Sialoadenitis esclerosante asociada a enfermedad por IgG4: reporte de un caso / Sclerosing sialadenitis associated with IgG4 disease: case report

Las enfermedades relacionadas con IgG4 (ER-IgG4) son entidades fibroinflamatorias e inmunomediadas, caracterizadas por la afección multiorgánica, con la formación de pseudotumores que provocan lesión tisular y daño orgánico subsecuente. Se describe el caso de un paciente de 43 años que presentó sialoadenitis esclerosante y cumplió todos los criterios diagnósticos de enfermedad relacionada con IgG4.

IgG4-related diseases (IgG4-RD) are fibroinflammatory immune-mediated entities characterized by multiorgan involvement with the development of pseudotumors that cause tissue injury and subsequent organ damage. We describe the case of a 43-year-old man who presented sclerosing sialadenitis and fulfilled the diagnostic criteria for IgG4-related disease.

Kuttner’s tumour: chronic sclerosing sialadenitis- a mimicker of malignancy

Background: Kuttner’s tumour is a condition of the submandibular gland which is underreported as a distinct entity. Also called as the chronic sclerosing sialadenitis it resembles a plasmocytic and lymphocytic inflammatory process and presents as a hard and painful mass which clinically mimics a malignancy and raises significant concerns. The objective of the present study was to evaluate cases of Kuttner’s tumor and discuss its clinical and pathological aspects to distinguish it from a definite malignancy and create a grown acceptance of the presence of such an entity in our setting.Methods: We collected 170 cases of submandibular swellings and evaluated in detail the clinical and pathological aspects of eight cases out of them which were later diagnosed to as Kuttner’s tumour.Results: The age of the patients varied between 23 to 61 years (mean age 42.5 years) with 3 males and 5 females. 6 patients reported with a firm to hard painless submandibular mass (5 left sided; 3 right sided) while 2 patients experienced intermittent discomfort. The mean duration of presentation of symptoms was 5.3 months. Fine needle aspiration cytology was done in 6 cases preoperatively. Following submandibular sialadenectomy histopathology showed salivary gland tissue with preserved lobular architecture, but with marked fibrosis, acinar atrophy, and a dense lymphoplasmacytic infiltrates.Conclusions: Kuttner’s tumor may be pre operatively distinguished from a malignancy with improved imaging and a good image guided FNA Cytology saving the clinician and the patient from a lot of dilemma. However, histopathology and immunohistochemistry would be the key in establishing the diagnosis.

Review of a novel disease entity, immunoglobulin G4-related disease

Immunoglobulin G4 (IgG4)-related dacryoadenitis and sialoadenitis (IgG4-DS) are part of a multiorgan fibroinflammatory condition of unknown etiology termed IgG4-related disease (IgG4-RD), which has been recognized as a single diagnostic entity for less than 15 years. Histopathologic examination is critical for diagnosis of IgG4-RD. CD4+ T and B cells, including IgG4-expressing plasma cells, constitute the major inflammatory cell populations in IgG4-RD and are thought to cause organ damage and tissue fibrosis. Patients with IgG4-RD who have active, untreated disease exhibit significant increase of IgG4-secreting plasmablasts in the blood. Considerable insight into the immunologic mechanisms of IgG4-RD has been achieved in the last decade using novel molecular biology approaches, including next-generation and single-cell RNA sequencing. Exploring the interactions between CD4+ T cells and B lineage cells is critical for understanding the pathophysiology of IgG4-RD. Establishment of pathogenic T cell clones and identification of antigens specific to these clones constitutes the first steps in determining the pathogenesis of the disease. Herein, the clinical features and mechanistic insights regarding pathogenesis of IgG4-RD were reviewed.

