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DOI:10.2214/AJR.08.1114
AJR 2008; 191:W299-W306
© American Roentgen Ray Society

Review

Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): Imaging Manifestations in the Head and Neck

Donald V. La Barge, III1, Karen L. Salzman1, H. Ric Harnsberger1, Lawrence E. Ginsberg2, Bronwyn E. Hamilton1,3, Richard H. Wiggins, III1 and Patricia A. Hudgins4

1 Department of Radiology, University of Utah, 30 N 1900 E, 1A71, Salt Lake City, UT 84132-2140.
2 Department of Diagnostic Imaging, The University of Texas M. D. Anderson Cancer Center, Houston, TX.
3 Department of Diagnostic Radiology, Oregon Health Sciences University, Portland, OR.
4 Department of Radiology, Emory University, Atlanta, GA.

Received April 24, 2008; accepted after revision June 23, 2008.

 
Address correspondence to D. V. La Barge III.

CME

This article is available for CME credit. See www.arrs.org for more information.

WEB

This is a Web exclusive article.


Abstract
Top
 Abstract
Introduction
 Materials and Methods
 Results
 Discussion
 Conclusion
 References
 
OBJECTIVE. We present the largest case series in the English-language imaging literature, emphasizing the variety of presentations of Rosai-Dorfman disease in the head and neck.

CONCLUSION. Rosai-Dorfman disease is a rare disorder with multiple sites of involvement in the head and neck.

Keywords: emperipolesis • histiocytosis • lymphadenopathy • lymphoma • Rosai-Dorfman disease • sinus


Introduction
Top
 Abstract
 Introduction
Materials and Methods
 Results
 Discussion
 Conclusion
 References
 
Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare but well-defined clinicopathologic entity first described in 1969 that affects predominantly children and adolescents. The most common presentation is bilateral painless cervical lymphadenopathy [1, 2]. Extranodal manifestations of the disease occur in up to 43% of patients; the most common sites affected are the soft tissues of the head and neck and the paranasal sinuses and nasal cavity [2–4].

Because of the rarity of the diagnosis, the extensive differential considerations, the nonspecific imaging findings, and the multiple sites of involvement, the diagnosis of SHML is often not considered, which leads to inappropriate treatment of this benign but often progressive disease. Further complicating the diagnosis, up to 85% of patients are in good general health and have no significant symptoms relating to their disease; patients may manifest extranodal disease in the absence of lymphadenopathy [2, 4, 5]. In patients with disease extrinsic to the lymph nodes, approximately 75% have disease in the head and neck region [6].

The purpose of this study is to present the largest (to our knowledge) case series in the head and neck imaging literature to increase familiarity with this entity and further define head and neck imaging manifestations.


Materials and Methods
Top
 Abstract
 Introduction
 Materials and Methods
Results
 Discussion
 Conclusion
 References
 
A multiinstitution retrospective review of pathologically proven cases of Rosai-Dorfman disease with head and neck imaging manifestations over the years 1997–2007 was combined with a review of the literature on the topic of Rosai-Dorfman disease and SHML in the head and neck with imaging. Clinical information, including demographic data and presenting features, in addition to CT and MRI studies of each case were reviewed at our institution. This study was declared exempt by our institutional review board.


Results
Top
 Abstract
 Introduction
 Materials and Methods
 Results
Discussion
 Conclusion
 References
 
Thirteen cases of pathology-proven Rosai-Dorfman disease were reviewed at our institution. Summary data are presented in Table 1, with clinical and imaging data provided individually in Tables 2 and 3. The patients, seven male and six female, ranged in age from 2.5 to 75 years (mean age, 33 years). The most common clinical presentation, in seven of 13 patients, was neck mass. The most common imaging finding was cervical lymphadenopathy in eight of 13 patients that ranged from multiple small lymph nodes (5/8) to massive lymphadenopathy (3/8). Other common imaging findings were paranasal sinus involvement (5/13) and salivary gland involvement (4/13), which included focal masses, diffuse enlargement, and cystic changes. Less common imaging findings were spread from the maxilla to the suprazygomatic masticator space (2/13), dural-based disease (2/13), subcutaneous involvement of the cheek (1/13), and primary osseous involvement (1/13). MRI, which was available in six of 13 patients, showed the masses to be predominantly isointense to skeletal muscle on T1-weighted imaging and hypointense to skeletal muscle on T2-weighted imaging. All soft-tissue masses showed contrast enhancement on CT or MRI.


