IgG4-related chronic sclerosing sialadenitis in a child with recurrent parotitis: a case report

Background IgG4-related disease (IgG4-RD) includes a group of immune-mediated diseases histologically characterized by lymphoplasmacytic infiltrate with a prevalence of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Autoimmune pancreatitis, sialadenitis, dacryoadenitis and retroperitoneal fibrosis are the most frequent manifestations. IgG4-related sialadenitis usually affects submandibular glands and is very rare in children. Here we report the case of IgG4-related sialadenitis in a six-year-old patient previously diagnosed as juvenile recurrent parotitis. Case presentation A six-year-old patient was referred to our Centre for left parotid swelling of 4 × 3 cm, that was tender, soft in consistency, with overlying red and warm skin. His general condition was good but he was subfebrile; general examination revealed mild enlargement of left cervical lymph nodes. In the last 2 years he had had five episodes of parotitis, diagnosed by another pediatric Center as juvenile recurrent parotitis. On ultrasound examination the left parotid gland appeared enlarged, inhomogeneous, with a colliquative intraparotid lymph node and no evidence of sialolithiasis. Laboratory tests showed an increase of white blood cells and anti-VCA IgM and IgG positivity, with anti-EBNA e anti-EA I negativity. The patient was initially treated with oral antibiotics, but after 10 days the parotid became fluctuating, requiring surgical biopsy and drainage. Postoperative course was regular, with complete remission under oral antibiotic and steroid therapy. Microbiological tests, including cultures for aerobic and anaerobic bacteria, mycobacteria and Bartonella, were negative. Surprisingly, histology showed marked fibrosis and histiocytic and lymphoplasmacellular infiltrate with polyclonal plasma cells mostly expressing IgG4 immunoglobulins. Thus, the diagnosis of IgG4 related chronic sialadenitis in recurrent parotitis and recent EBV infection was made. Conclusions IgG4-related sialadenitis is very unusual in children. Histology plays a key role in diagnosis, considering that up to 30% of patients have normal serum IgG4 levels, as shown in our case. The lack of previous histological data makes it impossible to attribute our patient’s previous episodes of parotitis to IgG4-RD, though it is a very consistent possibility.

The applicability of ultrasound in the diagnosis of inflammatory and obstructive diseases of the major salivary glands: a scoping review

Objectives: The purpose of the present scoping review was to determine the contribution of ultrasound (US) images in the diagnosis of inflammatory and obstructive diseases of the major salivary glands (MSG). Methods: A search of studies of ultrasonographic assessments of human samples was performed in several electronic databases and grey literature up to July 2021. The extracted data were the examined MSG; the diagnostic value of US (sensibility, specificity, positive and negative predictive value, accuracy); features of lesions, including number, echogenicity, echotexture, form, margins, size, posterior acoustic aspect, and location; and related clinical information, such as swelling, palpation, sensible to pain, salivation, lymph nodes, recurrence, duration, and causes. Results: After verifying the eligibility criteria, 91 articles focused on detecting inflammatory, and obstructive diseases of the MSG were gathered, with variable study designs and size samples. A wide variety of pathologies were assessed, including sialolitiasis (n = 45), acute sialadenitis (n = 30), chronic sialadenitis (n = 25), granulamatous diseases (n = 15), Kuttner’s tumor (n = 11), juvenile recurrent parotitis (n = 9), abscess (n = 7), post radiotherapy sialadenitis (n = 6), sialadenosis (n = 9), abscess (n = 7), post radiotherapy sialadenitis (n = 6), sialadenosis (n = 9), IgG4 related disease sialadenitis (n = 5), HIV-sialadenitis (n = 4), obstructive sialadenitis (n = 3), iodinated contrast-induced sialadenitis (n = 2), and pneumoparotitis (n = 1). Most studies were case reports or series of cases. Few studies exhibited data about the accuracy of US in detecting MSG diseases. Conclusions: The present scoping review concluded that US aspects of different MSG pathologies are similar but contribute to their differential diagnosis and can be considered as a valuable initial method for assessing the MSG of adults and children.

