Pathology reports of over 580 male patients with germ cell tumors treated between 1972 and 1982 were screened for teratomas in which malignant transformation was apparent. The diagnosis was established in 17 cases. The median age was 28 years (range, 14-52). For patients with disease limited to the testis, the median survival has not been reached, with all five patients surviving disease-free at 22+ to 120+ months. Among the 12 patients with metastatic disease, the median survival was 30.5 months (range, 12-69). All 12 patients were treated with cisplatin-containing regimens. Six had complete responses either to chemotherapy alone or to chemotherapy plus resection of residual disease. Four of the six complete responders relapsed, and three died of progressive disease. One patient with sarcomatous differentiation was treated with a doxorubicin-based regimen and had a partial remission, which lasted 8 months. Teratoma with malignant transformation, when found in metastatic sites, appeared to be a poor prognostic pathologic variant in male patients with germ cell tumors.

Teratoma with malignant transformation in germ cell tumors in men.

The clinicopathologic features of 46 patients with germ cell tumors with sarcomatous components (GCTSC) involving either the primary site or their metastases were studied. There were 43 men and 3 women aged 17 to 74 years. Twenty-three tumors arose in the mediastinum, 2 in the retroperitoneum, and 21 in the gonads. The germ cell component consisted of pure mature or immature teratoma (23 cases), teratoma mixed with other seminomatous or nonseminomatous components (17), pure seminoma (2), intratubular germ cell neoplasia (1), and yolk sac tumor (1). The SC included embryonal rhabdomyosarcoma (29), angiosarcoma (6), leiomyosarcoma (4), undifferentiated sarcoma (3), myxoid liposarcoma (1), malignant peripheral nerve sheath tumor (1), malignant "triton" tumor (1), and epithelioid hemangioendothelioma (1). Immunohistochemical studies were carried out in 34 cases with appropriate results supporting the diagnoses. Metastases containing both GCT and SC were observed in 6 cases, metastases of SC alone in 4, and metastases containing only GCT elements in 3. All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery. Clinical follow-up was available in 40 patients (1 to 96 mo; mean=24 mo). Thirty-two of 40 patients either died of tumor (25/40; 62.5%) or were alive with advanced, progressive disease (7/40; 17.5%), and only 8/40 (20%) were alive and free of disease between 5 to 40 months (mean=18 mo). Comparison of these patients with an age-matched and stage-matched control group of patients with GCT without SC showed statistically significant differences in survival between the 2 cohorts (P <or=0.001). On the basis of our findings, the presence of SC appears to represent a poor prognostic sign for GCTs of gonadal and extragonadal origin.

Germ Cell Tumors With Sarcomatous Components: A Clinicopathologic and Immunohistochemical Study of 46 Cases

The paratesticular region was the primary site in 20 of 289 children (7%) entered on the Intergroup Rhabdomyosarcoma Study. The 20 patients were 1.7- to 19-years-old at diagnosis. Fifteen underwent retroperitoneal node dissection (12) or biopsy (3), and 6/15 (40%) had nodal involvement by tumor. Nineteen of the 20 patients had no gross local or metastatic disease after surgery. All 20 received chemotherapy, and 13 also received radiotherapy. Treatment was effective: 16 of 18 evaluable patients (89%) were free of disease at a median of 23 months from diagnosis (range, 8-43 months). Since the incidence of tumor-involved retroperitoneal nodes is high, a dissection should be performed. If the nodes are free of tumor, retroperitoneal radiotherapy may not be necessary. Reduction of morbidity in patients with paratesticular rhabdomyosarcoma is desirable, because the disease has a good prognosis.

Paratesticular rhabdomyosarcoma in childhood.

A case of testicular teratoma metastasized to the retroperitoneum and after cytoreductive chemotherapy was noted to contain areas of frank sarcoma. Sarcomatous areas included embryonal rhabdomyosarcoma with a pattern of sarcoma botyroides, alveolar rhabdomyosarcoma, and fibrosarcoma. These areas differed markedly from areas of immature teratoma, which composed the remainder of the retroperitoneal lesion and which also characterized the primary tumor. These sarcomatous areas were characterized by numerous mitoses, marked cellular pleomorphism and diagnostic histologic, ultrastructural, and immunocytochemical features. Residual germ cell tumors following cytoreductive chemotherapy are traditionally categorized as teratoma only or teratoma with embryonal carcinoma or choriocarcinoma for therapeutic and prognostic reasons. This case does not conform to this simple categorization and raises serious questions concerning subsequent therapeutic decisions.

https://acsjournals.onlinelibrary.wiley.com/doi/pdf/10.1002/1097-0142%2819841101%2954%3A9%3C2015%3A%3AAID-CNCR2820540939%3E3.0.CO%3B2-B

F Alexander

Papers

Pure testicular rhabdomyosarcoma.