scholarly journals Paediatric ocular adnexal lymphoma: a population-based analysis

2020 ◽  
Vol 5 (1) ◽  
pp. e000483
Author(s):  
Giannis A Moustafa ◽  
Allan K Topham ◽  
Mary E Aronow ◽  
Demetrios G Vavvas
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Paediatric Population ◽  
Low Grade ◽  
Retrospective Case ◽  
Ocular Adnexal Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

ObjectiveTo investigate the incidence, clinicopathological characteristics and survival of ocular adnexal lymphoma (OAL) in the paediatric population.Methods and analysisIn this retrospective case series, the Surveillance, Epidemiology and End Results database was accessed to identify individuals with OAL ≤18 years of age, diagnosed between 1973 and 2015. OAL located in the eyelid, conjunctiva, lacrimal apparatus and orbit were included. Main outcome measures were the age-adjusted incidence rates (IRs) per 1 000 000 population at risk (calculated for the period 2000–2015) and descriptive statistics of demographic and clinicopathological features.ResultsThe IR of paediatric OAL was 0.12 (95% CI 0.08 to 0.16) per 1 000 000. Males (0.15; 95% CI 0.10 to 0.22) and blacks (0.24; 95% CI 0.13 to 0.42) had a higher tendency for OAL development. A total of 55 tumours in 54 children were identified. The majority were localised (78.4%), conjunctival (49.1%) lymphomas. Extranodal marginal zone lymphoma (EMZL, 45.5%, n=25) was the most frequent subtype, followed by diffuse large B-cell lymphoma (DLBCL, 9.1%, n=5), B lymphoblastic lymphoma (7.3%, n=4), follicular lymphoma (5.5%, n=3), Burkitt lymphoma (5.5%, n=3), anaplastic large cell lymphoma (ALCL, 3.6%, n=2), small lymphocytic lymphoma (1.8%, n=1), diffuse large B-cell lymphoma, immunoblastic (1.8%, n=1) and panniculitis-like T-cell lymphoma (1.8%, n=1). Localised, low-grade, conjunctival lymphomas were frequently treated with complete excision with or without radiation, while high-grade and distant tumours usually received chemotherapy. Only 29.1% of paediatric OAL cases were treated with radiation. Three out of five (60%) patients with DLBCL died of lymphoma at a median follow-up of 21 (range 10–86) months, and 1 out of 2 (50%) patients with ALCL died of lymphoma at 23 months from diagnosis.ConclusionOAL in the paediatric population is rare. The majority of OAL are EMZL and are characterised by excellent prognosis. The histological subtype was found to be the main predictor of outcome with cancer-specific deaths observed in patients with DLBCL and ALCL.

