Burkitt''s lymphoma: atypical localization

A case of small, borderline malignant biliary mucinous cystic tumor is presented. The patient initially presented to us 18 months earlier to undergo a sigmoid colon resection for sigmoid colon cancer. At that time, a liver cyst measuring 18 x 12 mm was detected. On a follow-up abdominal ultrasonography study for colon cancer, the liver cyst had enlarged to 21 mm in diameter and contained a fluid-fluid level 18 months after surgery. Histological examination of a needle biopsy specimen indicated possible adenocarcinoma. Lateral segmentectomy of the liver was performed. Histopathologically, the tumor was diagnosed as a mucinous cystic tumor, of border line malignancy, which had originated from a bile duct gland. It contained both mucinous and serous components, which were thought to have caused the formation of a fluid-fluid level within the cyst. In this case, the fluid-fluid level demonstrated by ultrasonography was beneficial in the early detection of a cystic tumor of the liver. This case may be the smallest reported cystadenocarcinoma of the liver yet published.     

Breast involvement in non-African Burkitt''s lymphoma

A patient with non-African Burkitt's lymphoma is reported in whom bilateral breast involvement was detected by both mammography and 67-Ga-citrate scintigraphy. The causes of breast uptake of 67Ga-citrate are reviewed, and it is noted that not only asymmetrical increases in uptake of 6767Ga-citrate are indicative of disease.     

A translocation t(8;14) and c-myc gene rearrangement associated with the histological transformation of B-cell acute lymphocytic leukemia (FAB-L2) into Burkitt''s type (FAB-L3) leukemia

During the late rainy season and winter season in 1990, outbreaks of suspected trypanosomiasis in native cattle (Bos indicus) occurred on 13 farms in Petchaboon province, Thailand. Forty-two cattle presented with nervous symptoms including circling, excitation, jumping, aggressive behavior, lateral recumbency, convulsion and finally death. Blood samples from 39 cattle on the two farms in which the outbreaks occurred were collected and examined for the presence of Trypanosoma evansi. It was found that all 16 blood samples from cattle on farm A were positive of T. evansi by mouse inoculation and indirect fluorescent antibody test (IFAT). In cattle from farm B, on the other hand only 37.5% and 39% of the samples were positive by mouse inoculation and IFAT, respectively. T. evansi was detected on impression smears of organs from the three cattle which died with nervous symptoms and also in smears made from their cerebrospinal fluid. In addition, trypanosomes were isolated from the cerebrum, cerebellum, pons and spinal cord by mouse inoculation.     

An aggressive case of Burkitt's lymphoma with t(8;14) and c-myc rearrangement transformed from CD5+ B-cell lymphoma

We experienced a case of Burkitt's lymphoma showing an unusual surface phenotype, CD5 expression, at an early stage of the disease. Initially, this patient showed massive abdominal para-aortic lymph node swelling which rapidly developed into leukemic change. Based on the clinical course and cytogenetic features of lymphoblasts in the bone marrow, which showed t(8;14) and c-myc gene rearrangement, the patient was diagnosed with Burkitt's lymphoma. Combination chemotherapy induced short-term remission, but central nervous system (CNS) involvement developed, followed by a regrowth of lymphoma cells in the bone marrow. The bone marrow at the end stage showed monotonous expansion of large cells with conspicuous vacuolation in the basophilic cytoplasm. The initial lymphoma cells showed pan-B markers and were CD5 positive but weakly CD10 positive; however, the lymphoma cells obtained from the bone marrow at the terminal stage did not express CD5. The chromosomal t(8;14) was seen, and identical rearrangement of immunoglobulin heavy chain joining gene and c-myc gene were detected by Southern blot analysis in the bone marrow lymphoblasts throughout the clinical course. This case is evidence that remarkable transformation of CD5-positive lymphoblasts to CD5-negative lymphoblasts occurred in an identical clone of Burkitt's lymphoma.     

BCL6 gene rearrangement and other cytogenetic abnormalities in diffuse large cell lymphoma

Evidence for rearrangement of the BCL6 gene at 3q27 has been documented in 20-30% diffuse lymphomas with a large cell component (DLLC), and was found to be of prognostic significance at the time of diagnosis. To incorporate these observations into current cytogenetic and clinical prognostic models, 76 cases of DLLC with known BCL6 status were analyzed. Cytogenetic indicators of progression, including trisomy 7, trisomy 12, del(6)(q21q25), and structural alterations of 17p were less frequent in BCL6 rearranged DLLC compared to BCL6 germline tumors. Despite a 93% overall survival at median follow-up of 30 months, a trend for continued relapse resulted in a projected freedom from progression for the BCL6 rearranged cohort of 66% at 4 years, compared to 39% for the BCL6 germline cohort. Six cases among the BCL6 rearranged group lacked additional cytogenetic indicators of progression and remained free of disease at follow-up in excess of 7 years, whereas BCL6 rearranged cases with increasing numbers of cytogenetic aberrations showed decreased intervals free from progression of disease. These results suggest that BCL6 rearrangement should be combined with other known clinical and cytogenetic indicators in prognostic analyses of patients with DLLC.     

