No abstract

Gonadal function was evaluated in 25 boys treated for Hodgkin's disease according to the DAL-HD-85 protocol with OPA- or OPA/COMP-chemotherapy (vincristine-prednisone-adriamycine/cyclophosphamide-vincristine-m ethotrexate- prednisone). All boys were in first continuous complete remission for 6 to 45 months at chronological ages varying from 14.0 to 18.9 years. Testosterone, basal and GnRH-stimulated LH- and FSH-levels were measured. Gonadal function was normal in 16 patients treated with 2 cycles of OPA-chemotherapy in Hodgkin stages I-IIA. 9 patients were treated with 2 OPA- and 2 or 4 COMP-cycles of chemotherapy and had received mean cyclophosphamide doses ranging from 2004 to 3722 mg/m2. Again, no major testicular damage was noted, though some patients had increased stimulated LH-levels possibly indicating compensated Leydig cell-insufficiency. Our results demonstrate, that testicular function is not severely affected when patients are treated for Hodgkin's disease without procarbazine even if cyclophosphamide is given in cumulative doses below 3800 mg/m2. The previously documented severe testicular damage in boys treated according to the DAL-studies HD-78 and HD-82 is thus a result of the gonadotoxic action of procarbazine.

Hamartomas of the tuber cinereum are tumour-like collections of normal tissue in abnormal location. They are benign lesions with slow or absent growth and without any tendency to neoplastic evolution. Due to their neurosecreting properties they usually cause precocious puberty. Further neuroendocrine disturbances, seizures, or psychoneurological symptoms may be associated in some cases. Cisternography and CT are the most conclusive radiologic procedures in all cases. The typical feature is a well circumscribed round-shaped isodense soft tissue mass without contrast enhancement. Usually the tumour is small, rarely exceeding 2 cm. in diameter. If CT diagnosis is not conclusive, examination in the coronal plane or CT cisternography are recommended. Although CT does not permit a histological diagnosis the clinical and radiological features together are sufficient to make a highly suggestive diagnosis. The treatment of choice is medical therapy. Surgery should be restricted to those tumours which damage surrounding structures by their size and cause other symptoms than precocious puberty.

Testicular function was evaluated in 8 boys with acute lymphoblastic leukemia (ALL) and testicular relapse following another course of intensive chemotherapy with unilateral or bilateral orchidectomy and/or testicular irradiation. LH- and FSH-secretion was studied in all using a standardized LHRH-test. In addition, a HCG-test was performed in 6 boys. In prepuberty, all boys examined showed normal LH- and FSH-values. Beginning at 9 to 10 years, elevated basal and/or stimulated LH- and FSH-values were occasionally noted in contrast to the consistently elevated values after the age of 12. Using the HCG-test, we found a testosterone response only in patients receiving gonadal irradiation of 1 100 and 1 500 rads (2 patients). No response was elicited in those with radiation doses of 2 400 and 3 000 rads. We conclude that high dose gonadal irradiation and chemotherapy cause temporary and possibly permanent impairment of spermatogenesis and Leydig cell function in boys with ALL and testicular relapse.