Figure - available from: Case Reports in Hepatology
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Epigastrium CT before treatment. Note: the liver outline was significantly more prominent. The liver edge was less smooth. Liver parenchymal density was uneven. Patchy low-density shadows were observed, especially in the left live. Multiple quasicircular low-density foci appeared in the right lobe of the liver. The spleen was enlarged to about seven rib units. The density was uniform.
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Objective
. Primary hepatic lymphoma is a rare disease. And the clinical manifestations of this disease are nonspecific. The objective of this paper is to improve clinicians’ understanding of this disease.
Methods
. We analyzed the clinical characteristics of a case of primary hepatic lymphoma in association with hepatitis B virus infection and rev...
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Primary hepatic lymphoma is a rare disease, accounting for only 0.1% of malignant liver tumors. The subtype of diffuse large B-cell lymphoma (DLBCL) is more infrequent. In contrast to hepatitis C virus, the association between hepatitis B virus and lymphoma is less clear. Here, we report the case of a 52-year-old patient followed for chronic hepati...
Citations
... As shown in Table S1, systemic combination chemotherapy is commonly used as the main therapeutic approach, which could usually achieve disease remission (89-91). Moreover, chemotherapy with CHOP-based regimens (cyclophosphamide, doxorubicin, vincristine, and prednisone) is the first-line treatment (92,93). The combination of rituximab with conventional chemotherapy can prolong survival of PHL patients who are positive for CD20 (69,(94)(95)(96). ...
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.
... Primary hepatic lymphoma (PHL) is a rare disease, with an incidence of only 0.1% of malignant liver tumors. The subtype of diffuse large B-cell lymphoma (DLBCL) is more infrequent [2]. Hepatitis C virus has been the most frequently studied hepatitis virus as a risk factor of lymphoma development. ...
... Primary liver non-Hodgkin's lymphoma is extremely rare, accounting for 0.4% of all primary extranodal lymphoma and 0.016% of all cases of NHL [2]. PHL may occur at any age but is more common in males aged about 50 years. ...
... The most frequent signs are fatigue, loss of appetite, night sweats, fever, and weight loss. Some patients show hepatomegaly, abdominal pain, or liver function abnormalities [2]. ...
Primary hepatic lymphoma is a rare disease, accounting for only 0.1% of malignant liver tumors. The subtype of diffuse large B-cell lymphoma (DLBCL) is more infrequent. In contrast to hepatitis C virus, the association between hepatitis B virus and lymphoma is less clear. Here, we report the case of a 52-year-old patient followed for chronic hepatitis B complicated by cirrhosis, associated with a primary hepatic DLBCL, with a good response to chemotherapy.
... It is typically characterized by an old age at presentation, female predominance, a high frequency of bone marrow involvement, advanced stage, and higher IPIs. 4 The symptoms are nonspecific, varying from loss of appetite, fatigue, low-grade fever, and night sweats to liver function abnormalities, hepatomegaly, and abdominal pain. 8 The typical radiologic characteristics of the disease are space-occupying lesions in the liver or spleen. 3 Some patients exhibit diffuse lesions, displaying hepatosplenomegaly. ...
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.
... PHL is a rare disease and accounts for 0.4% of all extranodal lymphoma and 0.016% of all non-Hodgkin's lymphoma (NHL) [2]. PHL of diffuse large Bcell lymphoma (PHL-DLBCL) is more infrequent [3]. ...
... PHL typically occurs in middle-aged men, and usually the presenting symptoms, blood investigations and imaging findings are nonspecific [1]. This condition is difficult to distinguish from primary liver cancer, liver metastases, granulomatous pseudotumor, and other liver diseases therefore it is easily misdiagnosed [3]. Histopathology is mandatory and confirms the diagnosis. ...
... Most PHL patients show a two to three-fold increase in ALT and AST levels, with an increase in bilirubin and HDL levels. However, AFP and CEA levels are usually normal, in contrast to those in primary liver cancer and liver metastasis [3]. The presented patient had normal liver enzyme and bilirubin values. ...
Introduction. Primary hepatic lymphoma (PHL) is a rare disease and represents lymphoproliferative disorder confined to the liver parencyme. This condition is difficult to distinguish from other liver diseases. Histopathology is essential and confirms the diagnosis. Treatment options for PHL include surgery, chemotherapy, radiation or combinations of these modalities. The objective of this this report is to present a case of primary hepatic lymphoma and to discuss disease features and treatment options in order to facilitate diagnostics and therapy. Case Outline. A 72-year-old female was presented with abdominal pain, nausea, weight loss and fatigue. Computed tomography (CT) revealed hypoattenuating solitary lesion in liver segment VIII. Detected liver lesion showed elevated 18F-FDG uptake on PET-CT examination. Extrahepatic disease was not detected. The tumor board opted for surgery and atypical resection of liver segments VII and VIII was performed. Pathohistological examination of the resected tumor demonstrated liver infiltration with non-Hodgkin?s lymphoma (NHL), diffuse large B-cell type (DBLCL). Postopertive recovery was complicated with fluid collections in the right subphrenic space successfully managed by percutaneous drainage. Subsequently patient was treated with chemotherapy and attained a complete remission documented by negative computed tomography. Conclusion. PHL can easily be misdiagnosed as another more frequent primary liver tumor due to its non-specific clinical manifestations, laboratory and imaging findings and therefore should be considered in the differential diagnosis of a hepatic lesion.
