1/13. Autoimmune liver disease in patients with neoplastic diseases.BACKGROUND: Development of de novo autoimmune liver disease has not been well documented in patients with malignant diseases. methods/RESULTS: In this paper we report on a series of six patients with neoplastic disorders who acquired liver disease with autoimmune features. Five patients had suffered from haematological neoplasms and one from colonic cancer. In two patients, liver disease was detected at the time of presentation with malignancy. In the remaining four, all of whom were successfully treated for malignancies, features of liver disease presented at intervals 24-72 months after the cancer diagnosis. Twelve liver specimens (11 biopsies and one hepatectomy specimen) were obtained at time intervals of 1-76 months after initial presentation of neoplastic disease. Biopsies from three patients showed features of hepatitis (one acute, one sub-acute, one chronic). Two patients had histological features suggestive of an overlap syndrome (one autoimmune hepatitis/primary biliary cirrhosis, one autoimmune hepatitis/primary sclerosing cholangitis). The sixth patient had features of autoimmune cholangiopathy. All but one responded well to steroid therapy with complete clinical and biochemical remission obtained 4 weeks to 8 months after steroid introduction. We discuss briefly possible aetiologies of autoimmune liver disease in these patients. CONCLUSIONS: Autoimmune liver disease may be precipitated by therapy for neoplastic disease or malignant disease itself. The unusually heterogeneous clinicopathological findings in this group as well as the response to treatment support the concept of a wide spectrum of manifestations of autoimmune liver disease. The results may also suggest that autoimmune liver disease may be possibly added to the list of paraneoplastic syndromes. Further prospective studies are required to confirm a causal association and to determine whether the mechanisms involved are disease- or treatment-related.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/13. heart transplantation for radiation-associated end-stage heart failure.radiation-induced heart disease is an increasingly recognized late sequela of mediastinal radiation therapy for malignant neoplasms. We report four cases of heart transplantation for end-stage heart failure induced by mediastinal radiation therapy. Short-term and intermediate-term results are excellent with all four patients currently surviving a mean of 48 months after transplantation. Neither a second malignancy nor recurrence of the primary malignancy has been observed to date. The early results of heart transplantation for end-stage, radiation-induced heart disease are encouraging.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/13. Interstitial deletion of the short arm of chromosome 12 during clonal evolution in myelodysplastic syndrome with t(5;12)(q13;p13) involving the ETV6 gene.We report here a 65-year-old man with a myelodysplastic syndrome (MDS), refractory anemia with excess of blasts. He had received chemotherapy with tegafur for renal carcinoma. Chromosome analysis of bone marrow cells revealed complex karyotypes; del(5)(q13) was observed in all 20 metaphase spreads, and two related aberrations, add(12)(p11) and add(12)(p13), were detected in 13 and 7 cells, respectively. fluorescence in situ hybridization (FISH) analysis with chromosome-specific DNAs revealed that these alterations originated from a reciprocal translocation (5;12)(q13;p13). Therefore, del(5)(q13), add(12)(p11), and add(12)(p13) were revised as der(5)t(5;12)(q13;p13), der(12)del(12)(p11p13)t(5;12)(q13;p13), and der(12)t(5;12)(q13;p13), respectively. fluorescence in situ hybridization with a series of cosmid probes spanning the ETV6 gene showed that the 12p13 breakpoint on the der(12)t(5;12)(q13;p13) was located in intron 1, but the exon 1 signal was deleted. Our results suggest that a fusion gene was generated between the 5'-end of an unidentified partner at 5q13 and the 3'-end of ETV6 by t(5;12)(q13;p13), and that the interstitial deletion (12)(p11p13) occurred following t(5;12) during clonal evolution. del(12)(p11p13), including the rearranged ETV6 gene, may be implicated in the progression of MDS.- - - - - - - - - - ranking = 0.0064605899148084keywords = carcinoma (Clic here for more details about this article) |
4/13. Maternal pulmonary adenocarcinoma metastatic to the fetus: first recorded case report and literature review.We report the first maternal pulmonary adenocarcinoma metastatic to the fetus as well as an updated literature review. review of the literature revealed that there have been only 67 cases of maternal malignancy metastatic to the products of conception. These were mostly malignant melanoma and hematopoietic tumors. A 46-year old multiparous woman with metastatic pulmonary adenocarcinoma, diagnosed at 23 weeks gestation, delivered a male infant who appeared normal at birth. The mother died 2 days after delivery. The child developed multiple scalp tumors a 2 weeks of age. The tumors recurred rapidly after initial resection. Wide local excision of the involved scalp and skin graft coverage was performed at 14 weeks of age. Histopathology of these tumors was identical to that of the maternal tumor. The maternal origin of these tumors was confirmed by fluorescence in situ hybridization (FISH). The child is now 5 years old and free of disease.- - - - - - - - - - ranking = 0.038763539488851keywords = carcinoma (Clic here for more details about this article) |
5/13. skin manifestations in CD4 , CD56 malignancies.CD4 CD56 hematologic neoplasms were recently individualized. We report three cases of CD4 CD56 malignancies with cutaneous lesions in three cases and also bone marrow involvement in two cases. Two patients relapsed 2 and 3 months after polychemotherapy. Two patients died within 3-10 months. A constant immunophenotype was observed with the co-expression of CD4 and CD56, the absence of B and T-cell markers. The salient fact of this report is the presence of T-cell clonal rearrangement. The clinical and pathological features closely resemble the specific cutaneous manifestations in acute leukemia with monocytic differentiation, especially the granulocytic sarcoma. Because of the positivity of the CD56, natural killer cell proliferations were discussed. Since 1994, 50 cases of CD4 , CD56 cutaneous neoplasms have been reported with specific clinical, cytologic and immunohistochemical features. The diagnosis is more difficult when the cutaneous location is exclusive; on the contrary, the cytological features of the blood and medullar cells with cytoplasmic vacuoles and pseudopodia are characteristic of this hematologic neoplasm. The presence of CD123 antigen in most of the cases is an argument for a plasmacytoid dendritic cell proliferation and it is also a good marker for primary cutaneous lesions.- - - - - - - - - - ranking = 3keywords = neoplasm (Clic here for more details about this article) |
