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1.
PURPOSE: Extranodal marginal zone B-cell lymphoma (low-grade B-cell lymphoma of mucosa-associated lymphoid tissue [MALT] type) is a distinctive type of lymphoma that usually arises in association with mucosa or other epithelial structures and has an indolent clinical course. The frequency and clinical features of MALT lymphomas in the ocular adnexa have not been well studied. METHODS: The authors examined the clinicopathologic features of ocular adnexal lymphoma, identified a subset of cases with MALT characteristics, and determined patient outcome. RESULTS: The 42 patients, 16 men and 26 women age 35-89 years (mean, 64) were followed an average of 4.8 years. Thirty-two patients had ocular adnexal involvement at presentation (primary ocular adnexal lymphoma) and 10 had a history of lymphoma that relapsed in the orbit (secondary ocular adnexal lymphoma). In the primary group, 23 patients had lymphoma confined to the ocular adnexa, 3 had a single lesion that invaded adjacent structures, and 6 had distant spread at the time of presentation. Twenty-five patients achieved a complete remission. Nine patients, including 6 patients whose disease was localized initially, had progression or relapse of disease in distant sites. At last follow-up, 21 patients were free of disease, 9 were alive with disease and 2 had died of lymphoma. In the secondary group, at last follow-up, 1 patient had died of other causes, free of lymphoma, 3 patients were alive with disease and 5 had died of lymphoma (outcome not known in 1 case). Using the recently described revised European-American lymphoma classification, we found 16 MALT lymphomas, 8 diffuse large B cell, 12 follicular center, 3 mantle cell, 1 B-small lymphocytic lymphoma, and 2 unclassifiable low-grade lymphomas. The most common type of primary lymphoma was MALT type (15 of 30 classifiable cases), and the most common secondary lymphoma was follicular center (6 of 10). No increased frequency of conjunctival or lacrimal gland involvement by MALT lymphomas was found. All 33 lymphomas with immunophenotyping were of B lineage. CONCLUSIONS: Ocular adnexal lymphomas are B-cell tumors that develop in older adults, predominantly among women. Primary orbital lymphomas have a favorable prognosis; a high proportion of them have MALT characteristics.  相似文献   

2.
An analysis was made of the incidence of various intrathoracic abnormalities noted on plain chest radiographs and tomograms in a consecutive series of 300 patients with untreated Hodgkin's disease and nonHodgkin's lymphoma. Those with Hodgkin's disease have a higher incidence of intrathoracic disease at presentation than those with non-Hodgkin's lymphoma (67% vs. 43%). Bulky superior mediastinal lymphadenopathy is the hallmark of Hodgkin's disease. Lung involvement was more common in Hodgkin's disease (11.6% vs. 3.7%) and was always accompanied by mediastinal and/or hilar lymphadenopathy.  相似文献   

3.
纵隔灰区淋巴瘤(mediastinal gray zone lymphoma)作为一个疾病实体,常不能依据现有的诊断标准进行分类.这类淋巴瘤同时具有纵隔弥漫大B细胞淋巴瘤(PMBL)和经典霍奇金淋巴瘤(cHL)的特征.在2008年WHO造血与淋巴组织肿瘤分类中将其命名为"B细胞淋巴瘤,不能分类,具有介于弥漫大B细胞淋巴瘤和经典霍奇金淋巴瘤之间的特征(BCLu)".BCLu具有独特的临床特点、免疫表型和分子遗传学特征,临床过程更具侵袭性,预后较差.目前尚无达成共识的治疗方案,可参照侵袭性B细胞淋巴瘤的方案化疗.  相似文献   

