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Rosai-Dorfman disease presenting as an isolated extranodal mass of the carotid sheath: a case report.


Rosai-Dorfman disease is a rare, benign granulomatous disease that typically presents with massive cervical lymphadenopathy. In less than 50% of cases, other soft-tissue manifestations may also be found in the head and neck. Rosai-Dorfman disease can be difficult to diagnose because of its rarity and its ability to mimic, both clinically and radiologically, more common diseases such as lymphoma. The histopathologic diagnosis can also be difficult to make, particularly when the disease exhibits extranodal manifestations. We present a case of isolated extranodal Rosai-Dorfman disease involving the carotid sheath, without the typical massive adenopathy.


Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare, benign, systemic, granulomatous disease. It has been classified as a reactive disorder, although no infectious or immunologic agent has yet been identified. (1) It is most commonly found in young adults, with a mean age at onset of 20 years and a slightly higher prevalence in males. (1,2) RDD is usually characterized by the presence of painless, massive cervical lymphadenopathy (LAD), which is most commonly bilateral and may be so large as to cause respiratory compromise. (1,3) Axillary and inguinal LAD are less frequently found. (2) Other systemic symptoms include the presence of fever, malaise, and weight loss, none of which were observed in our patient. (2-5) Abnormal serologic findings may include hypergammaglobulinemia, elevated erythrocyte sedimentation rate, elevated white blood cell (WBC) count, and anemia, although these are more commonly found in patients with massive LAD. (3,4,6,7)

Approximately 43 % of patients with typical nodal RDD also have extranodal involvement; this is probably more common in patients with immune dysfunction) Extranodal RDD most frequently (75%) occurs in the head and neck and, particularly, in the paranasal sinuses, salivary glands, oral cavity, and pharynx. (1,3,7-10) Isolated extranodal involvement without massive LAD, as seen in our patient, is an uncommon presentation of RDD. (1,3) Isolated extranodal RDD has been previously described and involved the sinonasal region, orbit, thyroid, and brain. (1,3,11) In all cases, the diagnosis has been difficult to make clinically, radiologically, and pathologically. The absence of massive LAD makes clinical diagnosis difficult because of the nonspecific nature of symptoms and laboratory findings. In addition, the radiologic appearance of nodal or extranodal RDD on computed tomography and magnetic resonance imaging (MRI) mimics other, more common, entities such as lymphoma. In the current case, the infiltrative nature of this mass along the carotid sheath suggested an extranodal process but revealed no additional specific findings.

Case Report

A 42-year-old white man presented with a 4-month history of a progressively enlarging left neck mass and a recent history of neck pain elicited when he tilted his head to the left. The neck pain did not respond to nonsteroidal anti-inflammatory medications. The patient reported some odynophagia but no dysphagia, and he had experienced no significant weight loss or voice changes. He was a moderate smoker, occasionally drank alcohol, and had no recent history of foreign travel.

Physical examination was notable for a large, firm 4 x 3-cm neck mass located along the anterior border of the left sternocleidomastoid muscle. The mass was mobile, nonpulsatile, and nontender to palpation. There were no palpable lymph nodes. On endoscopic examination, a right posterior nasopharyngeal mass was also observed. As the nasopharyngeal and left neck masses were suggestive of neoplasia, fine-needle aspiration biopsy (FNAB) of the left neck mass and MR/were ordered to further determine the nature of these lesions. The patient's WBC count and liver function tests were normal, and his purified protein derivative test was negative.


FNAB of the left neck mass revealed a lymphocytic aspirate suggestive of a reactive lymph node, but it also showed atypical features. For example, the smear showed a mixed population of lymphocytes and numerous sinus histiocytes, and some histiocytes appeared to have abundant cytoplasm containing intact lymphocytes (lymphophagocytosis). No neoplasia was seen.

MRI demonstrated a homogeneously enhancing infiltrative mass within the left carotid space, measuring 3 x 3 x 5 cm (figure, A). The mass encased the distal left common carotid artery and proximal left external and internal carotid arteries (figure, B-D). No evidence of necrosis or significant flow voids within the mass was found. At its superior aspect, the mass spread into the submucosal parapharyngeal space and bulged into the left oropharyngeal wall. There was prominence of the nasopharyngeal soft tissues, but no infiltrative or discrete mass was seen in the nasopharynx on MRI. A single left retropharyngeal lymph node and several small left supraclavicular lymph nodes were present, but no lymph nodes were enlarged.

Because the MRI findings were most suggestive of a lymphoproliferative disorder rather than reactive adenopathy, and because there were concerns about inadequate tissue sampling, a second FNAB of the left neck mass was performed with cytometric flow analysis. Staining was positive for CD68 and S-100. These again revealed only reactive features and no malignant characteristics. Direct laryngoscopy showed smoothly contoured, right posterior nasopharyngeal fullness with normal-appearing mucosa. Biopsy of the nasopharynx revealed benign respiratory mucosa with reactive lymphoid hyperplasia.