6/13. Treatment of patients with hematologic neoplasm, fever, and neutropenia.Choices of empirical antibiotic therapy for patients with febrile neutropenia must be made with very little information about the source and site of infection. The clinician is aided by recognition of the subtle signs and symptoms of infection in immunocompromised patients. National guidelines should be applied according to the microbiological patterns and trends in drug resistance at each institution. Case studies are provided to illustrate these challenges in daily practice.- - - - - - - - - - ranking = 4keywords = neoplasm (Clic here for more details about this article) |
7/13. osteonecrosis of the jaws in periodontal patients with a history of bisphosphonates treatment.BACKGROUND/AIM: osteonecrosis of the jaws is being increasingly reported in patients with bone metastasis from a variety of solid tumours and disseminated multiple myeloma receiving intra-venous bisphosphonates. The signs and symptoms that may occur before the appearance of clinical evident osteonecrosis include changes in the health of periodontal tissues, non-healing mucosal ulcers, loose teeth and unexplained soft-tissue infection. A series of nine periodontally involving patients showing osteonecrosis of the jaws that appeared following the intra-venous use of bisphosphonates is reported. MATERIAL AND methods: Nine consecutive patients with osteonecrosis of the jaws were prospectically studied. patients' past medical histories and the drugs that they had received for their malignant disease were systematically documented. Clinical, histopathological and radiographic features and proposal for treatment modalities of osteonecrosis are also reported. RESULTS: Of the nine patients (six women and three men) observed, all had osteonecrosis in the mandible; two had maxillary involvement as well. All nine patients had a history of extraction of periodontally hopeless teeth preceding the onset of osteonecrosis. In two patients, the lesions also appeared in edentulous areas spontaneously. All the patients had received intra-venous bisphosphonates as treatment for their disseminated haematological neoplasms or metastatic bone disease. The duration of bisphosphonate therapy at presentation ranged from 10 to 70 months (median: 33 months). CONCLUSIONS: jaw osteonecrosis appears to be associated with the intra-venous use of bisphosphonates. Dental professionals should be aware of this potentially serious complication in periodontal patients receiving long-term treatment with bisphosphonates.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/13. CD4 /CD56 haematodermic neoplasm: a preculsor haematological neoplasm that frequently first presents in the skin.CD4 /CD56 hematodermic neoplasm, formerly known as blastic NK cell lymphoma, is an aggressive and rare preculsor hematologic neoplasm recently recognized by the WHO-EORTC classification consensus for cutaneous lymphomas. The neoplasm tends to affect elderly patients, who usually present with skin lesions but often have a disseminated disease, including bone marrow involvement. Although the lesions are composed of cells with a lymphoblast-like morphology and an NK-cell phenotype, exhibiting a CD4 , CD56 positive immunophenotype, recent studies support a relationship to plasmacytoid dendritic cells. Because of the rarity of this disease, we describe two patients suffering a CD4 /CD56 hematodermic neoplasm.- - - - - - - - - - ranking = 12keywords = neoplasm (Clic here for more details about this article) |
9/13. Mass-forming extramedullary hematopoiesis diagnosed by fine-needle aspiration cytology.Extramedullary hematopoiesis (EMH) is usually a microscopic finding. However, it may present as a mass-forming lesion making it amenable to fine-needle aspiration biopsy (FNAB). When mass-forming EMH occurs, it can simulate a neoplasm clinically and radiologically. Additionally, the megakaryocytes can mimic malignant neoplastic cells, particularly if EMH is not a considered diagnosis. We report six cases of mass-forming EMH diagnosed by FNAB and evaluate the utility of FNAB in diagnosing EMH. Four patients had prior diagnoses of hematologic disorders, one patient had malignant mastocytosis who presented with lymphadenopathy and one patient had a history of carcinoma. The patients' ages ranged from 46 to 78 yr with an equal sex distribution. Aspirate smears showed trilineage hematopoiesis. The cytomorphologic differential diagnosis included metastatic carcinoma, Hodgkin lymphoma and myeloid sarcoma. No special stains were necessary due to the classic cytologic findings and prior hematologic history.- - - - - - - - - - ranking = 1.0129211798296keywords = neoplasm, carcinoma (Clic here for more details about this article) |
10/13. rhabdomyosarcoma presenting as acute hematologic malignancy: case report and review of the literature.The authors describe a case of undifferentiated rhabdomyosarcoma from unknown primary site, presenting as an acute hematologic malignancy with generalized lymphadenopathy, extensive bone marrow involvement and clinical and laboratory features of disseminated intravascular coagulation. Such a peculiar behaviour is known for rhabdomyosarcoma but is rare and can be a serious diagnostic problem for the clinician and the pathologist. The importance of a large spectrum immunohistochemistry as first diagnostic approach to any undifferentiated small-cell malignant tumor is stressed, together with the knowledge of the different immunoreactivity patterns. desmin, MS-actin and myoglobin are the most reliable markers of this type of myogenic sarcoma.- - - - - - - - - - ranking = 0.29534876297905keywords = undifferentiated (Clic here for more details about this article) |
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