Rosai-Dorfman disease affecting the maxilla

Rosai-Dorfman disease (RDD), formerly called sinus histiocytosis with massive lymphadenopathy, is a non-neoplastic proliferative histiocytic disorder with behavior ranging from highly aggressive to spontaneous remission. Although the lymph nodes are more commonly involved, any organ can be affected. This study aimed to describe the features and the follow-up of a case of extranodal RDD. Our patient was a 39-year-old woman who was referred with an 11-month history of pain in the right maxilla. On clinical examination, some upper right teeth presented full mobility with normal appearance of the surrounding gingiva. Radiographic exams showed an extensive bone reabsorption and maxillary sinus filled with homogeneous tissue, which sometimes showed polypoid formation. An incisional biopsy demonstrated a diffuse inflammatory infiltrate rich in foamy histiocytes displaying lymphocytes emperipolesis. Immunohistochemistry showed positivity for CD68 and S-100, and negativity for CD3, CD20, and CD30. Such features were consistent with the RDD diagnosis. The patient was referred to a hematologist and corticotherapy was administrated for 6 months. RDD is an uncommon disease that rarely affects the maxilla. In the present case, the treatment was conservative, and the patient is currently asymptomatic after 5 years of follow-up.

Rosai-Dorfman disease of the paranasal sinuses and orbit

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a benign indolent disorder, characterized by enlarged lymph nodes filled with histiocytes. Extranodal involvement is uncommon. The disease rarely affects the nose and paranasal sinuses. We report a case that presented with a right nasal mass, extending into all the paranasal sinuses and right orbit without any accompanying lymphadenopathy. Because of the absence of lymphadenopathy it posed a diagnostic challenge until the pathology was confirmed on histopathological examination

Doença de Rosai-Dorfman como diagnóstico diferencial de linfadenopatia cervical / Rosai-Dorfman disease in the differential diagnosis of cervical lymphadenopathy

A Doença de Rosai-Dorfman ou Histiocitose Sinusal com Linfadenopatia Maciça (SHML) é uma entidade clínica idiopática, rara e benigna, geralmente apresentando linfonodomegalia cervical. Usualmente acomete pacientes jovens. Manifestações extranodais na região da cabeça e pescoço são mais comuns em pacientes com anormalidades imunológicas. É uma doença autolimitada e, na maioria dos casos, não há necessidade de tratamento. Para os que requerem tratamento devido à persistência ou piora dos sintomas têm sido tentados tratamento cirúrgico, quimioterápico, radioterápico e corticoterapia. Os autores relatam um caso de um paciente do sexo masculino, 43 anos, com história de massas cervicais bilaterais, obstrução nasal, emagrecimento importante, febre e perda progressiva de acuidade visual há 6 meses. Trata-se de um paciente ex-tabagista e etilista crônico, onde a suspeita inicial foi de carcinoma espinocelular de rinofaringe. Realizada propedêutica, foi feito diagnóstico de doença de Rosai-Dorfman. Instituída a terapêutica, o seguimento de 6 meses revelou melhora do estado geral do paciente onde mantém as massas cervicais em regressão lenta. A apresentação clínica, as características histológicas, a patogênese e o tratamento são discutidos neste artigo.

Rosai-Dorfman Disease or Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is a rare benign disease of unknown etiology, which presents with cervical lymphadenopathy. It is usually seen in younger patients. The extranodal form affect various regions of the head and neck, and is more common in patients with immune abnormalities. It is a self-limited and seldom life-threatening disease, rendering therapy unnecessary in most cases. For those who require therapy because of persistent or worsening symptoms, treatments modalities include surgery, chemotherapy, radiotherapy and steroids. The authors describe one case of a 43-year-old man with bilateral cervical masses, nasal obstruction, fever, weight loss and decreased vision with 6 months duration. As his social history was positive for tobacco and alcohol use, the initial diagnosis was a possible rhinopharyngeal malignant tumor. Medical investigation established the diagnosis of SHML. After therapy, the 6-month follow-up evidenced the patient's clinical improvement, although cervical masses persisted. The clinical presentation, histological features, pathogenesis and treatment of this case are discussed.