1/176. Secondary glioblastoma remarkably reduced by steroid administration after anaplastic transformation from gliomatosis cerebri--case report.A 45-year-old female presented with gliomatosis cerebri manifesting as hemiballismus-like involuntary movement in the arm, motor weakness in the leg, and hypesthesia in her left side. Computed tomography showed only diffuse swelling of the right cerebral hemisphere, but T2-weighted magnetic resonance imaging revealed a diffuse lesion spreading from the right thalamus to the temporal, parietal, and occipital lobes on the same side. No abnormal enhancement was recognized. cerebral angiography showed no specific finding. A right occipital lobectomy was performed to confirm the diagnosis of gliomatosis cerebri. Anaplastic transformation was recognized 5 months later. The disease did not resolve with radiation or interferon administration, but steroid therapy achieved remarkably effective tumor regression. The patient died due to pneumonia. autopsy showed the features of diffuse glioblastoma. Steroid therapy may be an effective treatment for gliomatosis cerebri before the terminal stage.- - - - - - - - - - ranking = 1keywords = spread (Clic here for more details about this article) |
2/176. Progression of a Nelson's adenoma to pituitary carcinoma; a case report and review of the literature.A 25-year-old woman developed Nelson's syndrome, 3 years after successful bilateral adrenalectomy for Cushing's disease. Despite pituitary surgery and radiotherapy the tumour showed invasive growth, leading to visual disturbance, paresis of the oculomotor nerve and, 34 years after adrenalectomy, to death by widespread purulent leptomeningitis. autopsy revealed a large adenohypophyseal carcinoma with a metastasis attached to the dura, both tumours showing immunocytochemical staining for ACTH and TSH. We review the literature on metastatic adenohypophyseal carcinoma in Cushing's disease and Nelson's syndrome and discuss the role of proliferation markers as indicators of malignant progression.- - - - - - - - - - ranking = 1keywords = spread (Clic here for more details about this article) |
3/176. Two case studies of chronic idiopathic neutropenia preceding acute myeloid leukaemia.Chronic idiopathic neutropenia is regarded as a benign disorder without risk of malignant transformation. We present two patients with chronic idiopathic neutropenia who showed disease progression to acute myeloid leukaemia. sequence analysis of the granulocyte-colony stimulating factor receptor (G-CSFR) gene from leukaemic DNA did not reveal any mutations and microsatellite analysis provided no evidence of microsatellite instability or loss of constitutional heterozygosity. These case studies suggest that chronic idiopathic neutropenia may constitute a preleukaemic condition in some patients. Alterations of the G-CSFR or defective dna mismatch repair do not appear to be involved in malignant transformation.- - - - - - - - - - ranking = 3.0108982507098keywords = colony (Clic here for more details about this article) |
4/176. Malignant transformation of an optic disk melanocytoma.PURPOSE: To report a case of malignant transformation of an optic disk melanocytoma with a second melanocytoma in the ciliary body. methods: Clinical data including visual acuity, visual fields, color fundus photographs, fluorescein angiogram, and ultrasonogram and histopathologic studies of this case were reviewed. RESULTS: The right eye of a 65-year-old white woman was diagnosed with melanocytoma of the optic nerve. Four years later, the tumor became significantly larger. The best-corrected visual acuity declined from 20/40 to counting fingers and the size of the tumor increased fourfold in 2 years. The right globe was enucleated. Histopathologic studies demonstrated moderately pigmented spindle-B malignant melanoma cells adjacent to and within a population of large, polyhedral, heavily pigmented melanocytoma cells that extended to the lamina cribrosa and optic nerve. There was also a deeply pigmented melanocytoma in the ciliary body. CONCLUSION: This is a rare case of malignant melanoma transformed from an optic disk melanocytoma. Periodic follow-up of the patient with optic disk melanocytoma is necessary.- - - - - - - - - - ranking = 8.1257984968218keywords = count (Clic here for more details about this article) |
5/176. Microcystic meningioma arising in a mixed germ cell tumor of the testis: a case report.We report a case of a microcystic variant of meningioma arising in a mixed germ cell tumor of the testis composed predominantly of mature and immature teratoma with elements of seminoma and embryonal carcinoma. We believe this is the first such case of a meningioma arising in a teratoma within a gonadal or extragonadal site. The meningiomatous component showed positive immunohistochemical staining for epithelial membrane antigen and a lack of staining for cytokeratin, factor viii, CD31, and alpha-fetoprotein. Recognition of a non-germ cell tumor arising in the setting of a teratoma in the testis may be prognostically important depending on the nature of the non-germ cell component and whether it has spread beyond the testis.- - - - - - - - - - ranking = 1keywords = spread (Clic here for more details about this article) |
