Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Esthesioneuroblastoma Follow-up

  • Author: Michael Somenek, MD; Chief Editor: Jules E Harris, MD, FACP, FRCPC  more...
 
Updated: Apr 14, 2015
 

Further Outpatient Care

Nasal irrigation with sterile isotonic sodium chloride solution begins within a few days after packing removal. Removal of nasal crusting should be performed regularly during initial postoperative visits. This problem is aggravated during radiotherapy but decreases in most patients after 1-2 years. It is a lifelong burden for which no good treatment or prevention is available.

Craniotomy sutures are removed 7-10 days after surgery. If a facial incision has been used, sutures are removed after 5 days

Next

Further Inpatient Care

In the first 48 hours following craniofacial resection, regular neurosurgical evaluation is required. ICU monitoring for at least 24 hours also is necessary. Duration of the lumbar drainage is controversial, varying from intraoperative only to as long as 7 days.

Aspiration of the nasal trumpets should be performed several times a day. Nasal packing is left in place for 5-7 days. Removal of large nasal packing is uncomfortable, and some pain medication could be administered before the procedure.

Previous
Next

Inpatient & Outpatient Medications

Antibiotic prophylaxis is usually started before surgery for esthesioneuroblastoma and includes coverage for gram-positive bacteria. Because good intracerebral penetration is preferred, ceftriaxone often is a good choice. Postoperatively, the authors continue antibiotics while the nasal packing remains in place.

Previous
Next

Complications

Craniofacial resection for esthesioneuroblastoma (ENB) has been associated with a complication rate of 10-15%. Higher rates of complications (up to 30%) have been observed in cases of revision or salvage surgery or surgery following radiation or chemoradiation therapy.

The most frequent problems are infections, including abscess surrounding the bone flap; meningitis; and, less often, subdural or epidural abscess.

Meningitis sometimes is related to the presence of a cerebrospinal fluid leak, because the closure of the anterior skull base is not always watertight.

Pneumocephalus has been another difficult problem to prevent. Although self-limiting in most cases, pneumocephalus can result in brain compression and requires needle aspiration through the trephine bony holes.

Blindness, secondary to optic nerve injury, and death either from intracerebral bleeding or internal carotid injury have become exceptional.

Inform patients that olfactory function is likely to be altered or absent after surgery. In addition, nasal crusting is a long-lasting problem that requires daily nasal irrigation for several years.

Delayed complications include necrosis of the frontal bone flap, lacrimal drainage obstruction, and frontal sinus mucocele.

Radiation-related complications also are rare and include lacrimal duct blockage and tearing, postradiation cataract, osteoradionecrosis of the frontal bone flap, radiation retinopathy, and blindness.

Previous
Next

Prognosis

See the list below:

  • Treatment results before the availability and use of modern diagnostic techniques were flawed, probably by the inclusion of cases of sinonasal undifferentiated carcinoma and sinonasal neuroendocrine carcinoma, 2 aggressive diseases associated with poor survival. The importance of correctly differentiating these cancers is highlighted in a recent report by Rosenthal et al. [37] For a discussion of posttreatment prognosis, see Kane et al. [8]
  • In recent series, the 5-year patient survival rates have varied from 50-80%, with the majority of large studies indicating patient survival rates of higher than 70%. In the 2001 meta-analysis by Dulguerov et al, the average 5-year survival was 45 ± 22% (range, 0-86%). [18]
  • Whether the Kadish classification system has a definitive prognostic value remains unknown, but the available survival data for stages A, B, and C are 72%, 59%, and 47%, respectively. According to the Dulguerov staging system, the survival rates were 81% for T1, 93% for T2, 59% for T3, and 48% for T4.

Prognostic survival factors that emerged in the meta-analysis by Dulguerov et al include lower Hyams histopathologic grade (56% for grades I-II vs. 25% for grades III-IV) and absence of neck lymph node metastasis (64% for N0 vs. 29% for N+).[18]

Other possible but unconfirmed prognostic factors include a low proliferative index, the completeness of the surgical resection, and tumor shrinkage after induction chemotherapy.

Survival data categorized according to treatment modality favored regimens that include surgery.

