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Esthesioneuroblastoma

esthesioneuroblastoma, esthesioneuroblastoma survival rate
Esthesioneuroblastoma, also called "olfactory neuroblastoma", is a rare cancer of the nasal cavity Arising from the upper nasal tract, esthesioneuroblastoma is believed to originate from sensory neuroepithelial cells, also known as neuroectodermal olfactory cells1 Fewer than 700 cases have been documented in the United States2 Due to the location of the tumor and its proximity to the cranial cavity, esthesioneuroblastoma can be highly invasive and challenging to treat There is no consensus on appropriate treatment approach of esthesioneuroblastoma because of the rarity of the disease Most studies reported cranial surgical resection with radiotherapy or chemotherapy to target the tumor3

Esthesioneuroblastoma was first characterized in 192445

Contents

  • 1 Signs and Symptoms
  • 2 Pathophysiology
  • 3 Incidence and prevalence
  • 4 Diagnosis
  • 5 Treatment
    • 51 Surgical Approaches
    • 52 Radiotherapy
    • 53 Chemotherapy
  • 6 Prognosis
    • 61 Classification
  • 7 Notable cases
  • 8 References

Signs and Symptomsedit

Esthesioneuroblastoma will first frequently present as a nasal mass6 The most common signs and symptoms of esthesioneuroblastoma are nasal obstruction 70% and epistaxis 50%78 Less common symptoms include hyposmia loss of smell, headache, rhinorrhea, vision loss, proptosis, facial pain, diplopia double vision, masses in the neck and changes in mental status9 Esthesioneuroblastoma occurs in the upper nasal cavity, near the optic nerves and optic chiasm Thus, tumor growth can impinge nerve function and result in vision loss and diplopia10 As the tumor metastasizes to the oral cavity, there can be tooth pain and tooth mobility11

Pathophysiologyedit

Esthesioneuroblastoma is of neurocrest origin, arising from olfactory sensory cells in the olfactory epithelium12 The olfactory epithelium consists of olfactory sensory cells, sustentacular cells and basal cells13 Esthesioneuroblastoma consists of lobular sheets with neurofibrullar fibers and rosettes14 Hyam's classifications are an important way of determining prognosis15 Cancer is the mutation and unregulated cellular growth of tissues There is limited research on the genetic role in esthesioneuroblastoma development Of the research to date, the sonic hedgehog pathway, MYC and KDR genes are implicated for esthesioneuroblastoma1617

Hyam's Histopathological Grades for Esthesioneuroblastoma18

Grade Lobular Architexture Preservation Mitotic Index Nuclear Polymorphism Fibrillary Matrix Rosettes Necrosis
I + none none prominent Homer Wright HW rosettes none
II + low moderate present HW rosettes none
III +/- moderate prominent low Flexner-Wintersteiner rosettes rare
IV +/- high marked absent none frequent

Incidence and prevalenceedit

Esthesioneuroblastoma accounts for 2% of all intranasal tumors with an incidence of 04 cases per million people19 Fewer than 700 cases of esthesioneuroblastoma have been seen in the US since 198820 Fewer than 400 unique cases have been reported globally2122 Esthesioneuroblastoma can occur at any time, with peak occurrence reported in the second and sixth decade of life23

Diagnosisedit

Esthesioneuroblastoma can resemble small blue cell tumors like squamous cell carcinoma, sinonasal undifferentiated carcinoma, extranodal NK/T cell lymphoma, nasal type, rhabdomyosarcoma, Ewing/PNET, mucosal malignant melanoma and neuroendocrine carcinomas NEC that occur in the intranasal tract24 Compared to other tumors in the region, esthesioneuroblastoma has the best prognosis, with an overall 5 year survival rate of 60-80%25 Fewer than 700 cases have been documented in the United States alone26 Esthesioneuroblastoma is characterized by neurofibrillary stroma and neurosecretary granules that are not seen concurrently by any other pathologies in the region27 Histological tests such as keratin, CK5/6, S-100 protein or NSE can be run to further differentiate esthesioneuroblastoma from other tumors28

