1/258. Cutaneous waldenstrom macroglobulinemia in transformation.waldenstrom macroglobulinemia is a low-grade B-cell lymphoproliferative disorder of the elderly with characteristic monoclonal IgM-producing neoplastic infiltrates of the bone marrow, lymph node, and spleen. Cutaneous manifestations are usually nonspecific such as purpura, ulcers, and urticarial lesions. These lesions are caused by hyperviscosity of the blood, immune complex-mediated vascular damage, paraprotein deposition, and amyloid deposition. Specific skin lesions occur rarely and generally consist of translucent, flesh-colored papules composed of monoclonal IgM deposits. Rarely, there may be violaceous lesions composed of low-grade lymphoplasmacytic infiltrates characteristic of waldenstrom macroglobulinemia. Both cutaneous manifestations of the disease, as well as disease transformation to high-grade, large cell lymphoma are rare. We report two very unusual cases of waldenstrom macroglobulinemia with documented skin disease that demonstrated transformation to high-grade lymphoma. Both patients were elderly men with long-standing waldenstrom macroglobulinemia involving the bone marrow, who subsequently developed skin involvement by the disease. waldenstrom macroglobulinemia can rarely manifest as cutaneous disease, sometimes as a high-grade transformation of low-grade waldenstrom macroglobulinemia elsewhere. Distinction of cases of transformed waldenstrom macroglobulinemia from de novo cutaneous large cell lymphoma may be important, because the two entities are likely biologically different.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
2/258. Balloon cell change in cellular blue nevus.Balloon cells are altered melanocytes with clear vacuolated cytoplasm caused by a defect in the process of melanogenesis. Although rare, balloon cell change has been observed in a variety of melanocytic proliferations, particularly intradermal melanocytic nevi and melanoma. When present, such features may lead to difficulties in diagnosis, particularly with other clear cell neoplasms. We report an unusual case of the development of balloon cell change in a cellular blue nevus, a phenomenon that has not been extensively addressed in the literature. The importance of recognizing this change in cellular blue nevus to avoid misinterpreting the lesion as malignant is discussed.- - - - - - - - - - ranking = 2.5152744912025keywords = neoplasm (Clic here for more details about this article) |
3/258. The inv(11)(p15q22) chromosome translocation of therapy-related myelodysplasia with NUP98-DDX10 and DDX10-NUP98 fusion transcripts.Chromosomal abnormalities involving the 11p15 or 11q22-23 bands have been reported in several types of human neoplasms including hematopoietic malignancies. The abnormalities are observed in therapy-related malignancies and less frequently in de novo myeloid malignancies. Abnormality of the MLL gene located on chromosome 11q23 has been well known in therapy-related myeloid malignancies, but it has been reported only recently that the inv(11)(p15q22) in de novo or therapy-related myeloid malignancies results in the fusion of NUP98 on chromosome 11p15 and DDX10 on chromosome 11q22. NUP98 is a nucleoporin that composes the nuclear pore complex and is the target gene in leukemia with the t(7;11)(p15;p15). The DDX10 gene encodes a putative adenosine triphosphate-dependent DEAD box rna helicase. Here we present another patient with acute myelocytic leukemia (M4) transformed from chronic myelomonocytic leukemia with the inv(11) chromosome who had been treated with etoposide for a germ cell tumor. By reverse transcription polymerase chain reaction (RT-PCR) of the rna from the leukemic cells of the patient, DDX10-NUP98 and NUP98-DDX10 fusion transcripts were detected. Our case confirms that the inv(11) is a rare chromosomal translocation that is associated with therapy-related or de novo myeloid malignancy and involves NUP98 and DDX10 but not MLL. RT-PCR of the fusion transcripts might be applied to the detection of a small number of leukemic cells in the bone marrow or blood of patients in remission or in the cells harvested for autologous transplantation.- - - - - - - - - - ranking = 2.5152744912025keywords = neoplasm (Clic here for more details about this article) |
4/258. Combined modality treatment for malignant transformation of a benign ovarian teratoma.Malignant transformation of a mature teratoma is a rare event. patients often remain free of symptoms until the tumor burden makes the prognosis poor, due to pelvic and peritoneal metastases. We present a case of squamous cell carcinoma arising from a teratoma, with bowel and peritoneal invasion. The patient was treated by radical surgery followed by whole pelvic radiation and chemotherapy. This regimen, usually given for squamous cell tumors, such as cervical cancer, led to a 19-month persistent disease-free survival.- - - - - - - - - - ranking = 1.8071573838694keywords = cancer (Clic here for more details about this article) |
5/258. Squamous cell carcinoma arising in a Warthin's tumor.Warthin's tumor is a well-defined salivary gland neoplasm consisting of epithelial and lymphoid components. However, malignant transformation is extremely rare. Such a patient who developed squamous cell carcinoma within a Warthin's tumor of the parotid gland is described and possible pathogenesis is discussed.- - - - - - - - - - ranking = 2.5152744912025keywords = neoplasm (Clic here for more details about this article) |