4.
BACKGROUND: Children with large anterior mediastinal masses frequently present with severe respiratory compromise and often pose a difficult diagnostic dilemma. A biopsy is preferred for diagnosis before treatment can begin; however, many of these children are at risk of acute clinical deterioration and cardiovascular arrest with the induction of anesthesia. The authors noted a correlation between pleural effusions and lymphoblastic lymphoma and recently diagnosed three cases of lymphoblastic lymphoma in children with a large anterior mediastinal mass and pleural effusion through cytological and flow cytometric examination of the pleural fluid. METHODS: To focus on this problem, 101 pediatric patients presenting with an anterior mediastinal mass between January 1980 and September 1994 were reviewed to determine if pleural effusions occur more frequently at initial presentation with lymphoblastic lymphoma than with Hodgkin's disease, thus offering a means of diagnosis in children with severe respiratory compromise. The patients' chest radiographs and/or computed tomograms for the 88 cases in which they were available were reviewed retrospectively in a blinded fashion to identify those children with pleural effusions at the time of presentation. RESULTS: In this study, 71% of patients with lymphoblastic lymphoma (10 of 14) had a pleural effusion at presentation, whereas only 11.7% of patients with Hodgkin's disease (7 of 60) had a pleural effusion on initial presentation. (P < .002 Fisher's Exact test). CONCLUSION: This retrospective review suggests that there is a significantly greater association of pleural effusions in patients with lymphoblastic lymphoma than with Hodgkin's disease. Our experience supports the conclusion that thoracentesis may provide a means of diagnosis in children presenting in severe respiratory compromise obviating the need for anesthesia and open biopsy.  相似文献   

5.
Primary adrenal lymphoma is a rare entity, with only 16 cases reported in the last 40 yr. Although 67Ga scintigraphy has been extensively used to evaluate patients with other types of lymphomas, there are no reports of its use in patients with this disease entity. A man with primary adrenal lymphoma and no evidence of extraadrenal spread who was evaluated from presentation to remission with gallium scintigraphy and CT is presented. Gallium scintigraphy was valuable in assessing response to therapy.  相似文献   

6.
PURPOSE: To evaluate the clinical features of presentation and the response to two different third-generation regimens (F-MACHOP and MACOP-B) of primary mediastinal large B-cell lymphoma (MLBCL), a recently defined distinct clinicopathological entity of non-Hodgkin's lymphoma (NHL). PATIENTS AND METHODS: Thirty-seven consecutive patients with MLBCL, eight male and 29 female (F/M ratio 1:3.5) with a median age of 35 years, were enrolled in the present study. Thirty-five (94.5%) patients presented disease confined to thorax, with chest symptoms of a rapidly enlarging mass in the mediastinum in 70% and superior vena cava syndrome (SCVS) in 43% of these patients. The first 10 patients received F-MACHOP and the succeeding 27 patients MACOP-B chemotherapy, associated in 24 (88.8%) with involved field radiation therapy (IFRT). 67Gallium scan was routinely performed pre- and post-IFRT in 18 patients. RESULTS: All 37 patients were assessable for response: 10 of 10 (100%) in the F-MACHOP and 26 of 27 (96.3%) in the MACOP-B group achieved overall responses (CR + PR). Three of 24 (12.5%) patients in PR after chemotherapy obtained CR after IFRT. Persistent Gallium avidity was observed in 16 patients after chemotherapy and in only four patients after IFRT. Thus far, four of the 10 F-MACHOP and two of the 26 MACOP-B responders have presented disease progression. The probability of progression-free survival (PFS) was 91% and 60% (P < 0.02) while overall survival (OS) was 93% and 70% (P = n.s.) at a mean follow-up of 27 and 52 months in the MACOP-B + IFRT and F-MACHOP groups, respectively. CONCLUSION: MACOP-B + IFRT has proved to be a highly effective and less toxic therapeutic approach for primary MLBCL and appears to be superior to other third-generation chemotherapy regimens.  相似文献   

