Review of Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease) of Head and Neck

Abstract

The entity known as sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease (RD disease), is an uncommon benign proliferation of hematopoietic and fibrous tissue that often presents in the head and neck region. Its initial manifestations most often include a roughly symmetric, painless, bilateral cervical adenopathy, although extranodal disease may develop in a minority of patients. The key histologic feature of SHML is the presence of various numbers of large, pale histiocytic cells that contain within their cellular borders apparently engulfed lymphocytes ("emperipolesis"); these distinctive large, pale cells - RD cells - are S-100 protein-positive by immunostaining and so differ from ordinary histiocytes. Despite its sometimes impressive clinical presentation, SHML is a benign and self-limited disease, whose treatment is aimed largely at controlling its local manifestations (most often by surgical therapy). The microscopic differential diagnosis, particularly in extranodal disease, is at times challenging and can include Langerhans' cell histiocytosis, Hodgkin's disease, non-Hodgkin's lymphoma, metastatic carcinoma, and metastatic malignant melanoma.

Extranodal (cutaneous) Rosai Dorfman: a rare presentation

“…The neck lymph nodes are the most frequently involved, followed by inguinal, axillary and mediastinal lymph nodes. 4 In 85% of cases, patients with Rosai-Dorfman disease are in good general health without significant symptoms of the disease. 5 Treatment depends upon the patient individually and is planned after thorough testing to determine the extent of disease.…”

Adhesion and transcellular migration of neutrophils and B lymphocytes on fibroblasts

“…The engulfment of lymphocytes by non-phagocitic cells, observed several decades ago, was called emperipolesis [33][34][35][36]. In the Rosai-Dorfman disease, histological examination of the lymph nodes revealed that lymphocytes are internalized by histiocytes [37,38]. In addition, another cellular phenomenon, called pseudoemperipolesis, has been described in which lymphoma cells have physical interaction and migrated beneath adherent bone marrow stromal cells [39,40].Whether or not emperipolesis and pseudoemperipolesis correspond to a similar event, but taken at different time points, remains to be evaluated.…”

Doença de Rosai-Dorfman como diagnóstico diferencial de linfadenopatia cervical

“…4 The neck lymph nodes are the most frequently involved, followed by inguinal, axillary and mediastinal lymph nodes. 5 The most common extranodal sites are the skin, the upper respiratory tract and bones. Head and neck involvement -about 22% of extranodal disease 4,5 -include involvement of the nasal cavity, the paranasal sinuses, the nasopharynx, submandibular glands, the parotid, the larynx, the temporal bone, the intratemporal fossa, the pterygoid fossa, the meninges and the orbit.…”

“…Head and neck involvement -about 22% of extranodal disease 4,5 -include involvement of the nasal cavity, the paranasal sinuses, the nasopharynx, submandibular glands, the parotid, the larynx, the temporal bone, the intratemporal fossa, the pterygoid fossa, the meninges and the orbit. 5 The skin is commonly affected; half of these patients have another associated extranodal site. Orbit and ocular glove involvement have been reported, usually as a retroorbitary mass and proptosis.…”

“…Immunohistochemical features include positive S-100, alpha-antichymotrypsin and CD1a and CD68 antigens. 4,5 Imaging (CT and magnetic resonance imaging) may be used to assess disease extension. If there is cervical lymph node enlargement, FNAB or lymph node biopsies may be useful for the diagnosis.…”