Esthesioneuroblastoma

Esthesioneuroblastoma
Objawy

Esthesioneuroblastoma to rzadki, złośliwy nowotwór wywodzący się z nabłonka węchowego w górnej części jamy nosowej, charakteryzujący się zmiennym przebiegiem klinicznym od powolnego wzrostu do agresywnego rozrostu z szybkim przerzutowaniem. Wczesne objawy obejmują jednostronną niedrożność nosa (70% pacjentów), krwawienia z nosa (około 50%), utratę lub zaburzenia węchu oraz wyciek z nosa, które mogą utrzymywać się od 6 do 12 miesięcy, a nawet lat przed diagnozą. W miarę progresji guza pojawiają się objawy związane z naciekaniem oczodołu (ból oka, diplopia, proptosis), przedniego dołu czaszki i mózgu (bóle głowy, zmiany stanu psychicznego, drgawki), a także objawy ze strony ucha i twarzy. Przerzuty do węzłów chłonnych szyi występują u 10-44% pacjentów, a odległe przerzuty u około 10%, najczęściej do płuc, kości, wątroby i skóry.

Symptomy Esthesioneuroblastoma

Esthesioneuroblastoma (znany również jako neuroblastoma węchowy) to rzadki, złośliwy nowotwór wywodzący się z nabłonka węchowego w górnej części jamy nosowej. Ze względu na swoją lokalizację i rzadkość występowania, jego objawy mogą być początkowo mylone z bardziej powszechnymi schorzeniami nosa i zatok, co często prowadzi do opóźnienia w diagnozie.¹²

Wczesne objawy nosowe

Wczesne objawy esthesioneuroblastoma są związane głównie z jamą nosową i często naśladują objawy pospolitych chorób nosa i zatok. Do najczęstszych wczesnych objawów należą:

Niedrożność nosa – najczęstszy objaw, występujący u 70% pacjentów, często jednostronna¹²
Krwawienia z nosa (epistaxis) – występują u około 50% pacjentów, mogą być nawracające¹²
Utrata węchu (anosmia) lub jego zaburzenia (hyposmia) – częsty objaw wynikający z zajęcia płytki sitowej¹²
Wyciek z nosa – może być przezroczysty, krwisty lub ropny¹

Co istotne, objawy te mogą utrzymywać się przez miesiące, a nawet lata przed postawieniem diagnozy. Średni czas od pojawienia się pierwszych objawów do diagnozy wynosi od 6 do 12 miesięcy, a w niektórych przypadkach diagnozy są opóźnione o lata.¹²

Objawy związane z miejscowym rozprzestrzenianiem się guza

Wraz z rozwojem guza i jego rozprzestrzenianiem się na okoliczne struktury, mogą pojawić się dodatkowe objawy:

Objawy oczne

Gdy guz rozrasta się w kierunku oczodołu, mogą wystąpić:¹²

Ból oka
Podwójne widzenie (diplopia)
Wytrzeszcz gałki ocznej (proptosis)
Nadmierne łzawienie (epifora)
Zaburzenia widzenia, w tym utrata wzroku
Obrzęk powiek

Objawy neurologiczne

Przy rozroście guza do przedniego dołu czaszki i mózgu mogą pojawić się:¹²

Bóle głowy – często zlokalizowane w okolicy czołowej
Zmiany stanu psychicznego
Nudności i wymioty
Drgawki
Zaburzenia pamięci
Zmiany osobowości

Inne objawy miejscowe

Ból ucha i wysiękowe zapalenie ucha środkowego – często wynikające z zablokowania trąbki Eustachiusza¹
Ból i obrzęk twarzy¹
Objawy stomatologiczne – ruchomość zębów, ból zębów¹²

Zespół paraneoplastyczny

W rzadkich przypadkach esthesioneuroblastoma może powodować zespoły paraneoplastyczne, w tym:¹²

Zespół nieadekwatnego wydzielania hormonu antydiuretycznego (SIADH)
Ektopowe wydzielanie ACTH
Hiperkalcemia nowotworowa
Nadciśnienie spowodowane wydzielaniem katecholamin przez guz

