Primary Hepatic Lymphoma: Dilemmas in Diagnostic Approach and Therapeutic Management

Abstract

Primary hepatic lymphoma (PHL) is a very rare malignancy and is characterized by liver involvement at presentation with no affectation of the spleen, lymph nodes, peripheral blood, bone marrow, or other tissues until at least 6 months after diagnosis. PHL should be considered in the differential diagnosis in a patient with space-occupying liver lesions and normal levels of alpha-fetoprotein and CEA. A computed tomography (CT) scan is the commonly used modality for staging lymphomas. The widespread use of positron emission tomography/ CT results in the improvement in the accuracy of detecting the extent of disease, response evaluation, and prognostication. The liver biopsy, due to its pleomorphic appearances in the needle biopsy specimen, can be very challenging. Current literature favors the combination of chemotherapy as the frontline treatment for its least invasiveness and improved survival. Favorable prognosis of PHL can be obtained by early surgery combined with chemotherapy in strictly selected patients. However, the optimal therapy is still unclear and the outcomes are uncertain.

Primary hepatic lymphoma: is it a disease entity?

“…[20,21] Some authors defined primary hepatic lymphoma as a very rare malignant tumor with the features of liver involvement and without involvement of other organs and tissues including bone marrow, lymph nodes, the spleen and peripheral blood until at least 6 months after diagnosis. [14,22] However, Caccamo's criteria which many authors have cited for the diagnosis of primary hepatic lymphoma were proposed in 1986 and based on the data of a single patient who was complicated with liver cirrhosis, Kaposi's sarcoma, and involvement of gastric mucosa and abdominal lymph nodes. [22,23] In many of the case reports, the patient follow-up was not long enough as "at least 6 months after diagnosis" and some diagnoses were determined just after the biopsy even when there were enlarged retroperitoneal lymph nodes and bone marrow infiltration by lymphoma.…”

“…[22,23] In many of the case reports, the patient follow-up was not long enough as "at least 6 months after diagnosis" and some diagnoses were determined just after the biopsy even when there were enlarged retroperitoneal lymph nodes and bone marrow infiltration by lymphoma. [11,14,15] Is there indeed a disease entity named primary hepatic lymphoma? The definition of the word or special term "primary" is the key point to answer the question.…”

“…[13] With the advance in imaging technology, more and more lesions of lymphoma were detected involving the liver at presentation. [14] Currently, a number of authors have published case reports or case series in which these lesions are defined as primary hepatic lymphoma and described as a malignancy that is very rare and frequently misdiagnosed, but has a rather better prognosis by the combination of surgery and chemotherapy as the treatment than primary hepatic carcinoma. [15][16][17] This paper discusses several aspects of the entity of primary hepatic lymphoma, especially the dilemmas in diagnosis.…”

Hypercalcemia due to Primary Hepatic Lymphoma

“…Other pathologies are rare but have been reported in the literature. These include mucosa-associated lymphoid tissue (MALT), lymphoblastic, mantle cell, follicular, and Burkitt lymphomas [5]. …”

“…On the lymphoma spectrum, those with non-Hodgkin's lymphoma [3–5], chronic myeloid leukemia (blast phase) [6], and adult T-cell leukemia-lymphoma may have PTHrP-induced hypercalcemia [7, 8]. Teriparatide administration was unlikely because the effects of hypercalcemia are transient, usually resolving sixteen hours after the last administration [9].…”