Primary low-grade, B-cell, mucosa-associated lymphoid tissue lymphoma presenting as lymphomatous polyposis

This study explored perspective taking in social phobics' images of past anxiety-provoking social situations and non-social situations. For images of social situations, social phobics were significantly more likely than non-patient controls to take an observer perspective, viewing themselves as if from an external point of view. In contrast, social phobics and controls did not differ in the perspective taken in images of non-social situations, which were generally viewed from a field perspective (seeing the situation as if looking out through their own eyes). The clinical significance of these findings is briefly discussed.     

A study of the association of Epstein-Barr virus with Burkitt''s lymphoma occurring in a Chinese population

Several (carbamoylalkenyl)- and (carbamoylalkenyl)phenyloxy carboxylic acids (Table 1) and some of their ethyl esters (Table 2) were synthesized and evaluated in vitro as inhibitors of steroid 5 alpha-reductase. Inhibitors of this enzyme may be useful in treating dihydrotestosterone-related diseases such as prostate cancer and benign prostatic hyperplasia. Using an enzyme preparation obtained from human prostate carcinoma tissue, the inhibition values ranged from 0 to 57% at the given dose of 100 microM. In the series of free acids, surprisingly, the compounds showed only modest inhibitory potency (0-26%). By contrast, the ethyl esters displayed inhibition values up to 57%. Structure-activity relationships are discussed.     

Primary intraocular lymphoma

Primary intraocular lymphoma can arise as an isolated clinical entity or in combination with lymphomas in the central nervous system. The symptoms are usually floaters in the visual field and reduced visual acuity. The condition is commonly misdiagnosed as chronic uveitis in spite of unresponsiveness to corticosteroids. The authors discuss a case of primary intraocular lymphoma diagnosed in a 65 year old male patient. He had bilateral intraocular lesions in addition to two brain tumours. He went into complete remission after radiation therapy, but experienced later a recurrence in one eye. A course of chemotherapy using cytarabin (cytosine arabinoside) intravenously has kept the patient in remission for four months. The authors also discuss the diagnostic and therapeutic problems connected with this rare condition.     

Primary colorectal lymphoma

OBJECT: To analyze our experience in the treatment of primary colorectal lymphomas. PATIENTS: During the recent period from 1989 to 1995, a total number of 406 cases of malignant neoplasms of large intestine were treated at Department of Surgery, Hospital del S.A.S de Jerez de la Frontera. There were 5 cases of primary colorectal Non-Hodgkin's lymphomas, constituting 1.2% of all colorectal malignancies. METHODS: Medical records of all five patients were carefully reviewed. Surgical pathology specimens were retrospectively reexamined to confirm the diagnosis, and immunohistochemistry staining methods were performed. Dawson, Cornes and Morson criteria were used to distinguish primary colonic lymphoma. For tumor staging, a modification of the Ann-Arbor system for gastrointestinal lymphoma proposed by Musshoff and Schmidt-Vollmer was used. RESULTS: All cases were Non-Hodgkin's lymphomas. There were no cases of lymphoma of the rectum. There were 2 males and 3 females. Their ages ranged from 12 to 73 years, with an average age of 31 years. The cecum was the site of the lesions in 4 patients and the other was located in the sigmoid colon. Acute abdomen in three cases, undiagnosed abdominal mass in one and intussusception in the other one were the clinical presentation. Inmunohistochemical studies showed B-cell phenotype in 4 and non-B non-T Ki-1-positive phenotype in 1 patient. Chemotherapy was administered following resection in 100% of patients. CONCLUSIONS: Intraoperative diagnosis of lymphoma was not suspected in all cases but the malignancy was always present. The cecum is the commonest site of involvement. Both of our two patients presented with perforated lymphoma were associated with a poor short term prognosis. It is necessary to unify the staging system and the histologic classifications enabling a comparison of several studies.     

Primary laryngeal lymphoma

We report a new case of supraglottic primary laryngeal lymphoma. Fewer than 30 cases have been reported in the English-language literature. The absence of any other lymphoma site was confirmed by post-mortem study.     

Successful treatment with high dose chemotherapy following peripheral blood stem cell transplantation for relapsed Burkitt''s lymphoma with central nervous system involvement

BACKGROUND: The family of uncoupling proteins is thought to play an important role in the regulation of energy metabolism by uncoupling the respiratory chain reactions from ATP synthesis. The recently discovered uncoupling protein 2 (UCP2) is upregulated in genetically obese rodent models and during long term high fat feeding. AIM: We have examined the UCP2 mRNA levels in liver, heart and white adipose tissue (WAT) of obese ventromedial hypothalamus (VMH)-lesioned rats, during the dynamic and the early stage of the static phase of obesity, before the appearance of most of the metabolic perturbations associated with long term established obesity. RESULTS: The amount of UCP2 mRNA was not increased in any tissue of VMH-lesioned rats relative to control animals during the dynamic phase nor during the early static phase of obesity. CONCLUSION: These results indicate that in the rat, obesity does not necessarily lead to an increase in UCP2 expression and suggest that the up-regulation of UCP2 described in other models may be secondary to metabolic perturbations, rather than to a direct adaptative response to the increased adipose tissue content of the organism.     

