RHABDOMYOSARCOMA IN A PATIENT TREATED FOR METASTATIC GERM CELL TUMOUR OF THE TESTIS CONTAINING TERATOMA—A CASE REPORT

A patient who developed a rhabdomyosarcoma following apparently successful chemotherapy for metastatic germ cell testicular carcinoma is presented. This newly recognized association may be seen particularly in patients whose initial germ cell malignancy contains immature teratoma. Possible reasons for this are discussed. The findings in this patient suggest that re-biopsy of recurrent disease be undertaken wherever possible, particularly where immature teratoma was a feature of the initial histopathology. A proportion of relapsing patients as described may not in fact have recurrent germ cell malignancy, but may have developed high grade, and often chemoresistant sarcomas. These second tumours appear to have an extremely poor prognosis, unless amenable to complete surgical resection.     

CLINICAL EXPERIENCE WITH YOLK SAC TUMORS AND TERATOMA IN CHILDREN

Between 1971 and 1993, 12 children with testicular germ cell tumors were treated at the Department of Urology, Faculty of Medicine, Kyoto University. Seven patients had yolk sac tumors and 5 had mature teratoma. Of the 7 patients with yolk sac tumors, 6 had stage I and 1 had stage III tumors. Initial management of the stage I tumors consisted of high orchiectomy in 5 patients and high orchiectomy plus retroperitoneal lymph node dissection in 1 patient. Of these 6 patients, 4 were cured by surgery alone but lung metastases developed in the other 2 patients. One of them was salvaged with thoracotomy and chemotherapy but the other died of tumor. The patient with stage III tumor had bulky tumor spread to lung and retroperitoneum, but seems to have been cured by chemotherapy followed by resection of the residual mass although follow-up is still inadequate (14 months). Six of the 7 patients (85.7%) are alive 13 months to 21 years after diagnosis. Five patients with mature teratoma were treated by high orchiectomy or, more recently, enucleation and all are alive 4 months to 22 years after surgery.     

A CASE OF SPONTANEOUS THROMBOSIS OF THE PAMPINIFORM PLEXUS OF THE TESTIS

A case of spontaneous thrombosis of the pampiniform plexus of the testis in a young man is presented. The literature is briefly reviewed.     

ORGAN SPARING SURGERY FOR MALIGNANT GERM CELL TUMOR OF THE TESTIS

PURPOSE: We evaluate the indication, technique and long-term outcome of organ preserving tumor resection rather than standard orchiectomy in 73 patients with bilateral testicular germ cell tumors or tumors of a solitary testicle. MATERIALS AND METHODS: Tumor resection was performed in 73 patients with 52 and 17 metachronous and synchronous bilateral testicular germ cell tumors, respectively, and 4 testicular germ cell tumors of a solitary testicle. Histology of the enucleated germ cell tumor revealed seminoma in 42 (57.5%) patients, embryonal carcinoma in 14 (19.2%), mature teratoma in 11 (15.1%), and mixed and combined germ cell tumors in 6 (8.2%). Mean tumor diameter was 15 mm. (range 5 to 30). Associated testicular intraepithelial neoplasia was diagnosed in 82% of patients who underwent local radiation with 18 Gy. RESULTS: After a median followup of 91 months (range 3 to 191) 72 (98.6%) patients had no evidence of disease and 1 died of systemic tumor progression. No local relapse occurred in 46 patients with associated testicular intraepithelial neoplasia treated with local radiation. However, local recurrence did develop in 4 patients after 3, 6, 12 and 165 months without radiation but all survived after undergoing inguinal orchiectomy. Testosterone levels were normal in 62 (84.9%) patients, hypogonadism developed in 7 (9.6%) and preoperative levels remained low in 4 (5.5%). Of the 10 patients who postponed local radiation for paternity reasons 5 had successfully fathered a child after organ sparing surgery. CONCLUSIONS: After a long-term followup of greater than 7 years organ sparing surgery represents a viable therapeutic approach to bilateral testicular germ cell tumor with an excellent postoperative outcome. Tumor enucleation might be considered a standard approach if the guidelines are respected, including cold ischemia, organ confined tumor less than 20 mm., multiple biopsies of the tumor bed, adjuvant local irradiation postoperatively to avoid local recurrence, close followup and high compliance.     

MANAGEMENT OF A PHAEOCHROMOCYTOMA IN A CHILD

The management of a case of phaeochromocytoma in a child usingalpha and beta receptor blocking drugs is described. This reportconfirms that estimations of plasma catecholamine concentrationsand direct measurement of arterial pressure are of considerablevalue. Methoxyflurane would appear to be a most suitable anaestheticfor patients with this condition.