Parotid Gland Involvement Research Articles

Chronic sclerosing sialadenitis of the parotid gland mimicking a neoplasm

Chronic sclerosing sialadenitis or Küttner tumor is a benign chronic inflammatory disease of the salivary gland, most commonly involving the submandibular salivary gland in middle aged adults. We describe the magnetic resonance imaging features of a rare case of Küttner tumor with isolated parotid gland involvement in a child, to emphasize on the need for increased awareness among radiologists regarding the possibility and imaging features of this condition, and to enable its timely diagnosis and appropriate management.

Impact of Adjuvant Interferon Therapy on Survival Outcomes for Cutaneous Melanoma With Parotid Involvement.

To determine the relative 5-year overall survival (OS) and 5-year recurrence-free survival (RFS) outcomes for adjuvant interferon therapy in the treatment of head and neck cutaneous melanoma (HNCM) with parotid gland involvement. A retrospective cohort study was conducted at a single tertiary care institution to analyze patients undergoing parotidectomy for cutaneous head and neck melanoma involving the parotid gland from 2000 to 2014. Time-to-event analyses were performed using Kaplan-Meier curves with log-rank p-values and Cox proportional hazards models. The sample consisted of 82 patients who underwent surgical resection of stage III HNCM with parotid involvement. The mean follow-up was 67.8 months (SD 65) after diagnosis. Twenty-one patients received adjuvant interferon therapy, 12 patients received adjuvant radiation therapy, and 49 patients received no adjuvant therapy. Crude 5-year OS rates were 95.0% for interferon therapy, 33.3% for adjuvant RT, and 40.4% for no adjuvant therapy. Crude 5-year RFS rates were 75.2%, 19.5%, and 40.8% respectively. In the fully adjusted model, adjuvant interferon therapy was associated with improved 5-year OS compared to adjuvant RT (HR 0.10, 95% CI 0.011-0.837; p = 0.034). There was no significant association between adjuvant interferon therapy and 5-year RFS in the fully adjusted model. Adjuvant interferon therapy for surgically resected stage III cutaneous melanoma with parotid gland involvement may be associated with improved survival outcomes. These findings support the growing evidence for the use of immunotherapy in melanoma, and potentially a unique role for when melanoma involves the lymphatic-rich parotid gland. 3 Laryngoscope, 2024.

Comprehensive management and classification of first branchial cleft anomalies: An International Pediatric Otolaryngology Group (IPOG) consensus statement

ObjectiveFirst branchial cleft anomalies are rare congenital head and neck lesions. Literature pertaining to classification, work up and surgical treatment of these lesions is limited and, in some instances, contradictory. The goal of this work is to provide refinement of the classification system of these lesions and to provide guidance for clinicians to aid in the comprehensive management of children with first branchial cleft anomalies. Materials and methodsDelphi method survey of expert opinion under the direction of the International Pediatric Otolaryngology Group (IPOG) was conducted to generate recommendations for the definition and management of first branchial cleft anomalies. The recommendations are the result of expert consensus and critical review of the literature. ResultsConsensus recommendations include evaluation and diagnostic considerations for children with first branchial cleft anomalies as well as recommendations for surgical management. The current Work classification system was reviewed, and modifications were made to it to provide a more cogent categorization of these lesions. ConclusionThe mission of the International Pediatric Otolaryngology Group (IPOG) is to develop expertise-based recommendations based on review of the literature for the management of pediatric otolaryngologic disorders. These consensus recommendations are aimed at improving care of children presenting with first branchial cleft anomalies. Here we present a revised classification system based on parotid gland involvement, with a focus on avoiding stratification based on germ layer, in addition to guidelines for management.

Unusual Presentation of Extra Pulmonary Tuberculosis in Middle aged Female: A Unique Case Report

Pulmonary tuberculosis (TB) is a leading cause of morbidity and mortality among all infectious diseases especially in Pakistan where TB is endemic while extra pulmonary TB often encountered. However, primary salivary gland specifically unilateral parotid gland involvement is exceedingly uncommon. We present a case of 38-year old woman with six months duration of right parotid lump. The lump was firm and non-tender with bilateral cervical lymphadenopathy. No other constitutional symptoms of TB in our patient except weight loss. We presumed it a parotid neoplasm but after workup it reveals parotid TB which responds to first line anti-tuberculous therapy (ATT). So, careful exclusion of parotid neoplasm prevented unwanted surgery in our patient and patient recovered without any residual disease. We proposed that the differential diagnosis of a parotid gland swelling should include the infrequent possibility of parotid gland tuberculosis.

