Abstract
Introduction
Submandibular gland sarcoidosis is rare and little is known about its clinical presentation besides the usual neck swelling. The aim of the study was to extract clinical knowledge on submandibular sarcoidosis from the literature.
Methods
A systematic review was performed using a search in Medline with the key-words “sarcoidosis,” “submandibular,” “submaxillary.”
Results
Forty-six articles fitting the search criteria were found, whereas 31 had to be excluded because they did not report submandibular gland sarcoidosis. Twenty cases of submandibular gland sarcoidosis were considered suitable for analysis. Almost all reported cases concerned female patients. In some cases submandibular gland’s swelling is the first and only manifestation of the disease.
Conclusion
Sarcoidosis should be considered in the differential diagnosis of all progressive and painless swellings of the submandibular gland, especially in women. Rarely, it may be the first manifestation of the disease.
1
Introduction
Sarcoidosis is a systemic granulomatous disease of unknown etiology. Almost all systems of the human body may be affected and there is a great variety in the presenting symptoms. Consequently, the range of otolaryngological manifestations of the disease is wide. Submandibular gland swelling is commonly seen in daily ear, nose, and throat outpatient clinics and the differential diagnosis ranges from infectious, traumatic, congenital, and malignant to inflammatory causes. However, sarcoidosis in this gland is rather uncommon and more often seen in parotid swelling. Thus, there are no clinical characteristics which could draw the physician’s attention to suspect more particularly sarcoidosis. The present systematic review aimed to extract the available knowledge on submandibular gland sarcoidosis to identify possible common clinical presentation characteristics. Besides the reported cases in the literature, we also added a recent case of submandibular gland sarcoidosis treated in our department.
2
Material and methods
2.1
Systematic review: materials and methods
A search was performed in MEDLINE (from 1950 to February 2009) using the terms: “sarcoidosis,” “submandibular,” “submaxillary,” and “gland.” These terms were combined using Boolean operators. Searches were restricted to English-, French-, or German-language articles. Reference lists from identified articles were searched and cross-referenced to obtain further relevant articles. The authors searched for articles of submandibular gland sarcoidosis independently and blinded from each other. Patients’ characteristics such as age, sex, affected side and organs, symptoms, investigations, as well as treatment and evolution were extracted and recorded when available. In case of discrepancies, agreement was reached through discussion. Cases of submandibular lymph node sarcoidosis without submandibular gland involvement were excluded.
2.2
Case report
A 60-year-old woman presented to our outpatient clinic with a progressive swelling of the right submandibular area for the last 6 months. She had a 2-year history of sarcoidosis limited to the chest (radiological stage 2). One year after the initiation of oral corticosteroid treatment, the patient had a complete remission of the disease, and her pneumologist had decided to interrupt it. She was otherwise healthy, and the only other noteworthy disease in her medical records was a melanoma of the lower limb, excised more than 15 years ago. Physical examination revealed an elastic, mobile, and painless mass of the right submandibular region measuring 3 × 2 cm. The rest of the ear, nose, and throat examination was normal. Cervical ultrasound showed hypoechoic lesions in both submandibular glands ( Fig. 1 ). No diagnosis could be established by fine needle aspiration biopsy since the aspirated material was insufficient for cytological examination. In order to establish a diagnosis and to rule out malignancy, a resection of the right submandibular gland was performed. The pathology revealed multiple intraglandular noncaseating granulomas ( Fig. 2 ) with multinuclear giant cells ( Fig. 3 ), compatible with a sarcoidosis. The patient was referred to her pneumologist and further workup didn’t show any other lesions. An attitude of clinical surveillance was decided.
2
Material and methods
2.1
Systematic review: materials and methods
A search was performed in MEDLINE (from 1950 to February 2009) using the terms: “sarcoidosis,” “submandibular,” “submaxillary,” and “gland.” These terms were combined using Boolean operators. Searches were restricted to English-, French-, or German-language articles. Reference lists from identified articles were searched and cross-referenced to obtain further relevant articles. The authors searched for articles of submandibular gland sarcoidosis independently and blinded from each other. Patients’ characteristics such as age, sex, affected side and organs, symptoms, investigations, as well as treatment and evolution were extracted and recorded when available. In case of discrepancies, agreement was reached through discussion. Cases of submandibular lymph node sarcoidosis without submandibular gland involvement were excluded.
2.2
Case report
A 60-year-old woman presented to our outpatient clinic with a progressive swelling of the right submandibular area for the last 6 months. She had a 2-year history of sarcoidosis limited to the chest (radiological stage 2). One year after the initiation of oral corticosteroid treatment, the patient had a complete remission of the disease, and her pneumologist had decided to interrupt it. She was otherwise healthy, and the only other noteworthy disease in her medical records was a melanoma of the lower limb, excised more than 15 years ago. Physical examination revealed an elastic, mobile, and painless mass of the right submandibular region measuring 3 × 2 cm. The rest of the ear, nose, and throat examination was normal. Cervical ultrasound showed hypoechoic lesions in both submandibular glands ( Fig. 1 ). No diagnosis could be established by fine needle aspiration biopsy since the aspirated material was insufficient for cytological examination. In order to establish a diagnosis and to rule out malignancy, a resection of the right submandibular gland was performed. The pathology revealed multiple intraglandular noncaseating granulomas ( Fig. 2 ) with multinuclear giant cells ( Fig. 3 ), compatible with a sarcoidosis. The patient was referred to her pneumologist and further workup didn’t show any other lesions. An attitude of clinical surveillance was decided.
3
Results
We identified 46 articles, of which 13 were eliminated because of the language criterion or they did not focus on sarcoidosis. Eighteen more articles were excluded as reviews with no cases of sarcoidosis reported or as reports of sarcoidosis without submandibular gland involvement (see flow chart; Fig. 4 ). Only 15 reports were fulfilling our systematic review’s inclusion criteria (see Table 1 ) . These articles were including 20 cases of submandibular gland sarcoidosis, to which our case was added (see details on Table 1 ). Most of the articles was written in English, each one of them reporting 1 or 2 cases. There was only one article reporting 4 cases .