Primary and Secondary Ocular Adnexal Lymphoma: A Single Institutional Experience

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4956-4956
Author(s):  
Darcie Deaver ◽  
Kenneth S. Zuckerman ◽  
Celeste M. Bello ◽  
Eduardo M. Sotomayor ◽  
Salvador Bruno ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Staging System ◽  
Low Grade ◽  
Time To Progression ◽  
Ocular Adnexal Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Abstract Abstract 4956 Background: The incidence of ocular adnexal lymphoma (OAL) is rare and usually presents in the setting of central nervous system (CNS) involvement. There are no rigid guidelines for the treatment of OAL, most probably because of the variety of characteristics of the disease. Objectives: To analyze clinical pathological features, therapy and outcomes of patients with primary and secondary OAL. Research Design and Methods: Retrospective chart review of 17 consecutive patients diagnosed with OAL at Moffitt Cancer Center from 2004–2011. Characteristics of the participants were median age 68 years, 15 (88%) white, 2 (12%) Hispanic, and 11 (65%) male. Chlamydia serology testing was negative in all patients tested. Secondary OAL patients were staged per the Ann Arbor Staging System, 2 (22%) stage III and 7 (78%) stage IV. The primary OAL patients were staged utilizing the TNM staging system for OALs (Coupland et al, Arch Pathol Lab Med, 2009). Six (75%) patients were stage T1 and 2 (25%) patients were stage T2. Results: Seventeen patients with a diagnosis of OAL were evaluated in our institution. Patients with OAL are commonly stratified into 2 groups, primary and secondary. Eight (50%) of the patients were diagnosed with primary OAL; of these there were 4 (50%) marginal zone, 3 (37%) diffuse large B cell, and 1 (12%) follicular lymphoma. Nine (50%) patients were diagnosed with secondary OAL; 4 (44%) marginal zone, 1 (11%) diffuse large B cell, 1 (11%) mantle cell, 1 (11%) CLL, and 2 (22%) progressed from low grade to diffuse large B cell lymphoma. In the primary OAL, radiation in combination with systemic chemotherapy was the preferred treatment in diffuse large B cell lymphoma and radiation was preferred in patients with low-grade lymphoma. In secondary OAL, systemic chemotherapy was the preferred treatment for aggressive lymphoma. The choice of systemic Rituximab, radiation, or observation was the preferred treatment of low-grade lymphoma. Aggressive primary OAL had a relapse rate of 2 (66%) patients with a median time to progression of 8 months. Aggressive secondary OAL demonstrated a relapse rate of 50% with median time to progression 6 months. All patients who experienced relapsed disease received salvage chemotherapy. No cases of relapse were observed in the low-grade, primary or secondary, OAL patients. Median duration of response in low-grade primary lymphoma was 6 months and the low-grade secondary lymphoma was 54 months. Conclusion: In our patient population diffuse large B cell lymphoma and marginal zone lymphoma were the most common diagnoses. Ocular adnexal lymphoma has been associated with the presence of CNS disease and it is estimated that 80–90% of patients diagnosed with OAL will experience progression to the CNS. Treatment depends on the extent of disease and the subtype of lymphoma that is histologically identified. Treatment may consist of involved field radiation in localized disease and has approximately less than 10% local recurrence rate. Intravitreal methotrexate and itraorbital injections of Rituximab or a combination of localized radiation and systemic high dose methotrexate are also options for treatment. In the event that there is systemic disease, single agent IV Rituximab or standard chemotherapy regimens such as CHOP-R or CVP-R in conjunction with ocular directed therapy. When disease is localized to the ocular compartment, the burden of disease is low and there is a greater chance of eradicating the disease. The delay in diagnosis increase the risk of CNS involvement and decreases overall survival. Disclosures: No relevant conflicts of interest to declare.

Lacrimal sac lymphoma: A series of Japanese patients

2018 ◽  
Vol 29 (6) ◽  
pp. 678-684 ◽  
Author(s):  
Maria Suzanne Sabundayo ◽  
Yasuhiro Takahashi ◽  
Hirohiko Kakizaki
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Japanese Patients ◽  
Nasolacrimal Duct ◽  
Lacrimal Sac ◽  
Retrospective Case ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Purpose: To present cases of primary lacrimal sac lymphoma in a Japanese population. Methods: In this retrospective case series, five cases (two males and three females; mean age: 49.8 years; age range: 38–62 years) were included. Clinical features, diagnostic findings, and treatment outcomes were collected and reviewed. Results: Most patients presented with a swelling or mass in the medial canthal area. Only one patient presented with epiphora, while another patient also complained of pain which was due to inflammation. Imaging studies revealed a lacrimal sac mass with involvement of the nasolacrimal duct and variable involvement of the nasal cavity. Histopathological findings revealed three cases of diffuse large B-cell lymphoma, one case of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, and one case of follicular lymphoma. Four cases were treated with a combination of chemotherapy and immunotherapy, with one case necessitating additional radiotherapy. One case was treated with immunotherapy alone. The mean follow-up was 23 (range: 3–50) months. Four cases showed complete remission, while the remaining patient is still ongoing treatment. Conclusion: Diffuse large B-cell lymphoma is the most common type of lacrimal sac lymphoma in this series. Lacrimal sac tumors should be ruled out in the presence of epiphora, dacryocystitis, or a mass in the medial canthus, even in the absence of pain or bleeding. Combined chemotherapy and immunotherapy is an effective treatment for these cases.