To address the ongoing concern regarding the possibility of a neoplastic process and because two FNABs had been nondiagnostic, an incisional biopsy of the left neck mass was then performed. The mass was encountered just underneath the anterior border of the stemocleidomastoid muscle. Because the mass appeared to encase the carotid artery, the lesion was not excised; instead, multiple biopsies were taken without complication. The final pathology from these biopsies revealed sinus histiocytosis, or RDD.

Six months after biopsy, the patient reported new bitemporal headaches and left eye pain without vision alteration. On examination, he was noted to have left-sided Homer's syndrome, suggesting involvement of the left sympathetic chain ganglia; his cranial nerves were otherwise intact. Dexamethasone therapy has resulted in marked improvement in the size of the neck mass and some improvement in the Homer's syndrome. The patient has not received any additional chemotherapy and continues to be followed by medical oncology.


The difficulty of making a histologic diagnosis of both nodal and extranodal RDD has been well described. (12-14) FNAB may suggest a granulomatous or reactive process, and some cases are pathologically similar to lymphomatous tissue. (4) The distinguishing histologic feature, however, is evidence of lymphocytophagocytosis (emperipolesis), in which histiocytes with abundant cytoplasm contain intact lymphocytes. Extranodal disease may be more difficult to identify histopathologically, as the emperipolesis may be obscured by extensive fibrosis. (15)

The clinical course of RDD is usually marked by a waxing and waning of LAD, although many RDD cases will resolve spontaneously. About 17% of patients will have persistent, asymptomatic adenopathy, and another 17% will have residual symptoms for up to 10 years. (16) Close follow-up and surveillance are warranted in all patients, however--not only for detection of recurrence, but also for the rare case of transformation to lymphoma-like disease or other immune-deficiency states. (10,15,17) Extranodal disease does not clearly portend a poorer prognosis, although the presence of disseminated nodal disease, multiple organ system involvement, and underlying immune dysfunction are considered unfavorable prognostic factors. (7,9,18-21)

A number of different chemotherapeutic agents, alpha interferon, thalidomide, and corticosteroids have been used for the treatment of RDD. (5,8,17,22,23) No treatment modality or combination effectively treats the manifestations or prolongs the remission from RDD. While endoscopic resection has been reported to be effective for both symptomatic and locoregional control of sinonasal RDD, (23) surgical resection and/or radiation therapy are generally reserved for life-threatening manifestations of disease caused by compression of vital organs or the airway. Unfortunately, surgical resection may only provide short-term improvement because local recurrence and disease progression may still occur. (17)

Because carotid artery flow was apparently not compromised in our patient, the risk of a neurovascular complication from resection was considered too significant. Ultimately, steroid therapy has been effective in reducing the size of this patient's neck mass and improving his symptoms, with minimal adverse effect.

Although RDD classically presents with LAD, it must be considered in the differential diagnosis for all patients who present with unusual soft-tissue neck masses. Pathologic identification and clinical suspicion are the mainstays of diagnosis. When a workup for infection and malignancy does not lead to an answer, it is perhaps judicious to broaden the differential to encompass lymphoid tissue or immune dysregulatory diseases, including RDD. Had RDD not been diagnosed in this patient, he might have unnecessarily undergone more invasive surgical techniques in his carotid triangle, at the risk of carotid artery sacrifice or endangerment. Complete resection would have been difficult and, hence, there would have been ongoing potential for disease recurrence.

In conclusion, in our case of extranodal Rosai-Dorfman disease, which presented as an infiltrative mass of the carotid sheath, the diagnosis was particularly difficult to make because it was an unusual presentation of a rare disease. Indeed, to the best of our knowledge, this is also the first reported case of Homer's syndrome in association with RDD. This case illustrates the importance of pursuing the pathologic identification of a disease when the clinical presentation and the imaging are equivocal.


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Lisa Lee, MD; Christine M. Glastonbury, MBBS; Doris Lin, MD

From the Department of Otolaryngology-Head and Neck Surgery, University of Cincinnati Medical Center, Cincinnati, Ohio (Dr. Lee) and the Department of Radiology (Dr. Glastonbury) and Department of Otolaryngology (Dr. Lin), University of California, San Francisco Medical Center.

Reprint requests: Christine M. Glastonbury, MBBS, Associate Professor of Clinical Radiology, University of California, San Francisco, Box 0628, Room L-358,505 Parnassus Ave., San Francisco, CA 94143-0628. Phone: (415) 353-2486; fax: (415) 353-8593; e-mail
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Author:Lee, Lisa; Glastonbury, Christine M.; Lin, Doris
Publication:Ear, Nose and Throat Journal
Article Type:Report
Date:Oct 1, 2007
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