Extracranial and nonvaginal extragonadal malignant germ cell tumors: 12 cases at a Chinese institution over the last 38 years.
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PURPOSE: To provide a comprehensive understanding and propose a strategy for the management of extragonadal malignant germ cell tumors (EMGCTs) arising from extracranial and nonvaginal sites. METHODS: We retrospectively reviewed the cases of 12 patients with EMGCTs arising from extracranial and nonvaginal sites treated in our center over the past 38 years. Data on clinicopathological characteristics, treatment modalities, and follow-up information were analyzed. RESULTS: Among 209 patients diagnosed with EMGCTs, 12 women (5.7%) with EMGCTs of extracranial and nonvaginal sites were identified. These patients had tumors in the sacrococcygeal region (n = 4), abdominal cavity (n = 3), groin region (n = 2), uterus (n = 2), and mediastinum (n = 1). The median age at diagnosis was 23 years. Symptoms included abnormal uterine bleeding (n = 3), abdominal discomfort (n = 3), compression symptoms (n = 3), palpable mass (n = 2), and asymptomatic (n = 1). Yolk sac tumors (YSTs) were the most common histologic type. The median level of serum alpha-fetoprotein (AFP), a sensitive tumor marker, was 8216 ng/ml (2.7-74,157 ng/ml). One patient started bleomycin/etoposide/cisplatin without a pathologic diagnosis based on clinical diagnosis (high AFP levels and imaging findings), and 11 patients started chemotherapy following tumor biopsy or surgical resection. During the follow-up, one patient suffered a recurrence, two patients were alive with disease, and nine patients were disease-free. CONCLUSIONS: Extracranial and nonvaginal EMGCTs are a heterogeneous group of tumors due to their varied onset ages, location, and clinical presentation. An all-around clinical evaluation is crucial for selecting appropriate treatment. Most patients achieve a good prognosis after surgical resection and chemotherapy. Patients with these rare diseases may benefit from individualized treatment and timely referral to experienced medical centers.