Treatmentedit

The preferred treatment for esthesioneuroblastoma is surgery followed by radiotherapy to prevent reoccurrence of the tumor29

Surgical Approachesedit

Several surgical approaches have been described,3031323334 but post-excision recurrence rates have remained relatively high Studies suggest better results with a bilateral approach For cases with cribriform plate involvement, tumors are resected bilaterally using a transfacial and craniotomy approach35 In a research study, the craniofacial approach decreased reoccurrence of esthesioneuroblastoma by 20%36 Craniofacial resection can help preserve the optic nerves and brain while removing the cribriform plate, olfactory bulb, dura surrounding the bulb and even the orbital periosteum37

Radiotherapyedit

Radiotherapy alone is reserved only for small lesions not appropriate for either surgery or chemotherapy38 Both photon and proton radiotherapy have been used effectively to treat esthesioneuroblastoma3940 Proton radiotherapy has recently been shown to be effective in a 10-person study with Kadish C tumors, while delivering less toxicity to the nervous system41

Chemotherapyedit

Chemotherapy is used in a multimodality treatment plan generally for more advanced, unresectable or reoccurring tumors42 Cyclophosphamide, vincristine and doxorubicin have been used as neoadjuvant chemotherapy drugs for grade C esthesioneuroblastoma before surgical resection, producing fair outcomes Cisplatin and etoposide are often used to treat esthesioneuroblastoma as neoadjuvants or adjuvants with radiotherapy or surgery434445 Study results are promising In advanced stage esthesioneuroblastoma in pediatric patients, where surgery is no longer possible, aggressive chemotherapy and radiotherapy has resulted in some tumor control and long term survival46

Prognosisedit

Esthesioneuroblastoma is a slow developing but malignant tumor with high reoccurrence rates because of its anatomical position47 The tumor composition, location and metastatic characteristics as well as the treatment plan determine prognosis Common clinical classification systems for esthesioneuroblastoma include the Kadish classification and the Dulguerov classfictation Histopathological characteristics on top of Kadish classification can further determine cancer prognosis In severe, Kadish class C tumors, Haym's grades of pathology are important for prognosis Patients with low grade Kadish class C tumors have a 10-year survival rate of 86 percent compared to patients with high grade class C tumors who have a survival rate of 28 percent48 Surgically treated patients with high grade tumors are more likely to experience leptomeningeal metastases or involvement of the cerebral spinal fluid unlike patients with low grade tumors who usually only see local recurrence49 Survival rates for treated esthesioneuroblastoma are best for surgery with radiotherapy 65%, then for radiotherapy and chemotherapy 51%, just surgery 48%, surgery, radiotherapy and chemotherapy 47 and finally just radiotherapy 37%50 From the literature, radiotherapy and surgery seem to boast the best outcome for patients However, it is important to understand that to some degree, prognosis is related to tumor severity More progressed, higher grade tumors would result in chemotherapy or radiotherapy as the only treatment It is no surprise that the prognosis would be worse in these cases

Classificationedit

The Kadish classification is used for clinical classification of sinonasal tumors including esthesioneuroblastoma Subsequent research articles have been published to determine prognosis based on tumor grade

Modified Kadish Classification515253

Stage Description 5 Year Survival
A Tumor confined to nasal cavity 75-91
B Nasal cavity and paranasal sinuses 68-71
C Tumor extends beyond nasal cavity and paranasal sinuses, including skull base, orbit or cribiform plate 41-47
D Tumor metastasizes to cervical lymph nodes and beyond <40

Dulguerov Classification54

Stage Characteristics
T1 Tumour involving the nasal cavity and/or paranasal sinuses excluding sphenoid, sparing the most superior ethmoidal cells
T2 Tumour involving the nasal cavity and/or paranasal sinuses including the sphenoid with extension to or erosion of the cribriform plate
T3 Tumour extending into the orbit or protruding into the anterior cranial fossa, without dural invasion
T4 Tumour involving the brain
N0 No cervical lymph-node metastasis
N1 Any form of cervical lymph-node metastasis
M0 No metastases
M1 Distant metastasis

