James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
Am J Surg Pathol. 2021 Jun 1;45(6):727-741. doi: 10.1097/PAS.0000000000001625.
One hundred malignant mixed germ cell tumors of the ovary that occurred in patients 3 to 55 years (mean: 20 y) of age are described. The clinical presentation was usually that of any highly malignant tumor of the ovary (abdominal pain and distension), but rarely (3 cases) endocrine manifestations were present. The tumors were usually unilateral (96%), ranged from 4 to 38 cm (mean: 16 cm), and were uniformly solid or, more often, solid and cystic; occasionally the typical appearance of dysgerminoma could be appreciated. The most common tumor type was yolk sac tumor (91%), followed by dysgerminoma (61%), immature teratoma (58%), embryonal carcinoma (38%), and choriocarcinoma (11%). A variety of admixtures were encountered; dysgerminoma and yolk sac tumor was the most common combination (25% of the tumors) with the 2 components often being sharply demarcated. Immature teratoma and yolk sac tumor was the next most common pairing (20%) followed by yolk sac tumor and embryonal carcinoma, with or without immature teratoma (16%). Tumors with a choriocarcinoma component had the most varied combinations of tumor types. Embryoid bodies were seen in 21% of the tumors, most often as fragmented forms arranged in a nodular manner with yolk sac tumor and/or embryonal carcinoma; uncommonly they occurred singly or in clusters. Numerous confluent well-formed embryoid bodies (polyembryoma) were prominent in 2 tumors. Three tumors had a focal diffuse embryoma pattern. The specific tumor types showed the known diverse spectrum of microscopic appearances, but the frequent haphazard arrangement of 2 or more subtypes often resulted in complex morphology. Overgrowth of another neoplastic component, most often primitive neuroectodermal tumor, occurred in 10% of the tumors further complicating the histologic picture. This is the largest series of ovarian malignant mixed germ cell tumors reported and details their characteristics including associations of their subtypes and the frequent apparent role of embryoid bodies in giving rise to yolk sac tumor and embryonal carcinoma components.
描述了 100 例发生于 3 至 55 岁(平均 20 岁)患者的卵巢恶性混合生殖细胞肿瘤。临床表现通常为卵巢任何高度恶性肿瘤(腹痛和腹胀),但罕见(3 例)存在内分泌表现。肿瘤通常为单侧(96%),大小为 4 至 38cm(平均 16cm),均为实性或更常见的实性和囊性;偶尔可以观察到典型的卵黄囊瘤外观。最常见的肿瘤类型是卵黄囊瘤(91%),其次是未成熟畸胎瘤(58%)、胚胎癌(38%)和绒毛膜癌(11%)。还遇到了各种混合类型;未成熟畸胎瘤和卵黄囊瘤是最常见的组合(25%的肿瘤),这两种成分通常界限分明。其次是未成熟畸胎瘤和卵黄囊瘤的组合(20%),其次是卵黄囊瘤和胚胎癌,伴有或不伴有未成熟畸胎瘤(16%)。具有绒毛膜癌成分的肿瘤具有最多样的肿瘤类型组合。胚状体见于 21%的肿瘤中,最常见的形式是与卵黄囊瘤和/或胚胎癌一起呈结节状排列的碎片形式;罕见的是它们单独存在或成簇存在。在 2 个肿瘤中可见大量融合形成的胚状体(多胚瘤)。3 个肿瘤具有局灶性弥漫性胚胎瘤模式。特定的肿瘤类型显示出已知的广泛微观表现,但 2 种或更多亚型的偶然杂乱排列通常导致复杂的形态。另一种肿瘤成分(最常见的原始神经外胚层肿瘤)过度生长,发生于 10%的肿瘤中,进一步使组织学图像复杂化。这是报道的最大系列卵巢恶性混合生殖细胞肿瘤,详细描述了它们的特征,包括它们的亚型的关联以及胚状体在产生卵黄囊瘤和胚胎癌成分中的常见作用。