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  		TABLE 1: Summary of 13 Patients with Rosai-Dorfman Disease

 

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  		TABLE 2: Clinical Presentation of 13 Patients with Rosai-Dorfman Disease

 

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  		TABLE 3: Imaging Presentation of 13 Patients with Rosai-Dorfman Disease

 

Nodal Involvement: Cervical Lymphadenopathy
Patients with cervical lymphadenopathy (8/13) ranged in age from 2.5 to 75 years (mean age, 31 years). The most common clinical presentation was neck swelling or mass; all patients underwent contrast-enhanced CT (7/8) or MRI (2/8). Imaging revealed massive cervical lymphadenopathy in three of eight patients (Fig. 1). Submandibular, deep cervical, retropharyngeal, and mediastinal lymphadenopathy was seen.


Figure 1
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  		Fig. 1 —Contrast-enhanced CT scan in 5-year-old girl with neck masses reveals massive bilateral cervical lymphadenopathy. Pathology proved this to be Rosai-Dorfman disease.

 
Nodal and Extranodal Involvement
Paranasal sinuses —Five of eight patients (age range, 12–75 years; mean, 43 years) with cervical lymphadenopathy also had extranodal disease; the most common extranodal sites identified were the paranasal sinuses (5/13) and the salivary glands (4/13) (Figs. 2A and 2B). Patients presenting with cervical lymphadenopathy and paranasal sinus disease most commonly had involvement of the maxillary sinuses (4/5) and the ethmoid air cells (3/5). One patient also exhibited findings of extension of paranasal sinus disease into the suprazygomatic masticator space (Figs. 3A, 3B, and 3C).


Figure 2
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  		Fig. 2A —CT scan in 30-year-old woman with neck masses and elevated erythrocyte sedimentation rate shows lymphadenopathy and sinus disease. Axial unenhanced CT scan shows bilateral cervical lymphadenopathy.

 

Figure 3
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  		Fig. 2B —CT scan in 30-year-old woman with neck masses and elevated erythrocyte sedimentation rate shows lymphadenopathy and sinus disease. Coronal CT scan of sinus shows diffuse mucoperiosteal thickening of ethmoid air cells (straight arrow) and maxillary sinuses (curved arrow). Pathology proved this to be Rosai-Dorfman disease involving lymph nodes and paranasal sinuses.

 

Figure 4
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  		Fig. 3A —19-year-old woman with facial swelling and paranasal sinus disease. T1-weighted unenhanced image reveals opacification of left maxillary sinus and extension posteriorly into suprazygomatic masticator space on left (curved arrow). Retromaxillary fat is replaced. Compare with normal retromaxillary fat on right (straight arrow).

 

Figure 5
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  		Fig. 3B —19-year-old woman with facial swelling and paranasal sinus disease. Axial T2-weighted image with fat saturation shows T2 shortening in maxillary sinus that is characteristic of Rosai-Dorfman disease (arrow).

 

Figure 6
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  		Fig. 3C —19-year-old woman with facial swelling and paranasal sinus disease. Contrast-enhanced axial T1-weighted image with fat saturation shows enhancement in suprazygomatic masticator space (arrow). Pathology proved this to be Rosai-Dorfman disease of sinus with extension into suprazygomatic masticator space.

 

Salivary gland involvement —Salivary gland involvement was evident in four of 13 patients who ranged in age from 12 to 75 years (mean, 44 years). Findings consisted of lymphoid hyperplasia in the parotid gland or adjacent to the submandibular gland or diffuse enlargement of the submandibular glands (Figs. 4A and 4B). Cystic changes of the parotid gland were evident in one patient (Figs. 5A, 5B, 5C, and 5D).