Notable casesedit

The disease was brought into prominence by the case of Chantal Sébire, who was suffering from the disease and ended her life after being denied euthanasia55

Referencesedit

  1. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  2. ^ Stokes, W A, Camilon, P R, Banglawala, S M, Nguyen, S A, Harvey, R, Vandergrift, W A, & Schlosser, R J Is sex an independent prognostic factor in esthesioneuroblastoma American journal of rhinology & allergy, 5, 369–372
  3. ^ Jiang, G Y, Li, F C, Chen, W K, Liu, A M, & Cai, W Q 2011 Therapy and prognosis of intracranial invasive olfactory neuroblastoma Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery, 6, 951–955
  4. ^ "Esthesioneuroblastoma" Archived from the original on April 9, 2007 Retrieved 2008-03-22 
  5. ^ Berger L, Luc G, Richard D L'esthésioneuroépithéliome olfactif Bull Assoc Franç Étude Cancer 1924;13:410-421
  6. ^ Ghaffar, S, & Salahuddin, I 2005 Olfactory neuroblastoma: a case report and review of the literature Ear, nose, & throat journal, 3, 150–152
  7. ^ Sheehan, J M 2011 Esthesioneuroblastoma In J A Jane Ed, Youmans Neurological Surgery 6th ed Retrieved December 7, 2016, from https://www-clinicalkey-com/content/book/3-s20-B9781416053163001465
  8. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  9. ^ Sheehan, J M 2011 Esthesioneuroblastoma In J A Jane Ed, Youmans Neurological Surgery 6th ed Retrieved December 7, 2016, from https://www-clinicalkey-com/content/book/3-s20-B9781416053163001465
  10. ^ Oskouian, R J, Jane, J A, Dumont, A S, Sheehan, J M, Laurent, J J, & Levine, P A 2002 Esthesioneuroblastoma: clinical presentation, radiological, and pathological features, treatment, review of the literature, and the University of Virginia experience Neurosurgical focus, 5, e4
  11. ^ von Zeidler, S V, Guidi, R, Alencar, R d e C, Aguiar, R, Mendonça, E F, Batista, A C, & Ribeiro-Rotta, R F 2014 Atypical esthesioneuroblastoma invading oral cavity: a case report and review of the literature Diagnostic pathology, , 10
  12. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  13. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  14. ^ Sheehan, J M 2011 Esthesioneuroblastoma In J A Jane Ed, Youmans Neurological Surgery 6th ed Retrieved December 7, 2016, from https://www-clinicalkey-com/content/book/3-s20-B9781416053163001465
  15. ^ Kaur, G, Kane, A J, Sughrue, M E, Madden, M, Oh, M C, Sun, M Z, Safaee, M, El-Sayed, I, Aghi, M, McDermott, M W, Berger, M S, & Parsa, A T 2012 The prognostic implications of Hyam's subtype for patients with Kadish stage C esthesioneuroblastoma Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia, 2, 281–286
  16. ^ Mao, L, Xia, Y P, Zhou, Y N, Dai, R L, Yang, X, Wang, Y J, Duan, S J, Qiao, X, Mei, Y W, & Hu, B 2009 Activation of sonic hedgehog signaling pathway in olfactory neuroblastoma Oncology, 3-4, 231–243
  17. ^ Weiss, G J, Liang, W S, Izatt, T, Arora, S, Cherni, I, Raju, R N, Hostetter, G, Kurdoglu, A, Christoforides, A, Sinari, S, Baker, A S, Metpally, R, Tembe, W D, Phillips, L, Von Hoff, D D, Craig, D W, & Carpten, J D 2012 Paired tumor and normal whole genome sequencing of metastatic olfactory neuroblastoma PloS one, 5, e37029
  18. ^ VJ Hyams, JG Baksakis, L Michaels Eds, Tumors of the upper respiratory tract and ear, Armed Forces Institute of Pathology, Washington DC 1988, pp 240–248