Integrative approach to diagnosis of lymphoproliferative disease during long-term Sjogren’s disease. Clinical observation

During long-term autoimmune processes, most commonly in patients with Sjogren’s disease (SD), lymphoproliferative disorders (LPD) can develop. The risk of mucosa-associated lymphoid tissue lymphoma (MALT lymphoma) with involvement of parotid salivary glands is increased 1,000-fold compared to other immunomorphological variants. Some studies show that the main factors predisposing patients with SD to LPD are long-term enlargement of parotid salivary glands, generalized lymphadenopathy, cryoglobulinemic purpura. Application of an integrative approach to LDP diagnosis in patients with SD, identification of high-risk group for this malignant neoplasm promote its early detection.A clinical observation of a female patients with long-term SD which was diagnosed along with MALT lymphoma using an integrative approach to diagnosis of both diseases is presented.

Common Tumor in an uncommon location: Pleomorphic Adenoma of nasal cavity - a case Report.

Pleomorphic adenoma (PA) is the most common benign salivary gland tumor of which parotid gland involvement is the most common. PA may arise from minor salivary glands as well, however, PA is very rare in the sinonasal and nasopharyngeal areas. It usually affects middle aged females. They are frequently misdiagnosed due to high cellularity and myxoid stroma, leading to delay in diagnosis & further appropriate management. Here we present a case report of a female who presented with gradually progressive nasal obstruction, on examination found to have a nasal mass in right nasal cavity. Imaging was done and the nasal mass was excised. Histopathological report revealed a PA. Common tumor in an uncommon location: Pleomorphic adenoma of the nasal cavity - a case report.

A Rare Case of Adenoid Cystic Carcinoma of the External Auditory Canal

Malignancy of the external auditory canal (EAC) is rare, and adenoid cystic carcinoma (ACC) of EAC is even rarer. It is generally managed by radical resection with a clear oncological margin, followed by radiotherapy. However, local recurrence and distant metastasis are still the major concerns during follow-up. The authors present a case in a 32-year-old female, presenting with a mass in the EAC which was endoscopically removed. Histopathological examination revealed an ACC. Further imaging confirmed absence of distant metastasis and parotid gland involvement. The patient was treated with right modified lateral temporal bone resection and achieved clear surgical margins and thus planned for a long-term follow-up.

Metastatic retinoblastoma at presentation: Clinical presentation, treatment, and outcomes.

The aim of this study was to retrospectively determine clinical features, treatment outcomes, and overall survival in four patients with metastatic retinoblastoma at presentation. The mean age at diagnosis was 63 months (range: 24-108 months). Three patients had overt orbital disease of at least one eye and one patient had microscopic orbital disease with scleral infiltration on histopathology. Metastatic sites included regional lymph nodes (RLN) (n = 4), bone marrow (BM) (n = 2), and cerebrospinal fluid (CSF) (n = 1). The most common sites of RLN were ipsilateral preauricular nodes (two patients) and contralateral parotid gland involvement (one patient). The treatment administered included primary enucleation (n = 1), high-dose intravenous chemotherapy (n = 4), secondary enucleation (n = 2), orbital external beam radiotherapy (n = 3), and intrathecal chemotherapy (n = 1). High-risk features included massive choroidal and microscopic scleral infiltration in the eye that underwent primary enucleation. At a mean follow-up of 33 months (range, 4-68 months), one patient with CSF involvement deceased in 4 months. The remaining three patients were alive and disease-free at the last mean follow-up period of 43 months (range, 18-68 months). The results of our study showed that RLN and BM metastasis respond well to treatment while CSF metastasis is associated with poor prognosis.

Diagnostic performance of gray-scale ultrasound and shear wave elastography in assessing salivary gland involvement in patients with primary Sjögren's syndrome.

