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.
References
Cummings CW (2005) Cummings otolaryngology: head and neck surgery, 4th edn. St. Louis, Mosby
Bradley PJ, Jones NS, Robertson I (2003) Diagnosis and management of esthesioneuroblastoma. Curr Opin Otolaryngol Head Neck Surg 11:112–118
Berger L, Luc H, Richard R (1924) L’esthesioneuro epitheliome olfactif. Bull Assoc Fr Etud Cancer 13:410–420
Taxy JB et al (1986) The spectrum of olfactory neural tumors: a light-microscopic immunohistochemical and ultrastructural analysis. Am J Surg Pathol 10:687–695
Unger F, Walch C, Stammberger H, Papaefthymiou G, Haselsberger K, Pendl G (2001) Olfactory neuroblastoma (esthensioneuroblastoma): report of six cases treated by a novel combination of endoscopic surgery and radiosurgery. Minim Invasive Neurosurg 44:79–84
Kadish S, Goodman M, Wang CC (1976) Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer 37:1571–1576
Morita A, Ebersold MJ, Olsen KD et al (1993) Esthesioneuroblastoma: prognosis and management. Neurosurgery 32:706–715
Christopher Miyamoto R et al (2000) Esthesioneuroblastoma and sinonasal undifferentiated carcinoma: impact of histological grading and clinical staging on survival and prognosis. Laryngoscope 110:1262–1265
Simon JH, Zehn W, McCulloch TM et al (2001) Esthesioneuroblastoma: the University of Iowa experience 1978–1998. Laryngoscope 111:488–493
Ogura JH, Schenek NL (1973) Unusual nasal tumors: problems in diagnosis and treatment. Otolaryngol Clin North Am 6:813–837
Townsend C (2004) Sabiston textbook of surgery, 17th edn. Saunders, St. Louis
Tsue TT, Bailet JW, Barlow DW, Makielski KH (1997) Bilateral sinonasal papillomas in aplastic maxillary sinuses. Am J Otolaryngol 18:263–268
Ejaz A, Wenig B (2005) Sinonasal undifferentiated carcinoma: clinical and pathologic features and a discussion on classification, cellular differentiation, and differential diagnosis. Adv Anat Pathol 12:134–143
Rastogi M (2006) Esthesioneuroblastoma treated with non craniofacial resection surgery followed by combined chemotherapy and radiotherapy: an alternative approach in limited resources Jpn J Clin Oncol 36(10):613–619
De Franciscis V et al (1991) Preferential expression of the dbl protooncogene in some tumors of neuroectodermal origin. Cancer Res 51:4234–4237
Navarro S et al (1993) dbl oncogene expression in childhood tumors and tumor cell lines. Diagn Mol Pathol 2(3):158–162
Colucci-D’Amato GL et al (1995) Dbl expression driven by the neuron specific enolase promoter induces tumor formation in transgenic mice with a p53(±) genetic background. Biochem Biophys Res Commun 216(3):762–770
Rovigatti U et al (1998) Transgenic mice as research tools in neurocarcinogenesis. J Neurovirol 4:159–174 (review)
Loy AH, Reibel JF, Thomas CY et al (2006) Esthesioneuroblastoma: continued follow-up of a single institution’s experience. Arch Otolaryngol Head Neck Surg 132:134–138
Mishima Y et al (2004) Combination chemotherapy (cyclophosphamide, doxorubicin, and vincristine with continuous infusion cisplatin and etoposide) and radiotherapy with stem cell support can be beneficial for adolescent and adults with esthesioneuroblastoma. Cancer 101(6):1437–1444
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We are grateful to Rete tumori Rari staff, National Cancer Institute of Milan, for the scientific support.
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Turano, S., Mastroianni, C., Manfredi, C. et al. Advanced adult esthesioneuroblastoma successfully treated with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine. J Neurooncol 98, 131–135 (2010). https://doi.org/10.1007/s11060-009-0052-9
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DOI: https://doi.org/10.1007/s11060-009-0052-9