Diffuse large B-cell lymphoma: clinical implications of extranodal versus nodal presentation--a population-based study of 1575 cases

Differences in genetic origin between nodal and extranodal diffuse large B-cell lymphomas (DLBCL) exist. Using population-based data from the registry of the Danish Lymphoma Group, the present study is the first to analyse clinical implications of nodal versus extranodal presentation of DLBCL. Of 4786 newly diagnosed non-Hodgkin's lymphoma patients in a 16-year period, 1575 (33%) had DLBCL. The annual incidence rate was 2.9 per 100 000; 40% were extranodal. The clinical profile of patients with extranodal DLBCL was different from the nodal DLBCL patients. Extranodal DLBCL was associated with older age and poorer performance score, but also lower tumour burden. In extranodal DLBCL, 51% of the cases were stage I and 36% were stage IV, whereas the patients were relatively equally distributed between the four stages in nodal DLBCL. For stage I patients, extranodal DLBCL was independently associated with poor survival (P = 0.003). In contrast, among stage IV patients those with extranodal DLBCL survived longer (P = 0.009). We conclude that there are important clinical differences between nodal and extranodal DLBCL. The addition of these clinical results to the existing aetiological and genetic data suggests that the distinction between nodal and extranodal DLBCL is not only pathogenetically but also clinically important.     

PRAD1 gene over-expression in mantle-cell lymphoma but not in other low-grade B-cell lymphomas, including extranodal lymphoma

Summary. Employing Northern blot analysis and the polymerase chain reaction, we investigated PRAD1 gene over-expression in the tumour tissues of 58 patients with B-cell lymphoma. These findings were then examined in relation to the patients' clinical and immunohistological characteristics. The over-expression of this gene was detected in 6/8 patients with mantle cell lymphoma (MCL) and in only 1/50 other lymphomas, indicating its close association with MCL. The patients with MCL had common clinical findings of advanced disease with generalized lymphadenopathy on admission, and they had a CD5+CD10–IgD+ phenotype. The patients with chronic lymphocytic leukaemia (CLL) also showed findings indicating a distinctive disease entity: a CD5+CD10–IgD+ phenotype and lack of PRAD1 over-expression. In contrast, most patients with diffuse low-grade lymphoma other than MCL and CLL had localized extranodal disease, expressed a CD5-CD10–IgD– phenotype, and lacked PRAD1 over-expression. These findings suggest that extranodal low-grade lymphomas differ from nodal MCL and are not part of the spectrum of CLL.     

Primary lymphoma of the liver showing immunohistochemical evidence of T-cell origin

Summary Primary malignant lymphomas of the liver are extremely rare. Thus far, 28 cases (1–13) of this entity have been described, but after careful clinical and histological staging, no other sites of extrahepatic involvement were demonstrated in 17 of these cases (2, 3, 5–13). We recently had the opportunity to investigate and manage a case of true primary malignant peripheral T-cell lymphoma of the liver in a 22-year-old man. To our knowledge this is the first reported case of a primary T-cell lymphoma of the liver. The patient was successfully treated with a right trisegmentectomy and no tumor recurrence has appeared after five years.     

原发皮肤结外NK/T细胞淋巴瘤一例并文献复习

报道1例原发皮肤结外NK/T细胞淋巴瘤,并复习文献。患者,女,42岁。全身皮肤瘀斑、皮下结节20余天,发热4 d。右股内侧皮损组织病理示:大量淋巴细胞及浆细胞呈弥漫性浸润。免疫组化结果:CD3、CD43、CD56、颗粒酶B(Granzyme B,GгB)、细胞毒性蛋白(TIA)-1均(+)、Ki-67 LI约60%阳性;原位杂交EBER(+)。本病恶性程度高,需尽早进行组织病理检查及免疫组化染色以帮助诊断。     

累及皮肤的结外鼻型NK/T细胞淋巴瘤

报告1例累及皮肤的结外鼻型NK/T细胞淋巴瘤.患者男,72岁.确诊结外鼻型NK/T细胞淋巴瘤8年后,鼻根部出现红肿、破溃.根据皮损、组织病理检查和免疫组化染色结果确诊.     

