[1148] Kttner's Tumor Is a IgG4 Associated Disease

J Turbiner, BZ Pilch, NL Harris, JH Stone, A Ganguly, JA Ferry, V Deshpande. Massachusetts General Hospital, Boston

Background: Autoimmune pancreatitis (AIP) is a inflammatory disease of the pancreas. Recently, the spectrum of this IgG4-associated disease has expanded to include sclerosing lesions of biliary tract, kidney, retroperitoneal soft tissue, and orbit. Some examples of Kuttner's tumor (KT) or chronic sclerosing sialadenitis have shown abundant IgG4 plasma cells; however, this association has not been reported in the Western literature. In this study we explore the association of tissue IgG4 with KT, chronic sialadenitis, and Sjgren's syndrome (SS).
Design: Ten cases of KT received between 1992 and 2008 were retrieved from the department of pathology and were compared with 16 cases of chronic sialadenitis (CS) and 8 cases of clinically proven SS collected during the same period. Immunohistochemistry for CD138, IgG and IgG4 was performed. IgG4-positive plasma cells were quantified per high power field.
Results: Six patients with KT were female and 4 were male. Their median age was 60 years (range 13-76). All 10 KT cases involved the submandibular gland, with bilateral involvement in 2 cases. Morphologically these specimens had marked sclerosis, follicular hyperplasia, and numerous plasma cells. Obliterative phlebitis was observed in 4 cases. A ductocentric pattern of inflammation was seen in one case. The histological features were reminiscent of autoimmune pancreatitis. Nine cases showed increased numbers of IgG4 plasma cells with median of 182/HPF (range 160-608). The one patient whose biopsy lacked IgG4 positive plasma cells showed evidence of cytomegalovirus infection. KT showed significantly higher number of plasma cells (p<0.05) than SS and CS. Patients with CS had a median number of 16 (range 2-62) IgG4 positive plasma cells, while SS patients had a median of 1 (range 0-2) cell.
Conclusions: KT shows significant morphological overlap with AIP, and is characterized by IgG4 positive plasma cells. IgG4 emerges as a powerful diagnostic marker for KT. KT is part of the widening spectrum of IgG4 associated diseases.
Category: Head & Neck

Tuesday, March 10, 2009 2:15 PM

Platform Session: Section H, Tuesday Afternoon