Unusual Involvement of IgG4-Related Sclerosing Disease in Lacrimal and Submandibular Glands and Extraocular Muscles

Chronic sclerosing sialadenitis, also known as Kuttner tumor, is a chronic inflammatory disease of the salivary glands that is reported in a few cases in medical literature. Recent reports suggest that certain aspects of sclerosing diseases, including chronic sclerosing sialadenitis or dacryoadenitis, should be classified under immunoglobulin G4 (IgG4)-related sclerosing disease based on immunohistochemical studies. This study reports an unusual case of IgG4-related sclerosing disease appearing simultaneously in the lacrimal glands, submandibular glands, and extraocular muscles. A 56-year-old male presented with complaints of bilateral eyelid swelling and proptosis that began two years ago. Computed tomography confirmed that bilateral submandibular enlargements also existed five years ago in the subject. Orbital computed tomography and magnetic resonance imaging revealed bilateral lacrimal gland enlargement and thickening of extraocular muscles. Typical findings of chronic sclerosing dacryoadenitis were revealed upon pathologic exam of the right lacrimal gland. Immunostaining revealed numerous IgG4-positive plasma cells. Through these clinical features, we make a diagnosis of IgG4-relataed sclerosing disease in the subject.

Various Sonographic Findings of Kuttner Tumors of the Bilateral Submandibular Gland and Right Parotid Gland

Chronic sclerosing sialadenitis or a Kuttner tumor is a benign inflammatory condition characterized by firm swelling of the salivary gland. This disease may clinically mimic Sjogren's syndrome or a salivary gland origin neoplasm. Various sonographic findings in patients with a Kuttner tumor were described in previous studies. Here, we describe a rare case of a 60-year-old man presenting with a non-tender right-sided parotid mass and swelling of both submandibular glands. A pathologic examination of the lesions in the right parotid gland and both submandibular glands indicated the presence of Kuttner tumors of various sonographic appearances.

Fine Needle Aspiration Cytology of Kuttner Tumor of the Submandibular Gland: A Case Report

Kuttner tumor is a relatively uncommon benign tumor-like lesion of the salivary gland that clinically mimics neoplasm because of its presentation as a hard mass. It is also known as chronic sclerosing sialadenitis or cirrhosis of the submandibular gland. We present here the aspiration cytological findings of a case of Kuttner tumor in a 58-year old woman. The aspiration specimen of this lesion showed numerous lymphoid cells that were similar to lymph node aspirates, with several scattered benign ductal cells and eosinophilic fibrous tissue. The lymphoid cells were composed of mature bland lymphocytes and follicular center cells. Any acinar cells were not identified. The excised mass was ill-demarcated, hard and fibrotic, and it histologically exhibited an intense lymphocytic infiltration with irregular lymphoid follicle formation, dense periductal and stromal sclerosis, and loss of acini. No evidence of neoplasm or sialolithiasis was discovered, and this led to the diagnosis of Kuttner tumor.

Sialoadenite esclerosante crônica (tumor de Kuttner): relato de caso clínico / Chronic sclerosing sialadenitis (Kuttner tumour): clinical case report

A sialoadenite esclerosante (tumor de Kuttner) representa um processo inflamatório crônico incomum de glândula salivar, que acomete com maior freqüência a glândula submandibular. O objetivo deste trabalho é relatar o caso clínico de um paciente que apresentou sialoadenite esclerosante crônica associada a quadro de sialolitíase. Essa entidade patológica é pouco freqüente, clinicamente pode ser confundida com uma neoplasia e, embora seja resolvida com a exérese da lesão, causa transtornos importantes ao paciente, como dor intensa, disfonia e disfagia.

Chronic sclerosing sialadenitis (Kuttner tumour) is an unusual chronic inflammatory process of the salivary gland that affects principally the submandibular gland. The aim of this work is to report a case of chronic sclerosing sialadenits associated with sialolithiasis. Kuttner tumour is an uncommon entity that clinically may not be distinguished from neoplasia and though it can be resolved by surgical excision of the lesion, it causes important upset for the patient, as intense pain, dysphonia and dysphagia.