ABSTRACT
BACKGROUND: Spermatocytic seminoma (SS) is a distinct testicular germ cell tumor, representing less than 1% of testicular cancers. The clinical features that distinguish ss from classical seminoma are an older age at presentation and a reduced propensity to metastasize. The aim of our work is to underline the epidemiological, clinical, histological, therapeutical and prognostic features of this tumor. FINDINGS: A retrospective analysis of patients referred to the national institute of oncology with seminoma, identified from the institutional tumor registry, between January 1996 and February 2009, was performed. Information reviewed included demographics, clinical, pathological staging, surgical management, adjuvant treatment and last follow-up. We studied four cases of spermatocytic seminoma, which represented 1% of testicular tumor and 6,4% of all seminoma treated at our institution during the study period. Median age at diagnosis was 45 years (range: 42-48). Mean delay before consulting was 9 months and the mean tumor size was 13,75 cm (10-18 cm). No patient had a history of maldescended testis. The main clinical complaint was unilateral testis mass with low progression. Pathology showed that tumors had a polymorphic appearance with small, intermediate and large cells. In all cases, the tumor was limited to the testis. immunohistochemical studies showed that tumors were negative for all the classical antibodies tested (LCA, cytokeratins, PLAP, lymphoid markers, CD117). Thoraco-abdomino-pelvic CT scan and tumor markers (AFP and hCG) were normal. All patients were Stage I. Treatment consisted on an orchidectomy associated with adjuvant radiotherapy in one patient. After a median follow-up of 6 years ranging from 2 to 15 years, we did not note any relapse or metastasis. CONCLUSION: The diagnosis of spermatocytic seminoma must be considered in all patients aged of more than 50 with testicular tumor. With only three cases of metastatic disease confirmed in the literature, this is a subgroup of patients in whom radiotherapy can safely be omitted.
ABSTRACT
Immunoproliferative small intestinal disease (IPSID), also known as alpha chain disease, is a rare disease. In the recent WHO classification of hematopoietic and lymphoid tissue, IPSID is considered as a variant of extranodal mucosa-associated lymphoid tissue (MALT) lymphoma. Campylobacter jejuni is a specific pathogen, found to be related to IPSID. Diagnosis is based on histology and immunochemistry (± fluorescent in situ hybridization), with presence of many variable levels of abnormal immunoglobulin in the serum, identified to be truncated alpha-heavy chains. Early-stage disease is treated by antibiotics (tetracyclines). Chemotherapy is recommended up front for patients with advanced disease at presentation or refractory to antibiotics. The chemotherapy schedule used is the CHOP (cyclophosphamide, vincristine, doxorubicin, and prednisone) regimen.
