Advanced adult esthesioneuroblastoma successfully treated with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine

Abstract

The esthesioneuroblastoma is a rare neuroendocrine tumor that derives from the olfactory cells. In the last 20 years, around 1,000 cases have been described, with an overall survival rate of 60-70% at 5 years. The most common symptoms are nasal bleeding, nasal clogging and, in locally advanced cases, signs/symptoms of intracranic hypertension such as papilla edema, cefalea, and vomiting. The standard treatments are surgery and radiotherapy. Chemotherapy can be used in an adjuvant/neoadjuvant setting and in the metastatic phase, even if its role is still not established with certainty. Here, the case is reported of a young man (38 years old) with a locally advanced esthesioneuroblastoma. Two months before coming to our clinic, he had been treated elsewhere with debulking surgery through bilateral frontal craniotomy. After surgery, MRI showed residual disease in the nasal cavities and in the medial wall of the orbits responsible for blindness and bilateral exophthalmos within a month: a very short time. Octreoscan and whole body CT scan confirmed a locally advanced disease, in the absence of metastases. Chemotherapy was begun with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine with granulocyte colony-stimulating factor (G-CSF) support after every cycle. Soon after the first cycle, an important reduction of pain and decrease of the exophthalmos and vertigos was observed. No improvement in blindness was seen. The patient is still stable after 24 months of follow up.

Esthesioneuroblastoma: A case with prolonged disease control

“…Analogous to other related neuroendocrine tumors, cisplatin-based schedules (mainly associated with etoposide) are most frequently used [3,[36][37][38] . The use of non-platinum combinations such as irinotecan + docetaxel, [38] or doxorubicin + ifosfamide + vincristine [40] have also been reported. In our patient, doi: 10.18282/amor.v2.i5.112…”

Unexpectedly Durable Palliation of Metastatic Olfactory Neuroblastoma Using Anti-Angiogenic Therapy with Bevacizumab

“…16 Palliative cytotoxic chemotherapy most commonly yields only modest and temporary symptom improvement and with the goal of less toxicity. 17,18 …”

Neoadjuvant chemotherapy in the setting of locally advanced olfactory neuroblastoma with intracranial extension

“…16 Various chemotherapeutic combination regimens have been used involving alkylating agents and anthracylines with significant toxicities. 13,16 Response to chemotherapy may be dependent upon the Hyam's grading of the original tumor. McElroy et al noted that high-grade olfactory neuroblastoma was sensitive to cisplatin-based chemotherapy.…”

“…11–13 The capacity of induction chemotherapy to convert a previously unresectable presentation to one that is capable of complete surgical extirpation has been reported. 11 The following is a case report that is emblematic of successful multimodality management in a patient with locally advanced olfactory neuroblastoma with intracranial spread.…”