Obstructive sinonasal granuloma: a rare complication of systemic sarcoidosis

Abstract

Sarcoidosis is a granulomatous disorder which presents with pulmonary involvement in >90% of cases. Sinonasal involvement is rare, occurring in 1% of all patients with sarcoidosis. It typically presents in the setting of active pulmonary disease. Here, we present a rare case of sinonasal sarcoidosis (SNS) in a patient with latent pulmonary sarcoidosis. The patient presented to our clinic with several years of nasal obstruction, rhinorrhea and cough unresponsive to medical management. Eventually, radiographic imaging was performed, revealing a large obstructive mass in the left nasal cavity. The mass was resected, and pathology demonstrated epithelioid granulomas, consistent with sarcoidosis. The patient was subsequently placed on systemic therapy with improvement in her symptoms. In summary, SNS is a rare clinical entity that infrequently presents in patients without active pulmonary involvement. Clinicians should have a low threshold to obtain imaging in patients with sarcoidosis who present with sinonasal complaints.

Background

Sarcoidosis is a systemic granulomatous disorder characterised by the formation of non-caseating granulomas most commonly found in the lungs but can be present in any organ system.¹ In 1974, Siltzbach et al developed a staging system for this disease based on chest radiograph findings, ranging from no visible abnormality (stage 0) to end-stage fibrosis (stage V).² Since the advent of high-resolution chest CT scans, this staging system has fallen out of favour. Chest CT may show classic findings such as hilar and right paratracheal lymphadenopathy and perilymphatic upper lung zone–predominant micronodules.³ Pulmonary involvement can lead to symptoms of cough, dyspnea and chest pain.⁴ Extra-pulmonary manifestations of sarcoidosis occasionally present to the attention of otolaryngologists, with head and neck involvement occurring in 9% of cases, and sinonasal sarcoidosis (SNS) occuring in just 1% of all cases.⁵ The presentation of SNS can be quite variable and difficult to diagnose. It typically presents alongside active pulmonary disease, but in extremely rare cases, it can present in patients without any pulmonary involvement.⁶ In addition, SNS often presents a therapeutic challenge; compared with other patients with sarcoidosis, those with sinonasal involvement typically require systemic corticosteroid treatment at higher maintenance doses and symptoms are often refractory to medical management.⁶ For recalcitrant disease, sinus surgery is an effective treatment option.⁷ Here we report a unique presentation of SNS involving a giant granuloma obstructing the left nasal cavity which was successfully removed with improvement in the patient’s symptoms. Interestingly, this granuloma occurred in the absence of active pulmonary sarcoidosis.

Case presentation

The patient is a female with a medical history of hyperlipidemia, osteoarthritis, stage II breast cancer status post mastectomy and chemotherapy, severe obstructive sleep apnea, and pulmonary sarcoidosis. Her sarcoidosis was diagnosed in 2005 after mediastinal and hilar lymphadenopathy was incidentally found on chest imaging. Mediastinoscopy with biopsy confirmed the diagnosis, at which point her sarcoidosis was considered stage I (Siltzbach classification). In 2014 she developed a persistent cough, which was attributed to upper airway cough syndrome and reactive airway disease after imaging studies and pulmonary function tests showed no evidence of active pulmonary sarcoidosis. She was treated with inhaled steroids, nasal antihistamine spray, pantoprazole and a long acting β-agonist for several years. Her cough failed to improve, and in 2017 she developed left-sided nasal obstruction and yellow rhinorrhea. Intranasal steroid sprays and oral antihistamines were added to her regimen without improvement. Ultimately a CT sinus was obtained, showing a large obstructive mass in the left nasal cavity measuring 1.4×3.2×2.5 cm with bony remodelling of the medial left maxillary wall (figure 1). At this point, she was referred to Otolaryngology for further evaluation. Physical examination demonstrated cobblestoning of the mucosa in the right nasal cavity and a large mass in the left nasal cavity corresponding with the lesion seen on CT. She was advised to undergo excisional biopsy of the mass as well as endoscopic sinus surgery. Intraoperative findings revealed cobblestoning with multiple granulomas on the right middle turbinate, right inferior turbinate and right posterior septum. On the left side, a large mass originating from the inferior aspect of the left middle turbinate extending laterally to the nasal sidewall, inferiorly to the floor of the nose and posteriorly to the nasopharynx was found (figure 2). The mass was resected and pathology confirmed the presence of multiple epithelioid granulomas consistent with SNS (figures 3 and 4). The patient did well postoperatively without any complications and was discharged home the same day.

Figure 1.

