Caracterização clínica e patológica dos linfomas hepáticos: um estudo retrospetivo unicêntrico / Clinical and pathological characterization of hepatic lymphomas: a retrospective single-center study

ABSTRACT Background: The incidence of hepatic lymphoma has been increasing recently and diagnosis can be challenging as clinical presentation and radiological findings are usually variable and non-specific. Objective The aims of this study were to describe their main clinical, pathological and imaging characteristics and identify poor prognostic factors. Methods A retrospective study that included all patients with histological diagnosis of liver lymphoma over a 10-year period at our center was performed. Results A total of 36 patients were identified, with mean age of 56.6 years and male predominance (58%). There were three patients with primary liver lymphoma (8.3%) and 33 with secondary liver lymphoma (91.7%). The most common histological type was diffuse large B-cell lymphoma (33.3%). The most common clinical manifestations included fever, lymphadenopathy, weight loss, night sweats and abdominal discomfort; three patients (11.1%) were asymptomatic. Computed tomography scan revealed heterogenous radiological patterns including a single nodule (26.5%), multiple nodules (41.2%) or diffuse infiltration (32.4%). The mortality rate during follow-up was 55.6%. Higher levels of C-reactive protein (P=0.031) and absence of treatment response (P<0.001) were significantly associated with higher mortality. Conclusion Hepatic lymphoma is a rare disease that may involve liver as part of a systemic disease or, less commonly, be confined to this organ. Clinical presentation and radiological findings are often variable and non-specific. It is associated with high mortality and poor prognostic factors include higher levels of C-reactive protein and absence of response to treatment.

RESUMO Contexto A incidência de linfoma hepático tem aumentando recentemente e o diagnóstico pode ser desafiante, na medida em que a apresentação clínica e os achados imagiológicos são geralmente variáveis e inespecíficos. Objetivo: Os objetivos deste estudo foram descrever suas principais características clínicas, patológicas e de imagem e identificar fatores de mau prognóstico. Métodos: Foi realizado um estudo retrospetivo que incluiu todos os pacientes com diagnóstico histológico de linfoma hepático num período de 10 anos no nosso centro. Resultados: Foram identificados 36 pacientes, com média de idade de 56,6 anos e predomínio de género masculino (58%). Havia três pacientes com linfoma hepático primário (8,3%) e 33 com linfoma hepático secundário (91,7%). O tipo histológico mais comum foi o linfoma difuso de grandes células B (33,3%). As manifestações clínicas mais comuns incluíram febre, linfadenopatia, emagrecimento, hipersudorese noturna e desconforto abdominal; 3 (11,1%) pacientes eram assintomáticos. A tomografia computadorizada revelou padrões radiológicos heterogêneos, incluindo um único nódulo (26,5%), múltiplos nódulos (41,2%) ou infiltração difusa (32,4%). A taxa de mortalidade durante o seguimento foi de 55,6%. Níveis mais elevados de proteína C reativa (P=0,031) e ausência de resposta ao tratamento (P<0,001) foram significativamente associados a maior mortalidade. Conclusão O linfoma hepático é uma doença rara que pode envolver o fígado como parte de uma doença sistêmica ou, menos comumente, estar confinado a este órgão. A apresentação clínica e os achados radiológicos são frequentemente variáveis e inespecíficos. Associa-se a elevada mortalidade e fatores de mau prognóstico incluem níveis mais elevados de proteína C reativa e ausência de resposta ao tratamento.

Primary hepatic lymphoma- A diagnostic dilemma

Primary non-Hodgkin lymphoma of liver is a very rare malignancy. Here we report the case of a 50 year old female who presented with dull ache in the right hypochondrium and decreased appetite since 1 month. CT scan of abdomen and pelvis showed an enlarged liver with an ill- defined soft tissue lesion arising from left lobe measuring 13 × 9 cm suggestive of primary hepatic neoplasm. CT scan of chest, abdomen, and pelvis and whole body positron emission tomography showed no involvement of bone marrow, lymph nodes, spleen, or any other organ. Her liver function tests, alpha fetoprotein and carcinoembryonic antigen levels were normal. Serology was negative for viruses. Pathological examination favored diagnosis of Burkitt's lymphoma. Cytogenetic studies for MYC translocation t (8;14) is suggested for confirming the diagnosis since Ki 67 index is > 70% and not nearly 100% which is characteristic of Burkitt's lymphoma.