Intravascular lymphoma presenting as symmetric polyarthritis

Intravascular lymphoma (IVL) is an uncommon neoplastic disorder characterized by monoclonal intravascular expansion of lymphoid cells. Occlusion of small vessels in various organ systems probably accounts for the broad clinical spectrum of this type of lymphoma, which can closely mimic a variety of diseases, especially vasculitic disorders, and thus lead to delayed clinical diagnosis. This is the first report of a patient who presented with a predominant symptom of symmetric polyarthritis accompanied by fever. While her initial systemic symptoms, such as fever, improved rapidly after initiation of corticosteroid therapy, the response of the polyarthritic joint manifestations was only transient. The patient died of progressive lung involvement and was diagnosed as having IVL by histologic analysis of tissue samples obtained postmortem.     

Primary HIV-1 infection presenting with transient neurological deficit

An apocrine hidrocystoma occurring on the finger of a 55-year-old man is reported. The lesion presented as a solitary, painless cystic nodule on the dorsal aspect of the middle phalanx of the index finger. Apocrine hidrocystoma is a benign tumour developed from apocrine sweat glands, but the location of the lesion was, however, not consistent with reported locations of apocrine glands in the adult.     

Primary gastric tuberculosis

Intestinal tuberculosis (TB) comprises 5% of all cases of TB and may be a major problem in immigrant communities, although the incidence of pulmonary TB is declining. Gastric TB is rare, constituting 0.1-2% of all cases of TB. Gastric TB usually develops secondary to other tuberculous lesions, most commonly pulmonary. On endoscopy antral infiltrative lesions are found. Primary gastric TB is very rare, only 8 cases having been reported in the English literature. We report a case of primary gastric TB in a 55-year-old woman who presented with abdominal pain and gastric outlet obstruction. The diagnosis was confirmed by endoscopic biopsies which showed granulomas, but no acid-fast bacilli. The Mantoux test was positive, acid-fast bacilli were found in the gastric juice, and a positive culture for TB was obtained on gastric lavage. There was an excellent response to antituberculous chemotherapy. With the relative rate of extra-pulmonary TB increasing, primary gastric TB should be taken into account in the differential diagnosis of infiltrative lesions of the antrum.     

Prognostic significance of Bcl-2 protein expression and Bcl-2 gene rearrangement in diffuse aggressive non-Hodgkin's lymphoma

The prognostic significance of Bcl-2 protein expression and bcl-2 gene rearrangement in diffuse large cell lymphomas (DLCL) is controversial. Bcl-2 protein expression prevents apoptosis and may have an important role in clinical drug resistance. The presence of a bcl-2 gene rearrangement in de novo DLCL suggests a possible follicle center cell origin and perhaps a distinct clinical behavior more akin to low-grade non-Hodgkin's lymphoma (NHL). The purpose of this study was to determine the impact of Bcl-2 protein expression and bcl-2 gene rearrangement (mbr and mcr) on survival of a cohort of patients with DLCL who were uniformly evaluated and treated with effective chemotherapy. Patients included the original MACOP-B cohort (n = 121) and the initial 18 patients treated with the VACOP-B regimen (total = 139). All patients had advanced-stage disease, were 16 to 70 years old, and corresponded to Working Formulation categories F, G, or H. No patients had prior treatment, discordant lymphoma, or human immunodeficiency virus seropositivity. Paraffin sections from diagnostic biopsies were analyzed for bcl-2 gene rearrangement including mbr and mcr breakpoints by polymerase chain reaction and Bcl-2 protein expression by immunohistochemistry. With a median follow-up of 81 months, overall (OS), disease-free (DFS), and relapse-free survival (RFS) were measured to determine the prognostic significance of these parameters. Analyzable DNA was present in 118 of 139 (85%) cases, with 14 demonstrating a bcl-2 rearrangement (11 mbr, 3 mcr). All 14 of these bcl-2 gene rearrangement-positive cases were found in the 102 patients with a B-cell immunophenotype, but the presence of this rearrangement had no significant influence on survival. Bcl-2 protein expression was interpretable in 116 of 139 (83%) cases, with immunopositivity detected in 54 of 116 (47%). Using a cut-off of greater than 10% Bcl-2 immunopositive tumor cells for analysis, positive Bcl-2 protein expression was seen in 28 of 116 (24%) patients and the presence of this expression correlated with decreased 8-year OS (34% v 60%, P < .01), DFS (32% v 66%, P < .001), and RFS (25% v 59%, P < .001). Bcl-2 protein expression remained significant in multivariate analysis that included the clinical international prognostic index factors and immunophenotype (P < .02). In conclusion, although bcl-2 gene rearrangement status could not be shown to have an impact on outcome, Bcl-2 protein expression is a strong significant predictor of OS, DFS, and RFS in DLCLs.