281/2277. Testicular (gonadal stromal) fibroma: case report and review of the literature. A 25-year-old man presented with complaint of a painless enlargement in his left testis. The solid, encapsulated, circumscribed and grayish-white testicular mass displayed the characteristics of testicular fibroma histologically. It was composed of acellular collagenized plaques and hypercellular areas of fibroblastic spindle cells. Immunohistochemically, the neoplastic cells were positive for vimentin and smooth muscle actin, but not for cytokeratin, S-100 protein, desmin, CD99/MIC2 (a protein expressed by sertoli cells and granulosa cells) and CD34. Only 18 cases of testicular (gonadal stromal) fibroma composed exclusively of spindle cells have been reported to date. An additional case of fibroma, which lacks definite neoplastic sex cord elements, and its differential diagnosis from other mesenchymal lesions of testis are discussed here, together with other cases in the literature. ( info) |
282/2277. Persistent Mullerian Duct Syndrome (PMDS) with testicular seminoma. Persistent Mullerian Duct Syndrome (PMDS) is characteristically associated with unilateral or bilateral cryptoorchidism. Like other undescended testis, these gonads are at an increased risk of malignant transformation. We report a case of intra abdominal seminoma in cryptorchid testis of a patient with the Persistent Mullerian Duct Syndrome, hitherto uncommonly reported in india. ( info) |
283/2277. Solitary delayed contralateral testicular metastasis from renal cell carcinoma. We report a 60 year old male presenting with contralateral testicular metastasis 7 years following radical nephrectomy for renal cell carcinoma. Testicular metastases from renal cell carcinoma reported in literature are predominantly ipsilateral and invariably on the left side. Usually these are present simultaneously with the renal primary or precede the diagnosis of renal tumors. Delayed contralateral testicular metastatic has not been reported to the best of our knowledge. The case highlights the unique behaviour of renal cell carcinoma with an unusual site of recurrence. The clinical presentation, pathogenesis and management of this rare presentation along with review of recent literature are discussed. ( info) |
284/2277. seminoma arising in cryptorchid testis 25 years after orchiopexy: case report. A 33-year-old man who underwent right orchiopexy at age 8 for undescended testis had a swelling develop at the same site 25 years later. He underwent right high orchiectomy. Histopathologic examination of the resected specimen suggested seminoma, and he was treated with chemotherapy (cisplatinum, etoposide, and bleomycin.) Even though the ideal age at orchiopexy is unknown, early intervention is advocated to preserve fertility and prevent malignant transformation. The management of cryptorchid testis is controversial. In view of the persistent malignant potential of this condition, early orchiopexy and lifelong follow-up are recommended. ( info) |
| The Wilms' tumor gene (WT1) encodes a zinc-finger transcription factor involved in the development of the kidneys and gonads and their subsequent normal function. Mutations in the WT1 gene were identified in patients with WAGR (Wilms' tumor, aniridria, genitourinary abnormalities, and mental retardation), denys-drash syndrome, and frasier syndrome (FS). Constitutional heterozygous mutations of the WT1 gene, almost all located at intron 9, are found in patients with FS. This syndrome is characterized by female external genitalia in 46,XY patients, late renal failure, streak gonads, and high risk of gonadoblastoma development. We report a male with FS with an unusual phenotype characterized by normal penis size with perineal hypospadias, end-stage renal failure at the age of 19 yr, normal adult male serum T levels, extremely elevated gonadotropin levels, para-testicular leiomyoma, unilateral testicular germ cell tumor, bilateral gonadoblastoma, and absence of gonadal dysgenesis. Automatic sequencing identified the IVS9 4C>T mutation in the WT1 gene, which predicts a change in splice site utilization. WT1 transcript analysis showed reversal of the normal positive/negative KTS (lysine, threonine, and serine) isoform ratio, confirming the diagnosis of FS. This patient with FS presents an external genitalia of denys-drash syndrome, suggesting that these two syndromes are not distinct diseases but may represent two ends of a spectrum of disorders caused by alterations in WT1 gene. This case expands the spectrum of phenotypes associated with WT1 mutations, by including predominantly male ambiguous genitalia and absence of gonadal dysgenesis, extremely high gonadotropin levels, and delayed adrenarche, and presence of a para-testicular leiomyoma, bilateral gonadoblastoma, and germ cell neoplasia. ( info) |