7.
This study retrospectively reviewed the findings in laparoscopic biopsy specimens from 51 consecutive patients with suspected abdominal lymphoproliferative disorders. Histologic evaluation was supplemented (as necessary) by paraffin-section or frozen-section immunohistochemical analysis or by Southern blot hybridization. The laparoscopic procedure was diagnostic of a lymphoproliferative disorder in 24 patients (47%), of other neoplasms in 5 patients (10%), and of reactive tissue in 11 patients (22%); no tissue could be obtained for technical reasons (adhesions and inaccessible lesions) in 11 patients (22%). The 24 patients with lymphoproliferative disorders diagnosed by laparoscopic techniques included 14 patients with a new diagnosis of lymphoma and 10 patients with recurrent disease; pathologic findings were diagnostic of diffuse large cell lymphoma (11 patients), follicular lymphoma (11 patients), chronic lymphocytic leukemia (1 patient), and lymphocyte-predominant Hodgkin's disease (1 patient). Previous abdominal cytologic or core-needle biopsy specimens from 11 lymphoma patients did not yield an unequivocal diagnosis or subtype of lymphoma. The 11 patients (22%) in whom laparoscopic techniques did not produce a tissue sample needed laparotomy (10 patients) or femoral lymph node biopsy (1 patient) to document the diagnosis of large cell lymphoma (2 patients), follicular lymphoma (5 patients), composite lymphoma (1 patient), myeloma (1 patient), neurofibroma (1 patient), and reactive lymph nodes (1 patient). In the majority of patients with suspected abdominal lymphoma, laparoscopic techniques provide sufficient tissue for the diagnosis and classification of lymphoma and for the diagnosis of other causes of abdominal lymphadenopathy.  相似文献   

8.
BACKGROUND AND DESIGN: Primary cutaneous follicular center cell lymphomas represent a distinct type of cutaneous B-cell lymphoma, clinically characterized by localized skin lesions on the head or trunk and an excellent prognosis. Histologically similar lymphomas may occur on the legs. The clinical behavior of this group is still undefined, and controversy exists whether these lymphomas should be classified as follicular center cell lymphoma or B-immunoblastic lymphoma. We reviewed the clinical, histologic, and follow-up data of 18 patients with primary cutaneous large B-cell lymphoma of the legs. RESULTS: Primary cutaneous large B-cell lymphoma of the legs generally occurred in elderly patients (median age at diagnosis, 76 years), in particular women (male-female ratio, 7:2), and preferentially affected the lower legs (14 of 18 patients). Radiotherapy and/or systemic polychemotherapy resulted in complete remissions in 16 of 17 patients. Follow-up data demonstrated estimated 2- and 5-year survival rates of 77% and 58%, respectively. Histologic evaluation showed diffuse dermal infiltrates with variable proportions of centroblasts (large noncleaved cells), large centrocytes (large cleaved cells), and B immunoblasts. Seventeen of 18 patients were diagnosed as having primary cutaneous follicular center cell lymphoma; only 1 patient, whose histologic examination showed more than 30% immunoblasts, was diagnosed as having B-immunoblastic lymphoma. CONCLUSIONS: Primary cutaneous large B-cell lymphoma of the legs is a distinct clinicopathologic entity that mainly affects elderly patients and has an intermediate prognosis. Although most cases have a follicular center cell origin, primary cutaneous large B-cell lymphoma is proposed as the most appropriate term for this type of cutaneous lymphoma.  相似文献   

9.
We describe the radiological findings at presentation and follow up in 24 adult patients with Burkitt's and Burkitt-like lymphoma, age range 17-67 years. This is an older age group than previously described in North American series, but the clinical and imaging characteristics appear similar. Disease confined to the abdomen was seen in 12 (50%) at presentation, of whom 11 had bowel or mesenteric tumours. Of those with bowel involvement, five patients had disease that had arisen in the ileocaecal region. Intra-abdominal disease was large volume (greater than 5 cm) in the majority of patients. Four patients (17%) had small isolated masses in the head and neck region, two (8%) had isolated unilateral axillary lymphadenopathy. Six (25%) patients presented with disseminated disease, all with hepatic and/or splenic involvement, intra-abdominal and peripheral lymphadenopathy. Three of these patients with disseminated disease were human immunodeficiency virus (HIV) positive. Neurological symptoms were present in a total of five patients at presentation or relapse but positive imaging findings were present in only two patients who had MRI. Computed tomography (CT) head scans performed in four patients were normal. Disease recurrence most commonly occurred within the abdomen.  相似文献   

