Pediatric extranodal marginal zone B-cell lymphoma presenting as amyloidosis in minor salivary glands: A case report and review of the literature

Ali Gabali, Charles W. Ross, Paul C. Edwards, Bertram Schnitzer, Theodora E. Danciu

5 Scopus citations


We report an unusual case of an extranodal marginal zone B-cell lymphoma (EMZL) arising in the labial minor salivary gland in an immunocompetent 11-year-old boy. The initial histopathologic review favored localized amyloidosis. However, further evaluation supported the diagnosis of low-grade B-cell lymphoma with plasmacytic differentiation, surrounded by deposits of AL κ-type amyloid. Clinical management consisted of excision with no recurrence at 1-year follow-up. This case demonstrates that a diagnosis of lymphoma must be considered in cases of amyloidosis associated with minor salivary gland involvement, even in children. In addition, we provide a literature review of extranodal marginal zone B-cell lymphoma arising in salivary glands.

Original languageEnglish (US)
Pages (from-to)e130-e133
JournalJournal of Pediatric Hematology/Oncology
Issue number3
StatePublished - Apr 1 2013



  • Amyloidosis
  • Immunocompetent child
  • Lymphoma
  • Salivary glands

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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