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Ma J, Daou R, Bou Eid J, Fregonese B, El-Khoury J, Wijetunga NA, Imber BS, Yahalom J, Hajj C. Management approaches for primary hepatic lymphoma: 10 year institutional experience with comprehensive literature review. Front Oncol 2025; 15:1475118. [PMID: 40182049 PMCID: PMC11965623 DOI: 10.3389/fonc.2025.1475118] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2024] [Accepted: 01/28/2025] [Indexed: 04/05/2025] Open
Abstract
Purpose/objective Primary hepatic lymphomas (PHL) are an extremely rare form of non-Hodgkin Lymphoma (NHL) for which there are no established treatment guidelines, with available literature largely comprised of small case reports. Therefore, we evaluate our institutional experience treating PHL within the context of existing literature to better understand treatment modalities, role of radiotherapy (RT), and outcomes. Materials/methods We conducted a single institutional retrospective study of all patients with PHL diagnosed from 2000-2021, defined as a biopsy-proven liver lesion in the absence of other lymphomatous solid organ involvement, except for concurrently diagnosed hepatosplenic lymphomas. Subgroup analysis was performed for diffuse large B-cell lymphoma (DLBCL) and indolent lymphomas, which included marginal zone lymphoma (MZL), Grade 1-2 follicular lymphoma (FL), and low-grade B-cell lymphoma (BCL), NOS. Univariable (UVA) and multivariable analysis (MVA) for overall survival (OS) were performed using the Cox proportional hazards model. A literature review was conducted using key words "liver", "lymphoma", and "treatment" to identify relevant literature. Results We identified 30 patients with PHL within the institutional cohort and 192 patients from comprehensive literature review. Subgroup analysis of DLBCL included 15 patients. On MVA for OS, only ECOG score (p=0.02) and Lugano stage (p=0.04) remained significant. Subgroup analysis of the indolent lymphoma group included 9 patients. On MVA for OS, only age remained significant. Systemic therapy was the most common treatment modality overall (20 patients; 67%) with surgery, radiation and observation utilized in 4 patients (13%) each. Seventeen (57%) of patients were alive at the time of data collection, with 8 (27%) deceased and 5 (17%) lost to follow-up. Conclusion PHL are an extremely rare subtype of NHL for which there is no clear treatment consensus. Primary hepatic DLBCL appears to be treated mostly with chemotherapy with good disease control. For indolent PHL, low-dose RT appears to have good overall disease control with minimal toxicity. Our RT data is limited by the short duration of follow-up for patients receiving RT compared to those who received chemotherapy, surgery or observation. However, our results are encouraging for the use of RT for appropriate patients with indolent PHL.
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Affiliation(s)
- Jennifer Ma
- Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY, United States
| | - Remy Daou
- Department of Family Medicine, Saint Joseph University, Beirut, Lebanon
| | - Josiane Bou Eid
- Department of Family Medicine, Saint Joseph University, Beirut, Lebanon
| | - Beatrice Fregonese
- Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY, United States
| | - Joe El-Khoury
- Department of Family Medicine, Saint Joseph University, Beirut, Lebanon
| | - N. Ari Wijetunga
- Department of Radiation Oncology, University of North Carolina (UNC) School of Medicine, Chapel Hill, NC, United States
| | - Brandon S. Imber
- Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY, United States
| | - Joachim Yahalom
- Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY, United States
| | - Carla Hajj
- Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY, United States
- Department of Radiation Oncology, Cleveland Clinic, Abu Dhabi, United Arab Emirates
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Wang Q, Wu K, Zhang X, Liu Y, Sun Z, Wei S, Zhang B. Primary hepatopancreatobiliary lymphoma: Pathogenesis, diagnosis, and management. Front Oncol 2022; 12:951062. [PMID: 36110965 PMCID: PMC9469986 DOI: 10.3389/fonc.2022.951062] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2022] [Accepted: 08/08/2022] [Indexed: 11/28/2022] Open
Abstract
Primary hepatopancreatobiliary lymphoma (PHPBL) is extremely rare, which is defined as a lympho-proliferative disease confined to the hepatobiliary system and pancreas without any involvement of lymph nodes, bone marrow, or other organs. The clinical and imaging manifestations of PHPBL are variable and non-special, which are akin to those of tumors of the hepatobiliary and pancreatic systems. The overall prognosis and management of PHPBL differ from those of other tumors in the hepatobiliary system and pancreas. Proper diagnosis and prompt treatment are essential for improving clinical outcomes. Due to its rarity, the optimal treatment has not been issued. However, combination chemotherapy is considered as a standard treatment for them. This review provides an overview of the pathogenesis, diagnosis, pathology, and management of PHPBL and offers clinicians the diagnosis and management schedule for PHPBL.
