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ExtraGonadal Germ Cell Tumors

Goals. Review retroperitoneal and mediastinal primary germ cell tumorsReview primary therapeutic approach and outcome expectations for extragonadal sitesReview special biological associations- hematopoietic malignancies and non-germ cell elements. Characteristics of extragonadal germ cell tumor patients.

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ExtraGonadal Germ Cell Tumors

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    1. ExtraGonadal Germ Cell Tumors Craig R. Nichols, MD Earle Chiles Research Institute Robert Franz Cancer Research Center Providence Health Systems Portland, Oregon

    2. Goals Review retroperitoneal and mediastinal primary germ cell tumors Review primary therapeutic approach and outcome expectations for extragonadal sites Review special biological associations- hematopoietic malignancies and non-germ cell elements

    3. Characteristics of extragonadal germ cell tumor patients Hartmann, J. T. et al. Ann Oncol 2002 13:1017-1028; doi:10.1093/annonc/mdf176

    4. Characteristics of extragonadal germ cell tumor patients Hartmann, J. T. et al. Ann Oncol 2002 13:1017-1028; doi:10.1093/annonc/mdf176

    5. Characteristics of extragonadal germ cell tumor patients Hartmann, J. T. et al. Ann Oncol 2002 13:1017-1028; doi:10.1093/annonc/mdf176

    6. Basic Characteristics Extragonadal Seminoma Extragonadal seminomas mirror their testicular counterparts in marker elevation, sites of metastatic involvement and outcomes 90% of extragonadal seminomas are long term survivors

    7. Basic Characteristics Extragonadal Nonseminoma Retroperitoneal primary nonseminomas behave more similarly to testicular primary nonseminomas with similar IGCCC stage Mediastinal primary nonseminoma tend to have more frequent AFP elevation and are cured a minority of the time (40-45%)

    9. Factors with multivariate significance for OS in extragonadal non-seminoma *Presence of visceral disease (bone, liver, CNS metastasis), coefficient = 0.481, P = 0.008, HR = 1.62 (1.132.32) CI, confidence interval; CNS, central nervous system; HR, hazard ratio; OS, overall survival; -HCG, -human chorionic gonadotropin (n = 452 included, all patients with incomplete data have been excluded) Hartmann et al Ann Oncol

    10. Overall survival of patients with non-seminomatous extragonadal germ-cell tumors according to prognostic category

    11. Therapeutic Approach Extragonadal Seminomas Staging similar to testicular primary seminomas. Should include testicular exam and ultrasound to rule out occult testis primary in patients with presumed retroperitoneal primary Most patients present with disease > 5 cm and are most appropriate for primary chemotherapy dictated by ICCCC class (BEP X 3 for good risk, BEP X 4 for intermediate risk) Large post chemotherapy residual masses should be biopsied if PET avid and, if persistent disease, move on to salvage systemic therapy

    12. Therapeutic Approach Extragonadal nonseminomas Staging similar to testicular primary nonseminomas. Should include testicular exam and ultrasound to rule out occult testis primary in patients with presumed retroperitoneal primary Careful pathology review for non-germ cell elements Patients with retroperitoneal nonseminoma have treatment outcomes similar to intermediate risk IGCCC class despite having favorable prognostic features and probably are best served with therapy appropriate for intermediate prognosis patients (BEP X 4). All primary mediastinal nonseminomas should be treated as poor risk patients with BEP X 4 or on poor risk disease clinical trials

    13. Therapeutic Approach Extragonadal nonseminomas Patients obtaining PR marker negative status after chemotherapy should be considered for aggressive and complete resection of persistent disease Mediastinal primary patients with non-germ cell elements rarely respond to chemotherapy and, unless surgically resectable, are not curable

    14. Results of post chemotherapy resection of extragonadal nonseminoma RP Mediastinal n=225 n=287 Viable Tumor 23/92( 25%) 49/143 (34%) NEDter 37/92(36%) 37/143 (26%) NEDnec 54/92(59%) 53/143 (37%)

    15. 25 year man with Mediastinal Mass

    16. 25 year man with Mediastinal Mass

    17. Non germ cell elements 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 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).. All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery. 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). On the basis of our findings, the presence of SC appears to represent a poor prognostic sign for GCTs of gonadal and extragonadal origin.

    18. Non germ cell elements 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 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).. All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery. 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). On the basis of our findings, the presence of SC appears to represent a poor prognostic sign for GCTs of gonadal and extragonadal origin.

    19. Non germ cell elements 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 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).. All patients were treated by cisplatinum-based chemotherapy plus other agents followed by surgery. 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). On the basis of our findings, the presence of SC appears to represent a poor prognostic sign for GCTs of gonadal and extragonadal origin.

    20. Hematological Malignancies Associated with Mediastinal Nonseminoma Primary site Seminoma Nonseminoma Retroperitoneum 0/52 0/227 Mediastinum 0/51 *AML, mast cell disease, MDS with abnormal Megakaryocytes

    21. Salvage Therapy of Extragonadal Nonseminoma Primary Site n NED (%) Mediastinum 79/287 9/79 (8%) Retroperitoneum 61/227 19/61 (30%) Median follow-up 45 months for surviving pts

    22. Salvage Therapy of Extragonadal Nonseminoma Recurrent retroperitoneal nonseminoma should be managed in a manner similar to recurrent testicular nonseminoma eg high dose chemotherapy with post chemotherapy surgery in most cases Recurrent mediastinal nonseminoma without non germ cell elements Clinical trials, palliative care, possibly high dose chemotherapy Recurrent mediastinal nonseminoma with non germ cell elements-Palliative care

    23. Salvage Therapy of Extragonadal Seminoma Salvage programs similar to testicular primary sites should be employed with aggressive post chemotherapy management (including surgery) in selected cases

    24. Conclusions Patients experiencing multiple recurrences of germ cell tumor are rarely cured with any therapy and should be considered for desperation surgery or phase I/II trials of HDCT or novel compounds New biological understanding and new compounds are required

    25. Future Improvement in outcome will likely come from improved biologic understanding and new therapeutic compounds

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