To investigate the diagnostic performance of gray-scale ultrasound (US) and shear wave elastography (SWE) for determining salivary gland involvement primary Sjögren's syndrome (pSS). In this prospective study, the salivary glands of 72 healthy volunteers and 74 participants with pSS were examined by two blinded radiologists with consensus using gray-scale US and SWE. SWE parameters were compared between groups. The area under the curve (AUC), sensitivity, and specificity of gray-scale US and SWE was analyzed. The correlation between SWE and clinical findings was investigated. The SWE parameters of the parotid and submandibular glands were significantly higher in the pSS group, but did not differ significantly based on serologic assays, Schirmer test, minor salivary gland biopsy, and comorbidities. The AUC values for gray-scale US of the salivary glands were significantly lower than the AUC values for SWE. The elasticity modulus (kPa) of parotid gland had the highest AUC value (0.937; 95% CI, 0.901-0.973), with a sensitivity of 93.2% and a specificity of 83.3%. SWE had no correlation with age, disease duration, laboratory values, or disease activity. SWE provides excellent diagnostic performance for submandibular and parotid gland involvement in pSS and can be used to complement gray-scale US.

Symptom Outcomes After Sialendoscopy-Assisted Salivary Duct Surgery: A Prospective 6-Year Study.

The objective is to evaluate the long-term impact of sialendoscopic-assisted salivary duct surgery (SASDS) on sialadenitis symptoms using the Chronic Obstructive Sialadenitis Symptoms (COSS) questionnaire. The COSS questionnaire, which scores symptoms on a 0-100 scale, was administered prospectively to adult patients pre-operatively, 1-year,and 6-years post-operatively. We examined COSS scores and categories representing complete (<10), partial (10-25), and no (>25) resolution of symptoms with attention to factors significantly associated with incomplete resolution (≥10). Approximately 6-years after SASDS, 111 patients reported scores for 128 symptomatic glands (72 with sialolithiasis, 56 without sialolithiasis). For glands with sialolithiasis, the median COSS score pre-SASDS was 27.5 (interquartile range [IQR]: 13.5-43), which was significantly reduced to 1.0 (IQR: 0-5.5) at 1-year and 1.5 (IQR 0-5) at 6-years postoperatively. Glands without sialolithiasis had a median COSS score of 40.5 (IQR: 23-52.5) preoperatively, that significantly reduced to 13.5 (IQR 5-21) at 1-year and 14 (IQR 6.5-25.5) at 6-years post-operatively. There was no significant difference in scores from 1- to 6-years. Intraoperative presence of sialolith, absence of stenosis, stenosis in the distal duct, and submandibular gland involvement were significant predictors of complete resolution of symptoms at 6-years. Approximately 6-years after SASDS for sialadenitis, the majority of patients have durable symptom improvement. The sialolithiasis group had higher rates of complete resolution compared to the non-sialolithiasis group. Presence of ductal stenosis on sialendoscopy, non-distal stenoses, and parotid gland involvement were risk factors for persistent long-term sialadenitis symptoms. Laryngoscope, 133:792-800, 2023.

Clinical and Pathological Features and Outcome of Patients with Salivary Gland Cancer a Single Centre Report

Introduction: Salivary gland tumors are rare tumors that account for 3 to 4% of head and neck neoplasms. These tumors may arise from 6 major and numerous minor salivary glands. This study was designed to investigate the factors affecting the prognosis of salivary gland tumors. Methods: This study is a retrospective descriptive study in which the records of patients with salivary gland tumors referred to the Radiotherapy and Oncology Center of Namazi Hospital from 2005 to 2015 were reviewed. Results: 158 patients were included in this study with a mean age of 55.37 years. 49% of the patients were men. Adenoid Cystic Carcinoma was the most common histological type (30.57%) and Adenocarcinoma with a prevalence of 1.91% had the lowest prevalence rate in this study. 65.19% of salivary gland neoplasms in this study had parotid gland involvement and sublingual gland involvement had the lowest prevalence (3.8%). The results of survival analysis clearly illustrated that surgery, pathology type and stage was statistically significant in survival. Discussion: Clinical, pathological and therapeutic factors in people with malignant salivary gland neoplasms have a significant relationship with their survival. controversies on treatment and outcome continues.

Giant Facial Nerve Schwannoma with Extra-Temporal Involvement: A Series Of Two Cases.

Facial nerve schwannoma is a rare benign tumor of temporal bone arising from the schwann cells with incidence of 0.8%. They can arise from any segment of facial nerve with geniculate ganglion being the most common and labyrinthine segment the second commonest site. Intra-temporal location is common while only 9% of the cases have extra-temporal or parotid gland involvement. Bony remodeling or scalloping of the facial canal and the surrounding bone is the classic radiological feature of schwannoma. However schwannomas of temporal bone location can show bony erosions. The management option depends on site, extent, facial nerve function and hearing status. Surgery is reserved for large tumors with poor facial functions, hearing loss and giddiness. Giant facial nerve schwannoma with extra-temporal involvement is a rare entity with only handful number of cases reported in the literature. We present a series of two cases of giant facial nerve schwannoma with parotid gland involvement.

