Answer for BIR CoW 23 Apr 2023
LYMPHOEPITHELIAL CYSTS-SJOGREN’S SYNDROME
Findings
Mild enlargement both parotid glands (right > left) with complete replacement of the parenchyma by numerous cystic lesions in superficial and deep lobes of parotids. On contrast administration , enhancement of the residual parotid parenchyma noted. Few similar cystic lesions noted in bilateral submandibular glands and along bilateral mylohyoid muscles probably in sublingual Glands.A tiny cyst noted in the right lacrimal gland. Patchy diffusion restriction noted in both parotids and submandibular glands and bilateral mylohyoid muscles. IMPRESSION: Known case of benign lymphoepithelial lesion in bilateral parotids post superficial parotidectomy. Present study shows recurrence in bilateral parotid glands with additional involvement of bilateral submandibular glands and sublingual glands along mylohyoid muscles. Note: with history of dryness of mouth and eyes in the background of above mentioned findings – Sjogren’s syndrome to be considered.
FOLLOW UP: Patient tested anti SS-A - 3+ on follow up and started on treatment
Discussion
Benign lymphoepithelial lesions are mixed solid and cystic lesions that enlarge the parotid glands, and are usually associated with cervical lymph node enlargement, and nasopharyngeal lymphofollicular hyperplasia. Benign lymphoepithelial lesions refer specifically to a histopathological finding that is non-specific and seen in the context of lymphoepithelial sialadenitis (typical of Sjögren syndrome) and HIV-associated salivary gland disease. Although the most typical symptoms of Sjögren's syndrome are dry eyes and dry mouth, the clinical presentations vary widely. Approximately one-third of patients with primary Sjögren's syndrome also have diffuse parotid gland enlargement, and 80% of patients experience parotid gland enlargement during their lifetime. Parotid MRI images usually reveal multiple, mixed, hypointense, and hyperintense foci, described as a "salt and pepper appearance", which are considered strongly suggestive of Sjögren's syndrome Although multicystic parotid involvement is rare, this presentation should also trigger suspicion. The main differential diagnoses for bilateral parotid cysts, observed by imaging, are cystic Warthin tumors, HIV-related benign lymphoepithelial lesions, and branchial cysts
REFERENCES:
1. Gadodia A, Seith A, Sharma R. Unusual presentation of Sjogren syndrome: multiple parotid cysts. Ear Nose Throat J 2012;91:E17-E19.
2. Seo , Ju , Kwok , Oh . Unusual Sjögren's Syndrome with Bilateral Parotid Cysts. Arch Craniofac Surg. 2014;15(2):98-101.
3. Plaza G, Dominguez MP, Bueno A. Bilateral parotid cysts as presentation of Sjogren's syndrome. J Laryngol Otol 2003;117:151-152.
Findings
Mild enlargement both parotid glands (right > left) with complete replacement of the parenchyma by numerous cystic lesions in superficial and deep lobes of parotids. On contrast administration , enhancement of the residual parotid parenchyma noted. Few similar cystic lesions noted in bilateral submandibular glands and along bilateral mylohyoid muscles probably in sublingual Glands.A tiny cyst noted in the right lacrimal gland. Patchy diffusion restriction noted in both parotids and submandibular glands and bilateral mylohyoid muscles. IMPRESSION: Known case of benign lymphoepithelial lesion in bilateral parotids post superficial parotidectomy. Present study shows recurrence in bilateral parotid glands with additional involvement of bilateral submandibular glands and sublingual glands along mylohyoid muscles. Note: with history of dryness of mouth and eyes in the background of above mentioned findings – Sjogren’s syndrome to be considered.
FOLLOW UP: Patient tested anti SS-A - 3+ on follow up and started on treatment
Discussion
Benign lymphoepithelial lesions are mixed solid and cystic lesions that enlarge the parotid glands, and are usually associated with cervical lymph node enlargement, and nasopharyngeal lymphofollicular hyperplasia. Benign lymphoepithelial lesions refer specifically to a histopathological finding that is non-specific and seen in the context of lymphoepithelial sialadenitis (typical of Sjögren syndrome) and HIV-associated salivary gland disease. Although the most typical symptoms of Sjögren's syndrome are dry eyes and dry mouth, the clinical presentations vary widely. Approximately one-third of patients with primary Sjögren's syndrome also have diffuse parotid gland enlargement, and 80% of patients experience parotid gland enlargement during their lifetime. Parotid MRI images usually reveal multiple, mixed, hypointense, and hyperintense foci, described as a "salt and pepper appearance", which are considered strongly suggestive of Sjögren's syndrome Although multicystic parotid involvement is rare, this presentation should also trigger suspicion. The main differential diagnoses for bilateral parotid cysts, observed by imaging, are cystic Warthin tumors, HIV-related benign lymphoepithelial lesions, and branchial cysts
REFERENCES:
1. Gadodia A, Seith A, Sharma R. Unusual presentation of Sjogren syndrome: multiple parotid cysts. Ear Nose Throat J 2012;91:E17-E19.
2. Seo , Ju , Kwok , Oh . Unusual Sjögren's Syndrome with Bilateral Parotid Cysts. Arch Craniofac Surg. 2014;15(2):98-101.
3. Plaza G, Dominguez MP, Bueno A. Bilateral parotid cysts as presentation of Sjogren's syndrome. J Laryngol Otol 2003;117:151-152.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!