The most frequent recurrence is local, with rates from 20-40% (29% in the Dulguerov et al meta-analysis). Craniofacial resection followed by radiation seems to result in fewer recurrences, a rate of approximately 10%. Salvage after local recurrence is possible in one third to one half of cases.

Regional recurrence, while disease at the primary site remains under control, occurs in 15% of cases and is salvageable by further treatment in 25-50% of cases. Distant metastasis with locoregional control occurs infrequently (8%) and carries a dismal prognosis.

Recurrence may occur years after the completion of treatment, often more than 10 years; therefore, prolonged follow-up is required, although the optimal frequency of medical visits and the necessity of radiologic studies have not been precisely determined.

Previous
 
Contributor Information and Disclosures
Author

Michael Somenek, MD Physician, Facial Plastic and Reconstructive Surgery, Ruff Plastic Surgery

Michael Somenek, MD is a member of the following medical societies: Alpha Omega Alpha, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Guy J Petruzzelli, MD, PhD, MBA, FACS Physician-in-Chief and Vice President of Oncology Programs, Curtis and Elizabeth Anderson Cancer Institute at Memorial University Medical Center; Professor of Surgery-Head, Neck, and Endocrine Surgery, Mercer University School of Medicine-Savannah Campus

Guy J Petruzzelli, MD, PhD, MBA, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Association for the Advancement of Science, American College of Surgeons, American Head and Neck Society, American Medical Association, Chicago Medical Society, North American Skull Base Society, Society of Surgical Oncology, Society of University Otolaryngologists-Head and Neck Surgeons, SWOG, American Association of Clinical Anatomists, American Society of Clinical Oncology, International Academy of Oral Oncology, International Head and Neck Scientific Group, Georgia Society of Otolaryngology-Head and Neck Surgery

Disclosure: Nothing to disclose.

Nicholas C Shera, PhD Freelance Medical/Scientific Writer

Disclosure: Nothing to disclose.

Thomas C Origitano, MD, PhD, FACS Professor and Chair, Department of Neurological Surgery, Medical Director, Neuroscience Service Line, Co-Director, Center for Cranial Base Surgery, Loyola University Medical Center

Thomas C Origitano, MD, PhD, FACS is a member of the following medical societies: American Association of Neurological Surgeons, American College of Surgeons, North American Skull Base Society, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Jules E Harris, MD, FACP, FRCPC Clinical Professor of Medicine, Section of Hematology/Oncology, University of Arizona College of Medicine, Arizona Cancer Center

Jules E Harris, MD, FACP, FRCPC is a member of the following medical societies: American Association for the Advancement of Science, American Society of Hematology, Central Society for Clinical and Translational Research, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Additional Contributors

Robert C Shepard, MD, FACP Associate Professor of Medicine in Hematology and Oncology at University of North Carolina at Chapel Hill; Vice President of Scientific Affairs, Therapeutic Expertise, Oncology, at PRA International

Robert C Shepard, MD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for Physician Leadership, European Society for Medical Oncology, Association of Clinical Research Professionals, American Federation for Clinical Research, Eastern Cooperative Oncology Group, Society for Immunotherapy of Cancer, American Medical Informatics Association, American College of Physicians, American Federation for Medical Research, American Medical Association, American Society of Hematology, Massachusetts Medical Society

Disclosure: Nothing to disclose.

References
  1. Broich G, Pagliari A, Ottaviani F. Esthesioneuroblastoma: a general review of the cases published since the discovery of the tumour in 1924. Anticancer Res. 1997 Jul-Aug. 17(4A):2683-706. [Medline].

  2. Zhang M, Zhou L, Wang DH, Huang WT, Wang SY. Diagnosis and management of esthesioneuroblastoma. ORL J Otorhinolaryngol Relat Spec. 2010. 72(2):113-8. [Medline].

  3. Wenig BM, Prasad ML, Dulguerov P. Neuroectodermal tumors. In: Barnes L, Eveson JW, Reichart P, and Sidransky D, WHO Classification of Tumors. 2005.

  4. Guled M, Myllykangas S, Frierson HF Jr, Mills SE, Knuutila S, Stelow EB. Array comparative genomic hybridization analysis of olfactory neuroblastoma. Mod Pathol. 2008 Jun. 21(6):770-8. [Medline].

