The development of non-germ cell malignancies within germ cell tumors. A clinicopathologic study of 11 cases

Abstract

Eleven male patients had germ cell tumors of the testis (7), mediastinum (3), or retroperitoneum (1) in which non‐germ cell malignancies developed. Such malignant non‐germ cell elements were present in the primary excisions of five patients and were subsequently found in additional resected tissue in 10 of 11 patients. In the patients who had multiple pathology specimens examined, a progression from atypia to predominant non‐germ cell malignancy was often found. The authors believe these malignant elements arose within teratomatous foci, since eight of nine cases had teratoma in the primary tumor, and teratoma was found in subsequently resected tissue in one additional case. Cisplatin therapy frequently “unmasked” the non‐germ cell malignant elements by destroying the more chemosensitive germ cell cancers. The prognosis was worst for five patients who developed progressive embryonal rhabdomyosarcoma: two of these patients died of local spread of tumor, whereas a third died of metastatic sarcoma. Only one patient, who had total surgical excision of rhabdomyosarcoma, survived. Other forms of sarcoma that developed within germ cell tumors did not appear to adversely affect the prognosis beyond that of teratoma. It is currently recommended, when feasible, that patients with teratoma and sarcoma undergo total surgical excision. Further treatment with cisplatin regimens, after eradication of the germ cell component, has not been helpful. The role of other forms of chemotherapy remains speculative.

Testicular germ cell tumor with rhabdomyosarcoma successfully treated by disease-adapted chemotherapy including high-dose chemotherapy: case report and review of the literature

“…[1][2][3] The median OS in one retrospective study was 23 months. 4 Complete surgical resection of residual or recurrent disease is recommended when feasible.…”

“…4 Complete surgical resection of residual or recurrent disease is recommended when feasible. 1,4,6 The role of CHT has not been well defined. The CR rate was estimated at 30% for CHT alone, and at 38% for CHT and surgery.…”

“…[1][2][3] A derivation from preexistent mesenchymal elements of the teratomatous tissue is assumed. 4 Alternatively, the nongerm tissue may arise from the malignant transformation of totipotential germ cell subpopulations showing partial differentiation into somatic mesenchymal tissue.…”

“…6 Sarcomas can be present in the primary tumor or appear in metastases. 1,7 Teratomas with rhabdomyosarcomatous differentiation have a poor prognosis. 4 Here, we report a rare case of a combination of seminoma, teratoma and rhabdomyosarcoma.…”

First-line sequential high-dose VIP chemotherapy with autologous transplantation for patients with primary mediastinal nonseminomatous germ cell tumours: a prospective trial

“…Conventional cisplatin-based chemotherapy has demonstrated activity in patients with extragonadal GCT, and long-term survival rates approach those of patients with advancedstage metastatic gonadal GCT (Nichols et al, 1990b, Hidalgo et al, 1997. Primary nonseminomatous tumours of the mediastinum have a poor outcome with conventional chemotherapy and the presence of a mediastinal location defines the patient as 'poor prognosis' according to the IGCCCG classification irrespective of additional metastatic sites or elevated tumour marker concentrations (Ulbright et al, 1984;Toner et al, 1991;Bukowski et al, 1993;Childs et al, 1993;Harding et al, 1993;Saxman et al, 1994;Hidalgo et al, 1997;International Germ Cell Consensus Classification, 1997;Ganjoo et al, 2000). High-dose chemotherapy with autologous peripheral blood stem cell transplantation (HD-CT) is used as a therapeutic option with acceptable toxicity in patients with relapsed GCT, and has also been investigated as first-line therapy in patients fulfilling 'intermediate' or 'poor prognosis' criteria according to the IGCCCG classification (Motzer et al, 1993Bokemeyer et al, 1998).…”