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Endocrine Studies in a Male Patient with Choriocarcinoma and Gynecomastia
808
ONCOLOGY AND CHEMOTHERAPY
tomography scanning. Two small masses believed to be testes also were seen on the scan. A fine needle biopsy demonstrated malignancy of uncertain origin. The a-fetoprotein level was 1,899 ng./ml. and the /3-human chorionic gonadotropin was less than 3 mIU/ml. Presumptive diagnosis was retroperitoneal germ cell tumor, and chemotherapy with cisplatin, vinblastine and bleomycin was started. VP-16 was substituted for bleomycin after mucositis developed. Laparotomy 6 months later showed extensive retroperitoneal involvement with tumor, mostly embryonal carcinoma. There was no tumor in the testicles but several hyalinized scars were present. The patient eventually died of extensive disease. Given the propensity of cryptorchid testes to give rise to germ cell tumors and the universal presence of bilateral cryptorchidism in patients with the prune belly syndrome, it is surprising that no germ cell tumors have been reported in this patient population. Extragonadal germ cell tumors frequently are associated with testicular scarring and fibrosis. It is postulated that the scars in the testes represent regression of the primary tumor, while the primary retroperitoneal tumor may be a metastasis. G. F. S. 25 references
Endocrine Studies in a Male Patient With Choriocarcinoma and Gynecomastia
R. W. WHITCOMB, R. N. SCHIMKE, J. L. KYNER, B. P. LUKERT AND D. C. JOHNSON, Division of Endocrinology, Metabolism and Genetics, Department of Internal Medicine and Department of Physiology, University of Kansas School of Medicine, Kansas City, Kansas Amer. J. Med., 81: 917-920 (Nov.) 1986 The gynecomastia associated with choriocarcinoma is attributed to a decreased androgen-estrogen ratio. The reason for this alteration in nontesticular neoplasms is not always clear. A 41-year-old man presented with a 2-week history of painful bilateral gynecomastia and, subsequently, choriocarcinoma was found in the lung. The primary was believed to be either in the testes or mediastinum but it was undetected by current radiological techniques. Endocrine studies demonstrated elevated tumor levels of estradiol, testosterone and androstenedione. /3Human chorionic gonadotropin levels were elevated preoperatively, decreased for 2 weeks postoperatively and then began to increase again. Since /3-hurnan chorionic gonadotropin usually does not stimulate androgen production, the authors speculate that it had unusually high biological activity in this particular patient. They believed that the testosterone was elaborated by stimulated testes. They were able to demonstrate aromatase activity in the tumor and this enzyme is necessary for the conversion of androgen to estrogen. It is likely that the tumor did aromatize androgen and that the bulk of the estrogen derived from tumor conversion of androgen to estrogen. Although gynecomastia developed in the setting of increased androgen the androgen-estrogen ratio still was depressed. G. F. S. 1 figure, 2 tables, 19 references
Advances in the Treatment of Rhabdomyosarcoma
s. R. LACEY, T. C. JEWETT, JR., M. P. KARP, J.E. ALLEN AND D.R. COONEY, Department of Pediatric Surgery, State University of New York at Buffalo and The Children's Hospital of Buffalo, Buffalo, New York Sem. Surg. Oncol., 2: 139-146, 1986
Rhabdomyosarcoma is the most common soft tissue sarcoma in children less than 15 years old. In the past rhabdomyosarcoma was associated with an 80 per cent mortality rate. Improved 5-year survival rates of 70 per cent have been noted after combined modality therapy. Although the authors discuss all sites of rhabdomyosarcoma, major points of interest regarding genitourinary rhabdomyosarcomas are included. Genitourinary rhabdomyosarcoma occurs at the low rate of 0.5 to 0. 