Cases reported "Leydig Cell Tumor"

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1/111. Ten-year follow-up in a boy with leydig cell tumor after selective surgery.

    In a 4(8)/12-year-old boy with precocious puberty and an enlarged right testis, a leydig cell tumor was diagnosed. Surgical exploration revealed an encapsulated tumor which was selectively removed without orchiectomy. Within 1 year the signs of precocious puberty disappeared. Ten years later, the patient remained free of disease and had developed normal spontaneous puberty. Despite of highly advanced bone age at the time of diagnosis (13 years according to Greulich and Pyle), his height at age 15 was in the upper normal range and within the familial target height. Most of these prepubertal patients affected by this tumor underwent orchiectomy, although no malignant course of Leydig cell tumors before puberty has been reported. This work provides the first example of long-term follow-up of a prepubertal boy after testis-sparing surgery for a Leydic cell tumor. We conclude that selective removal of the tumor may be the procedure of choice in this entity.
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2/111. Metastatic leydig cell tumor with sarcomatoid differentiation.

    Leydig cell tumors of the testis are uncommon. Only about 10% of cases have a malignant course. It has been stated that the only definite criterion for malignancy is presence of metastasis. We present a 47-year-old patient with metastatic leydig cell tumor 17 years after initial diagnosis, to our knowledge the longest reported interval between diagnosis and the development of metastasis. The primary tumor did not exhibit convincing features of malignancy. The initial metastasis in the right perirenal fat tissue showed a biphasic tumor with sarcomatoid differentiation not described previously in a metastatic Leydig cell tumor.
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3/111. Malignant leydig cell tumor of the testis associated with Klinefelter's syndrome.

    We reported the case of a 35-year-old man with Klinefelter's syndrome and a malignant leydig cell tumor of the testis. Bilateral gynecomastia and right testicular enlargement led the patient to seek medical assistance. Despite initial orchidectomy two years later the patient developed lung and iliac lymph node metastases. The tumor appeared to be refractory to chemotherapy and to hormonal treatments including op'DDD. Finally, the patient died within 20 months of developing metastases. leydig cell tumor is an exceedingly rare tumor, especially when associated with Klinefelter's syndrome. This association as well as presentation, pathologic features, hormonal abnormalities, clinical course and response to therapy of malignant Leydig cell tumors are discussed.
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ranking = 2.5
keywords = testis
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4/111. Organ-sparing surgery for bilateral leydig cell tumor of the testis.

    Leydig cell tumors of the testis are uncommon, and bilateral lesions are extremely rare. We report a case of bilateral leydig cell tumor of the testis treated with radical orchiectomy and contralateral subtotal orchiectomy with the intent of preservation of hormonal function and fertility.
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keywords = testis
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5/111. cushing syndrome as the presenting feature of metastatic leydig cell tumor of the testis.

    We report a patient with a history of orchiectomy for leydig cell tumor of the testis who developed cushing syndrome. This syndrome was due to ectopic production of cortisol and was the primary feature of tumor recurrence.
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6/111. growth-arrest and inhomogenous echotexture of the affected testis after tumor enucleation for unilateral leydig cell tumor.

    The authors report on an 8-year-old boy with unilateral left-sided leydig cell tumor. After enucleation of the tumor, endocrine disturbances resolved. Long-time follow-up for more than 7 years was characterized by growth-arrest of the affected gonad and the unchanged appearance of a circumscribed hypoechogenic residual lesion within the testis.
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keywords = testis
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7/111. Intracytoplasmic and intranuclear Reinke's crystals in a testicular Leydig-cell tumor diagnosed by fine-needle aspiration cytology: a case report with review of the literature.

    We report on the cytopathologic findings of a Leydig-cell tumor of the testis in a young adult male with no evidence of endocrine dysfunction. The preoperative diagnosis was based on fine-needle aspiration cytology (FNAC) alone, which was subsequently confirmed on histopathology. The present case was of interest on account of the paucity of literature regarding the cytodiagnosis of this lesion. In addition, the finding of intracytoplasmic lipofuscin pigment and several intracytoplasmic as well as intranuclear Reinke's crystals served to clinch the diagnosis on FNA. Therefore, the use of FNAC, especially in the presence of diagnostic Reinke's crystals, may vitiate the need for more invasive biopsy procedures in the preoperative diagnosis of testicular Leydig-cell tumors.
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8/111. Development of true precocious puberty following treatment of a leydig cell tumor of the testis.

    We report a prepubertal boy who developed true precocious puberty following unilateral orchidectomy for the treatment of a leydig cell tumor.
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9/111. Malignant Leydig cell tumour of the testis.

    A case of malignant Leydig cell tumour is presented. It is a rare primary malignant tumour of the testis and occurs exclusively in adults. The present case is of interest because it occurred at the young age of 25 years which is rare. Histologically it showed almost all features which suggest malignancy and also had metastases to the lungs and liver. The clinical details and pathology of this tumour are discussed.
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ranking = 2.5
keywords = testis
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10/111. Mutational analysis of the luteinizing hormone receptor gene in two individuals with Leydig cell tumors.

    Inactivating mutations of the luteinizing hormone receptor (LHR) gene in males induce Leydig cell agenesis or hypoplasia, while activating mutations cause testotoxicosis. Recently, it was demonstrated that a somatic heterozygous activating mutation of the LHR gene (Asp578His), limited to the tumor, was the cause of Leydig cell adenomas in three unrelated patients. We describe the molecular study of two unrelated boys with gonadotropin-independent hypersecretion of testosterone due to Leydig cell adenomas. Genomic dna was extracted from the tumor, the adjacent normal testis tissue, and blood leukocytes. Both individuals exhibited an heterozygous missense mutation, limited only to the tumor, consisting of a guanine (G) to cytosine (C) substitution at codon 578 (GAT to CAT), turning aspartic acid into histidine. The presence of the same mutation in different ethnic groups demonstrates the existence of a mutational hot spot in the LHR gene. Indeed, this mutation occurs at the conserved aspartic acid residue at amino acid 578, where a substitution by glycine is the most common mutation observed in testotoxicosis and where a substitution by tyrosine has been linked to a more severe clinical phenotype where diffuse Leydig cell hyperplasia is found. Our results confirm the fact that somatic activating mutations of gonadotropin receptors are involved in gonadal tumorigenesis.
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