  5. Mhawech P, Berczy M, Assaly M, Herrmann F, Bouzourene H, Allal AS, et al. Human achaete-scute homologue (hASH1) mRNA level as a diagnostic marker to distinguish esthesioneuroblastoma from poorly differentiated tumors arising in the sinonasal tract. Am J Clin Pathol. 2004 Jul. 122(1):100-5. [Medline]. [Full Text].

  6. Carney ME, O''Reilly RC, Sholevar B. Expression of the human Achaete-scute 1 gene in olfactory neuroblastoma (esthesioneuroblastoma). J Neurooncol. 1995 Oct. 26(1):35-43. [Medline].

  7. Theilgaard SA, Buchwald C, Ingeholm P. Esthesioneuroblastoma: a Danish demographic study of 40 patients registered between 1978 and 2000. Acta Otolaryngol. 2003 Apr. 123(3):433-9. [Medline].

  8. Kane AJ, Sughrue ME, Rutkowski MJ, Aranda D, Mills SA, Buencamino R, et al. Posttreatment prognosis of patients with esthesioneuroblastoma. J Neurosurg. 2010 Aug. 113(2):340-51. [Medline].

  9. Magnavita N, Sacco A, Bevilacqua L, D'Alessandris T, Bosman C. Aesthesioneuroblastoma in a woodworker. Occup Med (Lond). 2003 May. 53(3):231-4. [Medline].

  10. Tseng J, Michel MA, Loehrl TA. Peripheral cysts: a distinguishing feature of esthesioneuroblastoma with intracranial extension. Ear Nose Throat J. 2009 Jun. 88(6):E14. [Medline].

  11. Rostomily RC, Elias M, Deng M, Elias P, Born DE, Muballe D, et al. Clinical utility of somatostatin receptor scintigraphic imaging (octreoscan) in esthesioneuroblastoma: a case study and survey of somatostatin receptor subtype expression. Head Neck. 2006 Apr. 28(4):305-12. [Medline].

  12. Min KW. Usefulness of electron microscopy in the diagnosis of "small" round cell tumors of the sinonasal region. Ultrastruct Pathol. 1995 Sep-Oct. 19(5):347-63. [Medline].

  13. Argani P, Perez-Ordonez B, Xiao H. Olfactory neuroblastoma is not related to the Ewing family of tumors: absence of EWS/FLI1 gene fusion and MIC2 expression. Am J Surg Pathol. 1998 Apr. 22(4):391-8. [Medline].

  14. Hyams VJ, Batsakis JG, Michaels L. Olfactory neuroblastoma. In: Tumors of the Upper Respiratory Tract and Ear, Atlas of Tumor Pathology. Vol 25. Washington DC: Armed Forces Institute Press; 1988:. 240-8.

  15. Kadish S, Goodman M, Wang CC. Olfactory neuroblastoma. A clinical analysis of 17 cases. Cancer. 1976 Mar. 37(3):1571-6. [Medline].

  16. Morita A, Ebersold MJ, Olsen KD, Foote RL, Lewis JE, Quast LM. Esthesioneuroblastoma: prognosis and management. Neurosurgery. 1993 May. 32(5):706-14; discussion 714-5. [Medline].

  17. Dulguerov P, Calcaterra T. Esthesioneuroblastoma: the UCLA experience 1970-1990. Laryngoscope. 1992 Aug. 102(8):843-9. [Medline].

  18. Dulguerov P, Allal AS, Calcaterra TC. Esthesioneuroblastoma: a meta-analysis and review. Lancet Oncol. 2001 Nov. 2(11):683-90. [Medline].

  19. Fitzek MM, Thornton AF, Varvares M. Neuroendocrine tumors of the sinonasal tract. Results of a prospective study incorporating chemotherapy, surgery, and combined proton-photon radiotherapy. Cancer. 2002 May 15. 94(10):2623-34. [Medline].

  20. Nichols AC, Chan AW, Curry WT, Barker FG, Deschler DG, Lin DT. Esthesioneuroblastoma: the massachusetts eye and ear infirmary and massachusetts general hospital experience with craniofacial resection, proton beam radiation, and chemotherapy. Skull Base. 2008 Sep. 18(5):327-37. [Medline]. [Full Text].