P27 Lymphoma-mimicker of IgG4-related dacryoadenitis and sialadenitis

Case report - IntroductionMajor salivary gland (sialadenitis) and lacrimal gland (dacryoadenitis) involvement can be a common feature of IgG4-related disease. There can be involvement of lacrimal and parotid gland which was previously called as Mikulicz disease and/or submandibular gland enlargement which was previously called Küttner tumour. These were previously mistakenly considered to be subcategories of Sjogren’s syndrome, but are now classified as IgG4-related disease. Here we discuss a case report of a patient who presented with bilateral dacryoadenitis and unilateral submandibular gland enlargement which initially was thought to be IgG4-related disease but turned out to be low-grade lymphoma.Case report - Case descriptionA 15-year-old boy presented with 6-month history of bilateral eyelid swelling. The swelling was more on the lateral side of the eyelids and was painless. It had come on suddenly over a couple of days. No history of weight loss, dry eyes, dry mouth, joint issues or skin rashes or any other symptoms. Physical examination revealed bilateral ptosis, no visual impairment and systemic examination revealed an enlarged right submandibular gland. He had initially been to a local hospital where he had investigations which included autoimmune screen including ANA, ENA, ANCA, dsDNA, serum ace and complement levels which were all negative. C1 esterase inhibitor was normal. Routine bloods including complete blood count, urea and electrolytes, thyroid stimulating hormone and erythrocyte sedimentation rate were within normal limits. Urine albumin to creatinine ratio was not raised. Hepatitis serology including Hepatitis B & C and HIV was negative. Ultrasound abdomen was unremarkable. CT scan of orbits showed bilateral enlarged lacrimal glands with patchy post contrast enhancement and the glands extending up to insertion of lateral rectus muscle. CT chest some enlarged axillary lymph nodes and nodes in lung query infective etiology. CT abdomen and pelvis was unremarkable. Ultrasound neck showed right submandibular node enlargement with colour doppler showing increased vascularity. Fine needle aspirate of the submandibular gland showed reactive lymphoid hyperplasia. He was given two short courses of steroids and each time the swelling rapidly responded to the steroids but recurred on cessation of the steroids. IgG subset analyses revealed elevated IgG4 levels of 1152mg/dl. The differential here was IgG4-related disease but as there was no clear tissue diagnosis a core biopsy of the right submandibular gland was done. This revealed tissue suspicious of low grade (extranodal marginal zone and mucosa-associated lymphoid tissue [MALT]) lymphoma and excision biopsy was performed for definitive diagnosis.Case report - DiscussionIgG4-related disease is an immune mediated fibroinflammatory condition which can affect a variety of organs and can present as tumour-like enlargement and/or organ dysfunction. The pathological findings in IgG4-related disease are lymphoplasmocytic infiltrates of IgG4-positive cells along with increased levels of serum IgG4 levels. Salivary and lacrimal glands can be commonly affected and present as enlargement, which is usually painless and bilateral. The combination of lacrimal gland enlargement with both parotid and submandibular gland enlargement is called IgG4-related Mikulicz disease.Apart from salivary glands, another commonly affected organ is the pancreas which can present as a pancreatic mass and painless jaundice, sclerosing cholangitis, retroperitoneal fibrosis, aortitis and periaortitis. Less commonly it can affect thyroid, kidney and lungs.Early recognition, diagnoses and treatment is important due to the fibroinflammatory nature of the disease. Malignancy is always in the differential and should be excluded. Steroids are the mainstay of treatment. If patients experience flare, rituximab can be added.Diagnosis should be confirmed with biopsy but histopathological findings are never alone diagnostic of IgG4-related disease and should be interpreted with clinical, serological and radiological findings.Case report - Key learning pointsAlthough this patient had typical presentation of IgG4-related disease with painless enlargement of salivary and lacrimal glands and elevated IgG4 serum levels, biopsy was imperative to get to the diagnoses of low-grade lymphoma and fine needle aspirate was not adequate. As mentioned above, biopsy in IgG4-related disease will confirm the diagnosis provided there are other supporting features (radiological and serological). However, it is imperative for excluding other important diseases like lymphoproliferative disorders.

