Neoadjuvant Concurrent Chemoradiation for Advanced Esthesioneuroblastoma: A Case Series and Review of the Literature

Abstract

is a rare malignancy arising from the olfactory epithelium of the nasal vault. ENB accounts for 3% to 6% of all intranasal tumors and has the best prognosis among sinonasal malignancies with neuroendocrine differentiation. 1-3 The optimal treatment continues to be controversial, but the benefit of adjuvant therapy, particularly radiotherapy, has been well described in the literature. The largest reported series evaluated neoadjuvant radiotherapy of 50 Gy, with or without neoadjuvant chemotherapy, and found improved resectability with improved patient survival. 4 We present two cases of advanced ENB at our institution treated with a preoperative concurrent radiochemotherapy in a manner akin to that of small-cell carcinoma of the lung. Case Reports A review of patient charts and electronic records was conducted. Histopathologic slides were reviewed, and the diagnosis of high-grade esthesioneuroblastoma was confirmed. Complications of adjuvant therapy were scored using the National Cancer Institute Common Terminology Criteria of Adverse Events (CTCAE) version 3.0. Case 1. A 38-year-old male patient presented with anosmia and nasal fullness. A nasal endoscopy with biopsies demonstrated ENB in the right nasopharynx, Hyams grade 3. 5 Figure 1 shows tumor cells positive for Synaptophysin immunohistochemical staining, at ϫ400 magnification. The staging work-up, including magnetic resonance imaging (MRI) and positron emission tomography (PET)/computed tomography (CT) scan, revealed the primary tumor involving bilateral cribriform plates, bilateral ethmoid sinuses, and right maxillary e358

Multimodality Treatment of Pediatric Esthesioneuroblastoma

“…Other reports describe the lack of viable tumor tissue on pathological examination after chemoradiotherapy [15, 16]. On the other hand, while describing the adult experience at the University of Virginia, Polin et al recommended removal of the floor of the anterior fossa even in patients with complete radiological response to reduce the chance of recurrence [17].…”

Sphenoidal esthesioneuroblastoma treated with sequential chemo radiotherapy: A case report and review of the literature

“…This approach needs, off course, further investigation. [13,15] However, what is unique in our case report is that with gross disease in the sphenoid, resectability is a very challenging consideration because of the proximity to the optic nerves, carotid arteries and the clivus and brainstem posteriorly. This makes this tumor more challenging to treat, and one might argue in favor of non-surgical treatment (definitive chemoradiotherapy in various combinations and schedules) as opposed to surgical treatment.…”

“…[10,11] A second possible theory was first suggested by Jakumeit in 1971. [12][13][14] This theory is based on the development and degeneration of the accessory olfactory systems during fetal life. The cells of these accessory systems eventually degenerate in fetal life.…”

Syndrome of Inappropriate Antidiuretic Hormone Secretion Associated With Olfactory Neuroblastoma

“…23 Most teams using chemotherapy also delivered sequential or concurrent radiotherapy. 22 Encouraging results have been obtained, indicating that the treatment of olfactory neuroblastoma must be multimodal. Hyams classification also seems to be predictive of the response to chemotherapy.…”

“…The molecules most commonly used are cisplatin and etoposide or doxorubicin or vincristine. 22 Neoadjuvant chemotherapy for advanced tumors, using cisplatin and 5-fluorouracil or etoposide, doxorubicin or vincristine, has also been reported in the literature. 23 Most teams using chemotherapy also delivered sequential or concurrent radiotherapy.…”