DIFFERENTIATED SOMATIC MALIGNANCIES IN GERM-CELL TUMORS (GCT) - CLINICOPATHOLOGICAL APPRAISAL AND LITERATURE REVIEW

Background: Germ cell tumors (GCTs) are rare diseases, but represent the most common solid tumors in young men. Uncommonly, non-germ-cell malignant tumors may arise in primary or metastatic GCTs, most likely derived from teratomas, the so-called teratoma with malignant transformation (TMT). Here we review our clinical experience with this entity. Methods: The records of patients (pts) with GCT and a non-germ-cell component treated between 1997 and 2017 at our Hospital were retrospectively reviewed. Histology, disease extent, treatment and outcomes were assessed. Results: In a series of 76 consecutive male pts with GCTs treated during a 20-y period, 6 patients were diagnosed as having TMT. The median age was 27.5 years-old (range 17-34). The primary GCT histology was pure mature teratoma in 3pts, pure immature teratoma in 1 pt, and embryonal carcinoma in 2 pt. Three pts had stage II, and 3 pts had stage III. All pts received first-line chemotherapy (4 pts treated with PEB; 1 pt treated with VIP; 1 pt treated with Epi + Ifo). The malignant transformation was documented in the pre-chemotherapy surgical specimen in 2 pts, and after the first-line treatment in 4 pts. The somatic malignancy was detected within the primary testicular tumor in 2pts, in the primary mediastinal tumor in 2 pts, and in the metastases in 2 pts. Sarcoma was the dominant histology: rhabdomyosarcoma was identified in 3 tumors; leiomyosarcoma was observed in 1 tumor; angiosarcoma in 1 tumor; anaplastic carcinoma in 1 tumor and adenocarcinoma in 1 pt. In addition to the diagnostic orchiectomy, other surgeries were done in all pts. One pt with metastasis showing predominantly rhabdomyosarcoma was treated with adapted chemotherapy (VAC protocol), resulting in disease control. With a median follow up of 83 months (range 11-106), four pts are alive, while 2 pts have died. Conclusions: Teratoma with malignant transformation (TMT) arises in 3-6% of patients with GCTs. Several non-germ cell histologies may occur in association with the germ cell tumor. The identification of somatic malignant transformation is important in many respects: TMT may be associated with a poor prognosis; surgical resection is the treatment of choice; chemotherapy targeted to the specific type of somatic neoplasm may be helpful.

Publication History

Article published online:
10 July 2025