Przebieg i progresja choroby

Charakterystyka wzrostu guza

Esthesioneuroblastoma charakteryzuje się zróżnicowanym przebiegiem klinicznym:¹²

Zmienny charakter biologiczny – od powolnego, indolentnego wzrostu (z przeżyciem pacjentów przekraczającym 20 lat) do wysoce agresywnego nowotworu zdolnego do szybkiego przerzutowania, z przeżyciem ograniczonym do kilku miesięcy
Najczęściej wolno rosnący guz – co przyczynia się do opóźnienia diagnozy
Miejscowo agresywny – z tendencją do naciekania okolicznych struktur

Guz ma tendencję do rozprzestrzeniania się wzdłuż płytki sitowej i może tworzyć charakterystyczny kształt „hantle” z „talią” w miejscu, gdzie przechodzi przez płytkę sitową.¹

Rozprzestrzenianie się miejscowe

Esthesioneuroblastoma często wykazuje wzorzec miejscowego rozprzestrzeniania się, w tym:¹²

Zatoki przynosowe – najczęściej sitowe, czołowe i szczękowe
Oczodół – prowadzące do objawów ocznych
Przedni dół czaszki – z możliwością naciekania mózgu

W momencie diagnozy około 25-30% pacjentów ma już rozrost guza do przestrzeni wewnątrzczaszkowej.¹

Przerzuty i rozprzestrzenianie się systemowe

Esthesioneuroblastoma może dawać przerzuty poprzez drogi krwionośne i limfatyczne:¹²

Węzły chłonne szyi – najczęstsze miejsce przerzutów, występujące u 10-44% pacjentów w momencie diagnozy i u około 35% pacjentów w przebiegu choroby¹²
Przerzuty odległe – występują u około 10% pacjentów i najczęściej dotyczą płuc, kości, wątroby i skóry¹²

U większości dzieci z esthesioneuroblastoma choroba jest już zaawansowana w momencie diagnozy.¹

Nawroty choroby

Nawroty są częstym problemem w przebiegu esthesioneuroblastoma:¹²

Częstość nawrotów – wynosi około 30-60% po skutecznym leczeniu guza pierwotnego¹
Czas do nawrotu – nawroty często występują z opóźnieniem, zazwyczaj w ciągu pierwszych 2 lat od wystąpienia (około 30% przypadków), ale mogą pojawić się nawet po latach od zakończenia leczenia¹²
Charakter nawrotów – najczęściej są to nawroty miejscowe, ale guz wykazuje również skłonność do nawrotów węzłowych i odległych niezależnie od początkowego stopnia zaawansowania¹
Wtórne nawroty – średni czas do wystąpienia wtórnego nawrotu jest znacząco krótszy niż czas do wystąpienia pierwotnego nawrotu (32 miesiące vs. 57 miesięcy)¹

Warto zauważyć, że pacjenci z dodatnimi marginesami resekcji mogą mieć nieco wyższe wskaźniki nawrotów i krótszy czas do nawrotu w porównaniu z pacjentami z ujemnymi marginesami.¹

Rokowanie

Rokowanie w esthesioneuroblastoma zależy od kilku czynników:¹²

Stadium zaawansowania – 5-letnie przeżycie guzów w stadium A wynosi 75-90%, podczas gdy w stadium C spada do 45%¹
Stopień złośliwości guza – guzy o wysokim stopniu złośliwości mają 25% 5-letniego przeżycia, podczas gdy guzy o niskim stopniu złośliwości mają 80% przeżycia¹
Negatywne wskaźniki prognostyczne – obecność przerzutów, wysokie stadium, wysoki stopień złośliwości guza, wiek powyżej 50 lub poniżej 20 lat w momencie diagnozy, rozprzestrzenianie wewnątrzczaszkowe, wysoka proliferacja, płeć żeńska oraz poliploidia/aneuploidia¹

Ogólnie, wskaźniki przeżycia 5-letniego wynoszą od 50% do 90%, w zależności od stadium choroby.¹ Szczególnie obiecujące wyniki obserwuje się w przypadku małych, zlokalizowanych guzów, które mogą osiągać wskaźniki wyleczenia do 85-90%.¹