Figure 7
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  		Fig. 4A —Axial contrast-enhanced CT in two adult men with neck mass. CT scan in 75-year-old man with enlarging left parotid mass reveals bilateral parotid masses (arrows) and cervical lymphadenopathy (not shown).

 

Figure 8
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  		Fig. 4B —Axial contrast-enhanced CT in two adult men with neck mass. CT scan in 56-year-old man with neck masses reveals diffuse enlargement of submandibular glands (arrows) and cervical lymphadenopathy. Pathology proved this to be Rosai-Dorfman disease of salivary gland.

 

Figure 9
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  		Fig. 5A —12-year-old girl with swelling of right eye. T1-weighted unenhanced (A) and fat-saturated contrast-enhanced (B) images reveal large enhancing right lacrimal gland mass (arrow).

 

Figure 10
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  		Fig. 5B —12-year-old girl with swelling of right eye. T1-weighted unenhanced (A) and fat-saturated contrast-enhanced (B) images reveal large enhancing right lacrimal gland mass (arrow).

 

Figure 11
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  		Fig. 5C —12-year-old girl with swelling of right eye. Axial T2-weighted image shows relative T2 hypointensity (arrow) and ethmoid sinus mucosal disease (asterisks).

 

Figure 12
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  		Fig. 5D —12-year-old girl with swelling of right eye. Axial contrast-enhanced T1-weighted image with fat saturation also shows parotid lymphadenopathy and multiple small parotid cysts (arrows). Pathology proved this to be Rosai-Dorfman disease of the orbit.

 
Orbital or lacrimal gland involvement— One patient, 12 years old, presented with swelling of the right eye and was found on MRI to have a mass involving the right lacrimal gland and orbit, isointense to the lacrimal gland on T1- and T2-weighted imaging, with enhancement similar to that of the normal contralateral lacrimal gland. Parotid lymphadenopathy and cystic changes of the parotid gland were also seen, as well as ethmoid sinus mucosal disease (Figs. 5A, 5B, 5C, and 5D).

Extranodal Involvement Without Lymphadenopathy
Dural involvement—Two patients, a 51-year-old man with left-sided headache and a 68-year-old man with difficulty walking, ataxia, and falls, were found to have dura-based involvement. Both patients underwent MRI that revealed extensive enhancing dura-based masses with relative T2 hypointensity (Figs. 6A, 6B, 6C, and 6D).


Figure 13
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  		Fig. 6A —Axial and coronal MRI in 68-year-old man with difficulty ambulating (A and B) and 51-year-old man with headache (C and D). Contrast-enhanced fat-suppressed T1-weighted MR images reveal extensive enhancing dura-based masses in suprasellar region (asterisk, A and B) with parasellar extension (arrows, A and B) and in cerebellopontine angles (asterisks, C and D) extending inferiorly. Note mass effect on medulla and cervicomedullary junction. Pathology proved this to be dura-based Rosai-Dorfman disease.

 

Figure 14
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  		Fig. 6B —Axial and coronal MRI in 68-year-old man with difficulty ambulating (A and B) and 51-year-old man with headache (C and D). Contrast-enhanced fat-suppressed T1-weighted MR images reveal extensive enhancing dura-based masses in suprasellar region (asterisk, A and B) with parasellar extension (arrows, A and B) and in cerebellopontine angles (asterisks, C and D) extending inferiorly. Note mass effect on medulla and cervicomedullary junction. Pathology proved this to be dura-based Rosai-Dorfman disease.

 

Figure 15
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  		Fig. 6C —Axial and coronal MRI in 68-year-old man with difficulty ambulating (A and B) and 51-year-old man with headache (C and D). Contrast-enhanced fat-suppressed T1-weighted MR images reveal extensive enhancing dura-based masses in suprasellar region (asterisk, A and B) with parasellar extension (arrows, A and B) and in cerebellopontine angles (asterisks, C and D) extending inferiorly. Note mass effect on medulla and cervicomedullary junction. Pathology proved this to be dura-based Rosai-Dorfman disease.

