Central Nervous System Germ Cell Tumors: Classification, Clinical Features, and Treatment With a Historical Overview

Abstract

Central nervous system germ cell tumors are neoplasms that affect children and young adults. They are subclassified into germinoma and nongerminomatous germ cell tumors. The latter include teratoma (mature teratoma, immature teratoma, teratoma with malignant transformation), choriocarcinoma, embryonal carcinoma, yolk sac tumors, and mixtures of these entities. Germinoma with syncytiotrophoblastic giant cells is a variant of germinoma. Germinomas respond well to radiation therapy, but late sequelae due to irradiation have been reported. The results of radiation treatment alone for nongerminomatous germ cell tumor are not satisfactory. Combination radiochemotherapy has been applied, and this yields a good outcome with less toxicity for germinomas and better survival of nongerminomatous germ cell tumors. This article also discusses other issues, including the controversy regarding spinal irradiation and the treatment of recurrent disease.

Clinical Reasoning: Multiple cranial neuropathies in a young man

“…6,7 Typically, CNS GCT present in the second decade of life. 7,8 GCT are broadly classified as germinomatous (composed solely of germinoma cells) or non-germinomatous (NGGCT). This distinction is clinically relevant as germinomas, like gonadal GCT, are highly curable with 5-year survival rates greater than 90%.…”

“…This distinction is clinically relevant as germinomas, like gonadal GCT, are highly curable with 5-year survival rates greater than 90%. [7][8][9] In contrast, NGGCT portend a worse prognosis, with survival of 30%-40% with radiation alone. The addition of platinum-based chemotherapy regimens and surgical resection has boosted survival rates in NGGCT to 90% at a mean of 96 months.…”

“…9 Thought to originate from primitive embryonal cells, GCT are divided histologically by their resemblance to primordial germ cells (germinoma), embryonic differentiated cells (teratoma), yolk sac endodermal cells (yolk sac tumor), blastocytes (choriocariocarcinoma), or embryonal pluripotent stem cells (embryonal carcinoma). 7,8 Mixed GCT are composed of various histologic types, with the most malignant component determining overall prognosis. 9 GCT are classically found in the pineal gland but can present anywhere within the CNS.…”

Diagnostic imaging of intracranial germ cell tumors. Review.

“…germinoma, teratoma (mature teratoma, immature teratoma, teratoma with malignant transformation), embryonal carcinoma, yolk sac tumor and choriocarcinoma [3,4], and some are composed of a mixture of these subtypes. Germinomas are the most frequent histological subtype, followed in order by teratomas, choriocarcinomas, embryonal carcinomas and yolk sac tumors.…”

Can stereotactic sample biopsies accurately diagnose mixed germ cell tumors?

“…Matsutani et al [3] reported that 32% of intracranial germ cell tumors had coexistence of more than two germ cell tumor components. Depending on the kinds of components present, the treatment strategies can vary, and the outcomes can differ significantly [1,3]. If mixed germ cell tumors were not diagnosed, we consider that it is too early to reach conclusions on the accuracy of pathological diagnosis with stereotactic biopsies.…”