Tuberculosis of the parotid gland associated with lower cranial neuropathy

Introduction: Parotid gland involvement as a form of extra pulmonary tuberculosis is a very rare manifestation. Extension of tuberculosis to skull base with multiple cranial nerve involvement is even rarer. We report a case of atypical presentation of tuberculosis with multiple cranial nerve palsies.

Bilateral parotid gland enlargement as a presenting manifestation of relapsed pediatric acute myeloid leukemia

Parotid gland involvement is a rare presentation of Acute Myeloid Leukemia (AML). We report a 10-year-old girl of acute myelomonocytic leukemia with normal cytogenetics and positivity for inversion 16, who after completion of first consolidation of the BFM 2004 AML protocol, presented with bilateral parotid gland enlargement. Bone marrow examination was suggestive of a relapse. Fine needle aspiration of the parotid gland showed presence of myeloblasts. Patient was given FLAG-IDA chemotherapy, with which the parotid swellings rapidly regressed and she achieved a remission and was planned for allogenic bone marrow transplantation. Acute myelomonocytic and monoblastic leukemias are known to be associated with tissue infiltration. However, exocrine gland involvement such as parotid enlargement is rare and is associated with a poor prognosis as was seen in our patient who despite having inversion 16 positivity had poor disease outcome. Keywords: acute myeloid leukemia; parotid gland enlargement; granulocytic sarcoma.

Mucoepidermoid carcinoma of the head and neck: CRTC1/3 MAML 2 translocation and its prognosticators

PurposeMucoepidermoid carcinoma (MEC) of the head and neck is a prevalent malignant salivary gland tumour with a reported good outcome. The aim of this study was to report the outcome in our centre.MethodsA retrospective chart analysis with survival analyses was performed combined with fluorescence in situ hybridization (FISH) analysis to assess CRTC1/3 MAML 2 fusion gene presence.ResultsSixty-four cases of MEC were identified. Median age at presentation was 51.4 years with a predominance for parotid gland involvement. Five, 10- and 20- year disease-free survival was 98%, 90% and 68%, respectively. Overall survival was 94%, 90% and 64%, respectively. Local recurrence was seen up to 14 years after primary diagnosis; distant metastases were diagnosed up to 17 years later. The overall recurrence rate was less than 20 per cent. CRTC1/3 MAML 2 fusion gene presence showed no survival benefit.ConclusionMEC of the head and neck has a favorable outcome with the exception of high-grade MEC. PNI and nodal involvement are not rare. CRTC1/3 MAML 2 fusion gene presence showed no survival benefit. The tendency for late onset of loco-regional and distant recurrence should not be underestimated.

Kimura's disease: a neoplasm mimicking, a rare benign form of chronic inflammatory disorder

Background: Kimura's Disease (KD) is a benign, rare form of chronic inflammatory disease of unknown origin, almost exclusively found in Asian males in their second to fourth decades of life. This rare condition is mostly present as soft tissue mass consists of multiple painless solitary subcutaneous nodules mostly localized in the head and neck region, with coexisting lymph node enlargement and eosinophilia. This case study aims to describe Kimura's Disease (KD) characteristics and give an insight into the role of Computed Tomography (CT) scan in identifying KD and its imaging characteristics.Case Presentation: We reported a case of an Asian man with a history of 18 years left-sided painless facial mass, gradually increase in size with no history of hoarseness, epistaxis, previous facial mass or malignancy, and familial malignancy. CT scan findings showed an inhomogeneous contrast enhancement soft tissue mass located on the left parotid-submandibular region with multiple neck lymphadenopathy and left parotid gland involvement. Eosinophilia was found persistently in the laboratory outcomes, and this mass had been confirmed with histological findings consistent with KD features.Conclusion: Recognizing the characteristics of KD and the imaging findings on Ultrasound and CT-scan as accessible and first-line diagnostic tools, might help in facilitating the diagnosis, making a decision and sparing the patient from unnecessary and harmful procedures.