 

Figure 16
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  		Fig. 6D —Axial and coronal MRI in 68-year-old man with difficulty ambulating (A and B) and 51-year-old man with headache (C and D). Contrast-enhanced fat-suppressed T1-weighted MR images reveal extensive enhancing dura-based masses in suprasellar region (asterisk, A and B) with parasellar extension (arrows, A and B) and in cerebellopontine angles (asterisks, C and D) extending inferiorly. Note mass effect on medulla and cervicomedullary junction. Pathology proved this to be dura-based Rosai-Dorfman disease.

 
Primary osseous involvement—A 20-year-old woman presented with a lytic mass on CT that involved the left maxilla. MRI revealed extension of abnormal enhancing soft tissue posteriorly into the suprazygomatic masticator space (Figs. 7A, 7B, 7C, and 7D).


Figure 17
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  		Fig. 7A —20-year-old woman with left facial pain. Contrast-enhanced CT scans in bone (A) and soft-tissue (B) algorithms reveal lytic mass (arrow).

 

Figure 18
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  		Fig. 7B —20-year-old woman with left facial pain. Contrast-enhanced CT scans in bone (A) and soft-tissue (B) algorithms reveal lytic mass (arrow).

 

Figure 19
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  		Fig. 7C —20-year-old woman with left facial pain. Axial (C) and coronal (D) contrast-enhanced fat-suppressed T1-weighted MR images reveal enhancement and extension beyond posterior maxillary wall into suprazygomatic masticator space (arrow). Pathology proved this to be osseous Rosai-Dorfman disease with extension into the suprazygomatic masticator space.

 

Figure 20
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  		Fig. 7D —20-year-old woman with left facial pain. Axial (C) and coronal (D) contrast-enhanced fat-suppressed T1-weighted MR images reveal enhancement and extension beyond posterior maxillary wall into suprazygomatic masticator space (arrow). Pathology proved this to be osseous Rosai-Dorfman disease with extension into the suprazygomatic masticator space.

 

Primary subcutaneous involvement—A 22-year-old woman with a right cheek mass underwent CT that revealed an enhancing lobular soft-tissue mass adjacent to the right maxilla and periosteal reaction along the right lateral maxillary wall. In addition, maxillary sinus mucosal disease with periosteal thickening was seen (Figs. 8A, 8B, and 8C).


Figure 21
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  		Fig. 8A —22-year-old woman with right cheek mass and known paranasal sinus disease. Contrast-enhanced CT scans using soft-tissue (A) and bone (B and C) algorithms reveal enhancing lobular soft-tissue mass (arrow, A) in right cheek and adjacent periosteal thickening along lateral maxillary wall (arrow, B and C). Mucoperiosteal changes in maxillary sinuses are also evident. Pathology proved this to be subcutaneous Rosai-Dorfman disease.

 

Figure 22
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  		Fig. 8B —22-year-old woman with right cheek mass and known paranasal sinus disease. Contrast-enhanced CT scans using soft-tissue (A) and bone (B and C) algorithms reveal enhancing lobular soft-tissue mass (arrow, A) in right cheek and adjacent periosteal thickening along lateral maxillary wall (arrow, B and C). Mucoperiosteal changes in maxillary sinuses are also evident. Pathology proved this to be subcutaneous Rosai-Dorfman disease.

 

Figure 23
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  		Fig. 8C —22-year-old woman with right cheek mass and known paranasal sinus disease. Contrast-enhanced CT scans using soft-tissue (A) and bone (B and C) algorithms reveal enhancing lobular soft-tissue mass (arrow, A) in right cheek and adjacent periosteal thickening along lateral maxillary wall (arrow, B and C). Mucoperiosteal changes in maxillary sinuses are also evident. Pathology proved this to be subcutaneous Rosai-Dorfman disease.