scholarly journals An Interesting Case of Lineage Switch to Histiocytic Sarcoma in a Case of Diffuse Large B Cell Lymphoma

2019 ◽  
Vol 05 (02) ◽  
pp. 093-096
Author(s):  
Manasi C. Mundada ◽  
Faiq Ahmed ◽  
Sudha Murthy ◽  
Krishna Mohan Mallavarapu
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Histiocytic Sarcoma ◽  
Lymphocytic Leukemia ◽  
Low Grade ◽  
Phenotypic Characteristics ◽  
Lineage Switch ◽  
Large B Cell Lymphoma ◽  
Large B Cell

AbstractLineage switch involves change in the phenotypic characteristics from one type to another. It is a rare phenomenon described in mature lymphoid neoplasms which transform to histiocytic/dendritic cell tumor, more commonly described in low-grade lymphoma like follicular, chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), wherein the neoplasm loses the phenotypic characteristics of non-Hodgkin lymphoma and acquires the markers of histiocytic differentiation. Here, we present a case of diffuse large B cell lymphoma transforming to histiocytic sarcoma post 6 months of start of therapy. Histiocytic sarcoma being a very aggressive tumor, the patient had a very rapid deteriorating course and succumbed to disease.

Clinical Features and Outcome of 147 Patients with Diffuse Large B-Cell Lymphoma and Hepatitis C Virus Infection.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 1921-1921
Author(s):  
Carlo Visco ◽  
Luca Arcaini ◽  
Michele Merli ◽  
Annalisa Andreoli ◽  
Sara Burcheri ◽  
...  
Keyword(s):  
Hepatitis C Virus ◽  
Hepatitis C ◽  
B Cell ◽  
Cell Lymphoma ◽  
International Prognostic Index ◽  
B Cell Lymphoma ◽  
Hcv Infection ◽  
Low Grade ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Abstract Hepatitis C virus (HCV) has been implicated in the pathogenesis of a subset of low-grade non-Hodgkin lymphomas. Furthermore, diffuse large B-cell lymphoma (DLBCL) has been correlated to HCV infection in several series from our geographical area (north-east of Italy), but little is known about the characteristics of such high-grade tumors. We analyzed presentation features of 147 previously untreated HCV-positive patients with DLBCL who presented to the three participating centers between 1993 and 2004. All patients were provided with complete clinical information, were HIV negative, and had been tested at tumor onset for HCV antibodies by ELISA and RIBA. Median age at presentation was 64 years old (range 29–88), 47% were males, ECOG performance status was >1 in 20%, Ann Arbor stage was I in 20%, II in 27%, III in 26%, IV in 27%, and B-symptoms were present in 37% of patients. The International Prognostic Index (IPI) value at diagnosis was low in 18%, int/low in 23%, int/high in 32%, and high in 27% of patients. Surprisingly, DLBCL transformed from a low-grade histology represented only 7% of the whole population, while primary mediastinal DLBCL were extremely rare (1/147, <1%). Patients frequently presented as primary extranodal DLBCL (65/147, 44%). Most involved extranodal sites were skin, liver, stomach, and spleen, with the latter being the most represented syte (33% of patients). Remarkably, spleen was the only extranodal involved organ in 20% of patients. Treatment was delivered with cure-intent, and consisted of CHOP-like regimens +/− Rituximab for the large majority of patients, except for 16 (11%) patients with cirrhosis or severe hepatic dysfunction, who received mono-chemotherapy or radiotherapy. Only three (2%) HCV-positive patients had to discontinue chemotherapy due to liver function impairment. The addition of Rituximab to chemotherapy did not seem to affect patients’ tolerance to treatment. With a median follow-up of 48 months for survivors, 5-year overall survival (OS) was 75%, while 5-year failure-free survival (FFS) was 51%. In particular, the 65 patients with primary extranodal DLBCL shared a better 5-year OS (83% vs 71%, p=0.01) and FFS (75% vs 39%, p=0.009) than their nodal counterpart. Nodal origin of the tumor resulted the strongest independent adverse factor both in terms of OS and FFS in multivariate analysis. The peculiar clinical behavior shared by HCV-positive DLBCL may disclose relevant biological features of these tumors, and may be relevant for future studies aiming to clarify the link between HCV infection and aggressive lymphoproliferative disorders.