7 per million children per year. Rhabdomyosarcoma of the paratesticular areas is the most common tumor and it is associated with an early metastasis rate of 40 per cent at diagnosis. In contrast, only 15 to 20 per cent of the patients with genitourinary tumors have nodal metastases when seen initially. Accurate staging is important using the most common classification of the Intergroup Rhabdomyosarcoma Study, which is based on the amount of residual disease postoperatively. Genitourinary and retroperitoneal rhabdomyosarcomas present commonly as group I or II lesions. Histological evaluation also is significant in terms of prognosis. A recent conceptual change is the recognition of unfavorable (alveolar or pleomorphic) or favorable (all others) types. Lower survival rates are associated with unfavorable lesions. Treatment includes a combination of surgery, radiation and chemotherapy. Although complete surgical resection remains the optimal goal, improved survival has been documented in patients with microscopic residual disease. Of the patients with paratesticular rhabdomyosarcoma 40 per cent have metastasis in the para-aortic lymph nodes. Therefore, an operation should consist of inguinal orchiectomy and para-aortic node dissections. Treatment for pelvic rhabdomyosarcoma is changing and controversial. Although surgical treatment has ranged from an exenterative operation to more conservative procedures each case must be individualized. The Intergroup Rhabdomyosarcoma Study III recommendation is that in cases of pelvic rhabdomyosarcoma a biopsy should be obtained followed by 8 weeks of chemotherapy. If the tumor is decreased in size by 50 per cent 12 more weeks of chemotherapy are given followed by partial cystectomy. If the tumor is present at 20 weeks radiation is recommended. If the treatment fails to decrease a tumor by 50 per cent resection of the tumor and its organ should be done followed by radiation therapy. Two years of chemotherapy then are given. This prot0col-results in a -7-2 per cent rate .free of disease in patients with bladder and prostatic primaries. Chemotherapy clearly is indicated in all patients. Drugs of choice are actinomycin D, vincristine, cyclophosphamide and doxorubicin. Radiation therapy may be a useful adjunct to an operation in selected cases. It is of no use and no longer recommended in cases of group I rhabdomyosarcoma. Current 2-year survival rates by group include group I-83 to 90 per cent, group 11-75 to 85 per cent, group IIl-65 to 75 per cent and group IV-28 to 35 per cent. R. K. 1 figure, 4 tables, 18 references
Testicular Tumors in Infants and Children D. M. GREEN, Department of Pediatrics, Roswell Park Memorial Institute, The Children's Hospital of Buffalo, and School of Medicine, State University of New York at Buffalo, Buffalo, New York
Sem. Surg. Oncol., 2: 156-162, 1986 Primary tumors of the testes are uncommon in infants and children, with an incidence of only 1.1 cases per million. Al-
ONCOLOGY AND CHEMOTHERAPY
tomography scanning. Two small masses believed to be testes also were seen on the scan. A fine needle biopsy demonstrated malignancy of uncertain origin. The a-fetoprotein level was 1,899 ng./ml. and the /3-human chorionic gonadotropin was less than 3 mIU/ml. Presumptive diagnosis was retroperitoneal germ cell tumor, and chemotherapy with cisplatin, vinblastine and bleomycin was started. VP-16 was substituted for bleomycin after mucositis developed. Laparotomy 6 months later showed extensive retroperitoneal involvement with tumor, mostly embryonal carcinoma. There was no tumor in the testicles but several hyalinized scars were present. The patient eventually died of extensive disease. Given the propensity of cryptorchid testes to give rise to germ cell tumors and the universal presence of bilateral cryptorchidism in patients with the prune belly syndrome, it is surprising that no germ cell tumors have been reported in this patient population. Extragonadal germ cell tumors frequently are associated with testicular scarring and fibrosis. It is postulated that the scars in the testes represent regression of the primary tumor, while the primary retroperitoneal tumor may be a metastasis. G. F. S. 25 references
Endocrine Studies in a Male Patient With Choriocarcinoma and Gynecomastia
R. W. WHITCOMB, R. N. SCHIMKE, J. L. KYNER, B. P. LUKERT AND D. C. JOHNSON, Division of Endocrinology, Metabolism and Genetics, Department of Internal Medicine and Department of Physiology, University of Kansas School of Medicine, Kansas City, Kansas Amer. J. Med., 81: 917-920 (Nov.) 1986 The gynecomastia associated with choriocarcinoma is attributed to a decreased androgen-estrogen ratio. The reason for this alteration in nontesticular neoplasms is not always clear. A 41-year-old man presented with a 2-week history of painful bilateral gynecomastia and, subsequently, choriocarcinoma was found in the lung. The primary was believed to be either in the testes or mediastinum but it was undetected by current radiological techniques. Endocrine studies demonstrated elevated tumor levels of estradiol, testosterone and androstenedione. /3Human chorionic gonadotropin levels