Clinicopathological study of salivary gland disorders in a tertiary care hospital, Lahore.

Objective: To describe the demographic and histological features of salivary gland pathologies at tertiary care hospital. Study Design: Retrospective Descriptive. Setting: Shalamar Institute of Health Sciences, Lahore. Period: February 2012 to February 2017. Material & Method: 17548 surgical specimens were received during this period. Among these, 50 cases were diagnosed as salivary gland disorders. Statistical analysis was carried out using SPSS version 22. Results: 50 cases were diagnosed as salivary gland disorders out of 17548 surgical resections. Salivary gland disorders were divided into non-neoplastic group (n= 15) and neoplastic group (n = 35), which was further divided into: (i) Benign tumors, (n= 26) and (ii) Malignant tumors, (n= 9). Conclusion: Salivary gland disorders are rare diseases to be encountered clinically. Chronic sialadenitis among non-neoplastic disorders, pleomorphic adenoma among benign tumor and mucoepidermoid carcinoma among malignant tumors were the commonest.

Long-Term Outcome of a cohort of 272 patients undergoing sialendoscopy

Objectives: To assess the cohort of patients undergoing sialendoscopic intervention for improvement of symptoms and gland-related quality of life at long-term follow up. Design: retrospective review of medical records with a prospective follow-up by questionnaire. Methods: All patients undergoing sialendoscopy between March 2008 and June 2020 were analyzed in detail regarding indications, technical aspects and postoperative course. To maximize follow-up, patients also received a questionnaire by e-mail. Results: 272 sialendoscopies were performed in 221 patients. Median follow-up time was 37 months. The sialendoscopies were performed in 130 patients for lithiasis, in 66 for stenosis, in 14 for recurrent parotitis of childhood, in 8 for recurrent sialadenitis of unknown origin and in 3 for radio-iodine induced sialadenitis. Complications occurred in 11 of 272 sialendoscopies (4%). Those were iatrogenic perforations, temporary lingual nerve paresthesia and swelling of the floor of the mouth. 53% of patients returned the questionnaire, for a total of 146 evaluable sialendoscopies. The majority of the responders indicated that sialendoscopy had improved their symptoms (83.6%). Salivary glands could be preserved in 89% of the responder group. The highest percentage of patients reporting residual symptoms was found in the RPC group (81.3%) and the lowest in the lithiasis group (16.2%). Besides age, no statistical differences in demographic and pathological features between the responder and non-responder groups were found, supporting generalization of the responders’ results to the entire cohort. Conclusions: This study confirms the good long-term outcomes of sialendoscopic interventions in patients with chronic sialadenitis of different etiologies and a high rate of gland preservation.

Mélange of Lymphoepithelial Lesions of Salivary Glands from a Tertiary Care Center of North East India: Diagnostic Conundrums

Background Lymphocytic infiltrates of the major salivary glands are involved in a spectrum of diseases that range from reactive to benign and malignant neoplasms. Occasionally, these pathologic entities present difficulties in the clinical and pathological diagnosis. Aim and Objective The aim of this study was to highlight the importance of meticulous cytopathological and histopathological examination (HPE) in solving the diagnostic challenges encountered in the analysis of these salivary gland lesions. Materials and Methods A retrospective analysis of salivary gland lesions was undertaken over a period of 5 years from 2013 to 2018 in the Department of Pathology at our institute. Salivary gland pathologies diagnosed either as chronic sialadenitis or reactive/benign/malignant lymphoepithelial lesions on fine-needle aspiration cytology (FNAC) and as lymphoepithelial carcinoma (LEC) were included in this study. Results A total of 86 cases of salivary gland lesions diagnosed as mentioned above were found during this period. Out of the 86 cases, 16 were subjected to HPE. Biopsy was not warranted in most of the cases diagnosed as chronic sialadenitis. HPE was concordant with the FNAC diagnoses in 13 out of the 16 cases (81.3%), with a single case misinterpreted as LEC on FNAC. Conclusion Benign and malignant lymphoepithelial lesions of salivary glands may sometimes be difficult to differentiate not only from one another on FNAC but also from other malignant lesions. FNAC is an effective tool for the diagnosis of nonneoplastic lesions, but in cases of benign lymphoepithelial lesions in the absence of salivary acini, biopsy is advisable.

