1/42. De novo CD5 burkitt lymphoma/leukemia.CD5 is a T-cell marker aberrantly expressed in B-cell chronic lymphocytic leukemia and mantle cell lymphoma. Other B-cell neoplasms, including burkitt lymphoma, are usually CD5-. We report 4 cases of de novo CD5 burkitt lymphoma/leukemia in elderly patients, all of whom were in a leukemic phase and had variable lymph node and splenic involvement. The blasts were typically medium sized, with folded nuclei, distinct but not prominent nucleoli, and moderate amounts of somewhat vacuolated basophilic cytoplasm; they were terminal deoxynucleotidyl transferase--negative and surface immunoglobulin--positive. All 4 cases demonstrated c-myc rearrangement, but none had t(14;18), t(11;14), or cyclin d1 overexpression or rearrangement. Only 1 patient achieved complete remission after hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) therapy. One patient responded poorly to hyper-CVAD, and 2 patients died during induction chemotherapy. These rare cases of aggressive lymphoid malignancy with CD5 positivity and molecular features associated with burkitt lymphoma/leukemia are best classified as Burkitt leukemia. However, the morphologic and immunophenotypic similarity to the blastoid variant of mantle cell lymphoma are diagnostically challenging. The diseases can be distinguished at the genetic level, since burkitt lymphoma involves the rearrangement of c-myc, and mantle cell lymphoma usually the overexpression or rearrangement of cyclin d1.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/42. De novo acute B cell leukemia/lymphoma with t(14;18).The t(14;18)(q32;q21) translocation is the most common translocation in B cell malignancies being found in 80% of follicular lymphomas and about 20% of diffuse large B cell lymphomas. Only rare cases of de novo acute B cell lymphoblastic leukemia with t(14;18) have been described. We describe five cases of this entity which appears to have very homogeneous clinical, phenotypic and genotypic features. None of these patients had prior history of follicular lymphoma. The disease was characterized by acute clinical features with nodal and/or extranodal disease, massive bone marrow infiltration and rapid increase of circulating blast cells of mature B cell phenotype. All patients disclosed complex chromosomal and molecular abnormalities involving at least the BCL-2 and c-MYC genes. Furthermore, three patients had evidence of BCL-6 involvement and one patient had a p53 mutation. Despite intensive chemotherapy, including for two patients allogeneic bone marrow transplantation in first complete remission, all patients died within a few months. Neuro-meningeal relapse occurred in three of the five patients in spite of neuro-meningeal prophylaxis. De novo leukemia/lymphoma with t(14;18) is a rare entity with a very poor prognosis. Whether early bone marrow transplant could modify the natural history of the disease remains to be determined. An intensive neuro-meningeal prophylaxis appears to be mandatory in these patients.- - - - - - - - - - ranking = 0.078654029805231keywords = complex (Clic here for more details about this article) |
3/42. Detection of diagnostically critical, often hidden, anomalies in complex karyotypes of haematological disorders using multicolour fluorescence in situ hybridization.Multicolour fluorescence in situ hybridization (M-FISH) simultaneously detects all 24 human chromosomes in unique fluorescent colours. The identification of diagnostically critical gene rearrangement(s) in complex karyotypes of haematological disorders continues to be a challenge. We present five cases in which t(9;11), complex t(8;22), t(12;21) and t(11;14) were detected primarily using M-FISH and were confirmed using locus-specific probes. We conclude that M-FISH can be effective in complete characterization of critical gene rearrangements in haematological disorders.- - - - - - - - - - ranking = 0.47192417883138keywords = complex (Clic here for more details about this article) |
4/42. Ovarian non-Hodgkin's lymphoma: a clinicopathologic study of eight primary cases.Primary (localized) non-Hodgkin's lymphoma (NHL) of the ovary is rare. We studied eight cases of primary ovarian NHL to better understand the clinicopathologic and immunophenotypic features of these tumors. The patients ranged in age from 29 to 62 years (mean 47 years). Pelvic complaints were the most common symptoms; however, three of eight neoplasms were discovered incidentally. All tumors were unilateral and Ann Arbor stage I(E). The three incidental NHL were microscopic (largest 1.2 cm), whereas the grossly evident lesions ranged from 7.5 to 20 cm (mean 13.3). Each tumor was classified according to the world health organization classification as follows: diffuse large B-cell lymphoma (three cases), follicular lymphoma (two cases), burkitt lymphoma (one case), T-cell anaplastic large cell lymphoma (one case), and precursor T-lymphoblastic lymphoma (one case). Six tumors were of B-cell lineage, and two tumors were of T-cell lineage. All three diffuse large B-cell lymphomas were positive for BCL-6, two were positive for CD10, and two were positive for BCL-2. Estrogen and progesterone receptors were negative in all NHLs assessed. patients were treated by various combinations of surgery, chemotherapy, and radiotherapy. Clinical follow-up ranged from 1.3 to 11.7 years (mean 5.2) and all patients were alive without disease at last follow-up. We conclude that most patients with primary ovarian NHL present with symptoms attributable to an ovarian mass, but in a subset of patients ovarian NHL may be detected incidentally. With appropriate therapy, patients appear to have a favorable prognosis although follow-up is short for some patients in this study.