YO17 - Olfactory Neuroblastoma with Cervical Lymph Node Metastases in a 21- Year old Filipino Woman: A Case Report

Case summary

Olfactory Neuroblastoma with Cervical Lymph Node Metastases in a 21- Year old Filipino Woman: A Case Report

Background

Olfactory Neuroblastoma (ONB) is a rare, malignant, neuroectodermal tumor arising from olfactory receptor cells high in the olfactory plate. Accounting only 2% of all sinonasal tract tumors, its clinicopathologic and immunohistochemical features can be difficult. It has a unimodal age distribution commonly in the 5th- 6th decades of life with a 5-year overall survival and cancer-specific survival rates of 69% and 78%. Cervical lymph node metastasis is variable with only few published reports. Lymphatic metastasis has shown to be a significant predictor of survival.

AIMS

To present a rare case ONB in a 21-year old woman presenting with proptosis and sudden onset blindness.

METHOD

Case Report

RESULT

We report the case of a 21-year old woman who presented with proptosis, sudden onset blindness and enlarged right cervical nodes. She was initially treated as a case of Hyperthyroidism with no improvement. The cervical neck mass biopsy revealed the mass to be consistent with ONB. Immunohistochemistry showed positive Synaptophysin and EMA and negative S-100 and CD-3. Cranial CT with intravenous contrast (CT-IVC) demonstrated a lobulated enhancing slightly hyperdense lesion 8 cm x 7.6 cm x 6.4 cm (lxwxap) in the nasoorbitosinofrontal area with destruction of the ethmoid, right orbitofrontal and sphenoidal walls, bilateral medial orbital walls, more in the right and dorsum sella. It extends from the right orbit and to the frontal and sellar areas. Neck CT-IVC showed right cervical mass of 3.5 cm x 3.7 cm x 5.6 cm, with multiple paracervical, prevertebral, and submandibular nodules. She underwent 4 cycles of chemotherapy with Cisplatin and Etoposide concurrent with 30 sessions of 50 Gy Linear Accelerator Radiotherapy. She showed a remarkable response with a 90% mass size reduction. The blindness, however, remains to be irreversible.

CONCLUSION

ONB rarely presents with ophthalmologic findings and cervical lymph node metastases. Good response is achieved with concurrent chemotherapy and radiation therapy. The importance of early recognition and a multidisciplinary approach are vital in ONB management.

Clinical trial identification

Editorial acknowledgement