were elevated preoperatively, decreased for 2 weeks postoperatively and then began to increase again. Since /3-hurnan chorionic gonadotropin usually does not stimulate androgen production, the authors speculate that it had unusually high biological activity in this particular patient. They believed that the testosterone was elaborated by stimulated testes. They were able to demonstrate aromatase activity in the tumor and this enzyme is necessary for the conversion of androgen to estrogen. It is likely that the tumor did aromatize androgen and that the bulk of the estrogen derived from tumor conversion of androgen to estrogen. Although gynecomastia developed in the setting of increased androgen the androgen-estrogen ratio still was depressed. G. F. S. 1 figure, 2 tables, 19 references
Advances in the Treatment of Rhabdomyosarcoma
s. R. LACEY, T. C. JEWETT, JR., M. P. KARP, J.E. ALLEN AND D.R. COONEY, Department of Pediatric Surgery, State University of New York at Buffalo and The Children's Hospital of Buffalo, Buffalo, New York Sem. Surg. Oncol., 2: 139-146, 1986
Rhabdomyosarcoma is the most common soft tissue sarcoma in children less than 15 years old. In the past rhabdomyosarcoma was associated with an 80 per cent mortality rate. Improved 5-year survival rates of 70 per cent have been noted after combined modality therapy. Although the authors discuss all sites of rhabdomyosarcoma, major points of interest regarding genitourinary rhabdomyosarcomas are included. Genitourinary rhabdomyosarcoma occurs at the low rate of 0.5 to 0. 7 per million children per year. Rhabdomyosarcoma of the paratesticular areas is the most common tumor and it is associated with an early metastasis rate of 40 per cent at diagnosis. In contrast, only 15 to 20 per cent of the patients with genitourinary tumors have nodal metastases when seen initially. Accurate staging is important using the most common classification of the Intergroup Rhabdomyosarcoma Study, which is based on the amount of residual disease postoperatively. Genitourinary and retroperitoneal rhabdomyosarcomas present commonly as group I or II lesions. Histological evaluation also is significant in terms of prognosis. A recent conceptual change is the recognition of unfavorable (alveolar or pleomorphic) or favorable (all others) types. Lower survival rates are associated with unfavorable lesions. Treatment includes a combination of surgery, radiation and chemotherapy. Although complete surgical resection remains the optimal goal, improved survival has been documented in patients with microscopic residual disease. Of the patients with paratesticular rhabdomyosarcoma 40 per cent have metastasis in the para-aortic lymph nodes. Therefore, an operation should consist of inguinal orchiectomy and para-aortic node dissections. Treatment for pelvic rhabdomyosarcoma is changing and controversial. Although surgical treatment has ranged from an exenterative operation to more conservative procedures each case must be individualized. The Intergroup Rhabdomyosarcoma Study III recommendation is that in cases of pelvic rhabdomyosarcoma a biopsy should be obtained followed by 8 weeks of chemotherapy. If the tumor is decreased in size by 50 per cent 12 more weeks of chemotherapy are given followed by partial cystectomy. If the tumor is present at 20 weeks radiation is recommended. If the treatment fails to decrease a tumor by 50 per cent resection of the tumor and its organ should be done followed by radiation therapy. Two years of chemotherapy then are given. This prot0col-results in a -7-2 per cent rate .free of disease in patients with bladder and prostatic primaries. Chemotherapy clearly is indicated in all patients. Drugs of choice are actinomycin D, vincristine, cyclophosphamide and doxorubicin. Radiation therapy may be a useful adjunct to an operation in selected cases. It is of no use and no longer recommended in cases of group I rhabdomyosarcoma. Current 2-year survival rates by group include group I-83 to 90 per cent, group 11-75 to 85 per cent, group IIl-65 to 75 per cent and group IV-28 to 35 per cent. R. K. 1 figure, 4 tables, 18 references
Testicular Tumors in Infants and Children D. M. GREEN, Department of Pediatrics, Roswell Park Memorial Institute, The Children's Hospital of Buffalo, and School of Medicine, State University of New York at Buffalo, Buffalo, New York
Sem. Surg. Oncol., 2: 156-162, 1986 Primary tumors of the testes are uncommon in infants and children, with an incidence of only 1.1 cases per million. Al-