POS0778 ULTRA HIGH-RESOLUTION ULTRASOUND (UHFUS) OF LABIAL GLANDS IS A STRONG PREDICTOR OF SALIVARY GLAND HISTOPATHOLOGY IN SJÖGREN’S SYNDROME (PSS)

Background:Last-generation ultra high-resolution ultrasound (UHFUS) transducers, producing frequencies up to 70 MHz and achieving tissue resolution up to 30 μm, are opening up new possibilities for the study of labial salivary glands (LSG) in patients clinically suspected with primary Sjögren’s syndrome (pSS).Objectives:To explore the value of LSG-UHFUS as a predictor of the intensity of the histological inflammation in LSG biopsy in an inception cohort of patients with sicca symptoms derived from daily clinical practice.Methods:Consecutive patients undergoing a LSG for clinically suspected pSS were included in this study from January 2018 to October 2020. UHFUS of LSG was performed by using VEVO MD, equipped with a 70 MHz probe, scanning first the central compartment of the inferior lip, and then both peripheral compartments. Parenchymal homogeneity was graded on a scale of 0 (normal) to 3 (evident). UHFUS imaging was used to locate the LSG for the US-guided biopsy. For each of the LSG specimens the area of the glandular tissue, the presence and number of foci and the focus score (FS) were assessed. Immunostaining for CD21 was performed to evaluate the presence of follicular dendritic cells (FDC) in foci.Results:We included a total of 171 patients with suspected pSS: out of them, 83 (48.5%) received a diagnosis of pSS (ACR 2016 criteria) and 88 (51.5 %) were diagnosed as no-SS sicca controls. Out of the 171 LSG biopsies, 73/171 (42.7%) were characterized by a nonspecific chronic sialadenitis (NSCS) whereas a focal lymphocytic sialadenitis (FLS) was described in the remaining 98/171 (57.3%). UHFUS-LSG scores were differently distributed in pSS patients with respect to no-SS sicca controls: a score 0 was detected in 16/88 no-SS sicca controls and in none of the 83 pSS patients, whereas a score 3 was detected in 12/83 pSS and in none of no-SS sicca controls. (p<0.001). The higher was the UHFUS grading, the higher was the number of foci (from a mean (S.D) = 0.38 (0.72) in UHFUS-score 0 to 4.58 (3.11) in UHFUS-score 3, p<0.001) and the focus score (from 0.17 (0.32) in UHFUS-score 0 to 1.88 (0.90) in UHFUS-score 3, p<0.001) of the samples. Overall, out of the 16 no-SS controls with LSG-UHFUS score 0, only 4/16 showed histological features of focal lymphocytic infiltrate; however, the mean FS of those 4/16 samples was 0.17 (0.32). In fact, considering a FS≥1, negative UHFUS-LSG was highly predictive of a negative histology (100 %). By contrast, out of the 12 pSS patients with the highest score at LSG-UHFUS (score 3), 10/12 presented FDC networks in their biopsies, with a positive predictive value of 83% of the severity of the infiltrate.Conclusion:UHFUS of LSG appeared feasible and sensitive in pSS; due to its striking negative predictive value this novel tool can help to identify negative patients on subsequent lip biopsy, thus avoiding invasive procedures in selected cases. Besides its diagnostic role, considering the association with the presence of FDC networks, UHFUS-LGS may also have a role in patients prognostic stratification.Disclosure of Interests:None declared

