A case of marginal zone B cell lymphoma mimicking IgG4-related dacryoadenitis and sialoadenitis

Miho Ohta, Masafumi Moriyama, Yuichi Goto, Shintaro Kawano, Akihiko Tanaka, Takashi Maehara, Sachiko Furukawa, Jun Nosuke Hayashida, Tamotsu Kiyoshima, Mayumi Shimizu, Yojiro Arinobu, Seiji Nakamura

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17 Citations (Scopus)


Background: IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease, is characterized by elevated serum IgG4 and infiltration of IgG4-positive plasma cells in glandular tissues. Recently, several studies reported both malignant lymphoma developed on the background of IgG4-associated conditions and IgG4-producing malignant lymphoma (non-IgG4-related disease). Case presentation: We report on the case of a 70-year-old man who was strongly suspected IgG4-DS because of high serum IgG4 concentration (215 mg/dl) and bilateral swelling of parotid and submandibular glands. Biopsies of cervical lymph node and a portion of submandibular gland were performed. These histopathological findings subsequently confirmed a diagnosis of marginal zone B cell lymphoma. Conclusion: Differential diagnosis of IgG4-DS is necessary from other disorders, including Sjögren's syndrome, sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, and cancer. We suggest that biopsy of swollen lesions is important for a definitive diagnosis of IgG4-DS and discuss the mechanism of development in this case.

Original languageEnglish
Article number67
JournalWorld Journal of Surgical Oncology
Issue number1
Publication statusPublished - Feb 21 2015

All Science Journal Classification (ASJC) codes

  • Surgery
  • Oncology


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