Długoterminowa obserwacja

Ze względu na wysokie ryzyko nawrotów, pacjenci z esthesioneuroblastoma wymagają długoterminowej obserwacji:¹²

Regularne wizyty kontrolne – zaleca się wizyty u zespołu medycznego co najmniej raz w roku przez resztę życia pacjenta
Badania obrazowe – regularne MRI, tomografia komputerowa i/lub PET w celu monitorowania nawrotów i przerzutów
Monitorowanie ogólnego stanu zdrowia – ze szczególnym uwzględnieniem objawów sugerujących nawrót choroby

Pacjenci powinni być świadomi, że nawet po zakończeniu leczenia mogą pojawić się późne nawroty, a nowe przypadki zespołu SIADH w okresie obserwacji mogą wskazywać na nawrót choroby.¹

Szczególne populacje pacjentów

Esthesioneuroblastoma u dzieci

Objawy esthesioneuroblastoma u dzieci mogą być podobne do tych u dorosłych, ale istnieją pewne różnice:¹²

Dzieci mogą nie wykazywać objawów, dopóki guz nie urośnie do znacznych rozmiarów
Większość dzieci jest diagnozowana w zaawansowanym stadium choroby
Dzieci są narażone na rozwój długoterminowych skutków ubocznych związanych z terapią

Objawy u dzieci, które powinny skłonić do konsultacji lekarskiej, obejmują: masę w nosie, trudności z oddychaniem przez nos, zmianę lub utratę węchu, krwawienia z nosa, bóle głowy, wytrzeszcz oka, problemy z widzeniem i ból oka.¹

Esthesioneuroblastoma zajmująca skórę

Esthesioneuroblastoma z zajęciem skóry wiąże się ze znacznie gorszymi wskaźnikami przeżycia:¹

5-letnie przeżycie całkowite: 0% vs. 45,5% u pacjentów bez zajęcia skóry
5-letnie przeżycie wolne od progresji: 0% vs. 31,3% u pacjentów bez zajęcia skóry

Podsumowanie najczęstszych objawów

Objawy esthesioneuroblastoma można podzielić na kilka kategorii w zależności od lokalizacji i stopnia zaawansowania guza:¹²

Objawy nosowe

Niedrożność nosa (70% pacjentów) – zazwyczaj jednostronna
Krwawienie z nosa (46-50% pacjentów)
Utrata węchu lub jego zaburzenia
Wyciek z nosa – może być przezroczysty, krwisty lub ropny

Objawy oczodołowe

Wytrzeszcz gałki ocznej
Podwójne widzenie
Pogorszenie ostrości wzroku
Ból oka
Nadmierne łzawienie

Objawy wewnątrzczaszkowe

Zmiany stanu psychicznego
Nudności i wymioty
Bóle głowy
Drgawki
Śpiączka (w skrajnych przypadkach)

Objawy związane z zablokowaniem trąbki Eustachiusza

Ból ucha
Wysiękowe zapalenie ucha środkowego

Objawy jamy ustnej

Ruchomość zębów
Niegojące się miejsca ekstrakcji zębów
Źle dopasowane protezy zębowe
Owrzodzenia

Należy pamiętać, że wczesne rozpoznanie esthesioneuroblastoma ma kluczowe znaczenie dla skutecznego leczenia i lepszych wyników. Pacjenci z utrzymującymi się lub nietypowymi objawami nosowymi, które nie reagują na standardowe leczenie, powinni zostać poddani dalszej ocenie medycznej.¹²