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Affiliation(s)
- Qianwen Wang
- Department of Surgery, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu, China
| | - Kangze Wu
- Department of Surgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Xuzhao Zhang
- Department of Hematology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
| | - Yang Liu
- Department of Surgery, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu, China
| | - Zhouyi Sun
- Department of Surgery, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu, China
| | - Shumei Wei
- Department of Pathology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- *Correspondence: Bo Zhang, ; Shumei Wei,
| | - Bo Zhang
- Department of Surgery, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China
- *Correspondence: Bo Zhang, ; Shumei Wei,
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Wu M, Wulipan F, Ma J, Qian W, Sun S, Chen P, Xu Y, Chen M, Yang W, Xie Y, Shen L. Clinical characteristics and prognostic factors of lymphoma patients initially presenting with fever of unknown origin. Am J Transl Res 2022; 14:2625-2636. [PMID: 35559398 PMCID: PMC9091104] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2022] [Accepted: 03/23/2022] [Indexed: 06/15/2023]
Abstract
BACKGROUND Lymphoma has been identified as the most common cause of non-infectious fever of unknown origin (FUO). However, clinical characteristics and prognostic factors in lymphoma patients with FUO are lacking. METHODS From January 1, 2013 to December 31, 2019, our center enrolled 185 patients who initially presented with FUO but were later diagnosed with lymphoma in Huadong Hospital of Fudan University. The FUO and matched non-FUO groups were compared in terms of clinical symptoms, laboratory examinations, overall survival (OS), and progression-free survival (PFS). The prognostic factors of OS and PFS in patients with FUO were assessed by Cox analyses. RESULTS In the FUO group (180 in total), B cell non-Hodgkin's lymphoma (B-NHL) cases were 88 (48.9%), T cell non-Hodgkin's lymphoma (T-NHL) was 60 (33.3%), NK/T cell lymphoma (NK/T-CL) was 24 (13.3%), and Hodgkin's lymphoma (HL) was 8 (4.4%). During the hospitalization, the maximum body temperature of the FUO group diagnosed with B-NHL, T-NHL, or NK/T-CL was statistically higher than that of the non-FUO group (P < 0.05). The differences in OS between the FUO and non-FUO groups were significant for HL (P = 0.006), B-NHL (P = 0.007), and T-NHL (P = 0.013). In the multivariate analyses, the log10 serum ferritin was an independent risk factor for all-cause death in patients with FUO (hazard ratio, 9.578; 95% confidence interval, 1.382-66.365; P = 0.022). CONCLUSION We found that the subtypes of lymphoma initially presenting with FUO were mostly B-NHL and T-NHL. The detection of ferritin levels during the hospital stay may help predict the long-term survival rate in patients with FUO.