... It is typically characterized by an old age at presentation, female predominance, a high frequency of bone marrow involvement, advanced stage, and higher IPIs. 4 The symptoms are nonspecific, varying from loss of appetite, fatigue, low-grade fever, and night sweats to liver function abnormalities, hepatomegaly, and abdominal pain. 8 The typical radiologic characteristics of the disease are space-occupying lesions in the liver or spleen. 3 Some patients exhibit diffuse lesions, displaying hepatosplenomegaly. ...
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
... [1]. ПЛПочень редкая патология: составляет около 0,016% от всех неходжкинских лимфом, 0,4% от всех первичных экстранодальных лимфом и 0,1% от всех злокачественных опухолей печени [2,3]. ...
... Кроме того, определялось повышение концентрации билирубина в 15 раз, печеночных фермен-Более 90% ПЛП происходит из В-клеток, Т-клеточные лимфомы (периферическая, анапластическая, гепатолиенальная) встречаются крайне редко. Среди В-клеточных ПЛП до 96% составляет диффузная В-крупноклеточная лимфома, описаны случаи развития MALT-лимфомы, лимфомы Беркитта с первичным поражением печени [2][3][4][5][6]. ...
... Marginal biopsy of residual liver foci (arrows) Рис. 7. Гистологическое исследование резидуального очага в печени: массивные очаги некроза, в которых просматривается структура печенирасширенные склерозированные портальные тракты, инфильтрированные лимфоцитами, гистио цитами, небольшим количеством плазмоцитов и гранулоцитов, вне полей некроза структура ткани печени сохранена звена иммунитета. В результате срыва локального Т/NK-клеточного контроля в печени индуцируется пролиферация В-лимфоцитов, что с высокой вероятностью приводит к развитию лимфатической опухоли [2][3][4][5][6]. ...
Aim of the case presentation. To demonstrate the rare case of extranodal lymphoma with primary extensive liver involvement which caused difficulties at diagnosis verification. Despite of rapid disease progression with development of severe liver failure, that resulted in poor prognosis, patient demonstrated significant response to high-dose chemotherapy and achieved long-term remission. Summary. Male 38-year-old patient developed acute symptoms: sweating, general weakness, febrile fever, weight loss (50 kg for 4 months), abdominal pain combined to hepatomegaly and multiple focal lesions in the liver according to abdominal US, with 5 unsuccessful attempts for 6 months to receive informative tissue sample. Laboratory tests revealed progressive signs of liver failure at normal level of alpha-fetoprotein and carcino-embryonic antigen; clinical signs included progressive manifestations of liver encephalopathy, skin hemorrhages, severe edema, ascites, pruritis and excoriations. Empirical therapy by prednisolon resulted in no responce and was complicated by development of steroid diabetes. The diagnosis of diffuse B-macrocellular lymphoma was established only via open liver biopsy. Four sessions of high-dose polychemotherapy according to mNHL-BFM-90 program resulted in rapid reduction of liver size, recovery of consciousness, resolution of encephalopathy signs, however the liver and the spleen remained enlarged with focal parenchimatous lesions that required laparoscopy with biopsy of the pathological foci, the latter revealed no remaining neoplastic cells. During 10 years of patient follow-up liver function remains compensated, focal lesions in the liver and the spleen are undetectable, that indicates achievement of 113-months complete tumor remission. Conclusion. Presence of multiple focal lesions in the liver and signs of progressive liver failure at normal level of tumor markers requires taking into account primary liver lymphoma in the spectrum of possible etiologies. Potentially poor life prognosis in similar clinical situations at detection of primary liver lymphoma can flip to favorable due to high efficacy of modern chemotherapeutic agents.
... Overall, there is no pathognomonic radiographic finding in PHL and therefore biopsy is considered the gold standard for the diagnosis of PHL. FNA should not be performed as the tissue may be necrotic and result in a false-negative result [6,15]. ...
... One study from the MD Anderson Cancer Center followed up 24 PHL cases for 20 years. Complete remission was achieved in 85% of cases and eventfree 5-year survival rate was 70% [15]. ...
A 65-year-old female with a history of mixed connective tissue disease and pulmonary fibrosis on azathioprine, hydroxychloroquine, and prednisone (osteoporosis on teriparatide) presented with a 1-month history of hypercalcemia. After discontinuation of teriparatide, the patient’s hypercalcemia persisted. Further evaluation revealed primary hepatic lymphoma as the source of her hypercalcemia.
Primary hepatic lymphoma is rare. Clinical and radiological presentations are not specific. The diagnosis is often late. Chronic hepatitis or cirrhosis, especially post-viral C usually precedes primary liver lymphoma. The differential diagnosis arises mainly with other hepatic tumors, such as atypical hypovascular cellular hepatocellular carcinoma when there is liver cirrhosis and with hypovascular hepatic metastases, especially colorectal, stomach and lung metastases. Other differential diagnosis are tuberculosis or sarcoidosis, particularly when there is multiple lesions.
We report the case of a 52-year woman, with a history of hepatitis C infection, presenting liver cirrhosis with multiple hepatic lesions. Radiological aspect was not specific which makes it difficult to distinguish from other hepatic tumors, especially hypovascular liver metastases.