10.
Anaplastic, CD30+, large-cell lymphoma is now a well-recognized pathologic entity that accounts for 2% to 8% of all lymphomas. Recent progress has been made in the understanding of certain biologic features found in anaplastic large-cell lymphoma, but information about its clinical behavior, in comparison to other large-cell lymphomas, is limited. The pathologic review of a large multicenter study of the treatment of aggressive lymphoma identified 146 cases of anaplastic large-cell lymphoma (ALCL) on the basis of morphology and CD30 expression. We compared initial presentation, immunophenotype, and clinical outcome of these cases with those of the 1,695 nonanaplastic diffuse large-cell lymphomas (non-ALCL) included in the same trial. Patients with ALCL were more likely to be male (P = .018) and were younger (P < .0001) than those with non-ALCL. B symptoms were more frequent in ALCL (P = .006). Skin (P < .0001) and lung (P < .05) involvement was also more frequent in ALCL, but frequency of bone marrow involvement was identical (P = . 5). Tumor cell phenotype was B in 56 cases (38%), T in 49 cases (34%), and null in 33 cases (22%). Response to chemotherapy (P = . 001), event-free survival (P = .006), and overall survival (P = . 0004) were better for ALCL than for non-ALCL. Multivariate analyses identified anaplastic character as an independent factor that predicted a longer survival. Tumor cell phenotype did not influence event-free survival (P = .72) or overall survival (P = .83). ALCL in adults is a clinicopathologic entity which, independent of its phenotypic characteristics, has a better outcome than other diffuse large-cell lymphomas.  相似文献   

11.
Forty-two fine-needle aspirates (FNA) of the mediastinum were reviewed from 1984-1995. The clinical, radiologic, pathologic, and cytologic material was studied. Twenty-five males and 17 females had an age range from 10-72 yr and a mean of 41 yr. Common complaints were chest pain, dyspnea, and cough. Thirty-eight tumors were in the anterior/superior mediastinum. Fifty-seven percent were primary neoplasms (Hodgkin's lymphoma, 7; non-Hodgkin's lymphoma, 6; thymoma, 3; germ-cell tumor, 3; thymic carcinoid and angiosarcoma, 1 each; and malignant not otherwise specified, 3). Twenty-four percent were metastatic tumors (carcinoma, 9; and sarcoma, 1). Twelve percent were benign conditions (granulomatous disease, 2; multinodular goiter, 1; extramedullary hematopoesis, 1; and one thymic cyst). Seven percent were inconclusive. FNA yielded adequate tissue for diagnosis in 83% and a correct diagnosis in 86%. There was one false-negative and no false-positive diagnosis. FNA is a useful tool for accurate tissue diagnosis of mediastinal masses.  相似文献   

12.
In a Danish population-based non-Hodgkin's lymphoma registry, 2687 newly diagnosed patients were registered from 1983 to 1992. 39 had testicular involvement (TL) (incidence 0.26/10(5)/year). Median age was 71 years. 24 cases had localised and 15 had disseminated disease. Histologically, all cases were diffuse (65% diffuse centroblastic type). Of the 27 tested, 11% were of T- and 89% of B-immunophenotype. In localised cases, where surgery was supplemented by combination chemotherapy (CCT), the relapse rate was 15.4%. The relapse rate for cases with localised disease treated with other regimens (orchiectomy and/or radiotherapy) was 63.6% (P < 0.05). Median relapse-free survival was 28 and 14 months, respectively. Overall 5-year survival for all cases was 17%. Adverse prognostic factors at the univariate level were stage IV, constitutional symptoms, serum lactic dehydrogenase elevation and performance score (WHO 3-4). It is suggested that the treatment of stage IE/IIE TL should include early CCT and CNS prophylaxis.  相似文献   

13.
PURPOSE: Teratomas with malignant transformation comprise up to 6% of metastatic teratomas. The prognosis of patients with these tumors can vary considerably. We delineate factors that may be related to prognosis in a cohort of men with teratoma with malignant transformation. MATERIALS AND METHODS: We analyzed pathological features, treatment, response, recurrence, time to recurrence, subsequent followup and survival for 21 patients (median age 28 years) diagnosed with teratoma with malignant transformation during a 7-year period at our institution. RESULTS: Malignant nongerm cell elements were present in the primary tumor in 11 cases (52%). Of 18 patients with testicular primaries 17 (94%) presented with metastatic disease. Despite aggressive treatment with surgery and chemotherapy 17 of 21 cases (81%) recurred (median time 6 months). Overall, 5 patients (24%) died of disease (median survival 23 months), 5 (24%) are alive with metastases (median followup 41 months) and 11 (52%) have no evidence of disease (median followup 50 months). Progression/recurrence was substantially greater for 2 of 2 cases with a mediastinal origin, 3 of 4 with rhabdomyosarcomatous differentiation and 5 of 6 with neural differentiation compared with the remainder of the cohort (p < 0.05). CONCLUSIONS: Teratomas with malignant transformation are usually metastatic at presentation, have a high recurrence rate and are more aggressive than teratomas without malignant transformation. Prognosis is especially poor for mediastinal teratomas with malignant transformation and for those with neural or rhabdomyosarcomatous differentiation. Complete surgical resection of residual or recurrent disease appears to offer the best chance for prolonged survival.  相似文献   

