Introduction  Inverted papilloma is a rare benign tumor of the nasal fossa, which usually originates from its lateral wall. Only 5% of cases demonstrate exclusive sinus involvement. Primary sphenoid sinus involvement is even rarer. Although considered a benign lesion, the tumor has a potentially invasive nature and has also been found to have an associated malignancy rate of 7 to 15%. Objectives  To report a case of inverted nasal papilloma originating in a rare location: the sphenoid sinus. Resumed Report  a 56-year-old woman, presented to our outpatient clinic complaining of frontal headache, occasional otalgia and recent forgetfulness. She was initially evaluated by a neurologist and then submitted to a head magnetic resonance imaging. A lesion was found to be filling both sphenoid sinuses. Sinus computed tomography showed an opacified sphenoid sinus with apparent bony integrity. The patient underwent sphenoidotomy through a transnasal endoscopic approach. A bleeding papillomatous lesion was identified. A biopsy was performed and histopathologic study suggested inverted papilloma. The lesion was then completely resected. The patient has been followed for 60 days after surgery; no signs of recurrence were found upon flexible nasofibroscope examination. Conclusion  Inverted Papilloma exclusively involving the sphenoid sinus is a rare entity. Non specific symptomatology and Clinical presentation make this kind of tumor a diagnostic and therapeutic challenge. The Endoscopic Sphenoidotomy has been the treatment of choice. Close follow-up is required in order to detect possible recurrences and malignant transformation.

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.

Os autores relatam o caso de uma paciente de 19 anos admitida para investigação de febre e anemia hemolítica, com dois meses de evolução. Durante a internação apresentou crises convulsivas tônico-clônicas e queda súbita no nível de consciência, evoluindo com hemoptise, hipoxemia e insuficiência respiratória. Os exames evidenciaram derrame pericárdico ao ecocardiograma e condensações alveolares bilaterais à radiografia de tórax. Foi levantada a hipótese diagnóstica de lúpus eritematoso sistêmico sendo solicitada a pesquisa do fator antinuclear e iniciada terapia com pulsos diários de metilprednisolona, a despeito da qual a paciente evoluiu para o óbito. Posteriormente o resultado do fator antinuclear mostrou-se positivo, em título de 1:5120, padrão puntiforme fino, preenchendo critérios para lúpus eritematoso sistêmico segundo o Colégio Americano de Reumatologia. A hemorragia pulmonar secundária a esta doença do tecido conjuntivo é uma forma incomum e grave de manifestação da doença que evolui com alta mortalidade, apesar do tratamento intensivo, conforme descrito na literatura.

The authors report a case of a 19-year-old woman admitted for the investigation of fever and hemolytic anemia for the previous 2 months. As an inpatient, she had convulsions and sudden loss of consciousness, developing hemoptysis, hypoxia, and respiratory insufficiency. Examination showed pericardial effusions on the echocardiogram and bilateral alveolar condensations on the thoracic radiograph. A hypothetical diagnosis of systemic lupus erythematosus was made, and measurement of the antinuclear factor was requested along with daily pulse therapy methylprednisolone, in spite of which the outcome was fatal. Afterwards, the result of the antinuclear factor test was positive, with a titer of 1:5120, showing a fine punctiform pattern, fulfilling the criteria for systemic lupus erythematosus according to the American College of Rheumatology. Secondary pulmonary hemorrhage in this connective tissue disease is an uncommon but serious complication that involves a high level of mortality in spite of intensive treatment, as is also reported in the literature.