PRIMARY TESTICULAR GRANULOCYTIC SARCOMA: CASE REPORT

A rare case of a 35 year old patient with primary testicular granulocytic sarcoma is described. In August 1997 the patient developed a painless testicular mass, low fever, dysuria, and bone pain treated unsuccessfully with antibiotics and NSAID. For thepersistence of these symptoms, a testicular biopsy and then an orchiectomy was performed: histological diagnosis of diffuse infiltration by an unclassifiable malignant tumor was made. During staging, bone marrow (BM) biopsy and peripheral blood (PB) was normal, while CT scan revealed abdominal lymph nodes enlargement. In January 98 the patient was admitted to our hospital for further management. After a few days, patient suddenly developed leukemia and diagnosis of AML (FAB M5a) was made. Testicular mass specimens were reviewed and myeloblastoma with amonoblastic differentiation diagnosis was made. Cytogenetic analysis was abnormal: (47XY,+8; XY, +8, add(9q). After treatmentwith idarubicin, cytosine-arabinoside and etoposide (ICE) a BM and PB remission was achieved but pelvic mass persisted. In April98 the patient had BM relapse and by reinduction treatment (ICE) a CR with disappearance of pelvic mass was obtained.In May 98 the patient underwent an allogeneic (aploidentical) bone marrow transplantation, after conditioning treatment with TBI and polichemoterapy. In October 1998 the patient presented a new BM and extramedullary relapse and therefore he was treated unsuccessfully with low dose cytosine arabinoside and infusion of total CD3+ donor lymphocytes. The disease was resistant to therapy and patient died in December 1998. Conclusion: Primary testicular granulocytic sarcoma is relatively rare, with only 4 documented cases reported in the literature. In our patient , primarytesticular involvement occurred 6 months before the leukemic bone marrow infiltration, with initial bone marrow and peripheralblood normal pattern. The clinical outcome of our case, despite allogeneic BMT, confirms the poor prognosis of this disease