 

Discussion
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
Conclusion
 References
 
First described by Rosai and Dorfman in 1969, SHML—later termed "Rosai Dorfman disease"—is now a well-defined clinicopathologic entity [1, 2]. Postulated causes include infectious causes, immunodeficiency, autoimmune disease, and a neoplastic process; however, none has been substantiated [1, 2, 4]. In reviewing the patient registry established by Juan Rosai and maintained at the Yale University School of Medicine [2], most of those afflicted are younger than 20 years (80%), with more than two thirds of registry patients younger than 10 years. There is also an increased incidence among male patients ranging from 1.4:1 to 3:1, as well as a greater incidence among patients of African and West Indian heritage [2, 6, 7].

Multiple strategies of therapy have been used with varying success, including radiation therapy, chemotherapy, steroids, and surgery [1–45]. For management of Rosai-Dorfman disease, clinical observation without treatment is preferred when possible [45]. Whenever infiltrates cause vital organ compression or important clinical signs, surgical debulking is preferred [45]. Earlier consideration of this diagnosis would thus facilitate appropriate management and obviate surgery for this benign condition.

Histopathologically, the Rosai-Dorfman disease infiltrate shows "sinusal" lymph node architecture with clustering of lymphocytes simulating the appearance of germinal centers. The characteristic histopathologic feature is emperipolesis (Fig. 9), in which histiocytes phagocytize lymphocytes, plasma cells, erythrocytes, or polymorphonuclear leukocytes. Intracytoplasmic eosinophilic globules (Russell bodies) are also seen in plasma cells. These features are often much less prominent in extranodal Rosai-Dorfman disease [1, 2, 4]. In addition, immunohistochemical analysis consistently shows S-100 protein positivity, particularly with the monoclonal antibody, and immunoreactivity against {alpha}-1-antichymotrypsin, CD68, and MAC387 antibodies [4].


Figure 24
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  		Fig. 9 — Lymphophagocytosis (emperipolesis). H and E stain at high-power shows predominantly intact lymphocytes phagocytosed in cytoplasm of a histiocyte (arrow), a characteristic histopathologic finding in Rosai-Dorfman disease. (Reprinted with permission from Macmillan Publishers Ltd. Andriko JW, Morrison A, Colegial CH, Davis BJ, Jones RV. Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. Mod Pathol 2001; 14:172–178 [46])

 

Patients most commonly present with cervical lymphadenopathy that manifests clinically as a neck mass. In our series, this was the most common clinical presentation, seen in seven of 13 patients. The most common imaging finding was deep cervical lymphadenopathy, present in eight of 13 patients. The differential diagnosis for this finding is broad and includes lymphomatous, pseudolymphomatous, and infectious causes.

The lymphadenopathy associated with Rosai-Dorfman disease is well shown on sonography, CT, and MRI. Nuclear imaging often reveals increased uptake of the radiopharmaceutical gallium [6] as well as increased metabolism of 18F-FDG on PET imaging [14, 15]. Ahuja and Ying [13] described the sonographic appearance of involved nodes as similar to that of malignant nodes, with histopathology necessary to yield a definitive diagnosis. Silvers et al. [17] describe a single case report of cervical lymphadenopathy with a peripheral rim of calcification (eggshell pattern) on CT after 1 year of interferon therapy in a patient with Rosai-Dorfman disease. An interesting case of involvement of a single pretracheal and thyroid isthmic lymph node has been reported. On sonography, the isthmic node appeared as a septate, avascular, cystic mass [26].

Although no specific imaging characteristics allow differentiation of lymphadenopathy in Rosai-Dorfman disease from myriad other disease processes, massive painless bilateral cervical lymph node enlargement, particularly when occurring in children and adolescents, should promote consideration of Rosai-Dorfman disease in the differential diagnosis [6, 12, 13].

Extranodal disease, most commonly occurring in the head and neck region, occurs in up to 43% of patients. However, Foucar et al. [2] emphasize that this may be an overestimation because of increased interest in publishing cases of extranodal disease. Of extranodal sites affected, the skin is the most common, followed by the paranasal sinuses and nasal cavity, subcutaneous tissue, orbit, eyelids, and bone [2]. Skin involvement was noted to occur more commonly in patients 40 years and older [16]. Multiple additional sites of extranodal involvement in the head and neck have been described, including the salivary glands, oral cavity, pharynx, tonsils, and trachea [6, 11]. Okada et al. [44] reported a case of tracheal involvement confined to the mucosa, without involvement of the tracheal cartilage. Additional lesions in the carina and left main bronchus were also present, as well as enlarged mediastinal lymph nodes [44].

