Marginal Zone Lymphoma (Low-grade B-cell Lymphoma of Mucosa-associated Lymphoid Tissue Type) of Skin and Subcutaneous Tissue

Abstract

Extranodal low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type occurs in the gastrointestinal tract, salivary gland, thyroid, orbit, lung, and breast. We report 15 patients with MALT-type lymphomas involving skin and subcutaneous tissue. All patients had tumors with histologic features of low-grade B-cell lymphoma of MALT type, including marginal zone cells (15 of 15 cases), plasmacytic differentiation (10 of 15 cases), Dutcher bodies (three of 15 cases), and reactive germinal centers (10 of 15 cases). All expressed pan B-cell antigens and monotypic immunoglobulin. Seven patients (five women, two men) aged 29 to 86 years (median, 53 years) had primary MALT-type lymphoma of skin (6) or subcutaneous tissue (1). One patient had persistent disease, and four patients had relapses involving skin, subcutaneous tissue, breast, orbit, and lymph node. At last follow-up (11-121 months; median, 36 months), one patient was alive with disease, and six patients had no evidence of disease. Three patients (two women, one man) aged 36 to 67 years (median, 57 years) had concurrent MALT-type lymphoma involving both subcutaneous tissue and extracutaneous sites at primary diagnosis, including lung, breast, orbit, lymph node, and bone marrow. One patient responded to treatment but relapsed with lymphoma of the skin and breast. The other two patients had persistent disease despite treatment. One patient died of disease at 25 months, and, at last follow-up (7 and 46 months), two patients were alive with disease. Five patients (four women and one man) aged 29 to 72 years (median, 63 years) had secondary skin or subcutaneous involvement by MALT-type lymphoma with primary tumors of ocular adnexa (3) or parotid gland (2). All five patients had relapses, which involved skin or subcutaneous tissue, parotid gland, lacrimal gland, breast, and lymph node. At last follow-up (61-137 months), two patients were alive with disease and three were alive with no evidence of disease. Low-grade B-cell lymphomas of MALT type may arise in or secondarily involve the skin and subcutaneous tissue and have a tendency to affect middle-aged to older women. These tumors are characterized by multiple extranodal relapses and are associated with long patient survival. Patients with primary MALT-type lymphoma of skin or subcutaneous tissue without extracutaneous involvement at diagnosis were more likely to experience prolonged disease-free survival than patients with extracutaneous spread at presentation (p < 0.03).

WHO-EORTC classification for cutaneous lymphomas

“…PCMZLs have a tendency to recur in the skin, but dissemination to extracutaneous sites is exceedingly rare. 131,[134][135][136] In some cases spontaneous resolution of the skin lesions may be observed. The development of anetoderma in spontaneously resolving lesions has been observed.…”

“…Monotypic plasma cells are often located at the periphery of the infiltrates and in the superficial dermis beneath the epidermis. 131,132,135,136 periodic acid-schiff (PAS) ϩ intranuclear or intracytoplasmic inclusions may be present in cases with a predominance of lymphoplasmacytoid cells. PCMZLs rarely show transformation into a diffuse large B-cell lymphoma, but a relative increase in large transformed cells can be seen in some cases.…”

“…The prognosis of PCMZL is excellent with a 5-year survival close to 100% (Table 2). [131][132][133][134][135][136] Therapy. Patients with a solitary or a few lesions can be treated with radiotherapy or surgical excision.…”

Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue

“…4 MALT lymphomas have also been described in various nongastrointestinal sites, such as salivary gland, thyroid, skin, conjunctiva, orbit, larynx, lung, breast, kidney, liver, prostate, and even in the intracranial dura. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Involvement of multiple mucosal sites is often present and disseminated disease appears to be more common in nongastrointestinal MALT lymphomas, in which one fourth of cases has been reported to present with involvement of multiple mucosal sites or nonmucosal sites such as bone marrow. 8,9,21,22 It has been postulated that this dissemination may be due to the specific expression of special homing receptors or adhesion molecules on the surface of the B cells of MALT.…”

Primary Central Nervous System Mucosa-Associated Lymphoid Tissue Lymphoma-Case Report-

“…MALT lymphomas most commonly occur in the gastrointestinal tract and lungs, 30) and also in the salivary glands, skin, thyroid, lung, ocular adnexa, liver, and breast. 3,5,8,9,20,23,32) MALT lymphomas are associated with infections (e.g. Helicobacter pylori of the stomach) and autoimmune disease (e.g.…”