Coronal CT sinus without contrast demonstrating large obstructive left nasal mass measuring 1.4×3.2×2.5 cm with bony remodeling of the medial left maxillary wall.

Figure 2.

(A) Intraoperative photo of sinonasal granuloma covered in rubbery yellow secretions prior to resection. (B) Intraoperative photo of sinonasal granuloma after partial resection. It was discovered that the mass was growing off the inferior aspect of the left middle turbinate and extended to the floor, lateral nasal sidewall and nasopharynx. (C) Intraoperative photo showing multiple small sinonasal granulomas in contralateral (right) naris. G, granuloma; IT, inferior turbinate; MT, middle turbinate; S, septum.

Figure 3.

Gross pathology specimen after resection.

Figure 4.

Hematoxylin and eosin (H&E) stain of surgical specimen at 200× magnification demonstrating epithelioid granulomas (orange arrow) in a background of small lymphocytes and multinucleated giant cells (blue arrow), consistent with sarcoidosis.

At her postoperative clinic visits, the patient reported significant improvement in her cough, nasal obstruction, rhinorrhea and sense of smell. Her pulmonologist started her on prednisone 20 mg daily for 1 month followed by a taper. Once tapered, the patient was maintained on hydroxychloroquine 200 mg two times per day. Interestingly, her pulmonologist noted that there was no evidence of active sarcoidosis in other organ systems, including the lungs. At her most recent otolaryngology follow-up appointment nearly 2 years after her surgery, flexible nasopharyngoscopy revealed no evidence of recurrent granulomas.

Discussion

SNS is a rare disease occurring in 1% of patients with sarcoidosis.⁴ It typically occurs in patients with active pulmonary sarcoidosis but in extremely rare cases can present as an isolated clinical entity.^{6 7} SNS typically presents as inflammatory rhinosinusitis with symptoms of anosmia, nasal crusts, epistaxis, nasal polyps, cough and purulent rhinorrhea.^{5 7} Evaluation by an Otolaryngologist is critical when SNS is suspected. Physical examination most frequently reveals mucosal hypertrophy and purple discolouration with granulomas on the septum and/or inferior turbinates.⁷ The gold standard for diagnosis is tissue biopsy confirming the presence of non-caseating granulomas. CT helps to evaluate for the presence of bony lesions, invasion into surrounding tissues, or obstructive granulomas, as described here.

Given the rarity of this clinical entity, it may go undiagnosed or misdiagnosed. The differential for sinonasal masses is broad, and can include vascular, inflammatory, infectious, autoimmune or neoplastic etiologies. Some common benign masses include polyp, mucocele, inverted papilloma and mycetoma, while common malignant lesions include squamous cell carcinoma, esthesioneuroblastoma, adenocarcinoma, nasopharyngeal carcinoma, and mucosal melanoma.

SNS presents a significant therapeutic challenge. Local and systemic corticosteroids are first-line treatments for this condition, but high doses may be required and often do not control the disease process.⁷ Steroid-sparing immunomodulatory agents such as hydroxychloroquine are also used with variable success. For patients with demonstrated sinonasal anatomic blockages such as our patient, surgery is an effective option that has been shown to significantly improve quality of life and reduce the need for systemic therapy.⁸ However, symptomatic relapse may occur after surgery, and long-term medical therapy may be required.⁹

Learning points.

• Sinonasal sarcoidosis (SNS) is a rare clinical entity that occurs in just 1% of all patients with sarcoidosis.

• Otolaryngologists should be aware of this condition and consider it as part of the differential diagnosis in patients presenting with inflammatory rhinosinusitis, destructive sinonasal lesions or a new sinonasal mass.

• This SNS case presentation is unique in that it involved a large obstructive granuloma occurring in a patient without evidence of active pulmonary sarcoidosis, which is rarely reported in the literature.

Patient’s perspective.

Before my surgery I was not able to breathe and I had a cough for about ten years. As my condition progressed I had trouble sleeping, headaches and earaches. The cough had become constant and I could not breathe from my nose. Approximately 5 years I sought help for my constant cough from (redacted). After a couple days of testing they confirmed the original diagnosis of GERD and I was told to continue taking pantoprazole but up the dosage to two pills a day. I continued to be miserable until nasal CT scan was done finding the giant granulomas and surgery was done to remove them. The coughing stopped, the headaches stopped and I could breathe freely again. I am so grateful for the relief from coughing and getting all of the nervous stares from people and if my case could keep some one else from experiencing what I went though, I would be so happy.

Ethics statements

Patient consent for publication

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