Interestingly, patients with extranodal head and neck disease appear to have a higher occurrence of immunologic abnormalities on serology. This is significant because the presence of immunologic dysfunction, ranging from circulating autoantibodies to arthritis, is also associated with increased morbidity and mortality [8, 9, 23]. In reviewing 14 known fatalities among registry patients, Rosai-Dorfman disease was determined to be the cause of death in only two patients, one with CNS involvement, the other with renal involvement. No evidence has been seen of malignant transformation of Rosai-Dorfman disease, and most registry patients have a long history of disease involvement [9].

In our series, extranodal involvement was identified in nine of 13 patients (77%). Our patients represented involvement of the paranasal sinuses and nasal cavity, salivary glands, lacrimal gland and orbit, maxillary bone, and dura. Extension into the suprazygomatic masticator space was evident in two of 13 patients, and previously unreported cystic changes in the parotid gland were identified in one of 13 patients.

After the skin, the paranasal sinuses are the most common extranodal site of involvement [2]. Patients often present with progressive nasal obstruction but may also present with facial pain, epistaxis, or hyposmia or anosmia. CT and MRI findings are nonspecific and typically reveal polypoid masses, mucosal thickening, or soft-tissue opacification of the paranasal sinuses or nasal cavity, with or without associated osseous erosion. The abnormal soft tissue generally shows associated contrast enhancement and may show increased activity on PET [5, 18–24]. On MRI, sinus lesions may also show marked hypointensity on T2-weighted imaging [19]. The differential diagnosis is broad and includes infectious (granulomatous) disease, Wegener's granulomatosis, other histiocytoses, Hodgkin's and non-Hodgkin's lymphoma, and fibroinflammatory lesions [4]. In general, Rosai-Dorfman disease does not show the bone and soft-tissue destruction evident with midline destructive lesions such as Wegener's granulomatosis and T-cell lymphoma; however, exceptions have been reported [4, 20].

The most common manifestation of disease involvement of the salivary glands in our patients (3/4 patients) was lymphoid hyperplasia in the parotid gland or adjacent to the submandibular gland. A unique feature present in one of our patients was cystic changes in the parotid gland. Whether this is a manifestation of Rosai-Dorfman disease is unknown. Lymphoid hyperplasia in the parotid gland can be seen in various disorders; however, when it is present in a young patient without significant clinical symptomatology, the diagnosis of Rosai-Dorfman disease should be considered [25]. Although disease involving the salivary glands typically manifests as lymphoid hyperplasia, diffuse enlargement of the salivary gland without significant lymphadenopathy can also rarely occur [27, 28]. Juskevicius and Finley [28] described a case of parotid gland involvement without significant lymphadenopathy in a 48-year-old patient with systemic lupus erythematosus.

The orbit, globe, and eyelid are well-known sites of involvement in Rosai-Dorfman disease, occurring in 7–10% of patients. Orbital involvement is most commonly unilateral [2, 6]. Imaging findings include extraconal soft-tissue masses with proptosis (most common); infiltration of the eyelids, extraocular muscles, and fat; lacrimal gland involvement; and rarely, involvement of the globe [5, 6, 18, 22, 29–35]. Ischemia of the globe can also occur [30]. Involvement of the optic nerve sheath has been reported as well as extension through and expansion of the superior orbital fissure and mass extension involving the optic chiasm, cavernous sinus with encasement of the internal carotid artery, and the suprasellar region [29–30, 32, 34]. Orbital masses are of soft-tissue attenuation and show uniform or heterogeneous contrast enhancement. The differential diagnosis is broad and includes optic nerve sheath tumor, leukemia, inflammatory pseudotumor, sarcoidosis, Wegener's granulomatosis, and infectious processes.