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Affiliation(s)
- Min Wu
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Fulati Wulipan
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Jiexian Ma
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Wensi Qian
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Shunrong Sun
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Pingping Chen
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Yu Xu
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Mingyue Chen
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Wenjing Yang
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Yanhui Xie
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
| | - Lin Shen
- Department of Hematology, Huadong Hospital, Fudan University Shanghai, China
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Hou H, Guo C, Que C, Nie L, Zhang Q, Zhao H, Nong L, Ma W, Wang Q, Liang Z, Wang B, Ma J, Wang G. Diffuse large B-cell lymphoma presenting as reversible intrapulmonary arteriovenous shunts with hypoxia, fever and progressive jaundice: a case report and literature review. BMC Pulm Med 2022; 22:89. [PMID: 35292006 PMCID: PMC8922084 DOI: 10.1186/s12890-022-01881-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/16/2021] [Accepted: 03/07/2022] [Indexed: 11/16/2022] Open
Abstract
Background Intrapulmonary arteriovenous shunts is rare seen in a patient without lung involvement. Case presentation This is the first report of reversible intrapulmonary arteriovenous shunts secondary to extrapulmonary lymphoma as one initial symptom. The patient presented as fever of unknown origin and dyspnea, and examinations of infection were negative. Diagnosis of DLBCL was finally confirmed through bone marrow and splenic biopsies. Intrapulmonary arteriovenous shunts were diagnosed through 100% oxygen inhalation test and transthoracic contrast echocardiography (TTCE). After the treatment of lymphoma, his respiratory failure was relieved. We rechecked the 100% oxygen inhalation test and TTCE, which both indicated that his intrapulmonary arteriovenous shunts had resolved. Conclusions We speculated the prominent inflammation from active DLBCL was the most possible mechanism associated with the reversible intrapulmonary shunt in this patient. These findings will assist us to better understand the mechanism of intrapulmonary shunts.
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Affiliation(s)
- Huan Hou
- Department of Respiratory and Critical Care Medicine, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China
| | - Cuiyan Guo
- Department of Respiratory and Critical Care Medicine, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China
| | - Chengli Que
- Department of Respiratory and Critical Care Medicine, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China
| | - Ligong Nie
- Department of Respiratory and Critical Care Medicine, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China
| | - Qi Zhang
- Department of Respiratory and Critical Care Medicine, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China
| | - Hong Zhao
- Department of Infectious Diseases, Center for Liver Disease, Peking University First Hospital, Beijing, China
| | - Lin Nong
- Department of Pathology, Peking University First Hospital, Beijing, China
| | - Wei Ma
- Department of Cardiology, Peking University First Hospital, Beijing, China
| | - Qian Wang
- Department of Hematology, Peking University First Hospital, Beijing, China
| | - Zeyin Liang
- Department of Hematology, Peking University First Hospital, Beijing, China
| | - Bingjie Wang
- Department of Hematology, Peking University First Hospital, Beijing, China
| | - Jing Ma
- Department of Respiratory and Critical Care Medicine, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China.
| | - Guangfa Wang
- Department of Respiratory and Critical Care Medicine, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China
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Hai T, Zou LQ. Clinical management and susceptibility of primary hepatic lymphoma: A cases-based retrospective study. World J Clin Cases 2021; 9:9417-9430. [PMID: 34877277 PMCID: PMC8610872 DOI: 10.12998/wjcc.v9.i31.9417] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2021] [Revised: 08/21/2021] [Accepted: 09/16/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The liver as a primary site of lymphoma is rarely seen, they are usually misdiagnosed as hepatocellular carcinoma, etc. In 2017, a review of primary hepatic lymphoma (PHL) was done in immunocompetent diffuse large B-cell lymphoma (DLBCL) patients. Yet questions that include treatment choosing or susceptibility of immunoincompetent patients remain disputable.
AIM To investigate the clinical characteristics of patients with PHL.
METHODS We collected PHL cases on PubMed, and extracted demographic and clinicopathological data to perform a systematic analysis. Survival analysis regarding age, lactate dehydrogenase (LDH), liver function abnormality (LFA), and treatment modalities were conducted. The Kaplan-Meier method and Cox regression were used to identify risk factors.