  19. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  20. ^ Stokes, W A, Camilon, P R, Banglawala, S M, Nguyen, S A, Harvey, R, Vandergrift, W A, & Schlosser, R J Is sex an independent prognostic factor in esthesioneuroblastoma American journal of rhinology & allergy, 5, 369–372
  21. ^ Sheehan, J M 2011 Esthesioneuroblastoma In J A Jane Ed, Youmans Neurological Surgery 6th ed Retrieved December 7, 2016, from https://www-clinicalkey-com/content/book/3-s20-B9781416053163001465
  22. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  23. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  24. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  25. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  26. ^ Stokes, W A, Camilon, P R, Banglawala, S M, Nguyen, S A, Harvey, R, Vandergrift, W A, & Schlosser, R J Is sex an independent prognostic factor in esthesioneuroblastoma American journal of rhinology & allergy, 5, 369–372
  27. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  28. ^ Thompson, L D 2009 Olfactory neuroblastoma Head and neck pathology, 3, 252–259
  29. ^ Dulguerov, P, Allal, A S, & Calcaterra, T C 2001 Esthesioneuroblastoma: a meta-analysis and review The Lancet Oncology, 11, 683–690
  30. ^ Roxbury, Christopher R; Ishii, Masaru; Gallia, Gary L; Reh, Douglas D 2016 "Endoscopic Management of Esthesioneuroblastoma" Otolaryngologic Clinics of North America 49 1: 153–165 ISSN 0030-6665 doi:101016/jotc201509010 
  31. ^ Vergani F, Pirola E, Fiori L, Pagni F, Parmigiani F, Sganzerla EP 2007 "Combined transcranial and endoscopic nasal resection for esthesioneuroblastoma Technical note" J Neurosurg Sci 51 2: 99–102 PMID 17571044 
  32. ^ Unger F, Walch C, Stammberger H, Papaefthymiou G, Haselsberger K, Pendl G 2001 "Olfactory neuroblastoma esthesioneuroblastoma: report of six cases treated by a novel combination of endoscopic surgery and radiosurgery" Minim Invasive Neurosurg 44 2: 79–84 PMID 11487789 doi:101055/s-2001-16000 
  33. ^ Cakmak O, Ergin NT, Yilmazer C, Kayaselçuk F, Barutcu O 2002 "Endoscopic removal of esthesioneuroblastoma" Int J Pediatr Otorhinolaryngol 64 3: 233–8 PMID 12090952 doi:101016/S0165-58760200036-8 
  34. ^ Prasad KC, Kumar A, Prasad SC, Jain D 2007 "Endoscopic-assisted excision of esthesioneuroblastoma" J Craniofac Surg 18 5: 1034–8 PMID 17912077 doi:101097/scs0b013e318157264c 
  35. ^ CA Spaulding, MS Kranyak, WC Constable, FM Stewart, Esthesioneuroblastoma: a comparison of two treatment eras, Int J Radiat Oncol Biol Phys, 15 1988, pp 581–590
  36. ^ CA Spaulding, MS Kranyak, WC Constable, FM Stewart, Esthesioneuroblastoma: a comparison of two treatment eras, Int J Radiat Oncol Biol Phys, 15 1988, pp 581–590
  37. ^ Dulguerov, P, Allal, A S, & Calcaterra, T C 2001 Esthesioneuroblastoma: a meta-analysis and review The Lancet Oncology, 11, 683–690
  38. ^ Sheehan, J M 2011 Esthesioneuroblastoma In J A Jane Ed, Youmans Neurological Surgery 6th ed Retrieved December 7, 2016, from https://www-clinicalkey-com/content/book/3-s20-B9781416053163001465
  39. ^ Dulguerov, P, Allal, A S, & Calcaterra, T C 2001 Esthesioneuroblastoma: a meta-analysis and review The Lancet Oncology, 11, 683–690
