Not All Kuttner Tumors Are IgG4-Related Disease (IgG4-RD)
- 1 December 2021
- journal article
- research article
- Published by Springer Science and Business Media LLC in Head and Neck Pathology
- Vol. 15 (4), 1322-1327
- https://doi.org/10.1007/s12105-020-01268-3
Abstract
Kuttner tumor is an uncommon cause of salivary gland enlargement that most frequently affects the submandibular gland. More recently it has been considered a manifestation of IgG4-related disease (IgG4-RD) and synonymous with chronic sclerosing sialadenitis (CSS). We present a series of cases to emphasize this clinical entity as a disease pattern and presentation that is separate from IgG4-RD. Retrospective case series of 3 patients with unilateral salivary gland enlargement, or "Kuttner tumor," histologically classified as "chronic sclerosing sialadenitis," The clinical history, radiology reports, laboratory studies, and pathology slides were reviewed for each case. Radiology showed discrete unilateral mass-like lesions in all three cases. Immunohistochemistry showed reduced tissue IgG4-positive plasma cells in two cases and increased numbers in one case, but insufficient to diagnose IgG4-RD. Storiform fibrosis was not seen in all cases and did not coincide with increased IgG4-positive plasma cells. A systemic workup, including serum IgG4 levels in two cases, was normal. A brief review of the literature on the spectrum of salivary gland involvement by IgG4-RD is presented. Kuttner tumor is not necessarily the same as chronic sclerosing sialadenitis and is not always associated with IgG4-related disease. This report includes the second documented case of Kuttner tumor of the sublingual gland.This publication has 20 references indexed in Scilit:
- The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4‐Related DiseaseArthritis & Rheumatology, 2019
- Differential diagnosis of IgG4-related sialadenitis, primary Sjögren syndrome, and chronic obstructive submandibular sialadenitisBritish Journal of Oral and Maxillofacial Surgery, 2016
- International Consensus Guidance Statement on the Management and Treatment of IgG4‐Related DiseaseArthritis & Rheumatology, 2015
- Increased Immunoglobulin (Ig) G4–Positive Plasma Cell Density and IgG4/IgG Ratio Are Not Specific for IgG4-Related Disease in the SkinAmerican Journal of Clinical Pathology, 2014
- Raised Numbers of IgG4-Positive Plasma Cells Are a Common Histopathological Finding in Orbital Xanthogranulomatous DiseaseOrbit, 2013
- Consensus statement on the pathology of IgG4-related diseaseLaboratory Investigation, 2012
- Pediatric Chronic Sclerosing Sialadenitis: Küttner TumorPediatric and Developmental Pathology, 2012
- IgG4-Related DiseaseThe New England Journal of Medicine, 2012
- Chronic Sclerosing Sialadenitis (Küttner Tumor) Is an IgG4-associated DiseaseThe American Journal of Surgical Pathology, 2010
- Tumors of the submandibular gland: Clinicopathologic analysis of 23 patientsJournal of Oral and Maxillofacial Surgery, 2004