Sarcoidosis presenting as a solitary parotid mass: an uncommon but real diagnostic challenge.

Editor:

I read with interest the report by McCormick et al, (1) and I would like to comment on it. Briefly, the authors described the case of a 51-year-old woman who presented with a solitary parotid mass and no other manifestation of sarcoidosis. The diagnosis was established on the basis of pathologic examination after parotidectomy.

Current diagnostic criteria. A diagnosis of sarcoidosis is based on the radiologic identification of both a distinctive lung infiltrate and a hilar lymphadenopathy. (2) A final diagnosis requires pathologic evidence of noncaseating granulomas that are typically non-necrotic, hyalinized, and small. (3) In addition, some laboratory studies--such as measurement of the serum angiotensin-converting enzyme level, which is elevated in as many as 80% of patients (4)--may help support the diagnosis. Computed tomography of the chest, pulmonary function testing, ophthalmologic examination, electrocardiography, analyses for hypercalcemia and hypercalciuria, and assays for antinuclear and antineutrophil cytoplasmic antibodies are also required.

The differential diagnosis of parotid gland enlargement includes systemic autoimmune diseases (e.g., Sjogren's syndrome), infectious diseases (e.g., tuberculosis, actinomycosis, and cat scratch disease), and a variety of neoplasms (e.g., Warthin's tumor and mucosa-associated lymphoid tissue lymphoma). (2-5) Also, known causes of granulomatous inflammation (e.g., Wegener's granulomatosis) must be ruled out. Although McCormick et al mentioned some of these diagnostic tests in the "Discussion" section, they did not mention them in the "Case report" section, so the reader does not know if these tests were performed and, if they were, what the results were.

Uncommon clinical presentation. Sarcoidosis is confined to the parotid salivary glands in as many as 6% of patients. (6) However, as many as 30% of patients with systemic disease could have parotid involvement. (5) A clinical pattern of sarcoidosis characterized by isolated granulomatous involvement of intraparotid lymphoid tissue with no parenchyma involvement and an absence of general symptoms of sarcoidosis would be extremely uncommon. This may be a clinical herald of silent sarcoidosis. (7)

Review of the English-language literature. The authors wrote, "To the best of our knowledge, no such presentation has been previously reported in the English-language literature." (1) However, their review was not complete. In 2005, my colleagues and I published a similar case?

References

(1.) McCormick JT, Newton ED, Geyer S, Caushaj PF. Sarcoidosis presenting as a solitary parotid mass. Ear Nose Throat J 2006;85: 664-5.

(2.) Mandel L, Surattanont F. Bilateral parotid swelling: A review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:221-37.

(3.) Shah UK, White JA, Gooey JE, Hybels RL. Otolaryngologic manifestations of sarcoidosis: Presentation and diagnosis. Laryngoscope 1997;107:67-75.

(4.) Baumgartner BJ, Sorensen DM, Willard CC. Pathology quiz case: Sarcoidosis of the parotid glands. Arch Otolaryngol Head Neck Surg 2002;128:195-6.

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

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

(7.) Mandel L, Kaynar A. Sialadenopathy: A clinical herald of sarcoidosis: Report of two cases. J Oral Maxillofac Surg 1994;52:1208-10.

(8.) Anton E, Lopez A, Echeverria M. Sarcoidosis presenting as isolated intraparotid lymphadenopathy. Sarcoidosis Vasc Diffuse Lung Dis 2005;22:79-80.

Enrique Anton, MD, PhD

Department of Internal Medicine

Hospital of Zumarraga

Zumarraga, Guipuzcoa, Spain

Response

We appreciate the concerns expressed by Dr. Anton regarding our case report. (1)

In explaining his first point of contention, Dr. Anton astutely notes the complexity of establishing the diagnosis of sarcoidosis. He states that the diagnosis is made by findings on radiographic imaging of the chest. However, this is not an absolute requirement for diagnosis, as evidenced by the fact that 6% of patients have disease confined to the parotid, (2) as Dr. Anton mentions in his second point of contention. The lungs and chest are involved in most cases, but not in all. Indeed, sarcoid can affect any organ system. Why the disease manifests differently among various patients is an unknown that is ripe for discovery.

The case we presented involved the finding of a discrete mass on computed tomography. Therefore, a more generic cause of parotid gland enlargement was not included in our differential diagnosis. Also, the various neoplasms that Dr. Anton discusses were not present on final pathology. Additionally, the pathology specimen did not demonstrate the vasculitis typically found in Wegener's granulomatosis. We believe that the differential diagnosis in our case was significantly more limited than what Dr. Anton suggests because the final pathology was simply not consistent with any of the other possibilities he lists.

We believe that this was an unusual case in that the sarcoidosis presented as a discrete solitary parotid mass. While we appreciate the fact that involvement can be confined to the parotid in as many as 6% of patients (2) and that as many as 30% of patients with systemic disease could have parotid involvement, (3) descriptions of presentations as a discrete mass are not prevalent in the literature. Finally, this case report was submitted for publication in 2002 and revised in 2004. The review was complete to the best of our knowledge at the time we drafted the manuscript.

We share Dr. Anton's intrigue with and bafflement over the complexity and difficulty involved in making a diagnosis of a disease as enigmatic as sarcoidosis. As is often the case, the availability of final pathology and deductive reasoning can allow the clinician to reach reasonable and solid conclusions. In writing this article, it was not our intention to answer the vast number of questions that surround this disease, but rather to raise awareness of it.

References

(1.) McCormick JT, Newton ED, Geyer S, Caushaj PF. Sarcoidosis presenting as a solitary parotid mass. Ear Nose Throat J 2006;85: 664-5.

(2.) James DG, Sharma OP. Parotid gland sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2000;17:27-32. (3.) Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med 1997; 336:1224-34.

James T. McCormick, DO

Vice-Chair and Chief, Department of Surgery

The Western Pennsylvania Hospital-Forbes

Regional Campus

Assistant Professor, Department of Surgery

Temple University School of Medicine 

Clinical Campus at the Western Pennsylvania Hospital

Pittsburgh

Philip F. Caushaj, MD, DMSc, FACS, FACG, FASCRS

Professor and Chair, Department of Surgery

Temple University School of Medicine

Clinical Campus at the Western Pennsylvania Hospital

Surgeon in Chief and General Surgery Residency Program Director

The Western Pennsylvania Hospital-Forbes Regional Campus

Adjunct Professor, Department of Surgery

University of Pittsburgh School of Medicine