286/2277. Follicular large cell lymphoma localized to the testis in children. PURPOSE: Primary follicular lymphoma of the testis in childhood is rare with only 6 cases previously reported. We present 3 additional cases. MATERIALS AND methods: We extensively analyzed primary follicular lymphoma of the testis in 3 boys. Clinical data were obtained by reviewing patient charts. RESULTS: The patients were 4, 5 and 11 years old, respectively. Two patients presented with painless unilateral testicular enlargement and 1 presented with unilateral hydrocele. Laboratory findings were within normal limits in all patients. Radical orchiectomy was done in all cases. The excised testes were partially or completely replaced by tumor. In all cases the features were those of follicular, large cell-type malignant lymphoma. Tumor cells in all cases were CD20 and CDw75 positive, focally CD23 positive and bcl-2 negative, while in 2 they were CD10 positive and bcl-6 positive. Surface Ig was absent in the 2 cases studied. karyotyping in 1 case showed a normal karyotype. Staging revealed no evidence of extratesticular disease. All patients underwent combination chemotherapy and were in complete remission 7 to 59 months after therapy. CONCLUSIONS: We present 3 cases of pediatric primary follicular lymphoma of the testis. Pathological findings and clinical features were similar to those in the 6 previously reported cases and suggest that primary pediatric testicular follicular lymphoma may represent unique subset of follicular lymphoma with a particularly good prognosis. ( info) |
287/2277. Testicular germ cell cancer despite previous local radiotherapy to the testis. BACKGROUND: Testicular intraepithelial neoplasia (TIN, also carcinoma in situ of the testis) is the uniform precursor of testicular germ cell cancer. Local radiotherapy to the testis with dosages of 18-20 Gy has been found to safely eradicate TIN and germ cells, too. Thus, the general assumption is that the development of invasive germ cell tumours can be prevented by this radiotherapy. patients AND methods: Herein, we report two patients with one-sided testicular tumour and biopsy-proven contralateral TIN. Both of them developed germ cell neoplasms in the remaining testis although local radiotherapy with 20 Gy had been applied to the testis. RESULTS: One patient developed pure seminoma 7 years after completion of radiotherapy, the other developed a combined tumour consisting of embryonal carcinoma and seminoma after 5 years. Treatment consisted of orchiectomy in each of the cases. Histologically, both had TIN in the testicular tissue surrounding the new growths. CONCLUSIONS: Pathogenetically, a small fraction of radioresistent TIN cells overcoming irradiation and progressing to full-blown germ cell cancer in the later course may be the histogenetic clue to explain these unexpected events. Other explanations, though less probable, could be technical radiotherapeutic failure due to targeting problems and a pre-existing radioresistent germ cell tumour in the irradiated testicle. ( info) |
288/2277. Case report: testicular secondary localization of a multiple myeloma. A 61-year-old man who had 40 months history of recurrent multiple myeloma presented a right testicular mass, which was diagnosed incidentally by scrotal sonography examination. Alpha-feto-protein and beta-human-chorionic-gonadotropin were normal. A right radical orchiectomy was performed. The histological examination showed an intermediate grade plasmacytoma. ( info) |
289/2277. Leydig cell tumour--a rare testicular tumour. Although Leydig cell tumour is a rare tumour which constitutes only 1-3% of all testicular tumours, still it is in the focus of interest because of the difficulties in determining its exact nature and subsequently the type of treatment and follow-up. We report a case of Leydig cell tumour with a review of the related literature. ( info) |
290/2277. Extragonadal endodermal sinus tumours. Report of a case. A case is described of endodermal sinus tumour originating in the sacro-coccygeal region in a man 20 years of age. Of the hitherto presented cases of endodermal sinus tumour of a gonadal origin, 24 out of 42 patients have died. Of 21 patients in whom the disease was of an extragonadal origin, 18 have died. Thus, the prognosis seems to be less favourable in patients with extragonadal tumours. The best prognosis has been reported in boys with endodermal sinus tumours of a testicular origin operated before the age of 2 years. ( info) |