Unlike gestational choriocarcinoma, nongestational choriocarcinoma is not related to pregnancy; these tumors can occur somatically as a component of a high-grade malignant tumor or originate from germ cells. Gestational choriocarcinoma is most commonly androgenetic but can also be biparental, whereas germ cell tumors and somatic neoplasms with choriocarcinomatous differentiation are genetically related to the patient. Nongestational choriocarcinomas of somatic origin, which typically affect postmenopausal women and have a poor clinical course, are rare but have been described in various sites, including the female genital tract. Germ cell-derived choriocarcinomas commonly affect children and young adults. These tumors may present as a pure form or as part of mixed germ cell tumors, predominantly found in the ovary. While nongestational choriocarcinoma of both somatic and germ cell origin is well accepted in the classification system, the recognition of nongestational placental site trophoblastic tumor (PSTT) and epithelioid trophoblastic tumor (ETT) has only emerged recently. This category should be recognized in the classification systems of female genital tumors, mirroring the inclusion of their counterparts in the testis. This is important for informing clinical management, as gestational and nongestational tumors, including choriocarcinoma, PSTT, and ETT, exhibit diverse genetic origins and pathogenesis, potentially necessitating distinct therapeutic approaches. This chapter discusses nongestational choriocarcinoma, PSTT, and ETT of both somatic and germ cell origin, with emphasis on origin/pathogenesis/etiology, clinical features, pathological characteristics (including immunohistochemical features), and differential diagnosis.

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Nongestational Trophoblastic Neoplasms Derived from Somatic Tumors or of Germ Cell Origin

  • Deyin Xing,
  • Brigitte M. Ronnett

摘要

Unlike gestational choriocarcinoma, nongestational choriocarcinoma is not related to pregnancy; these tumors can occur somatically as a component of a high-grade malignant tumor or originate from germ cells. Gestational choriocarcinoma is most commonly androgenetic but can also be biparental, whereas germ cell tumors and somatic neoplasms with choriocarcinomatous differentiation are genetically related to the patient. Nongestational choriocarcinomas of somatic origin, which typically affect postmenopausal women and have a poor clinical course, are rare but have been described in various sites, including the female genital tract. Germ cell-derived choriocarcinomas commonly affect children and young adults. These tumors may present as a pure form or as part of mixed germ cell tumors, predominantly found in the ovary. While nongestational choriocarcinoma of both somatic and germ cell origin is well accepted in the classification system, the recognition of nongestational placental site trophoblastic tumor (PSTT) and epithelioid trophoblastic tumor (ETT) has only emerged recently. This category should be recognized in the classification systems of female genital tumors, mirroring the inclusion of their counterparts in the testis. This is important for informing clinical management, as gestational and nongestational tumors, including choriocarcinoma, PSTT, and ETT, exhibit diverse genetic origins and pathogenesis, potentially necessitating distinct therapeutic approaches. This chapter discusses nongestational choriocarcinoma, PSTT, and ETT of both somatic and germ cell origin, with emphasis on origin/pathogenesis/etiology, clinical features, pathological characteristics (including immunohistochemical features), and differential diagnosis.