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/42. Mature B-cell leukemias with more than 55% prolymphocytes: report of 2 cases with burkitt lymphoma-type chromosomal translocations involving c-myc.CONTEXT: The molecular genetic events involved in the pathogenesis of mature B-cell leukemias with more than 55% prolymphocytes are not well characterized. We have encountered 2 such cases in which conventional cytogenetic analysis identified burkitt lymphoma-type chromosomal translocations involving 8q24. OBJECTIVE: To assess these 2 cases for involvement of the c-myc gene using fluorescence in situ hybridization analysis with probes specific for the c-myc and immunoglobulin heavy-chain (IgH) genes. RESULTS: In both cases, conventional cytogenetic analysis demonstrated complex karyotypes, including chromosomal translocations involving 8q24. In case 1, a case of de novo prolymphocytic leukemia, the t(8;14)(q24;q32) was detected. In case 2, a case of chronic lymphocytic leukemia in prolymphocytoid transformation, the t(8;22)(q24;q11) was identified. fluorescence in situ hybridization studies showed c-myc/IgH fusion signals in case 1, proving the presence of the t(8;14). Split c-myc signals without fusion to IgH were observed in case 2, proving c-myc gene rearrangement and consistent with the t(8;22). CONCLUSION: These results suggest that c-myc gene alterations may be involved in the pathogenesis of a subset of mature B-cell leukemias with more than 55% prolymphocytes.- - - - - - - - - - ranking = 0.078654029805231keywords = complex (Clic here for more details about this article) |
6/42. Burkitt's lymphoma in the base of the tongue: differential diagnosis and management.Burkitt's lymphoma is the most common malignancy in African children but can occur sporadically in every country. It is one of the most aggressive malignancies in the human body, and in the past the prognosis was very poor. However, complex chemotherapy regimens can now cure approximately 50-80% of adult patients with Burkitt's lymphoma or small noncleaved lymphoma, and in pediatric populations the cure rate is even higher. Although the African type has a preference to the head and neck region (whereas the sporadic type to the abdomen), involvement of the base of the tongue is extremely rare as only 1 case has ever been reported in the English literature. The present study describes a patient with Burkitt's lymphoma presenting as a single mass in the base of the tongue without any abdominal or other extra-abdominal involvement. The patient was submitted to chemotherapy (intravenous and intrathecal) and skull radiotherapy. Today, 17 months after the diagnosis, the patient is disease free. physicians should be aware of the extranodal manifestations of Burkitt's lymphoma and their differential diagnosis in order to achieve early diagnosis and treatment.- - - - - - - - - - ranking = 0.078654029805231keywords = complex (Clic here for more details about this article) |
7/42. Interdisciplinary approach to abdominal Burkitt's lymphoma.Burkitt's lymphoma is a high-grade, rapidly growing B-cell neoplasm. It is recognized by its aggressive course, brief median survival, and low rates of long-term survival. The authors discuss the case of a patient who acutely presented with intraabdominal complications from a new onset of Burkitt's lymphoma. The clinical and pathological features, staging, treatment options, and survival data are reviewed. In addition, the role of surgical intervention is carefully analyzed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/42. Multiple AIDS-related malignancies just in the era of potent antiretroviral therapy. A rare but intriguing finding.Two exceedingly rare cases of dual AIDS-associated neoplasms (Kaposi's sarcoma and non-Hodgkin's lymphoma), occurring in a short time as AIDS-defining diseases of two hiv-infected patients treated with highly active antiretroviral therapy (HAART) are reported and discussed in light of evidence from the available literature. A slowly progressive increase in neoplastic complications following the introduction of HAART and the consequent decline of opportunistic infections such as the main AIDS-related disorders has been observed, and combined cancer diseases may be expected in the next few years, due to the persistent dysregulation of the immune system, or a possible involvement of oncoviruses and hiv itself in the pathogenesis of hiv-associated cancer.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/42. Extensive peritoneal and omental lymphomatosis with raised CA 125 mimicking carcinomatosis: CT and intraoperative findings.Diffuse peritoneal and omental seeding are well-known forms of dissemination of metastatic carcinoma. A wide variety of primary neoplasms may cause peritoneal and omental carcinomatosis, most commonly carcinomas of the ovary, gastrointestinal tract and breast. Extensive involvement of the peritoneal cavity with lymphoma is, however, rare. The association of peritoneal lymphoma with a raised CA 125, a tumour marker which is commonly raised in ovarian carcinoma, is a highly challenging clinical situation, which to our knowledge has not been published before in the medical literature. Not being aware of the possibility of this unusual combination of clinical, laboratory and imaging findings can lead to an erroneous diagnosis, as in our case.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/42. Pediatric tumors of the parapharyngeal space. Three case reports and a literature review.Three cases of pediatric primary parapharyngeal space tumors, namely a Burkitt's lymphoma, lipoblastoma and a ganglioneuroma are described here, along with a literature review. These disorders are very rare, accounting for less than 20% of all parapharyngeal neoplasms. In comparison to adults, there are differences in pathomorphologic spectrum of pediatric parapharyngeal tumors. The authors describe the prevalence of malignant tumors, mainly soft tissues sarcomas. The salivary gland tumors and paragangliomas which dominate in adult populations, are extremely rare in pediatric populations where the most common benign tumor to be encountered is that of neurogenic origin. It follows that these features modify the diagnostic and therapeutic approach.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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