14.
We describe here the clinical and pathologic features associated with a specific translocation, t(2;13), in alveolar rhabdomyosarcoma. Tumor specimens from 14 patients with a t(2;13)-positive alveolar rhabdomyosarcoma were studied for cytogenetic-clinicopathologic correlations. Three patients had occult primary tumors; nine patients had primary tumors of the trunk (mediastinal, pelvic, or rectal). The presence of the t(2;13) was ascertained from examination of tumor involved bone marrow in ten patients who had widespread metastatic disease at the time of diagnosis. Marrow involvement was so extensive in three cases that they were initially diagnosed as acute leukemia. Response to therapy was poor, with only five patients achieving a complete response. Twelve patients have died of their disease at a median survival time of 6 months from diagnosis and one is living with recurrent disease; only one patient survives free of disease.  相似文献   

15.
We describe the case of a 26-year-old man with an elevated level of alpha-fetoprotein who had no evidence of intragonadal or extragonadal germ cell tumor. The patient was found to have Hodgkin's lymphoma within an anterior mediastinal mass. We review the significance of elevated alpha-fetoprotein levels and discuss the clinical relevance of this unusual presentation of a possible primary Hodgkin's lymphoma.  相似文献   

16.
PURPOSE: To provide further information about the presentation of thoracic involvement in Hodgkin disease and non-Hodgkin lymphoma and to compare chest radiography with chest CT findings. MATERIALS AND METHODS: We reviewed the chest radiographs and the CT images of 100 Hodgkin and 100 non-Hodgkin patients, all of them untreated. Our data were compared with those of literature series: the latest study comparing the different patterns of Hodgkin and non-Hodgkin disease appeared in 1976 and it compared chest radiography with conventional tomography, not with CT. RESULTS: Intrathoracic involvement (75% vs 48%) and adenopathy (74% vs 28%) were more frequent in Hodgkin than in non-Hodgkin lymphoma. Ninety-nine per cent of the patients with intrathoracic involvement (74/75) had nodal disease. Paratracheal/prevascular nodes were most frequently involved, namely in 72/74 Hodgkin (97%) and in 27/28 non-Hodgkin patients (96%). The lung parenchyma was more often involved in non-Hodgkin (24%) than in Hodgkin (8%) patients; it was associated with mediastinal/hilar adenopathy in all Hodgkin and in 10/24 (42%) non-Hodgkin cases. Parenchymal involvement was demonstrated with chest radiography in 7/8 Hodgkin (88%) and in 13/24 non-Hodgkin patients (54%). Chest radiography showed paratracheal/prevascular adenopathy more often in Hodgkin (54/72, 75%) than in non-Hodgkin (15/27, 56%) cases. Subcarinal and internal mammary adenopathy was poorly depicted with plain films, while hilar adenopathy was generally identified with both CT and chest radiography. Chest radiography usually missed posterior mediastinal and anterior diaphragmatic adenopathy. CONCLUSIONS: The differences in the presentation of Hodgkin vs non-Hodgkin disease are not sufficiently distinctive to permit radiographic differentiation of the two conditions, but some patterns are helpful. Recognizing the frequency of thoracic involvement and that of the additional CT findings in Hodgkin and non-Hodgkin patients makes a sound basis for lymphoma imaging.  相似文献   

