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457 Extracranial and nonvaginal extragonadal malignant germ cell tumors: a review of 12 cases at a Chinese institution over the last 38 years
  1. Xiao Ma,
  2. Jingwen Gan,
  3. Peng Peng and
  4. Dongyan Cao
  1. Peking Union Medical College Hospital, Beijing, China


Introduction/Background To provide a comprehensive understanding and propose a strategy for the management of extracranial and nonvaginal extragonadal malignant germ cell tumors (EMGCTs).

Methodology We retrospectively reviewed cases of 12 patients with extracranial and nonvaginal EMGCTs treated in our center over the past 38 years. Data on clinicopathological characteristics, treatment modalities, and oncological outcomes were analyzed.

Results Among 209 patients diagnosed with EMGCTs, 12 (5.7%) were diagnosed with extracranial and nonvaginal EMGCTs: 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 histological type. Serum alpha-fetoprotein (AFP) is a sensitive tumor marker for YSTs, and the median serum AFP level was 8216 ng/ml (2.7–74157 ng/ml). One patient started bleomycin/etoposide/cisplatin without a pathologic diagnosis because it was unresectable and difficult to biopsy, and 11 patients started chemotherapy following tumor biopsy or resection. One patient suffered a recurrence, two patients died, and nine patients were disease-free. We observed a strong correlation between bleomycin/etoposide/cisplatin (BEP) chemotherapy and overall survival.

Conclusion Extracranial and nonvaginal EMGCTs are a heterogeneous group of tumors due to their varied onset ages, locations, and clinical presentations. An all-around clinical evaluation is crucial for selecting appropriate treatments. Most patients achieve good prognosis after surgical resection and chemotherapy. Adequate BEP chemotherapy can improve the prognosis of patients with rare diseases.

Disclosures The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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