Primary hepatic lymphoma misdiagnosed as hepatic hemangioma: a case report and domestic literature review / 第二军医大学学报

Objective To explore the characteristics of diagnosis, treatment and prognosis of primary hepatic lymphoma (PHL), so as to better understand the tumor. Methods The diagnosis and treatment of a patient with PHL misdiagnosed as hepatic hemangioma were reported, and the domestic literatures were reviewed from 1975 to 2019 to sum up the diagnosis, treatment and prognosis characteristics of PHL. Results This patient had no specific clinical manifestations, and was misdiagnosed as hepatic hemangioma in other hospital. Magnetic resonance imaging (MRI) examination in our hospital showed that the tumor had malignant features and vessel floating sign, suggesting the possibility of malignancy. The patient was treated with resection of multiple hepatic tumors +hepatoduodenal lymph node dissection +cholecystectomy. Postoperative pathological results showed that the tumor was composed of large patches of lymphoid cells with few cytoplasm and atypical nuclei; the tumor had no obvious envelope; and the peripheral liver tissues were infiltrated. Immunohistochemical staining showed that the cells in follicular zone were mostly CD20 (+), and the cells in germinal center were CD10 (+), Bcl-6 (+) and Bcl-2 (+), Ki-67 (80%). Primary hepatic B-cell non-Hodgkin lymphoma (B-NHL) was diagnosed finally. A total of 376 PHL patients were reported in 91 domestic literatures, with an average age of 48.8±9.6 years and a male-to female ratio of 1.69:1. The imaging was characterized by single lesion and vessel floating sign, lack of blood supply. The levels of lactic dehydrogenase were increased in 146 cases (38.8%). Totally 132 cases (35.1%) were misdiagnosed and the final diagnosis depended on pathological findings. Survival analysis results showed that the prognosis of B-NHL patients was significantly better than that of T-cell non-Hodgkin lymphoma (T-NHL) patients (median overall survival time: 36.4 months vs 20.7 months, P=0.009); the prognosis of mucosa-associated lymphoid tissue lymphoma patients was significantly better than that of diffuse large B-cell lymphoma patients (52.0 months vs 30.0 months, P=0.049); and the prognosis of B-NHL patients treated with surgery combined with postoperative chemotherapy was significantly better compared with those treated with surgery alone and those treated with chemotherapy alone (45.6 months vs 23.9 months and 7.7 months, P=0.016). Conclusion Primary hepatic lymphoma is a rare tumor without characteristic clinical features, and its diagnosis needs to be combined with medical history, clinical manifestations, and imaging and laboratory examination. Once PHL is confirmed, it should be treated actively by surgery combined with chemotherapy or comprehensive chemotherapy..

A Hepatic Lymphoma Mimicking Multiple Liver Metastases or an Intrahepatic Cholangiocarcinoma / 대한내과학회지

A primary hepatic lymphoma (PHL) is a rare malignancy; misdiagnosis and mistreatment are very common. We report the case of a 56-year-old female who presented with a 2-week history of upper abdominal pain. She exhibited no risk factors for hepatocellular carcinoma (HCC) and her serum tumor marker levels were normal. A computed tomography scan and gadolinium-enhanced magnetic resonance imaging of the liver revealed multiple liver masses, suggestive of multiple liver and lung metastases or an intrahepatic cholangiocarcinoma with lung metastasis. A diagnosis of PHL (a diffuse large B cell lymphoma) was confirmed by biopsy followed by immunohistochemistry. This case emphasizes that a PHL must be considered in the differential diagnosis of space-occupying liver lesions in patients with no risk factors for HCC and normal levels of serum tumor markers. It is notable that neither B cell lymphoma symptoms nor an elevated lactate dehydrogenase level were apparent in this case. We thus report a case of PHL mimicking multiple liver metastases or an intrahepatic cholangiocarcinoma, and we review the literature.