Evaluation of salivary gland tumors- clinically radiologically and pathologically: prospective study

&lt;p&gt;&lt;strong&gt; &lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Background: &lt;/strong&gt;Salivary gland tumors are difficult to differentiate based solely on clinical presentation or cytological findings due to their overlapping pictures. Often inadequate samples from cytology pose a challenge in preoperative diagnosis. Histopathology is considered the gold standard in diagnosing these tumors. The purpose of this study was to determine clinical, radiological, and cytological findings of these tumors and to assess the accuracy of these results with that of histopathological diagnosis.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods: &lt;/strong&gt;Prospectively 52 patients with salivary gland swellings were enrolled between 2007-2009. Cases with inflammatory swelling were excluded from the study. Demographic, clinical history, preoperatively ultrasonography, cytology, and histopathological data were collected and analyzed.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results: &lt;/strong&gt;Most cases (65.38%) had parotid gland involvement. Benign tumors were common (80.76%) with pleomorphic adenoma as the most common one. Malignant tumors comprised 19.23%. Mucoepidermoid carcinoma and adenoid cystic carcinoma were common malignant tumors. Local swelling was the most common clinical presentation, and no facial nerve involvement was reported. The hard palate was the most common minor salivary gland affected predominantly by benign tumors. The diagnostic accuracy of fine-needle aspiration cytology was 96.15%, followed by clinical and radiological diagnostic accuracy of 92.31% and 86.54%, respectively. Using McNemar's test, a significant agreement was found between clinical and histological diagnosis (p=0.1336) and between FNAC and histological diagnosis (p=0.4975).&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Fine needle aspiration cytology is a highly accurate, sensitive, and specific screening technique. It is safe and reliable, though minimally invasive. Ultrasonography-guided cytology along with clinical, and radiological findings could enhance the pre-operative diagnostic accuracy in distinguishing salivary gland tumors.&lt;/p&gt;

Eosinophilic sialodochitis: An emerging atopic condition

To report the clinical characteristics of the largest single centre cohort of patients with eosinophilic sialodochitis. Analysis of data relating to 37 patients seen in a dedicated multidisciplinary clinic was performed. Demographic, clinical, haematological, cytological, histological and radiological features were collated. Response to trials of allergy treatment was assessed. Thirty-seven patients (30 female, seven male) were identified, 42% of whom were of Afro-Caribbean origin, with a mean age of 50.4years (range 28-80years). Mean symptom duration at presentation was 10years (range 2-33years). Parotid and submandibular gland involvement was equally reported. The most commonly reported symptoms were swelling (97%), itching of the overlying skin (92%), salivary gland discomfort (84%) and "string-like" mucus discharge from salivary duct orifices (76%). Twenty-three patients (62%) demonstrated atopic disease and serum IgE level elevated in 57%. All 37 patients had eosinophils present in aspirated duct contents samples while raised peripheral eosinophil count was seen in 41%. Anecdotal symptom improvement was reported with antihistamine, antileukotriene or steroid treatment. Eosinophilic sialodochitis should be considered in any patient presenting with recurrent salivary gland swelling. Further studies are needed to evaluate treatments directed at a likely allergic pathogenesis.

Differential diagnosis of immunoglobulin G4-related sialadenitis and Kimura’s disease of the salivary gland: a comparative case series

The aim of this study was to investigate key points for the differential diagnosis of immunoglobulin G4-related sialadenitis (IgG4-RS) and Kimura’s disease (KD) involving the salivary glands. The clinical, serological, radiological, histological, and immunohistochemical features of 85 IgG4-RS cases and 52 KD cases were evaluated comparatively. Seventy-two IgG4-RS cases had enlargement of multiple salivary and/or lacrimal glands; 67 patients had bilateral submandibular gland (SMG) involvement. Unilateral parotid gland involvement (59.6%) and comorbid skin lesions (61.5%) were common in KD. Serum IgG4 was elevated in 94.1% of IgG4-RS cases versus 19.0% of KD cases (cut-off value=266.5mg/dl). KD was more commonly associated with elevated eosinophil counts (86% vs 23.1%) and elevated IgE concentrations (95.5% vs 76.6%). Storiform fibrosis, irregular lymphoid follicles, and increased IgG4-positive cells (112.9±37.6/high-power field (HPF)) were common in IgG4-RS. Acellular fibrosis, regular lymphoid follicles, IgE-positive reticular networks, increased IgE-positive cells (43.4±26.7/HPF), and tryptase-positive mast cells (29.7±13.3/HPF) were usually detected in KD. Computed tomography showed that 85.7% of KD cases involved subcutaneous fat tissue. A superficial hypoechoic and reticular pattern with multiple hypoechoic foci were the sonographic features of the SMG in IgG4-RS. Despite numerous overlapping manifestations, histopathological examination showed meaningful differences in the types of fibrosis, eosinophils, and IgG4-positive cell counts. Comprehensive evaluation of clinical, serological, radiological, and histopathological features are crucial for the differential diagnosis.