  21. Madani I, Bonte K, Vakaet L, Boterberg T, De Neve W. Intensity-modulated radiotherapy for sinonasal tumors: Ghent University Hospital update. Int J Radiat Oncol Biol Phys. 2009 Feb 1. 73(2):424-32. [Medline].

  22. Sterzing F, Stoiber EM, Nill S, Bauer H, Huber P, Debus J, et al. Intensity Modulated Radiotherapy (IMRT) in the treatment of children and adolescents - a single institution''s experience and a review of the literature. Radiat Oncol. 2009 Sep 23. 4(1):37. [Medline]. [Full Text].

  23. Tselis N, Heyd R, Baghi M, Zamboglou N. Interstitial high-dose-rate-brachytherapy in advanced esthesioneuroblastoma. Laryngoscope. 2008 Nov. 118(11):2006-10. [Medline].

  24. Gupta S, Husain N, Sundar S. Esthesioneuroblastoma chemotherapy and radiotherapy for extensive disease: a case report. World J Surg Oncol. 2011 Oct 5. 9:118. [Medline]. [Full Text].

  25. Loy AH, Reibel JF, Read PW, Thomas CY, Newman SA, Jane JA, et al. Esthesioneuroblastoma: continued follow-up of a single institution's experience. Arch Otolaryngol Head Neck Surg. 2006 Feb. 132(2):134-8. [Medline].

  26. McElroy EA Jr, Buckner JC, Lewis JE. Chemotherapy for advanced esthesioneuroblastoma: the Mayo Clinic experience. Neurosurgery. 1998 May. 42(5):1023-7; discussion 1027-8. [Medline].

  27. Porter AB, Bernold DM, Giannini C, Foote RL, Link MJ, Olsen KD, et al. Retrospective review of adjuvant chemotherapy for esthesioneuroblastoma. J Neurooncol. 2008 Nov. 90(2):201-4. [Medline].

  28. Koka VN, Julieron M, Bourhis J. Aesthesioneuroblastoma. J Laryngol Otol. 1998 Jul. 112(7):628-33. [Medline].

  29. Mishima Y, Nagasaki E, Terui Y, Irie T, Takahashi S, Ito Y, et al. Combination chemotherapy (cyclophosphamide, doxorubicin, and vincristine with continuous-infusion cisplatin and etoposide) and radiotherapy with stem cell support can be beneficial for adolescents and adults with estheisoneuroblastoma. Cancer. 2004 Sep 15. 101(6):1437-44. [Medline]. [Full Text].

  30. Turano S, Mastroianni C, Manfredi C, Biamonte R, Ceniti S, Liguori V, et al. Advanced adult esthesioneuroblastoma successfully treated with cisplatin and etoposide alternated with doxorubicin, ifosfamide and vincristine. J Neurooncol. 2010 May. 98(1):131-5. [Medline].

  31. Castelnuovo P, Bignami M, Delù G, Battaglia P, Bignardi M, Dallan I. Endonasal endoscopic resection and radiotherapy in olfactory neuroblastoma: our experience. Head Neck. 2007 Sep. 29(9):845-50. [Medline].

  32. Castelnuovo PG, Delù G, Sberze F, Pistochini A, Cambria C, Battaglia P, et al. Esthesioneuroblastoma: endonasal endoscopic treatment. Skull Base. 2006 Feb. 16(1):25-30. [Medline]. [Full Text].

  33. Folbe A, Herzallah I, Duvvuri U, Bublik M, Sargi Z, Snyderman CH, et al. Endoscopic endonasal resection of esthesioneuroblastoma: a multicenter study. Am J Rhinol Allergy. 2009 Jan-Feb. 23(1):91-4. [Medline].

  34. Devaiah AK, Andreoli MT. Treatment of esthesioneuroblastoma: a 16-year meta-analysis of 361 patients. Laryngoscope. 2009 Jul. 119(7):1412-6. [Medline].

  35. Zanation AM, Ferlito A, Rinaldo A, Gore MR, Lund VJ, McKinney KA, et al. When, how and why to treat the neck in patients with esthesioneuroblastoma: a review. Eur Arch Otorhinolaryngol. 2010 Nov. 267(11):1667-71. [Medline]. [Full Text].

