A case of painful ophthalmoplegia with unilateral ocular pain, fixed eyeball to all directions of gaze, and loss of vision is presented. After intensive steroid therapy, conjunctival chemosis subsided markedly, but no improvement was seen in other clinical signs. We took a CT scan of orbit brain and performed nasopharyngeal biopsy and open biopsy through craniectomy. Based on the results of clinical features and findings of the CT scan and tissues, we diagnosed painful ophthalmoplegia secondary to nasopharyngeal carcinoma metastasized to orbital apex and brain.
Brain Neoplasms/pathology/secondary ; Carcinoma, Squamous Cell/*complications/pathology/secondary ; Female ; Humans ; Middle Aged ; Nasopharyngeal Neoplasms/*complications/pathology ; Ophthalmoplegia/*etiology ; Orbital Neoplasms/pathology/secondary ; Pain/*etiology

BACKGROUNDSinonasal malignancy with orbital invasion is rare. The objective of the current study was to evaluate the clinical and pathological features, treatment outcomes and survival rates for these malignancies.

METHODSNinety-three patients who were treated between 1997 and 2007 were retrospectively reviewed. Age, life style, symptoms, location of lesions, previous occurrences, histological subtypes, and treatment modalities were analyzed.

RESULTSNinety-three patients were evaluated, including 51 men and 42 women: the gender ratio was 1.2 men to 1.0 women. The median age was 40.5 years old. The nasal cavity (34.4%), the maxillary sinus (29.0%) and the ethmoid sinus (19.4%) were the most common primary malignant tumor sites. Almost half of the patients, 44.1% had squamous cell carcinoma, 13.9% had neuroectodermal carcinoma, 11.7% had rhabdomyosarcoma, 9% had adenocarcinoma and salivary gland-type carcinoma, and 11% had other malignancy. The majority of patients presented with T3/T4 (99%), N0 (93.1%), M0 (98%) disease. The distant metastasis rate was 20.9%. The overall survival and disease-free survival rates were 68.1% and 40.9% at three years, respectively. When the overall survival rate was computed according to the epicenter of the original malignant tumor, patients with nasal cavity malignancy and maxillary sinus had the best survival, and patients with ethmoid sinus malignancy had the worse survival (P = 0.03). According to their pathology classification, patients with rhabdomyosarcoma had worse overall survival than those with squamous cell carcinoma, or neuroendocrine carcinoma (P < 0.001). Squamous cell carcinoma and rhabdomyosarcoma invaded the orbit more often and malignancy of the nasal cavity invaded the orbit more than malignancy of the nasal sinus.

CONCLUSIONSFor nasal orbital tumors early diagnosis is crucial. Orbital exenteration and postoperative rehabilitation should be carefully considered. The current data suggest that surgical resection with postoperative radiation therapy may offer durable local control.

Adolescent ; Adult ; Aged ; Carcinoma, Squamous Cell ; complications ; epidemiology ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Male ; Middle Aged ; Nose Neoplasms ; complications ; epidemiology ; pathology ; Orbital Neoplasms ; epidemiology ; secondary ; Paranasal Sinus Neoplasms ; complications ; epidemiology ; pathology ; Young Adult