Primary bone marrow diffuse large B cell lymphoma: a case series and review

2010 ◽  
Vol 90 (7) ◽  
pp. 791-796 ◽  
Author(s):  
Hung Chang ◽  
Yu-Shin Hung ◽  
Tung-Liang Lin ◽  
Po-Nan Wang ◽  
Ming-Chung Kuo ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Primary Bone ◽  
Primary Bone Marrow ◽  
Large B Cell

scholarly journals Clinical manifestations of diffuse large B‑cell lymphoma in the skin and subcutaneous tissue a case series study

2020 ◽  
Vol 37 (5) ◽  
pp. 812-816
Author(s):  
Katarzyna Dulik ◽  
Grażyna Kamińska-Winciorek ◽  
Ryszard Swoboda ◽  
Anna Kwiatkowska-Pamula ◽  
Sebastian Giebel
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Subcutaneous Tissue ◽  
Clinical Manifestations ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Case Series Study ◽  
Series Study ◽  
Large B Cell

scholarly journals P024 The mighty mimic: intravascular large b-cell lymphoma masquerading as myositis: a case series

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Maria A Mirza
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Surrounding Tissue ◽  
Affected Area ◽  
Small Vessels ◽  
Large B Cell Lymphoma ◽  
Skin Biopsies ◽  
Large B Cell

Abstract Background/Aims  Intravascular large b-cell lymphoma (IVLBCL) is a rare and aggressive form of extranodal diffuse lymphoma which due to its heterogeneous manifestations is often diagnosed at post mortem. The disease is typically contained within the small vessels thus sparing the surrounding tissue. Indeed, the presentation of the disease will depend on the organ involved with classical involvement of the central nervous system and skin. Methods  We herein report two compelling cases of patients presenting with features of myositis, only to find on muscle biopsy evidence of IVBCL. Biopsy of the affected area and indeed random skin biopsies (RSB) may be a fundamental tool in establishing this elusive diagnosis. Results  Features of the two cases are discussed, along with their various presentations, investigations, treatment and overall trajectory. Conclusion  IVBCL is a rare disease that can present in various ways and often a delay in reaching a diagnosis leads to patient deterioration and death. A multidisciplinary approach involving the microbiologists, rheumatologists and haematologists led to a timely identification of the disease and subsequent management in these two cases. As the disease is characteristically contained within the blood vessels, the diagnosis is normally not confirmed from peripheral blood tests or even bone marrow biopsy. Biopsy of the affected area is what revealed the diagnosis in our two cases. Random skin biopsies may be very useful in establishing the diagnosis when suspecting IVBCL. Treatment may be very effective in controlling the disease as demonstrated by these two cases. Disclosure  M.A. Mirza: None.

scholarly journals Prognosis of Intravascular Large B Cell Lymphoma (IVLBCL): Analysis of 182 Patients from Global Case Series

2020 ◽  
Vol Volume 12 ◽  
pp. 10531-10540
Author(s):  
Ziyue Liu ◽  
Yuelun Zhang ◽  
Yicheng Zhu ◽  
Wei Zhang
Keyword(s):  
B Cell ◽  
Cell Lymphoma ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell
Sign in / Sign up

Export Citation Format

Share Document