phenylephrine-hydrochloride and Histiocytosis--Sinus

Rosai-Dorfman disease (RDD) is a rare and benign lymphoproliferative disorder that commonly presents as painless, bilateral neck swelling. Extranodal presentations are considered rare, but the most common extranodal locations involved include skin, subcutaneous followed by nasal/paranasal sinuses. Although it is a benign condition, it may be mistaken as a malignant lesion and requires a biopsy for diagnostic confirmation. In this study, we report a rare case of RDD with bilateral neck node and nasal/paranasal sinus involvement which initially presented with bilateral nasal obstruction. And, we reviewed the management in this unusual case and discussed the helpful role imaging studies play in the further workup and subsequent follow-up to treatment response.

Topics: Histiocytosis, Sinus; Humans; Nasal Obstruction; Nose; Nose Diseases; Paranasal Sinuses

Topics: Histiocytosis, Sinus; Humans; Nose; Skin Diseases; Steroids; Thalidomide

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman, RD) disease is a well known entity. This pathology is differentiated from other childhood histiocytosis with its distinct features. Many sites within the head and neck area may be involved with the disease. Multi-organ involvement is very rare. A patient with Rosai-Dorfman disease involving neck, nose and larynx is presented and clinical follow-up is given.

Topics: Adolescent; Histiocytosis, Sinus; Humans; Larynx; Male; Neck; Nose; Radiography

Rosai-Dorfman disease is a rare, idiopathic, benign histiocytic proliferation usually seen in younger patients. Massive lymphadenopathy most commonly involves the cervical lymph nodes, with a predominant infiltration of sinusoidal histiocytes. Nearly half of the patients will have extranodal involvement, 75% occurring in sites in the head and neck. Three cases of extranodal Rosai-Dorfman disease of the head and neck involving the nose, paranasal sinuses, and parotid gland are presented. The clinical presentation, histologic characteristics, radiographic findings, and treatment of the disease are discussed. Because of the scarcity of cases, the clinical and histopathologic features of this disease may be overlooked. Familiarity with its relatively frequent clinical manifestations in the head and neck, as well as with the diagnostic histopathology, should preclude confusion with other disease entities.

Topics: Adult; Child; Female; Head; Histiocytosis, Sinus; Humans; Male; Middle Aged; Neck; Nose; Paranasal Sinuses; Parotid Gland; Tomography, X-Ray Computed

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare entity characterized by the proliferation of cells belonging to the macrophage-histiocyte family, but whose exact origin is unknown. A case of SHML occurring in a 62-yr-old female is described in which the diagnosis is initially suggested by fine-needle aspiration (FNA) of a cervical node. The smears show a background of lymphocytes and plasma cells and large histiocytes with well-preserved lymphocytes in their cytoplasm (emperipolesis or lymphophagocytosis). Both nodal and extranodal (nasal mass) involvement are confirmed by surgical biopsy. The immunohistochemistry suggests that SHML cells are functionally activated macrophages. Eight months after diagnosis, the patient is clinically well, with partial improvement of lymphadenopathy.

Topics: Biopsy, Needle; Cell Nucleus; Coloring Agents; Cytoplasm; Female; Histiocytes; Histiocytosis, Sinus; Humans; Immunohistochemistry; Lymph Nodes; Lymphocytes; Middle Aged; Neutrophils; Nose; Plasma Cells