Sarcoidosis of the Head and Neck

Abstract

Sarcoidosis is a complex disorder that often times involves the head and neck. Despite the presence of strong clinical evidence, tissue diagnosis and imaging is needed for confirmation of the disease. Although typically managed medically, when found in the sinonasal tract or intracranially, it may necessitate the intervention of a rhinologist-skull base surgeon. This article seeks to provide a comprehensive review of head and neck sarcoidosis, as this fascinating disorder often poses a diagnostic and therapeutic challenge. A brief discussion of surgical treatment for pituitary lesions is also provided. Articles from 1997 to 2013 were selected and reviewed by three researchers utilizing the most recent literature regarding sarcoidosis in the head and neck. PubMed searches were conducted using search terms such as "sarcoidosis", "neurosarcoid", and "extra-pulmonary sarcoid", among many others. A large collection of articles was generated and reviewed by the team of authors, and appropriate information was extracted to compose a thorough and expansive review of the subject. 10-15 % of patients with sarcoidosis have head and neck manifestations. Sinonasal and pituitary sarcoidosis presents a diagnostic challenge owing to its non-specific symptoms. Although systemic steroid therapy is often the first time treatment, endoscopic surgery is commonly used to treat advanced pituitary sarcoidosis refractory to medical management. As tissue diagnosis and imaging is key, a multi-disciplinary team approach is advantageous. Our study collates the available literature on head and neck sarcoidosis to provide a comprehensive review of the subject. This provides helpful information to guide all practitioners involved in the care of these challenging patients, namely pathologists, radiologists, otolaryngologists, and skull base surgeons, in the workup and management of head and neck sarcoidosis.

Sarcoidosis from Head to Toe: What the Radiologist Needs to Know

“…Ten percent to 15% of patients with sarcoidosis have head and neck manifestations (77). Any site in the head and neck may be involved, including the orbits, sinonasal regions, pharynx, hypopharynx, salivary glands, thyroid gland, cervical nodes, and larynx (Figs 9-11) (78).…”

“…Orbital involvement may be seen in 11%-83% of patients with head and neck sarcoidosis (17,78,79). The uvea, optic nerve, lacrimal gland, extraocular muscles, orbital fat, and soft tissue may be affected (Fig 9).…”

“…Sinonasal sarcoidosis may manifest with nonspecific symptoms such as nasal obstruction and rhinosinusitis, which may result in delayed diagnosis (77,78). CT and MR imaging may show nodular thickening and enhancement in the nasal septum and turbinates.…”

Variability of Sarcoid Skin Manifestations Is Illustrated in a Patient With Multiple Concurrent Pinna Lesions

“…Skin manifestations may imitate other diseases, making sarcoidosis particularly challenging to diagnose. 2,7 Even synchronous sarcoidosis-related skin lesions in the same patient can vary greatly in appearance, as this case demonstrates. A high level of clinical suspicion for sarcoidosis is needed, and the presence of unexplained pinna lesions should prompt further investigation.…”

“…While the exact etiology of sarcoidosis is unclear, a currently favored theory states that sarcoidosis is caused by an environmental trigger in an individual with a genetic predisposition. [1][2] Certain HLA polymorphisms are associated with the development of sarcoidosis, suggesting that aberrant antigen presentation plays a role in the generation of an inappropriate immune response. [2][3] Sarcoidosis can involve almost any organ and tissue type, though there is a predilection for certain organs and anatomic sites.…”