Primary Salivary Gland Lymphoma: A Clinicopathologic Study of 23 Cases in Taiwan

Abstract

Twenty-three patients with primary salivary gland lymphoma were diagnosed between 1990 and 2001. The sites of involvement were the parotid gland in 13, the submandibular gland in 9 and the minor salivary gland in 1. The sites of lymphoma involvement beyond the salivary glands were the cervical lymph nodes in 7, bone marrow in 3, the axillary lymph nodes in 3, the nasopharynx in 2, the abdominal lymph nodes in 2, the palate, the subconjunctiva, and the spleen in 1 each patient. Histologically, 19 patients had lymphomas of mucosa-associated lymphoid tissue (MALT) with myoepithelial sialadenitis in 13, 3 patients had diffuse large cell lymphomas and 1 had follicular lymphoma. Six patients were in stage I, 4 in II, 1 in III and 12 in IV. Eight of 23 patients (35%) had autoimmune diseases before or after the diagnosis of NHL and all suffered from MALT lymphoma. Four patients with parotid MALT lymphoma had primary or secondary Sjögren’s syndrome. One each patient suffered from hyperthyroidism, systemic lupus erythematosus, membranoproliferative glomerulonephritis and cryoglobulinemia, respectively. All the 6 stage I patients had achieved complete remission (CR) without relapses 17–84 months (median 44 months) after treatment. Excluding a stage IV patient with follicular lymphoma who died at 3.5 months without treatment, CR was achieved in all of the remaining 16 patients. However, a high relapse rate (9/16, 56%) was noted in stage II–IV patients. These patients tended to relapse in the original sites, but achieved CR again after chemotherapy or radiotherapy. One patient with MALT lymphoma developed histologic transformation into diffuse large lymphoma during relapse and died of refractory disease. Overall, only 2 patients succumbed. The overall survival and relapse-free survival rates at 5 years were 94.7 and 51.4%, respectively. Thus, salivary gland lymphoma proved to be an indolent disease.

Salivary Gland Lymphoproliferative Disorders: A Canadian Tertiary Center Experience

“…This is in keeping with other studies that have reported a rate of 8-46 % of primary SG MALT-L patients with Sjogren's syndrome [10][11][12][13]. None of our lesions were bilateral.…”

“…Furthermore, the SG can be a secondary extranodal site of involvement for patients with systemic lymphoma. Primary salivary gland lymphomas usually occur in the parotid gland, can be bilateral, and most are classified as extranodal marginal-zone B-cell lymphoma, MALT-type (MALT-L) [2,[10][11][12][13]. Sjogren's syndrome, a chronic immune mediated disease localized to the exocrine glands that produce tears and saliva, is a well accepted risk factor for acquiring salivary gland lymphoma [14,15].…”

US of the Major Salivary Glands: Anatomy and Spatial Relationships, Pathologic Conditions, and Pitfalls

“…However, primary involvement of salivary glands is rare; they are usually one of the sites of systemic disease. Clinically, salivary lymphomas most often manifest as a painless, progressive swelling (107,108). They are usually associated with autoimmune disease, most often with Sjö gren syndrome, sometimes also with rheumatoid arthritis (62)(63)(64)109).…”

Extranodal Marginal Zone Lymphoma of the Parotid Gland

“…For this reason, it is not suspected clinically before FNAB or parotidectomy. Primary salivary gland lymphomas show parenchymal involvement of the parotid gland without lymph node differentiation [6,7]. Our case presented with slow growing, painless mass without symptom on the right parotid region.…”