6/258. Gastric adenocarcinoma associated with fundic gland polyps in a patient with attenuated familial adenomatous polyposis.Familial adenomatous polyposis (FAP) is a rare autosomal dominant precancerous condition of the colon caused by mutations in the adenomatous polyposis coli (APC) tumor suppressor gene. FAP is characterized by the appearance of innumerable adenomatous polyps throughout the large bowel. Fundic gland polyps are the most common gastric lesion in FAP. It is generally believed that fundic gland polyps have little or no potential for malignant transformation in the population at large, and only a few case reports describe the development of high grade dysplasia or gastric adenocarcinoma associated with diffuse fundic gland polyposis in patients with FAP. We report the second case of gastric adenocarcinoma intimately associated with fundic gland polyposis in a family with an attenuated form of FAP. The patient had undergone routine screening per current guidelines because of his known mutation in the APC gene. This suggests that malignant transformation of fundic gland polyps in patients with FAP occur more frequently than previously believed. Current screening recommendations may not be sufficient for patients with FAP or its attenuated forms.- - - - - - - - - - ranking = 1.8071573838694keywords = cancer (Clic here for more details about this article) |
7/258. Malignant transformation in non-irradiated juvenile laryngeal papillomatosis.A spontaneous cancer transformation of non-irradiated juvenile papillomas has been observed in a very small number of cases. We report on six more patients with juvenile laryngeal papillomatosis developing into a squamous-cell carcinoma. Three patients had childhood papillomatosis and three patients adult onsets of their papillomas. The average duration between onset of papillomatosis and cancer diagnosis was 33 years, while the average age at time of diagnosis of a laryngeal cancer was 50 years. All patients were cigarette smokers, but none of them had received prior irradiation. The typical histological picture of tumors showed an infiltrating keratinizing squamous carcinoma besides remaining benign papillomas within the larynx. Our study again illustrates the risk of malignant transformation of juvenile papillomatosis in long-lasting cases. Therefore, regular extensive biopsies and careful histopathological examination are required. The role of smoking as a co-factor in the development of carcinoma ex-papilloma is still not clear.- - - - - - - - - - ranking = 5.4214721516083keywords = cancer (Clic here for more details about this article) |
8/258. Case report: adenocarcinoma arising in a Crohn's stricture of the jejunum.patients with Crohn's disease affecting the small intestine appear to have an increased risk of developing adenocarcinoma. However, it remains an uncommon complication of an uncommon disease. The diagnosis is difficult to make both pre- and intra-operatively, and is most commonly made postoperatively on histopathology. Hence, at laparotomy, consideration should be given to performing a frozen section on all small bowel strictures due to Crohn's disease to define the presence of dysplasia or cancer. This will assist the surgeon in deciding whether to perform a stricturoplasty or a resection.- - - - - - - - - - ranking = 1.8071573838694keywords = cancer (Clic here for more details about this article) |
9/258. Classical Hodgkin's disease and follicular lymphoma originating from the same germinal center B cell.PURPOSE: Classical Hodgkin's disease and non-Hodgkin's B-cell lymphoma occasionally occur in the same patient. To clarify whether these different diseases share a common precursor cell, we analyzed the immunoglobulin rearrangements in tumor cells of the classical Hodgkin's disease and the follicular lymphoma that developed in the same patient 2 years apart. patients AND methods: polymerase chain reaction (PCR) for the detection of rearranged immunoglobulin genes was carried out on single reed-sternberg cells and on whole tissue dna extracted from the follicular lymphoma. PCR products were sequenced and compared with each other and with germ line immunoglobulin variable segments. Immunoglobulin heavy- and light-chain transcripts were analyzed by radioactive in-situ hybridization. RESULTS: The same monoclonal immunoglobulin gene rearrangement was found in both neoplasms. The variable region of the immunoglobulin heavy-chain genes of the Reed-Sternberg and of the follicular lymphoma cells were differently mutated, but six somatic mutations were shared by both lymphoma cells. Although the coding capacity of the immunoglobulin genes was preserved in both neoplastic cell populations, immunoglobulin heavy- (mu) and light- (kappa) chain expression was restricted to the follicular lymphoma cells, except for small amounts of kappa light-chain mRNA in some reed-sternberg cells. CONCLUSIONS: The neoplastic cells of the Hodgkin's disease and the follicular lymphoma that occurred in this patient derived from a common precursor B cell. Its differentiation stage could be identified as that of a germinal center B cell. Thus, transforming events can be more important than the cell of origin in determining a disease entity.- - - - - - - - - - ranking = 2.5152744912025keywords = neoplasm (Clic here for more details about this article) |
10/258. Blastic crisis in previously clinically silent chronic myelogenous leukemia.A patient is described in whom CML first presented as blastic crisis. The diagnosis of CML was based upon the findings of Ph(1) chromosome in the bone marrow, basophilia in the peripheral blood, absence of NAP activity in the leukocytes, elevated serum vitamin B(12) and an enlarged firm spleen. CML with blastic crisis as its first expression is relatively rare, as compared to CML in which blastic crisis appears as a phase of prolonged clinically manifest disease.- - - - - - - - - - ranking = 1keywords = spleen (Clic here for more details about this article) |
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