Diagnosis and treatment of primary hepatic lymphoma: report of one case / 白血病·淋巴瘤

Primary hepatic lymphoma (PHL) is an extremely rare disease without any unified diagnostic criterion.The symptoms are usually nonspecific.Liver biopsy remains the most valuable tool for diagnosis of PHL.The predominant histology of PHL is diffuse large B-cell lymphoma.The therapeutic modalities are variable,including surgery,chemotherapy,radiotherapy,or combination of the various processes.This article described a 33-year-old man with diffuse large B-cell PHL who was treated at the Affiliated Cancer Hospital of Shanxi Medical Univeitity Blood Disease Diagnosis and Treatment Center in February 2014.The patient benefited from eight-cycle chemotherapy.At present,the patient is disease-free and undergoes regular follow-up.

Hepatic Lymphoma Representing Iso-Signal Intensity on Hepatobiliary Phase, in Gd-EOB-DTPA-Enhanced MRI: Case Report

Image findings of hepatic lymphoma have been reported as variable, ranging from single or multiple small nodules to diffuse infiltrative patterns. On MRI, most hepatic lymphomas show T1 low signal intensity, T2 high signal intensity. Dynamic imaging reveals a hypointense appearance in the arterial phase, followed by delayed enhancement in the portal venous and transitional phase. In the hepatobiliary phase using a hepatocyte-specific contrast agent (which have recently aided in increasing the access to the focal liver lesions), hepatic lymphoma is known to exhibit low signal intensity. We report a case of hepatic lymphoma, which shows iso-signal intensity on hepatobiliary phase, using gadoxetic acid (Gd-EOB-DTPA).

Primary hepatic lymphoma mimicking acute hepatitis

No abstract available.

Positron emission tomography / computerized tomography evaluation of primary Hodgkin's disease of liver.

Occurrence of primary Hodgkin's lymphoma (PHL) of the liver is extremely rare. We report on a case of a 60-year-old male who presented with liver mass and B-symptomatology. Hepatoma or hepatic metastasis from a gastrointestinal primary was initially suspected. Tumor markers like AFP, CEA, Total PSA, and CA-19.9 were within normal limits. Positron Emission Tomography / Computerized Tomography (PET/CT) revealed a large hepatic lesion and a nodal mass in the porta hepatis. A liver biopsy was consistent with Hodgkin's lymphoma. There was complete regression of the hepatic lesion and evidence of shrinkage of the nodal mass following four cycles of chemotherapy. 18F Fluro -de-oxy Glucose (FDG) PET / CT in this case helped in establishing a primary hepatic lymphoma by demonstrating the absence of pathologically hypermetabolic foci in any other nodes or organs. PET / CT scan is a useful adjunct to conventional imaging and histopathology, not only to establish the initial diagnosis, but also to monitor treatment response in PHL.

A Case of Primary Hepatic Burkitt's Lymphoma / 대한소화기학회지

Burkitt's lymphoma is a rare disease that belongs to the aggressive non-Hodgkin's lymphoma. Herein, we report a case of primary hepatic Burkitt's lymphoma. A 19-year-old man visited the hospital for right upper quadrant pain. He felt fatigue for two months. Physical examination revealed hepatomegaly and no palpable lymph node. He had no fever, weight loss, or night sweating. Laboratory finding showed mild anemia (hemoglobin, 12.4 g/dL), mild elevated transaminase (ALT, 52 IU/L), elevated lactate dehydrogenase (LDH, 437 IU/L), and alkaline phosphatase (ALP, 129 IU/L). The viral marker was positive for HBsAg, HBeAg, anti-HBs, and anti-HBc (IgG), and negative for anti-HBe, anti-HCV, and anti-HIV. CEA, AFP, and CA19-9 levels were within normal ranges. The HBV DNA quantitation was 1.3x10(9) copies/ml. Abdominal-Pelvis CT scan and abdominal MRI finding were compatable with malignant lymphoma. Liver biopsy examination confirmed Burkitt's lymphoma. No metastasis was detected in the thoracic cavity, bone marrow, and spinal fluid. The patient was treated with the combination regimen of cyclophosphamide, doxorubicin, vincristine, prednisone and high dose methotrexate. Cytosine arabinoside and methotrexate were added for CNS prophylaxis by intrathecal installation. Chemotherapy was administered every 3 weeks for fifteen cycles. Serial follow-up CT scan showed a marked decrease in the size of hepatic lesions. Follow-up CT scan and PET-CT scan were perfomed 4 weeks after the final cycle disclosed no definite residual or active lesion confirming the state of complete remission.

