1/89. 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 = progression (Clic here for more details about this article) |
2/89. 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 = 5.5074665902353keywords = disease progression, progression (Clic here for more details about this article) |
3/89. leiomyomatosis peritonealis disseminata: does malignant transformation occur? A literature review.leiomyomatosis peritonealis disseminata (LPD) is a rare smooth muscle tumor. In the literature more than 100 cases have been described. LPD is characterized by multiple small nodules on the peritoneal surface, mimicking a malignant process with metastases, but generally demonstrates benign histologic features. Exposure to estrogen seems to play an etiologic role. Many patients have uterine leiomyomas as well. The diagnosis of LPD is easily made on biopsy. Reduction of estrogen exposure is generally sufficient to cause regression of LPD. Surgical castration or gonadotrophin releasing hormone agonists seem good alternatives in the case of progression or recurrence of LPD. In six patients a malignant leiomyosarcoma has been described shortly after the diagnosis of LPD was made. Five of these patients did not have uterine leiomyomas or exposure to exogenous or increased endogenous estrogen. The relationship with pregnancy in the sixth patient may be coincidental. Whether malignant transformation of LPD occurs remains uncertain. Characteristics of these patients differ from those of LPD patients and may indicate a high malignant potential, necessitating a different approach.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
4/89. 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 = progression (Clic here for more details about this article) |
5/89. Conversion of multiple solid testicular teratoma metastases to fatty and cystic liver masses following chemotherapy: CT evidence of "maturation".Testicular germ cell tumour metastases may undergo "retroconversion" to mature differentiated teratoma following chemotherapy or irradiation. We report a patient with testicular germ cell liver metastases in whom computed tomography (CT) scans following chemotherapy demonstrated a reduction in CT attenuation of the liver lesions to that of cystic and fatty density. This is believed to represent CT evidence of liver metastasis "retroconversion", which offers the potential for non-invasive monitoring of histological progression.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
6/89. 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 = 5.5074665902353keywords = disease progression, progression (Clic here for more details about this article) |
7/89. Molecular features of a human rhabdomyosarcoma cell line with spontaneous metastatic progression.A novel human cell line was established from a primary botryoid rhabdomyosarcoma. reverse transcription polymerase chain reaction investigations of this cell line, called RUCH-2, demonstrated expression of the regulatory factors PAX3, Myf3 and Myf5. After 3.5 months in culture, cells underwent a crisis after which Myf3 and Myf5 could no longer be detected, whereas PAX3 expression remained constant over the entire period. karyotype analysis revealed breakpoints in regions similar to previously described alterations in primary rhabdomyosarcoma tumour samples. Interestingly, cells progressed to a metastatic phenotype, as observed by enhanced invasiveness in vitro and tumour growth in nude mice in vivo. On the molecular level, microarray analysis before and after progression identified extensive changes in the composition of the extracellular matrix. As expected, down-regulation of tissue inhibitors of metalloproteinases and up-regulation of matrix metalloproteinases were observed. Extensive down-regulation of several death receptors of the tumour necrosis factor family suggests that these cells might have an altered response to appropriate apoptotic stimuli. The RUCH-2 cell line represents a cellular model to study multistep tumorigenesis in human rhabdomyosarcoma, allowing molecular comparison of tumorigenic versus metastatic cancer cells.- - - - - - - - - - ranking = 5keywords = progression (Clic here for more details about this article) |
8/89. Blastic/blastoid transformation of follicular lymphoma: immunohistologic and molecular analyses of five cases.Progression of follicular lymphoma to a higher-grade malignancy frequently heralds a poor prognosis. Clinical transformation is variably accompanied by a spectrum of histologic changes characterized by alteration in growth and cytology. Although several cytogenetic events and potential oncogenes have been documented in this progression, the underlying molecular mechanisms are largely unknown. We present five patients with an unusual histologic transformation of follicular lymphoma manifested by blastic/blastoid morphology. This transformation is histologically distinct from other types of transformation of follicular lymphoma. All five cases exhibited the t(14;18) translocation and expressed the BCL-2 protein. In addition, two of the five patients showed increased levels of the p53 protein within neoplastic cells implicating a possible role for this oncogene in blastic/blastoid transformation. The lack of BCL-1 and myeloid antigens by immunohistochemistry and flow cytometry studies served to distinguish blastic/blastoid transformation of follicular lymphoma from its morphologic mimics. This distinction is clinically important because lymphoblastic and myeloid leukemias require significantly different therapeutic modalities and show better prognosis. Moreover, the lack of Epstein-Barr virus-specific mRNA suggests that this virus is unlikely to participate in blastic/blastoid transformation of follicular lymphoma.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
9/89. Spontaneous keratinocyte cell lines representing early and advanced stages of malignant transformation of the epidermis.A unique series of epidermal cell lines representing different stages of malignant transformation were spontaneously derived from a single adult immunosuppressed individual. Four keratinocyte lines (PM1-4) established from forehead skin are here compared with 4 squamous cell carcinoma (SCC) lines (MET1-4) derived respectively from a primary cutaneous tumour, two local recurrences and a distant metastasis of invasive SCC. Despite altered growth properties, the PM lines retained many features of normal keratinocytes including keratin phenotype, differentiation capacity and non-tumorigenicity in athymic mice. In contrast, from early passage, the MET lines displayed markedly reduced growth requirements, abnormal differentiation, aberrant K18 expression and tumorigenicity in athymic mice. The abnormal keratin profile of individual MET lines closely reflected the keratin phenotype of the tumour of origin. Although unusual HPV types were identified in the original tissue, there was no evidence of persistent virus within any cell line and it appears that HPV is not critical for maintenance of the immortal phenotype. The PM lines were distinctly different from invasive SCC lines and are likely to be useful for studies of mutations important early in neoplastic progression. The SCC series represent primary, recurrent and metastatic carcinoma. Availability of such a series from the same individual will facilitate genetic analysis of the malignant process.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
10/89. Malignant melanoma arising from unusual conjunctival blue nevus.Cellular blue nevus is an uncommon pigmented tumor in the conjunctiva, where it generally appears as a deep, circumscribed, pigmented conjunctival mass. We report a case of conjunctival blue nevus that clinically resembled primary acquired melanosis and gave rise to conjunctival melanoma. A 41-year-old man developed a diffuse pigmented mass in the inferior fornix of his left eye. Over a 20-year period, he noted slight progression of the pigment. Foci of epibulbar pigmentation were also present. The lesion resembled primary acquired melanosis. Excisional biopsy and adjuvant cryotherapy were performed. Histopathologic examination disclosed an intense infiltrate of heavily pigmented dendritic melanocytes with aggregates of less pigmented plump cells in the substantia propria. The conjunctival epithelium was normal. Malignant cellular features consistent with melanoma were observed in some foci. Cellular blue nevus of the conjunctiva can simulate primary acquired melanosis and can give rise to malignant melanoma. Arch Ophthalmol. 2000;118:1581-1584- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
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