RESULTS Of 116 PHL patients with DLBCL (62.1%) as the most common subtype. Biopsy methods before surgery produced a 97% positive rate. Progression-free survival (PFS) was significantly shortened in patients with elevated LDH [Hazard ratio (HR): 3.076, 95% confidence interval (CI): 1.207-7.840, P = 0.018] or LFA (HR: 2.909, 95%CI: 1.135-7.452, P = 0.026). Univariate Cox regression analysis suggesting that LDH, liver function, B symptom, hepatosplenomegaly, and lesion were significantly associated with PHL patients survival (P < 0.05). Heavy disease burden was observed in deceased patients. A few PHL patients (3.4%) have slightly higher tumor markers.
CONCLUSION PHL patients with elevated LDH and LFA tend to have shorter PFS. Biopsy before treatment in undecided patients with no tumor markers exceeds upper limits has the most essential clinical significance, especially in immunoincompetent patients.
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Affiliation(s)
- Tao Hai
- Cancer Center, West China Hospital, Chengdu 610041, Sichuan Province, China
| | - Li-Qun Zou
- Department of Medical Oncology, Cancer Center, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China
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Shi X, Zhang T, Xu H, Zhang X, Zhao H, Liu X, Hou F, Yang G, Liu Z. Jaundice may be the only clinical manifestation of primary hepatosplenic diffuse large B-cell lymphoma: a case report and literature review. J Int Med Res 2020; 48:300060520938173. [PMID: 32762403 PMCID: PMC7416148 DOI: 10.1177/0300060520938173] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/14/2023] Open
Abstract
A 64-year old Chinese male patient was admitted to our hospital because of severe
jaundice that persisted for 2 months. No swollen lymph nodes or hepatosplenomegaly was
detected on physical examination. His laboratory data indicated high levels of direct
bilirubin, alkaline phosphatase, aspartate aminotransferase, and alanine aminotransferase.
No abnormality was revealed on abdominal computed tomography (CT). However, positron
emission tomography (PET)-CT revealed diffuse hypermetabolism in the liver and spleen.
Ultimately, liver biopsy guided by PET-CT was performed, revealing that atypical
lymphocytes diffusely infiltrated the liver. The immunohistochemical analysis demonstrated
that the tumor cells were positive for CD20, Bcl-2, Bcl-6, MUM1, and c-Myc but negative
for CD3, CD4, CD8, and CD10. Based on these findings, this patient was diagnosed with
primary hepatosplenic diffuse large B-cell lymphoma. After the definite diagnosis, he
received chemotherapy and remained in good health as of September 2019.
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Affiliation(s)
- Xue Shi
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Tingting Zhang
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Hong Xu
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Xiaoying Zhang
- Department of Emergency, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Hongguo Zhao
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Xiaodan Liu
- Department of Hematology, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Fang Hou
- Department of Hematology Research Laboratory, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Guangjie Yang
- Department of PET/CT Center, The Affiliated Hospital of Qingdao University, Qingdao, China
| | - Zhihe Liu
- Department of Lymphoma, The Affiliated Hospital of Qingdao University, Qingdao, China
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Ramachandran P, Sahni S, Wang JC. De Novo CD5+ Primary Gastrointestinal Diffuse Large B-Cell Lymphoma: Challenges With Treatment and Clinical Course. J Investig Med High Impact Case Rep 2019; 7:2324709619893546. [PMID: 31814435 PMCID: PMC6900610 DOI: 10.1177/2324709619893546] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/02/2022] Open
Abstract
The gastrointestinal tract is a common extranodal site for lymphomas. However,
primary gastrointestinal lymphomas are rare. Diffuse large B-cell lymphomas
(DLBCL) are the most commonly encountered type in the gastrointestinal tract.
Most of the DLBCL are CD5 negative. CD5+ DLBCL is very rare and a poor
prognostic subtype of lymphoma. We report a rare case of primary small bowel
CD5+ DLBCL that evolved from being a localized low International Prognostic
Index–scored disease into an advanced and aggressive disease primarily dictated
by the presence of CD5 antigen positivity.
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Affiliation(s)
| | - Sonu Sahni
- Brookdale University Hospital and Medical Center, Brooklyn, NY, USA
| | - Jen C Wang
- Brookdale University Hospital and Medical Center, Brooklyn, NY, USA
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