Sarcoidosis presenting as a solitary parotid mass.

Abstract

We describe an unusual case of sarcoidosis in which the patient presented with a discrete solitary parotid mass and no other manifestation of the disease. The diagnosis was based on the unexpected pathologic findings during examination of a superficial parotidectomy specimen. To the best of our knowledge, no such presentation has been previously reported in the English-language literature.

Introduction

Detection of a mass of the parotid gland is generally considered an indication for superficial parotidectomy. In most cases, pathology will identify a neoplastic process. We present a case in which the patient presented with a discrete solitary parotid mass that was found to represent sarcoidosis.

Case report

A 51-year-old woman presented with a right-sided neck mass posterior to the angle of the mandible. The size of the mass had gradually increased since the patient first discovered it 6 weeks earlier. She said the mass caused occasional discomfort, but she reported no pain, tenderness, fever, or chills. Her primary care physician had prescribed a trial of antibiotics, but no improvement ensued. Findings on the review of systems had been otherwise negative.

The patient's medical history included rheumatoid arthritis in remission, hyperlipidemia, gastroesophageal reflux disease, and irritable bowel syndrome. Her surgical history included laparoscopic cholecystectomy for biliary dyskinesia, hysterectomy for endometriosis, and bilateral breast reduction surgery. She had also undergone a tonsillectomy 5 years earlier, and the specimen demonstrated amyloid deposits. One year later, she underwent an excisional biopsy of a lymph node in the right side of her neck and biopsy of a subcuticular nodule in the abdominal fat pad. Pathologic examination of both specimens revealed benign nonspecific inflammation and fibrosis. The patient's current medications included an estrogen patch, omeprazole as needed, and a vitamin [B.sub.12] injection every 2 weeks.

On physical examination, the discrete, 1-cm nodule was freely mobile. No lymphadenopathy was noted, and findings on the remainder of the physical examination were normal. Computed tomography (CT) demonstrated a 7-mm mass in the tail of the right parotid gland (figure 1). The patient underwent a superficial parotidectomy. Pathologic examination of the specimen revealed chronic inflammation and fibrosis involving an intraparotid lymph node with noncaseating granulomas (figure 2). Stains for acid-fast bacilli with Grocott-Gomori methenamine-silver nitrate were negative. On the basis of these findings, the patient was diagnosed with sarcoidosis.

[FIGURES 1-2 OMITTED]

At the most recent follow-up, the patient was under no treatment and had not manifested any symptoms of systemic sarcoidosis. Her chest x-ray remained normal.

Discussion

Sarcoidosis is a multisystem disorder of unknown etiology characterized by the accumulation of T lymphocytes, mononuclear phagocytes, and noncaseating granulomas in involved tissues. (1) Lung and thoracic lymph node involvement occurs in more than 90% of cases, but any organ system can be involved. (2) Patients typically present with cough, dyspnea, and chest pain; other vague complaints include fatigue, weakness, malaise, fever, and weight loss.

Diagnosis is based on radiographic findings of bilateral hilar lymphadenopathy, with or without parenchymal infiltrates, and is supported by the presence of non-caseating granulomas on pathologic evaluation. The serum angiotensin-converting enzyme level is elevated in 75% of untreated patients with sarcoidosis. (3) It is important to rule out infectious and other granulomatous diseases on the basis of the history and physical examination, serologic testing, and special tissue-staining techniques. The American Thoracic Society recommends that patients who are diagnosed with sarcoidosis undergo a comprehensive evaluation that specifically details the patient's occupational and environmental exposures and includes a chest x-ray, pulmonary function testing, a peripheral blood count, a serum chemistry panel (including measurement of creatinine, calcium, and liver enzyme levels), a urinalysis, an electrocardiogram, a routine ophthalmologic examination, and a tuberculin skin test. (4) These investigations will assist in staging the disease and will provide a baseline from which to evaluate the effectiveness of treatment.

Corticosteroids are the mainstay of treatment for symptomatic patients and for those with progressive disease, although the dosage and duration of treatment are not well defined; no consensus exists as to whether treatment should be given to asymptomatic patients and patients with only mild disease. (5-7) In patients refractory to corticosteroid therapy, antimalarial agents and cytotoxic agents such as methotrexate should be considered. (8,9)

Sarcoidosis is well known to affect the salivary glands, typically causing bilateral diffuse enlargement. (10) Presentation as a discrete mass is unusual, particularly in the absence of any involvement of other organ systems. (11,12) Our review of the English-language literature revealed only 1 previous report of sarcoidosis causing a parotid mass, but that patient had overt disease on chest x-ray. (13)

References

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(2.) Baughman RE Teirstein AS, Judson MA, et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001;164:1885-9.

(3.) Studdy PR, Bird R. Serum angiotensin converting enzyme in sarcoidosis--its value in present clinical practice. Ann Clin Biochem 1989;26:13-18.

(4.) Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999; 160:736-55.

(5.) Sharma OR Pulmonary sarcoidosis and corticosteroids. Am Rev Respir Dis 1993;147:1598-1600.

(6.) Hunninghake GW, Gilbert S, Pueringer R, et al. Outcome of the treatment for sarcoidosis. Am J Respir Crit Care Med 1994;149: 893-8.

(7.) Baughman R, Lower E, Lynch J. Treatment modalities for sarcoidosis. Clinical Pulmonary Medicine 1994; 1:223-31.

(8.) Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med 1997; 336:1224-34.

(9.) Lynch JP III, McCune WJ. Immunosuppressive and cytotoxic pharmacotherapy for pulmonary disorders. Am J Respir Crit Care Med 1997;155:395-420.

(10.) James DG, Sharma OR Parotid gland sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2000; 17:27-32.

(11.) Gaisford JC, Hanna DC. Salivary gland tumors and miscellaneous associated problems: Diagnosis, pathology and treatment. Plast Reconstr Surg 1957;19:458-77.

(12.) Richardson GS, Dickason WL, Gaisford JC, Hanna DC. Tumors of salivary glands. An analysis of 752 cases. Plast Reconstr Surg 1975;55:131-8.

(13.) Katz AD. Unusual lesions of the parotid gland. J Surg Oncol 1975; 7:219-35.

James T. McCormick, DO; E. Douglas Newton, MD; Stanley Geyer, MD; Philip F. Caushaj, MD

From the Department of Surgery (Dr. McCormick, Dr. Newton, and Dr. Caushaj) and the Department of Pathology (Dr. Geyer), Temple University School of Medicine, Clinical Campus at The Western Pennsylvania Hospital, Pittsburgh.

Reprint requests: Philip F. Caushaj, MD, 4800 Friendship Ave., Pittsburgh, PA 15224. Phone: [phone]; fax: [phone]; e-mail: [e-mail]

Originally presented at the annual meeting of the Pittsburgh Surgical Society; Jan. 22, 2000; Pittsburgh.