Rosai-Dorfman Disease with Extranodal Manifestation in the Head

Abstract

The term sinus histiocytosis with massive lyrnphadenopathy (SHML) was introduc ed by Rosai and Dorfinan in 1969. Although SHML was initially described as low-pain cerv ical lymphadenopathy accompanied by fevet; leukocy tosis, an increased erythrocyte sedim entation rate, and hypergammaglobulinemia, extranodal involvement is observed in 25 to 40% of cases. This pathology is velY rare and involvement of the nasal fossae and paranasal sinus es is exceptional. We present two atypical cases of extranodal involvement exelusively confined to the head and review the literature. The first case showed a dramatic involvement offacial skin and tnuscles, orbit, and paranasal sim/ses. In the second case , the diseas e was limifed to the nasalfossae. Both cases showed exelusive extranodal involvement limit ed to the head region. In the absence oflymphadenopathy, diagnosing Rosai-Dorfman disease is dijJicult because ofthe lack ofthe most typical manifestation ofthe disease.

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

“…The abnormal soft tissue generally shows associated contrast enhancement and may show increased activity on PET [5,[18][19][20][21][22][23][24]. On MRI, sinus lesions may also show marked hypointensity on T2-weighted imaging [19].…”

“…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][30][31][32][33][34][35]. Ischemia of the globe can also occur [30].…”

Rosai-Dorfman disease: A rare clinico-pathological presentation

“…The background typically shows lymphocytes, plasma cells, and occasional neutrophils. 2,11 Extra nodal involvement is seen in 40 per cent of RDD cases which show similar morphological features to its nodal counterpart, although fewer histiocytes with emperipolesis are encountered. 2,12 The internalised lymphocytes are usually located within cytoplasmic vacuoles.…”

“…Extranodal involvement is seen in 40 per cent of RDD cases, which show similar morphological features to its nodal counterpart, although fewer histiocytes with emperipolesis are encountered. 2 Literature reviews show that up until 2004 about 600 cases of RDD had been reported, in all races but mainly in Caucasians (43 per cent), in any age group, but mainly in first and second decades (81 per cent), and more in males than females (2:1). 3 The most common presenting symptom of this disease is painless bilateral cervical lymphadenopathy which is seen in 90 per cent of the patients, and in 43 per cent of the cases the patients have at least one site of extra-nodal involvement.…”

Mandibular Reconstruction in a Patient With Rosai–Dorfman Disease

“…Pustular, psoriasiform, and acneiform presentations have also been documented. [3][4][5] The cutaneous RDD has a benign course usually with spontaneous regression in most cases.…”