鼻部NK/T细胞淋巴瘤1例

1例以鼻部皮肤受累为首发表现的结外NK/T细胞淋巴瘤(鼻型)患者,男,29岁,因左侧鼻翼肿胀伴间歇高 热1个月,口腔、左鼻孔口溃烂及左眼周红肿1周就诊。体格检查表现为左鼻翼部肿胀,左鼻孔下方皮肤浅表糜烂、 结痂。口腔上腭部及两侧颊黏膜可见多个大小不等的溃疡面。双侧眼睑红肿(左侧明显),双眼结膜充血,结合皮肤 组织病理学及免疫组织化学检查符合鼻部NK/T细胞淋巴瘤诊断。     

Lymphoid neoplasms of the sinonasal tract and their differential diagnoses

Many types of lymphoid neoplasms have been reported involving the sinonasal region including aggressive and indolent B-cell lymphomas, T/NK cell lymphomas and plasma cell neoplasms. They account for a small percentage of all lymphomas but as a whole is the second most common malignancy in the sinonasal region. Clinically and morphologically, they can mimic non-neoplastic and non-hematolymphoid malignant lesions in this region and must be included in the differential diagnoses when a sinonasal lesion is encountered. This article describes the histopathologic and molecular features of diffuse large B-cell lymphoma and extranodal NK/T cell lymphoma, the two main types of lymphomas found in the sinonasal tract, as well as plasmacytoma and plasmablastic lymphoma, which tend to locate in the head and neck region, and reviews the main differential diagnoses.     

Non- Hodgkin lymphoma of the lips: A rare entity

AimTo investigate clinico-pathological features of lymphoma of the lips, and review the literature.Materials and MethodsRetrospective analysis and review of English literature, 1996-2016.ResultsAnalysis included 23 cases, 7 new cases and 16 from literature, 12 M: 11 F, age 7–82 years. Four occurred in children, mean age 10.1; 19 in adults, mean 61.1 years.The lower lip was involved in the majority of cases (16, 69.56%). 14 (60.87%) were isolated to the lips, 8 (34.78%) were multifocal. Nine (39.13%) occurred in association with Sjogren's syndrome, of which one also had Hashimoto thyroiditis. IgG4-related disease and HIV were reported in one case each. The lip salivary glands were involved in most cases (19, 82.6%); 3 (13.6%) showed only cutaneous involvement.The typical presentation was single or multiple nodules (15, 65.21%), with surface ulceration in only two (8.69%). Constituent symptoms were absent in all cases, paresthesia was reported in one (4.34%). The majority (18, 78.26%) was extranodal marginal zone B-cell lymphoma - mucosa-associated lymphoid tissue lymphoma (EMZB-MALT), and one case each was mantle cell, NK-T cell, CD30 positive and plasmablastic lymphoma.ConclusionThe lips seem to have a unique pattern of non-Hodgkin lymphoma dominated by EMZB-MALT lymphoma, rarely other types. In more than half, neither Sjogren's syndrome nor other chronic inflammation was identified. Lesions tend to present as asymptomatic slowly progressing, non-ulcerated submucosal masses. Lymphoma should be considered even in the absence of constituent symptoms, as most cases showed none. Although the number of reported cases is rather small, disease course is usually prolonged and prognosis seems to be good.     

Predictive Value of Sonographic Features of Extranodal Extension in Axillary Lymph Nodes

Objective. The purpose of this study was to assess the accuracy of 5 sonographic features in the prediction of extranodal extension (ENE) in axillary lymph nodes (ALNs) of patients with biopsy‐proven breast cancer. Methods. A review of our institution's surgical and pathologic database was performed for patients with pathologically proven axillary ENE from October 1, 2003, to October 1, 2007. An equivalent number of patients without ENE were included. All patients had sonograms of ALNs available. A radiologist to whom the study was masked reviewed ALN images with specific attention to unclear margins, node matting, perinodal edema, and hilar effacement or replacement. Univariate and multivariate logistic regression analyses were used to obtain the sensitivity, specificity, and odds ratio (OR) for each feature. Results. Our review included a total of 131 patients (64 with ENE and 67 without ENE). The respective sensitivity and specificity estimates for each feature were as follows: node matting, 52% and 84%; perinodal edema, 34% and 87%; unclear margins, 64% and 75%; hilar replacement, 71% and 42%; and hilar effacement, 74% and 60%. Univariate analysis showed a statistically significant association between features and ENE with ORs as follows: matting, 5.4; perinodal edema, 3.4; unclear margins, 5.2; and hilar replacement, 4.3. Multivariate analysis showed that matting and unclear margins were independently associated with ENE. Conclusions. The sonographic features of unclear margins, node matting, perinodal edema, and hilar replacement have a statistically significant association with ENE. The sonographic features of unclear margins, node matting, and perinodal edema predict ENE with high specificity.