| Abstract|| |
Primary hepatic mucosa-associated lymphoid tissue lymphoma (MALToma) is a rare entity. Its coexistence with other malignancies is even rarer. Only few case reports of its association with other malignancies mostly gastric and colon cancer have been published. We report a case of primary MALToma of liver in an unusual setting of dual solid malignancy.
Keywords: Dual malignancy, liver, mucosa-associated lymphoid tissue lymphoma
|How to cite this article:|
Khurana A, Mukherjee U, Patil N. An unusual case of hepatic lymphoma with multiple epithelial malignancies. Indian J Pathol Microbiol 2018;61:585-6
| Introduction|| |
Primary hepatic lymphoma (PHL) implies the lymphomatous transformation confined to the liver with no evidence of involvement of the spleen, lymph nodes, bone marrow, or other lymphoid structures. PHL is rare, accounting for <1% of malignant lymphomas, the most common type being diffuse large B-cell lymphoma followed by rare occurrences of mucosa-associated lymphoid tissue lymphoma (MALToma), follicular lymphoma, Burkitt lymphoma, or Hodgkin lymphoma. Here, we report a unique case of MALToma liver coexisting with invasive breast cancer and papillary carcinoma of the thyroid.
| Case Report|| |
A 71-year-old female, with no comorbidities, presented with lump in the left breast of 2 months' duration. Local examination of the breast revealed a hard mass in the left breast along with palpable axillary nodes. The initial investigation included mammography and core needle biopsy of breast lump and fine needle aspiration cytology (FNAC) of the axillary lymph node. The trucut biopsy showed the presence of infiltrating carcinoma with axillary nodal metastases. Examination of the neck revealed a nodule in thyroid region, FNAC of which showed cytoarchitectural features consistent with papillary carcinoma of the thyroid. In the setting of two proven malignancies, she underwent positron emission tomography scan for the precise staging of disease which confirmed the clinical and pathological findings. Besides this, a fluorodeoxyglucose avid, hypodense lesion in the left lobe of the liver, measuring 1.3 cm × 0.7 cm was noted. The liver function tests (LFT) were within normal limits. The patient was taken up for left modified radical mastectomy and total thyroidectomy along with intraoperative evaluation of the liver nodule. Histopathological examination confirmed infiltrating duct carcinoma breast [Figure 1]a with axillary node metastasis and papillary carcinoma thyroid [Figure 1]b with cervical nodal metastasis. Diagnostic laparoscopy with excision of the liver nodule was performed and sent for frozen section. The liver nodule measured 1.5 cm × 1 cm grossly. Microscopic examination showed portal and periportal dense infiltrate of small lymphocytes. However, no evidence of metastases was noted from either of the two proven primaries. The final opinion was deferred to permanent sections which revealed a circumscribed nodular proliferation of small lymphoid cells which had interspersed lymphoid follicles with well-formed germinal centers [Figure 1]c. The infiltrate was portocentric and showed accompanying ductular proliferation. Occasional focus showed a lymphoepithelial lesion in the bile duct epithelium [Figure 1]d. The cellular population was expansile at places. Immunohistochemistry (IHC) was performed. CD23 stained the follicular dendritic meshwork. CD20 highlighted the nodular B-cell proliferation predominantly in the follicles and showed an expansile/irregular ductulocentric arrangement [Figure 1]e. CD3 and CD5 stained the accompanying T-cell population. Bcl-2 predominantly stained the native T cells, sparing the germinal centers. Cyclin D1 expression was not seen. Occasional CD138-positive plasma cell population was seen. Kappa and lambda light chain staining was noncontributory toward clonality assessment. Ki-67 proliferation was seen expectedly in the proliferating germinal centers along with interspersed cells in the perifollicular area. Differential diagnoses of nodular-reactive lymphoid hyperplasia (synonymous with pseudolymphoma of the liver) alongside a possibility of a primary lymphoid neoplasm were considered. In view of morphological and immunohistochemical overlap between the two entities, molecular testing with immunoglobulin gene rearrangement (multiplex PCR) was performed which demonstrated a clonal B-cell population with both immunoglobulin heavy (IgH) and light chain (IgK) rearrangement. Hence, the final diagnosis of low-grade extranodal marginal zone lymphoma was made.
|Figure 1: (a) Microphotograph showing infiltrating duct carcinoma, breast (H and E, ×400). (b) Microphotograph showing papillary carcinoma, thyroid (H and E, ×400). (c) Microphotograph showing proliferation of small lymphoid cells with interspersed lymphoid follicles having well-formed germinal centers (H and E, ×100). (d) Microphotograph showing lymphoepithelial lesion in the bile duct epithelium (H and E, ×400). (e) Microphotograph showing CD20 highlighting the nodular B-cell proliferation (DAB, ×400)|
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| Discussion|| |
MALToma is a low-grade lymphoma, commonly involving the stomach in the setting of Helicobacter pylori infection. Other sites of origin being intestinal tract, lungs, head, neck, skin, thyroid, breast, and liver.,, The incidence of PHL is known to be approximately 0.016% of all NHL cases. MALToma constitutes 3% of all hepatic malignant lymphomas. 68 cases of primary MALTomas have been reported in literature till date. Mean age of presentation is the sixth decade with slight female predilection. Hepatic MALTomas are mostly incidentally detected during surgery or on imaging workup. They present as solitary masses of size ≤3 cm. Radiologically, they are hypoechoic masses on ultrasound and low-density lesions on computerized tomography. Most of the cases in literature have shown deranged LFT while our case had normal LFT profile. Etiology of MALToma is unclear. Chronic inflammation is known to be the possible trigger including chronic gastritis associated with H. pylori infection. Sjogren syndrome, Hashimoto thyroiditis, primary biliary cirrhosis, hepatitis C, and hepatitis B viral infections are other associations.,,,,, Our case was evaluated for underlying autoimmune diseases but did not reveal any significant finding to corroborate an immune-mediated etiology. There are a very few cases quoted of their association with extrahepatic malignancies, gastric and colon cancer being the most commonly associated. Identifying and diagnosing this lesion on morphology and IHC can be challenging because of the overlapping findings with florid-reactive lymphoid hyperplasia. Reactive lymphoid hyperplasia (pseudolymphoma) of the liver is also an extremely rare condition. It is a benign lesion characterized by marked polyclonal infiltration of lymphocytes forming follicles with active germinal centers. Mean age at presentation is 58 years with marked female predominance, M: F = 1:7. It has asymptomatic presentation, incidentally detected on imaging. The precise etiology in these cases is also unclear, but half the cases are known to be associated with underlying inflammation and autoimmune disorders., B-cell receptor immunoglobulin gene rearrangement can play a decisive role in separating MALToma and nodular lymphoid hyperplasia occurring in such an unusual setting and uncommon site. To the best of our knowledge and available literature, this is the first case of primary MALToma of liver in the background of dual malignancies with no autoimmune association.
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Department of Laboratory Medicine, Max Super Speciality Hospital, Saket, New Delhi - 110 017
Source of Support: None, Conflict of Interest: None