袭性，但这些肿瘤在早期诊断时预后良好。