Longlasting Remission of Primary Hepatic Mucosa-associated Lymphoid Tissue (MALT) Lymphoma Achieved by Radiotherapy Alone

Primary hepatic lymphoma is a rare disorder representing less than 1% of all extranodal lymphomas. Histological examination of a primary hepatic lymphoma usually reveals a diffuse large B-cell lymphoma; there have been few reports of primary hepatic mucosa-associated lymphoid tissue (MALT) lymphomas. A 67-year-old man was being treated for a duodenal ulcer; while receiving therapy for the ulcer, a liver mass was incidentally found on abdominal ultrasonography. The pathologic diagnosis of the hepatic mass was an extranodal marginal zone B-cell lymphoma of MALT. The patient underwent radiotherapy with a total of 4,140 cGy delivered. The patient achieved complete remission and has been followed for 6 years with no recurrence of the disease. This report reviews the case of a primary hepatic extranodal marginal zone B-cell lymphoma of MALT successfully treated by radiotherapy alone.

A Case of Primary Hepatic Lymphoma Mimicking Hepatitis / 대한간학회지

We report here on a case of non-Hodgkin's lymphoma in which liver involvement was the predominant clinical manifestation. A healthy 44-year-old man presented with upper abdominal pain, hepatosplenomegaly, thrombocytopenia, elevated AST, ALT and bilirubin, and marked elevation of lactate dehydrogenase and alkaline phosphatase. The abdominal CT scan showed only diffuse hepatosplenomegaly and uneven contrast enhancement of the spleen without any definite mass of the liver and spleen. US-guided aspiration biopsy of liver and the histologic examination confirmed a diagnosis of non-Hodgkin's lymphoma, the diffuse large B cell type. Bone marrow biopsy showed the infiltration of malignant lymphoma cells. PET-CT showed an increased FDG uptake of the liver, spleen and long bones. The patient was treated with combination regimen of cyclophosphamide, doxorubicin, vincristine and prednisone chemotherapy. Even in the absence of a mass lesion or lymphadenopathy, primary hepatic or hepatosplenic lymphoma should be considered in differential diagnosis of hepatitis or liver cirrhosis, especially for patients with diffuse hepatosplenomegaly and markedly elevated LDH.

Burkitt's Lymphoma Representing Periportal Infiltrating Mass on CT

Imaging findings of secondary hepatic lymphoma have been reported as variable, ranging from single or multiple small nodules to diffuse infiltrative tumor patterns. We hear present a rare case report concerning aggressive B cells, secondary Burkitt's lymphoma in non-AIDS demonstrating a surprising periportal lymphoma infiltration, without upper abdominal lymphadenopathy or splenomegaly on the sonography and CT scans. Clinically, the case was characterized by atypical and highly aggressive course, with the patient presenting an abruptly developed obstructive jaundice with rapidly deteriorating hepatic function that could be indicative of cholestatic hepatitis, which differs in its clinical manifestations from hepatic lymphoma without functional deterioration in respect of its non-tissue destructive growth pattern. We suggest that hepatic lymphoma can sometimes be consistent with periportal infiltrating homogeneous mass, with no lymphadenopathy or splenomegaly on the imaging examination, with a predictable aggressive clinical course of the disease and poor prognosis.