A 30-year-old man with a testicular tumor ressembling a "round cell sarcoma" was treated for rhabdomyosarcoma. Complete remission was achieved but the patient relapsed and died of the disease. A retrospective diagnosis of granulocytic sarcoma was established using an anti-myeloperoxidase antibody, unfortunately not available at the time of the initial diagnosis, No hematological disorders were observed during the course of the disease, Four cases of granulocytic sarcoma of the testis have been reported in the literature, All these cases where accompanied or followed by leukemia. The present case seems to be the first case of granulocytic sarcoma of the testis not accompanied by hematological disorders.
The therapeutic indications for germ cell tumours of the testis depend on the histology (pure seminoma: 45 %, non-seminomatous germ cell tumour: 55 %), the extension and the severity of the prognosis. The well standardised approach to pure seminomas is less clear for non-seminomatous germ cell tumours. Stage I, IIAB pure seminomas (95 to 98 % of cases) should be irradiated. The dose and target volume are adapted to prophylactic (1) and curative (II) objectives. Rare seminomas with a large tumour bulk should be treated with chemotherapy. Survival is close to 100 %. Stage I non-seminomatous germ cell tumour offers several theoretical possibilities. Radiotherapy is not very popular and chemotherapy appears to be to aggressive, lumboaortic lymph node dissection is being replaced by new imaging modalities and simple follow-up requires a rigorous and disciplined approach. At the Val-de-Grace hospital (France) since 1987, we perform simple orchidectomy in favourable stage I disease : 80 % are cured with no other treatment, 20 % relapse and are cured by chemotherapy, in the unfavourable stage I cancers (histology, markers) or with uncertain follow-up, limited chemotherapy is performed (3 cycles of EP). Stage II and more advanced non-seminomatous germ cell tumours are divided into moderate forms (IIA, B, III, IVL1) and major forms (IIC, IVL2, L3, H+, CNS+). In the exclusive infradiaphragmatic involvement of moderate forms, some authors propose bilateral lumboaortic lymph node dissection which is invasive surgery with an efficacy declining from 90 % (IIA) to 50 % (IIB). The majority of teams, particularly Val-de-Grace, administer 3 or 4 courses of BEP followed by assessment (CT scan - markers) and salvage surgery. The 5-year survival of non-seminomatous germ cell tumours for all stages combined is close to 90 %, but the major forms have a higher failure rate. Progress is expected in the therapeutic trials requiring groups, reflection and cooperation.
In 1992, the staging and follow-up of testicular germ cell tumours is based on a combination of computed tomography and tumour markers. Due to the development of medical imaging over the last decade, abdominal and thoracic CT has now replaced the combination of lymphography and pulmonary tomographies. Testicular ultrasonography is valuable for the diagnosis and contributes to staging and follow-up. The chest x-ray is still performed and MR] has very exceptional indications. Tumour markers, essentially alpha-foetoprotein and the free beta-fraction of human chorionic gonadotrophin, are useful in more than 80 % of NSGCTs and about 15 % of seminomas (beta-HCG alone). A very high initial level often indicates a poor prognosis. Monitoring of markers is essential after exclusive orchidectomy and to assess the efficacy of chemotherapy.
There are two other treatments for germ cell tumours of the testis apart from surgery: radiotherapy and chemotherapy. Radiotherapy is ideally administered with a linear accelerator delivering photons and electrons. The dose is well established and smaller volumes are now irradiated. The precision is increased by CT and by the use of personalised shields. Radiotherapy is indicated in pure seminomas, with two exceptions: rare seminomas with a large tumour mass (2 %), rare palliative indications for non-seminomatous germ cell tumours. Chemotherapy, following the progress due to the combination of vinblastine and bleomycin, has been based, for the last 10 years, on cisplatin, which must be administered at the correct dose. VP 16, ifosfamide and other drugs have also been introduced. In forms with a poor prognosis and depending on the clinical course, this chemotherapy should be administered at high doses with the protection of autologous bone marrow transplantation. This requires an appropriate infrastructure and a well trained team. The short-term and long-term effects of radiotherapy are more clearly defined than effects of chemotherapy.