Rosai Dorfman's disease of the maxillary bone with soft tissue involvement of the cheek: Case Presentation and Literature Review / Enfermedad de Rosai Dorfman de maxilar con compromiso de partes blandas de mejilla: Presentación de caso y revisión de la literatura

The Rosai-Dorfman disease (RDD) is a bening entity, of unknown etiology, rare, characterized by the overproduction of histiocytes and their subsequent accumulation in the lymph nodes, which may also compromise other organs and systems. It predominantly affects young people. The histological study, which shows emperipolesis, is very indicative but not pathognomonic, but with the immunohistochemistry the diagnosis of the disease is reached, which does not have a well-protocolized treatment. Its evolution is highly variable, with spontaneous remission being a possibility, although in some cases the commitment is extensive, compromising important or vital anatomical structures that requiere vigorous treatment, which can be surgical, radiotherapy and even chemotherapy. We present a case of RDD that affected a 17-year-old girl with upper jaw and right maxillary sinus commitment, which began with loss of teeth, and subsequent appearance of a painless and deforming facial tumour that underwent surgical resection. In this case, the etiological diagnosis was not posible in the preoperative period despite multiple biopsies. An extensive review of the literature is carried out in order to a better understanding of this pathology.

La Enfermedad de Rosai-Dorfman (ERD) es una entidad benigna, de etiología desconocida, poco frecuente, caracterizada por la sobreproducción de histiocitos y su posterior acúmulo en los linfonodos, pudiendo comprometer también a otros órganos y sistemas. Afecta predominantemente a personas jóvenes. El estudio histológico, que muestra emperipolesis, es muy orientativa pero no patognomónica, aunque con la Inmunohistoquímica se llega al diagnóstico de la enfermedad que no tiene un tratamiento bien protocolizado. Su evolución es muy variable, siendo la remisión espontánea una posibilidad, no obstante, en algunos casos, el compromiso es extenso, comprometiendo estructuras anatómicas importantes o vitales que requieren de un tratamiento enérgico, pudiendo ser éste quirúrgico, radioterápico y aún quimioterápico. Se presenta un caso de ERD que afectó a una joven de 17 años, con compromiso de maxilar y seno maxilar derecho, que debutó con pérdida de piezas dentarias y posterior aparición de un tumor facial indoloro y deformante que fue sometido a resección quirúrgica. En este caso no fue posible el diagnóstico etiológico en el preoperatorio pese a múltiples biopsias. Se hace una extensa revisión de la literatura con el objeto de conocer mejor esta patología.

Solitary extranodal Rosai-Dorfman disease of the mandible: an exceedingly rare presentation

Sinus histiocytosis with massive lymphadenopathy, generally known by the name of Rosai-Dorfman disease is a rare benign condition principally affecting cervical lymph nodes. Concurrent extra-nodal disease frequently occurs, however, solitary extra-nodal disease involving the mandible is exceedingly rare with less than five reported cases in the English literature. We describe a case of primary involvement of the mandible in a 27-year-old female, and discuss the differential diagnosis of this disease with other histiocytic lesions.

Rosai-Dorfman disease with sinonasal mucosal involvement.

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is a rare benign disorder that typically manifests as massive lymphadenopathy and systemic symptoms. The authors report a 27-year-old man who presented with nasal mass and generalised cervical lymphadenopathy. Cytological and histopathological examination of lymph nodes demonstrated lymphophagocytosis (emperipolesis) consistent with a diagnosis of RDD. There was remarkable regression in the size of nasal polyps and lymph nodes after systemic corticosteroids. The clinical and histological aspects of the disease are discussed as a rare cause of generalised lymphadenopathy.

Rosai-Dorfman disease with pleural involvement

Sinus histocytosis with massive lymphadenopathy [SHLM] disease is considered to be indolent with self limiting pathology. However, severe morbidity and mortality have been attributed to complications of SHLM. Lower respiratory tract involvement, which is often unfavorable, is rarely reported and carries particularly grave prognosis. A case of sinus histocytosis with massive lymphadenopathy [SHLM] is reportedhere. The patient had lower respiratory and pleural involvement

Sinus histiocytosis with massive lymphadenopathy and subglottic stenosis: a case report.

An 18 year-old woman presented with massive cervical lymphadenopathy. She also had bilateral parotid gland enlargement, and later upper airway obstruction from subglottic mass compression. Surgical excision of lymph nodes and bilateral total parotidectomy with facial nerve preservation were selected. Direct laryngoscopy and CO2 laser excision were used to remove the subglottic mass. Airway was secured by tracheostomy and after that it was removed. Histologic finding was Sinus histiocytosis with massive lymphadenopathy (SHML) that involved the subglottic. SHML is very rare, probably less than 1000 cases reported in the literature and this is the 6th case in the literature that involved the subglottic. There are no standard treatment modalities. The treatment is comprised of surgery, steroid, chemotherapy and local control. In the present case the authors chose surgery, steroid and local control of disease in the subglottic by using CO2 laser excision. Now, the disease is under control.

Sinus histiocytosis with massive lymphadenopathy: complicated by autoimmune hemolytic anemia.

Sinus histiocytosis with massive lymphadenopathy is a rare histiocytic disorder with very few case reports in Indian literature. Immunological abnormalities have been documented in few cases. We report one such case of a child presenting with generalized lymphadenopathy and complicated by autoimmune hemolytic anemia, suggestive of an associated immune dysfunction.

Extranodal sinus histiocytosis with massive lymphadenopathy--a case report.

Sinus histiocytosis with massive lymphadenopathy involving organs other than the lymph nodes is a rare event. A case of SIIML presenting with multiple skin and subcutaneous nodules and multiple osteolytic lesions is described. A search of the Indian literature revealed many cases of nodal SIIML, but none of the reported cases had prominent extranodal involvement.