17.
To evaluate the potential value of endoscopic ultrasonography in primary gastric lymphoma, we compared endoscopic ultrasonography findings with endoscopic and histologic findings in 15 patients in whom the diagnosis had been established by radiography and endoscopy, including forceps biopsy. The patients were divided into four groups according to the endoscopic ultrasonography findings. The groups included the following: superficially spreading type (six patients), diffusely infiltrating type (three patients), mass-forming type (four patients), and mixed type (two patients). The endoscopic ultrasonography findings correlated well with the endoscopic, macroscopic, and histologic findings. The histologic findings in nine patients with the endoscopic ultrasonography superficially spreading type or diffusely infiltrating type revealed B-cell lymphoma arising from mucosa-associated lymphoid tissue, which shows slowly infiltrative growth. In four patients with the endoscopic ultrasonography mass-forming type, on the other hand, the tumor histologic finding consisted of diffuse large-cell or diffuse mixed-cell type. Our results indicate that endoscopic ultrasonography may provide information helpful for the management of primary gastric lymphoma.  相似文献   

18.
Microscopic evaluation of all adult tonsillar specimens has been considered essential despite the low incidence of unsuspected pathologic conditions. We evaluate whether routine histologic examination of clinically benign adult tonsillar specimens is indicated. We retrospectively reviewed pathology results from all tonsillectomies performed on patients ages 18 years or older at our institution from 1989 through 1996. Three groups were created on the basis of indications for tonsillectomy: (1) routine tonsillectomies for benign disease, (2) asymmetric tonsils, and (3) search for unknown primary lesions. Demographic data and pathologic findings in each group were analyzed. In 1280 tonsillectomies performed for benign disease there were no malignancies (0%) and 32 cases (2.50%) with clinically unsuspected benign pathologic conditions. In 31 cases of tonsillar asymmetry, two cases with malignant lymphoma (6.5%) and three cases with benign pathology (9.7%) were identified. In nine patients with squamous cell carcinoma metastatic to the neck, two occult primary lesions were identified in the ipsilateral tonsil. Our results suggest that histologic evaluation of adult tonsils removed for benign disease may be clinically unnecessary. The elimination of microscopic examination of tonsils removed from patients whose clinical presentation is entirely consistent with benign disease poses minimal risk of missing clinically significant pathologic conditions. Substantial costs for negative examinations may be avoided.  相似文献   

19.
We describe the morphologic, immunohistologic, and genotypic characteristics of 13 cases of true histiocytic lymphomas. Six cases presented with primary gastrointestinal involvement, five with lymphadenopathy, the other sites involved being the bone marrow and the skin. The neoplastic cells displayed large abundant eosinophilic cytoplasm, occasionally vacuolated with folded or bizarre-shaped nuclei with prominent nucleoli. Mitotic figures were numerous. Multinucleated cells were common. The pattern of growth was usually diffuse and noncohesive. Spindle cell sarcoma-like areas were evident in five cases, with a prominent foam cell component in four cases. All cases expressed histiocyte-associated markers (CD68, lysozyme, alpha-1-antitrypsin), CD45 or CD45RO, and were negative for CD1a, epithelial, and B- and T-cell lineage-specific markers. Reactivity for S-100 was observed in a variable proportion of cells in 11 cases. The proliferation fraction varied from 3 to 88%. Genotypic analysis for T-cell receptor or immunoglobulin gene rearrangement demonstrated a germline configuration in all cases. We demonstrate that true histiocytic lymphoma is a rare distinctive pathologic entity that may be defined by immunohistochemical criteria and that recognition among histiocytic disorders is important for clinical and prognosis reasons.  相似文献   

20.
PURPOSE: A child with a primary lymphoma of bone (PLB) with a t(3;22)(q27;q11) is described. METHODS: An 11-year-old boy had a 5-week history of back pain and a destructive lesion of S1 that contained an epidural component. Histologic evaluation of a biopsy confirmed the diagnosis of diffuse large B-cell non-Hodgkin lymphoma. Karyotypic analysis disclosed a t(3;22)(q27;q11), but the amount of tumor tissue was insufficient for molecular studies of the BCL-6 gene. RESULTS: The patient remains free of disease 4 years after completion of intensive systemic chemotherapy and intrathecal chemotherapy. CONCLUSIONS: The lymphoma in the patient described in this report is highly unusual because of the coexistence of pediatric PLB and a t(3;22)(q27q11).  相似文献   

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