6/176. Pronounced cystic transformation of the rete testis. MRI appearance.BACKGROUND: Cystic transformation of the rete testis is a little-known partial or complete obstruction of the spermatozoa-containing secretion of the head of the epididymis. Depending on its severity, either ectasia or a cystic transformation occurs, which spreads to the network of convoluted seminiferous tubules in the mediastinum testis. Findings in contrast-enhanced MRI examinations are characteristic and may help to differentiate this benign entity from malignant neoplasia. The authors present two pronounced cases in different stages, documenting the broad spectrum of possible involvement.- - - - - - - - - - ranking = 1keywords = spread (Clic here for more details about this article) |
7/176. Proliferative verrucous leukoplakia: report of two cases and a discussion of clinicopathology.Proliferative verrucous leukoplakia (PVL) is a recently delineated but poorly recognized form of multifocal leukoplakia that is premalignant and of unproven origin. PVL generally presents as a simple benign form of hyperkeratosis that tends to spread and become diffuse. Although slow-growing, the disease is persistent and irreversible. Clinically, PVL often presents as an exophytic wart-like form of leukoplakia that appears to be resistant to nearly all forms of therapy. PVL of the oral cavity is best-defined as a continuum of oral epithelial disease with hyperkeratosis at one end of a clinical and microscopic spectrum and verrucous carcinoma or squamous cell carcinoma at the other. The microscopic findings associated with PVL are dependent on the stage of the disease and the adequacy of the biopsy. Microscopic findings can be markedly variable. PVL is a clinicopathologic disorder that includes the microscopic entity known as verrucous hyperplasia as a component of its histopathologic progression. This article reports on two cases of PVL, describes the clinicopathology of the disease process, and presents therapeutic and etiologic considerations.- - - - - - - - - - ranking = 1keywords = spread (Clic here for more details about this article) |
8/176. Malignant transformation of mesenchymal hamartoma of the liver: case report and review of the literature.Here the first case in the literature of both mesenchymal hamartoma and malignant mesenchymoma occurring in a 6-year-old male child, at different times and at different sites in the liver, and also the possible malignant transformation of a mesenchymal hamartoma is reported. The tumor developed from a lesion in the right lobe that was overlooked initially during a left lateral segmentectomy at 18 months of age for a mesenchymal hamartoma. Malignant mesenchymoma is a rare and aggressive tumor. The origin of this tumor is not well understood. There has been no direct support to the hypothesis that malignant mesenchymoma may be the malignant counterpart of mesenchymal hamartoma. The authors provide clinical and histopathologic evidence in our case that suggests the possibility of malignant mesenchymoma arising from a mesenchymal hamartoma. This case emphasizes the need for complete removal of mesenchymal hamartoma and the need for long-term follow-up to detect multifocal lesion or malignant transformation.- - - - - - - - - - ranking = 8.1257984968218keywords = count (Clic here for more details about this article) |
9/176. Myelotoxicity of vincristine-prednisone therapy in treatment of chronic myelogenous leukemia in blastic transformation.Therapy with vincristine and prednisone (VP) has produced remissions in 30% of patients with chronic myelogenous leukemia in blast transformation (CML-BT). The possibility that therapy with VP can adversely affect the production of mature granulocytes in this setting has not been appreciated, as these drugs are generally considered free of myelotoxicity. In this report we review eight courses of VP administered to three patients with CML-BT. Granulocytopenia developed following all five courses in which granulocyte counts were normal prior to therapy; granulocytopenia worsened in two of three courses in patients who were granulocytopenic prior to therapy. Progressive leukemia in the marrow was excluded as a cause of granulocytopenia. It is important to recognize that VP therapy rather than disease progression may be a cause of granulocytopenia in CML-BT.- - - - - - - - - - ranking = 8.1257984968218keywords = count (Clic here for more details about this article) |
10/176. Aplasia cutis congenita with precancerous transformation - the first case. Why do these scars never develop invasive tumors?The term aplasia cutis congenita characterizes a heterogeneous group of diseases which have in common a focal absence of the skin. The defect may be limited to the epidermis but often involves the full thickness of the skin including the underlying bone. At birth the lesions present as erosive patches and they heal rather rapidly with a residual scar. Although more than 200 publications on aplasia cutis congenita have appeared in the medical literature between 1966 and 1999, surprisingly no case of malignant degeneration has been described. We observed a 58-year-old male patient with aplasia cutis congenita who developed crusted changes within the scar over the past 10 years. Repeated biopsies over the years have always documented a precancerous lesion without solar elastosis. Invasion has never been observed in this patient. We hypothesize that for invasive malignancies dermal-epidermal interactions are necessary. Such a cell to cell communication seems to be impossible in patients with aplasia cutis congenita, as the dermal-epidermal unit is not developed. Aplasia cutis congenita might serve as an interesting model for further investigations on the importance of epidermal-dermal interactions.- - - - - - - - - - ranking = 0.012161480212108keywords = unit (Clic here for more details about this article) |
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