  36. Beitler JJ, Fass DE, Brenner HA, Huvos A, Harrison LB, Leibel SA, et al. Esthesioneuroblastoma: is there a role for elective neck treatment?. Head Neck. 1991 Jul-Aug. 13(4):321-6. [Medline].

  37. Rosenthal DI, Barker JL, El-Naggar AK. Sinonasal malignancies with neuroendocrine differentiation: patterns of failure according to histologic phenotype. Cancer. 2004 Dec 1. 101(11):2567-73.

  38. Bradley PJ, Jones NS, Robertson I. Diagnosis and management of esthsioneuroblastoma. Curr OPin Otolaryngol Head Neck Surg. 2003. 11:112-118.

  39. Brodeur GM, Pritchard J, Berthold F, Carlsen NL, Castel V, Castelberry RP, et al. Revisions of the international criteria for neuroblastoma diagnosis, staging and response to treatment. Prog Clin Biol Res. 1994. 385:363-9. [Medline].

  40. Constantinidis J, Steinhart H, Koch M. Olfactory neuroblastoma: the University of Erlangen-Nuremberg experience 1975-2000. Otolaryngol Head Neck Surg. 2004 May. 130(5):567-74.

  41. Devaney K, Wenig BM, Abbondanzo SL. Olfactory neuroblastoma and other round cell lesions of the sinonasal region. Mod Pathol. 1996 Jun. 9(6):658-63. [Medline].

  42. Dias FL, Sa GM, Lima RA. Patterns of failure and outcome in esthesioneuroblastoma. Arch Otolaryngol Head Neck Surg. 2003 Nov. 129(11):1186-92. [Medline].

  43. Dulguerov P, Allal AS. Nasal and paranasal sinus carcinoma: how can we continue to make progress?. Curr Opin Otolaryngol Head Neck Surg. 2006 Apr. 14(2):67-72.

  44. Dulguerov P, Jacobsen MS, Allal AS, Lehmann W, Calcaterra T. Nasal and paranasal sinus carcinoma: are we making progress? A series of 220 patients and a systematic review. Cancer. 2001 Dec 15. 92(12):3012-29. [Medline].

  45. Frierson HF Jr, Mills SE, Fechner RE. Sinonasal undifferentiated carcinoma. An aggressive neoplasm derived from schneiderian epithelium and distinct from olfactory neuroblastoma. Am J Surg Pathol. 1986 Nov. 10(11):771-9. [Medline].

  46. Ganly I, Patel SG, Singh B, Kraus DH, Bridger PG, Cantu G, et al. Craniofacial resection for malignant paranasal sinus tumors: Report of an International Collaborative Study. Head Neck. 2005 Jul. 27(7):575-84. [Medline].

  47. Lund VJ, Howard D, Wei W. Olfactory neuroblastoma: past, present, and future?. Laryngoscope. 2003 Mar. 113(3):502-7. [Medline].

  48. Perez-Ordonez B, Caruana SM, Huvos AG. Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses. Hum Pathol. 1998 Aug. 29(8):826-32. [Medline].

  49. Silva EG, Butler J, MacKay B, et al. Neuroblastomas and neuroendocrine carcinomas of the nasal cavity. A proposed new classification. Cancer. 2006. 50:2388-2405.

 
Previous
Next
 
Esthesioneuroblastoma. Coronal CT scan of the orbits and sinuses shows a large, enhancing, and expansile mass occupying the ethmoid air cells that is invading the cribriform plate and breaking through to the left anterior cranial fossa. Image courtesy of Michael Lev, MD.
Esthesioneuroblastoma. A 39-year-old man presented with 1 month of decreased vision, left facial numbness, and swelling. Physical examination demonstrated left-sided exophthalmos and blindness. He had also lost his sense of smell. Contrast-enhanced T1-weighted MRI demonstrated a large lesion that originated in the paranasal sinuses and extended through the cribriform plate into the anterior cranial fossa. He underwent a bifrontal craniotomy for resection of this tumor.
Table. Histopathologic Grading According to Hyams [14]
Grade Lobular Architecture Preservation Mitotic Index Nuclear Polymorphism Fibrillary Matrix Rosettes Necrosis
I + Zero None Prominent HW rosettes None
II + Low Low Present HW rosettes None
III +/- Moderate Moderate Low FW rosettes Rare
IV +/- High High Absent None Frequent
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.