CLINICOPATHOLOGICAL AUDIT OF SUBMANDIBULAR GLAND SURGERY

Aim:- Aim of this study is to evaluate clinical profile and histopathological findings in patient undergoing submandibular gland excision (due to sialadenitis and primary tumors of submandibular gland benign or malignant). Method:-we carried out retrospective analysis of submandibular gland excisions performed in our hospital over five year period and analysed clinical data and postoperative histopathological findings of excised glands. Results:- In 43 cases of submandibular gland excision main presenting symptom was swelling of submandibular gland (100%). Patients presented with pain 26 (60%), swelling 43 (100%), recurrent swelling 30 (70%), persistent swelling 13 (30%). On palpation, firm consistency was seen in 42 cases (97.67%) and hard consistency in 1 case (2.3%). Swelling was tender in 20 Cases (46.5%) and non tender in 23 cases (53.4%). Most frequent indication of submandibular gland excision was sialadenitis with sialolithiasis (37 patients, 86%). Neoplastic pathology was present in 6 patients (14%). Benign pathology (4 patients, 9.3%) was more common than malignancy (2 patients, 4.7%). Most common benign tumour was pleomorphic adenoma of submandibular gland. Among the malignant tumour 2 cases were identified in histopathogy, one of adenoid cystic carcinoma and 1 of acinic cell carcinoma of submandibular gland. Overall benign/malignant ratio was 2:1.Conclusion:- Inflammatory submandibular gland swelling are mainly enlarged and tender while neolastic swellings are non tender. The most common pathology of the patients undergoing submandibular gland excision was chronic sialadenitis followed by neoplastic Pathology. In neoplastic pathologies benign tumors were more common than malignancy

Histopathological spectrum of salivary gland lesions - in a tertiary care centre

ABSTRACT Background: Histopathology is the most important means in diagnosis of salivary gland lesions. The objective of this study is, to study occurrence of salivary lesions during a period of one year from January 2019 to December 2019, to study age, sex and site distribution of various salivary gland lesions, to study histo-morphological aspect of these lesions.Methods: Surgically resected salivary gland specimens received at Department of Pathology, Government Stanley Medical College, Chennai were subjected to histopathological examination. Specimens were fixed in 10% neutral buffered formalin, processed, embedded, sections cut and stained with hematoxylin and eosin stain.Results: There were a total of 30 salivary gland specimens. Out of thirty, 25 were neoplastic and 5 were non-neoplastic. Pleomorphic adenoma was the commonest of all benign tumors. Mucoepidermoid carcinoma was the most common malignant tumor. Among the 21 benign tumors, 16 cases of pleomorphic adenoma and a case each of myoepithelioma, benign metastasising pleomorphic adenoma, basal cell adenoma, warthins tumor and schwannoma were found. Among the 4 malignant tumors, 2 cases of mucoepidermoid carcinoma, a case of carcinoma ex pleomorphic adenoma and a case of myoepithelial carcinoma were found. Among the 5 non neoplastic lesions a case each of chronic sialadenitis, Sjogren’s syndrome, inflammatory myofibroblastioc tumor, parotid abscess and lymphoepithelial cyst were found.Conclusions: The diagnosis of salivary gland lesions by histopathological examination is the most important method in diagnosis and differentiating benign and malignant lesions and to predict prognosis by typing, staging and grading of malignant neoplasms.

HISTOPATHOLOGICAL SPECTRUM OF SALIVARY GLAND LESIONS WITH CLINICAL CORRELATION

Salivary gland tumours account for 2-6.5% of all the neoplasms of the Head and Neck. Histopathology is most important in diagnosis of salivary gland tumors. AIMS and OBJECTIVES : To study age,sex and site distribution of various salivary gland lesions, to study the histomorphologic (gross &microscopic) aspect of these lesions to correlate clinical diagnosis with that of histopathologic features. MATERIALS AND METHODS: The study is conducted in the RAINBOW CLINICAL LABORATORY AND RESEARCH CENTRE, SIDDIPET during the period of September2018 to September 2020. Formalin fixed ,paraffin embedded sections and stained with hematoxylin and eosin slides were studied. RESULTS: Total number of specimens were 54. Out of these 39 were neoplastic (benign 70%,malignant 30%) and 15 were non neoplastic. Among the benign tumors majority are pleomorphic adenoma.Among the malignant tumors, mucoepidermoid carcinoma is the most common. Among the non neoplastic lesions chronic sialadenitis is most common. CONCLUSION: Histopathological examination of salivary gland lesions stands out to be the most important method in establishing the final diagnosis. It helps in differentiating non neoplastic and neoplastic lesions.