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Materiały źródłowe

#1 Esthesioneuroblastoma: Causes, Symptoms & Treatment
https://my.clevelandclinic.org/health/diseases/esthesioneuroblastoma
Esthesioneuroblastoma may make you feel like you have a sinus infection or other sinus issues, including: Anosmia (loss of sense of smell). […] It often comes back after treatment and may come back years after you’ve finished treatment. Recurrent esthesioneuroblastoma may come back in the original site or in nearby tissue. […] Without treatment, it can spread (metastasize) to your brain or other areas of your body. Studies show esthesioneuroblastoma most often spreads to your sinuses, but it can spread to lymph nodes in your neck, your lungs and your bones. […] Esthesioneuroblastoma can come back, so you should plan on seeing your care team at least once a year for the rest of your life. Your team will check on your overall health. They may do imaging tests to look for signs of recurring esthesioneuroblastoma.
#1 Esthesioneuroblastoma – Wikipedia
https://en.wikipedia.org/wiki/Esthesioneuroblastoma
Esthesioneuroblastoma frequently first presents as a nasal mass. The most common signs and symptoms of esthesioneuroblastoma are nasal obstruction (70%) and epistaxis (50%). 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 status. 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 diplopia. As the tumor metastasizes to the oral cavity, there can be tooth pain and tooth mobility. […] Esthesioneuroblastoma is a slow developing but malignant tumor with high recurrence rates because of its anatomical position. 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 classification. 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 percent. 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 recurrence. […] 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%).
#1 esthesioneuroblastoma
http://www.aboutcancer.com/esthesioneuroblastoma.htm
ONB occurs over a broad age range (three to 88 years) with a mean age of 45 years. Approximately one-fifth of cases occur between the ages of 11 and 20. There is no gender predilection. […] The most common symptom is unilateral nasal congestion or obstruction. In one series of 22 patients, the most common symptoms were nasal congestion (64 percent), anosmia (55 percent), and recurrent epistaxis and pain (36 percent each). Other symptoms included frontal headache and diplopia. The symptoms are related both to the site and local extent of the tumor: […] Anosmia is caused by involvement of the cribriform plate, while epistaxis reflects the marked vascularity of the tumor. […] Pain, proptosis, and excessive lacrimation are secondary to orbital extension. […] Ear pain and otitis media result from obstruction of the eustachian tube. […] Frontal headache suggests involvement of the frontal sinus. […] Early diagnosis is uncommon due to the nonspecificity of symptoms.
#1 Esthesioneuroblastoma – Prognosis and Treatmentphone iconphone iconGroup 49
https://www.barrowneuro.org/condition/esthesioneuroblastoma/
Frequent and unexplained nosebleeds can occur as the tumor grows and affects the nasal tissues. […] You might experience a discharge from one side of your nose, which can be clear, bloody, or resemble pus. […] As the tumor grows, it can press on surrounding structures and cause headaches. […] In advanced cases, if the tumor grows upwards into the brain or towards the eyes, it may cause changes in vision, such as double vision or a decrease in vision. […] The tumorâs growth can also lead to swelling or pain in the face, especially around the eyes or in the forehead. […] If the tumor affects the nerves or muscles around the eyes, it may cause difficulty moving the eyes, leading to double vision or a noticeable difference in eye positioning. […] Other, more benign conditions can also cause these symptoms. However, if you notice these symptoms persisting, especially if theyâre only on one side or if they donât improve with standard treatments, itâs crucial to see a healthcare provider for further evaluation. Early detection and treatment of esthesioneuroblastoma can significantly affect the outcome.
#1
https://med.uth.edu/orl/2012/09/07/esthesioneuroblastoma/
The presenting symptoms depend on the location and extent of the tumor. The most common symptoms are unilateral nasal obstruction, nasal bleeding, headaches, facial pain, and decreased sense of smell. […] Intracranial extension may lead to neurological symptoms such as changes in mental status, headaches, nausea and vomiting, seizures, and eventually coma and death. Other symptoms include ear pain and serous otitis media, which usually result from the obstruction of the eustachian tube. […] In most studies, the mean time from onset of symptoms to diagnosis of ENB ranges between 6-12 months. For this reason, many patients have advanced stage at the time of diagnosis. A high index of suspicion is therefore critical to achieve an accurate and timely diagnosis.
