Management of Esthesioneuroblastoma: A Retrospective Study of 6 Cases and Literature Review

Abstract

The esthesioneuroblastoma (ENB) is a rare malignant sinonasal tumor of neuroectodermal origin. This study aimed to improve the understanding of the clinical features by reviewing the literature and analyzing the medical records of patients diagnosed with ENB in our institution between 2012 and 2019. A total of 6 cases of ENB were available for analysis. The mean age at the time of diagnosis was 36 years. The main complaints at presentation were the rhinologic signs. Tumors were classified as stage C of Kadisk in 3 cases and stage D in the others. According to TNM (modified by Dulguerov), 2 patients were T3N0M0, one T4N0M0, one T3N1M0, and two T4N1M0. The diagnosis of ENB was based on pathological examination. According to Hyams histological grading, low-grade lesions (Hyams I and II) were seen in 2 cases, high-grade undifferentiated lesions (Hyams III and IV) were seen in 4. Of the 6 patients, 4 received surgery. The surgical approaches mainly included an endoscopic endonasal resection in 2 cases, a cranial-facial resection surgery in 1 case, and an expanded endoscopic endonasal approach in combination with craniotomy in 1 case. Four patients received adjuvant radiotherapy (RT). RT dose ranged from 60 to 70 Gy. A total of 3 patients had lymph node metastasis and received RT of the neck. Chemotherapy was delivered in 2 patients. After a mean follow-up of 4.5 years, 4 patients were free of recurrence. Unfortunately, 1 patient died from a progressive disease 6 months after RT. ENB is a rare locally aggressive tumor of the nasosinusal cavities. The first-line treatment for resectable tumors should include primary surgical resection with adjuvant RT. However, this tumor remains of poor prognosis. Therefore, long-term close follow-up based on symptoms, endoscopy, and imaging is essential.

Exophthalmos revealing an olfactory esthesioneuroblastoma: A case report

“…Ophthalmological involvement including orbital invasion, is observed in 20 to 30 % of cases, resulting in exophthalmos, visual disturbances, and even ophthalmoplegia. The presence of ocular signs indicates an advanced stage of the disease [ 11 , 12 ].…”

“…Differential diagnosis of ENB includes squamous cell-carcinoma, hemangioma, sinonasal undifferentiated carcinoma, inverting papilloma and metastasis. ENB is histologically similar to sympathetic ganglionic neuroblastoma and retinoblastoma [ 11 , 12 ].…”

Skull Base Tumors

“…As these tumors are amenable to direct endoscopic visualization, the initial step in diagnosis is generally endonasal biopsy 84 . The diagnosis hinges on the presence of characteristic histopathologic features, and although there are no pathognomonic immunohistochemical features, these tumors are generally positive for neuronal markers (eg, chromogranin, synaptophysin, neuron-specific enolase) and S100 11,80 . Esthesioneuroblastomas generally express somatostatin receptors.…”

“…Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a malignant neuroectodermal tumor of the sinonasal tract, originating from the olfactory epithelium; CNS involvement occurs through contiguous spread through the cribriform plate to involve the anterior cranial fossa, orbit, and brain 11,79–81 . This rare cancer represents 6% of sinonasal malignancies, with an estimated annual incidence of 0.4 per 1 million 79,82 .…”

“…This rare cancer represents 6% of sinonasal malignancies, with an estimated annual incidence of 0.4 per 1 million 79,82 . Esthesioneuroblastoma has a slight male predominance in some studies, and a bimodal age distribution, with one peak in patients 11 to 20 years old, but the highest incidence is in the sixth decade of life 11,79,80 …”