The most common intracranial manifestation of Rosai-Dorfman disease is that of a dura-based mass or masses easily confused with meningiomas. CNS involvement with Rosai-Dorfman disease often has no associated lymphadenopathy (up to 70% of patients), and it commonly occurs in adult men, usually in the fourth decade; however, it has also been reported in children [2, 29, 37, 43]. Common locations are the parasellar, cavernous sinus, petroclival, and parafalcine regions; the cerebellopontine angle; and posterior fossa [7, 19, 22, 29, 34, 36–42]. Although the imaging findings are similar to those of meningiomas, a discriminator may be very low signal intensity on T2-weighted images that is believed to be due to free radicals released by inflammatory macrophages [7]. The lack of hypervascularity on angiography, as is more typically seen with meningiomas, may also assist with the preoperative diagnosis [7, 19].

Konishi et al. [39] describe an interesting case of Rosai-Dorfman disease presenting as dural thickening with the disease arising in the subarachnoid space. Dura-based masses with invasion of the transverse and superior sagittal sinuses have also been reported [37], as well as extension of orbital Rosai-Dorfman disease into the cavernous sinus with encasement and subsequent occlusion of the internal carotid artery [29, 30]. Involvement of the trigeminal nerves at the level of the pons has been described [38], as well as masses in the choroid plexus of the left lateral ventricle trigone [38], atrium of the left lateral ventricle [22], and the fourth ventricle [22]. Case reports have also described involvement of the posterior pituitary gland [21] and thickening of the infundibulum [29, 34].

Primary osseous involvement with Rosai-Dorfman disease is rare but has been reported to involve the maxilla [20], as in one of our patients, as well as the sphenoid bone and the frontal bone [29]. Imaging findings may reflect a well-defined lytic lesion, often confused with Langerhans cell histiocytosis (LCH). However, during the healing phase, Rosai-Dorfman disease is reported to show ill-defined fuzzy margins rather than the well-defined sclerotic margin typical of LCH [29, 38]. Alternatively, frank osseous destruction may result from the progressive nature of adjacent disease invading bone, as in the case presented by Shemen et al. [20], in which disease invaded from the cheek through the premaxilla and hard palate into the nasal cavity.


Conclusion
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 Conclusion
References
 
Rosai-Dorfman disease is a benign, pseudolymphomatous entity with various imaging manifestations in the head and neck. Involvement of the cervical lymph nodes resulting in massive bilateral lymphadenopathy is the most characteristic finding described in the literature; however, massive cervical lymph node involvement was present in only three of 13 patients in this series. Although the paranasal sinuses are a well-known site of involvement, the term "sinus histiocytosis" refers to the original pathologic description of infiltration of the medullary sinus of the lymph node. Furthermore, extranodal disease occurs in up to 43% of patients and occurs in the absence of lymphadenopathy in approximately 23% of patients [2]. Although Rosai-Dorfman disease is most commonly diagnosed in children and adolescents presenting with cervical lymphadenopathy, this disease is a well-established diagnosis in all age groups with imaging manifestations throughout the head and neck. Imaging manifestations are nonspecific; however, decreased T2-weighted signal intensity in a dura-based mass and lack of arteriovenous shunting on angiography may help to distinguish Rosai-Dorfman disease from meningioma. Concomitant cervical lymphadenopathy with orbital or paranasal sinus masses may also assist in earlier consideration of this diagnosis, thus facilitating appropriate management of this benign but often progressive disease.


Acknowledgments
 
We thank Barton Branstetter, Bernadette Koch, and Kristine Mosier for contributing cases and expert opinions during the development of this manuscript.


References
Top
 Abstract
 Introduction
 Materials and Methods
 Results
 Discussion
 Conclusion
 References
 

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M.P. Lungren, J.R. Petrella, T.J. Cummings, and G.A. Grant
Isolated Intracranial Rosai-Dorfman Disease in a Child
AJNR Am. J. Neuroradiol., November 1, 2009; 30(10): E148 - E149.
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