  40. ^ Nichols AC, Chan AW, Curry WT, et al: Esthesioneuroblastoma: the Massachusetts Eye and Ear Infirmary and Massachusetts General Hospital experience with craniofacial resection, proton beam radiation, and chemotherapy Skull Base 2008; 18: pp 327-337
  41. ^ Nichols AC, Chan AW, Curry WT, et al: Esthesioneuroblastoma: the Massachusetts Eye and Ear Infirmary and Massachusetts General Hospital experience with craniofacial resection, proton beam radiation, and chemotherapy Skull Base 2008; 18: pp 327-337
  42. ^ Sheehan, J M 2011 Esthesioneuroblastoma In J A Jane Ed, Youmans Neurological Surgery 6th ed Retrieved December 7, 2016, from https://www-clinicalkey-com/content/book/3-s20-B9781416053163001465
  43. ^ Porter AB, Bernold DM, Giannini C, et al: Retrospective review of adjuvant chemotherapy for esthesioneuroblastoma J Neurooncol 2008; 90: pp 201-204
  44. ^ Bhattacharyya N, Thornton AF, Joseph MP, et al: Successful treatment of esthesioneuroblastoma and neuroendocrine carcinoma with combined chemotherapy and proton radiation Arch Otolaryngol Head Neck Surg 1997; 123: pp 34-40
  45. ^ Kim DW, Jo YH, Kim JH, et al: Neoadjuvant etoposide, ifosfamide, and cisplatin for the treatment of olfactory neuroblastoma Cancer 2004; 101: pp 2257-2260
  46. ^ Bisogno G, Soloni P, Conte M, et al: Esthesioneuroblastoma in pediatric and adolescent age A report from the TREP project in cooperation with the Italian Neuroblastoma and Soft tissue sarcoma Committees BMC Cancer 2012; 12: pp 117
  47. ^ Kaur, G, Kane, A J, Sughrue, M E, Madden, M, Oh, M C, Sun, M Z, Safaee, M, El-Sayed, I, Aghi, M, McDermott, M W, Berger, M S, & Parsa, A T 2012 The prognostic implications of Hyam's subtype for patients with Kadish stage C esthesioneuroblastoma Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia, 2, 281–286
  48. ^ Kaur, G, Kane, A J, Sughrue, M E, Madden, M, Oh, M C, Sun, M Z, Safaee, M, El-Sayed, I, Aghi, M, McDermott, M W, Berger, M S, & Parsa, A T 2012 The prognostic implications of Hyam's subtype for patients with Kadish stage C esthesioneuroblastoma Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia, 2, 281–286
  49. ^ Malouf GG, Casiraghi O, Deutsch E, Guigay J, Temam S, Bourhis J Low- and high-grade esthesioneuroblastomas display a distinct natural history and outcome Eur J Cancer 2013;496:1324-34
  50. ^ Dulguerov P, Allal AS, Calcaterra TC Esthesioneuroblastoma: a meta-analysis and review Lancet Oncol 2001;211:683-90
  51. ^ Kadish, S, Goodman, M, & Wang, C C 1976 Olfactory neuroblastoma A clinical analysis of 17 cases Cancer, 3, 1571–1576
  52. ^ Sheehan, J M 2011 Esthesioneuroblastoma In J A Jane Ed, Youmans Neurological Surgery 6th ed Retrieved December 7, 2016, from https://www-clinicalkey-com/content/book/3-s20-B9781416053163001465
  53. ^ Kadish, S, Goodman, M, & Wang, C C 1976 Olfactory neuroblastoma A clinical analysis of 17 cases Cancer, 3, 1571–1576
  54. ^ Dulguerov, P, Allal, A S, & Calcaterra, T C 2001 Esthesioneuroblastoma: a meta-analysis and review The Lancet Oncology, 11, 683–690
  55. ^ "BBC NEWS - Europe - Tumour woman's death not natural" BBC 21 March 2008 Retrieved 1 November 2016 

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    Esthesioneuroblastoma beatiful post thanks!

    29.10.2014


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