Esthesioneuroblastoma with Widespread Distant Metastasis

SESSION TITLE: Student/Resident Case Report Poster - Lung Cancer I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Esthesioneuroblastoma (ENB) is a rare, slow growing malignant tumor of neural crest origin comprising less than 5% of sinonasal malignancies. Since 1924, approximately 1400 cases have been reported affecting people mainly in their 2nd and 5-6th decades of life. Classically, the presentation is related to its site of origin and extent of invasion. The most common presenting symptoms are obstructed nasal breathing, rhinorrhea, and epistaxis. Headache and diplopia have also been reported with extension of the tumor into the skull base. ENB’s are thought to arise from the basal layer of olfactory epithelium with reports of ectopic ENB’s located in areas lacking normal olfactory neuro-epithelium. Due to the rarity of this malignancy and its local, slow growing nature, metastatic disease is not recognized as a common presentation. CASE PRESENTATION: A 30-year-old woman with a history of metastatic ENB to the neck and parotid gland after surgery and adjuvant chemoradiation presented with complaints of bilateral leg pain of one month duration with worsening right sided leg swelling for 3 days. Venous duplex scan of the lower extremities confirmed right-sided non-obstructive deep vein thrombosis in the common femoral vein. CT angiogram of the chest showed a large right-sided pleural effusion with an enlarged right anterior mediastinal mass measuring 3.6 cm, compressing the superior vena cava. It also showed a 3.3 cm right supraclavicular lymph node and needle biopsy confirmed a malignant neoplasm consistent with ENB. A CT of the abdomen and pelvis revealed a 10.8 cm adnexal/pelvic mass, biopsy of which was also consistent with metastatic ENB. DISCUSSION: Esthiesioneuroblastomas are rare and the presence of metastatic disease is even more rare, with incidences reported between 10-40%. There are only nine reported cases of metastases of ENB to the lungs diagnosed by tissue biopsy. We present another such case, while introducing the only case of metastasis to the pelvis per our literature review. Regardless of location, ENB metastasis carries a very poor prognosis. The 5-year survival rate of patients who present with cervical metastasis is 0%. Unfortunately, our patient further deteriorated shortly after VATS was attempted for recurrent right pleural effusion. The best chance of survival remains in treating ENB early on in the course of the disease. CONCLUSIONS: Due to the unusual spread of ENB in this patient, we suggest early and frequent monitoring of disease metastasis in patients with a history of ENB who present with unusual symptoms. Reference #1: Ow T J, Bell D, Kupferman M E, Demonte F, Hanna E Y. Esthesioneuroblastoma. Neurosurg Clin N Am. 2013;24(1):51-65 Reference #2: Faragalla H, Weinreb I. Olfactory neuroblastoma: a review and update. Adv Anat Pathol.2009;16(5):322-331 DISCLOSURE: The following authors have nothing to disclose: Manraj Khosla, Annie Do, Christina Pecci, Taaran Cariappa Ballachanda Subbaiah, Lee Mcghan, Mahesh Seetharam, Richard Sue No Product/Research Disclosure Information