#1 Esthesioneuroblastoma – EyeWiki
https://eyewiki.org/Esthesioneuroblastoma
Esthesioneuroblastoma (ENB) is a rare malignant tumor of the upper nasal cavity and anterior skull base first described in 1924 by Berger et al. Local invasion into the orbit, sinuses and central nervous system is common. Signs and symptoms vary and depend on location and degree of invasion. […] Diagnosis begins with a thorough history as most patients will have chronic nasal obstruction and congestion refractory to standard medical treatment. Epistaxis is also common. The average delay between appearance of initial symptoms and diagnosis is 6 months as initial symptoms tend to be subtle and frequently found in common nasal diseases such as rhinosinusitis and allergic polypoid sinus disease. […] Clinically, symptoms depend on location and extent of the tumor. Initially, most symptoms tend to be those found in common nasal diseases such as congestion and obstruction. As the tumor grows, symptoms worsen depending on location and extent of disease. It is common to have local invasion into the orbit, sinuses and intracranial cavity through erosion of the cribriform plate. Worsening sinus invasion commonly causes facial pain and epistaxis, a symptom often indicating a malignant process. Extension into the orbit can cause injection, ptosis, diplopia, proptosis, decreased vision and epiphora. Intracranial extension can present with headaches, seizures and neurologic complications. […] Esthesioneuroblastoma requires lifelong surveillance for recurrence and metastasis. Most surgeons obtain regular imaging such as CT/MRI and PET scan to help monitor disease progression.
#1 Esthesioneuroblastoma: Types, Symptoms, Causes, Diagnosis.
https://www.medicoverhospitals.in/diseases/esthesioneuroblastoma/
Recognizing the symptoms of esthesioneuroblastoma is pivotal for early diagnosis and management. However, the nonspecific nature of its presentation often complicates timely detection. […] One of the most prevalent symptoms, nasal obstruction, is often mistaken for benign conditions such as sinusitis or allergic rhinitis. […] Recurrent nosebleeds are a hallmark symptom, frequently prompting further investigation. […] The loss of the sense of smell can occur as the tumor impinges upon the olfactory structures. […] As the tumor progresses, it may invade surrounding structures, leading to facial pain or numbness. […] In cases where the tumor extends into the orbit, patients may experience visual disturbances or proptosis. […] In advanced cases, esthesioneuroblastoma can lead to more severe manifestations, including intracranial extension, which may result in neurological deficits or altered mental status.
#1 Esthesioneuroblastoma Symptoms | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD
https://www.aaroncohen-gadol.com/en/patients/esthesioneuroblastoma/symptoms
In rare cases where the tumor invades the brain, symptoms such as confusion, memory loss, or changes in personality can occur. […] Esthesioneuroblastoma can spread to neck lymph nodes, causing noticeable swelling. […] Though less common, esthesioneuroblastoma can sometimes affect the teeth and gums, leading to dental symptoms. […] Early detection of esthesioneuroblastoma greatly improves the chances of successful treatment. […] If symptoms persist or seem unusual, especially when they dont respond to typical treatments, should prompt further medical evaluation. […] Addressing symptoms early not only helps with early diagnosis but also can provide better outcomes and potentially reduce the risk of recurrence after treatment. […] Nasal symptoms such as persistent congestion, nosebleeds, and loss of smell are common early signs of esthesioneuroblastoma.
#1 New Onset SIADH Masquerading Disease Progression during Treatment of Esthesioneuroblastoma
https://www.cpho.or.kr/journal/view.html?uid=885&pn=lastest
Esthesioneuroblastoma (ENB) behaves as a great imposter due to variable clinical presentation which have insidious onset. Diagnosis is often made in late stage as a result of non-specific symptoms including nasal obstruction, epistaxis, sinusitis, anosmia and headache. […] Rarely, ENB may be associated with paraneoplastic syndromes (PNS) including endocrinological syndrome of inappropriate ADH secretion (SIADH), ectopic ACTH syndrome, humoral hypercalcemia of malignancy, hypertension due to catecholamine secretion by tumour and neurological (opsoclonus-myoclonus-ataxia; cerebellar degeneration). […] Here, we describe a pediatric case of ENB in whom SIADH detected during ongoing primary treatment reflected disease progression that was confirmed radiologically. […] It has been observed that treatment of primary tumour usually resolves the associated PNS as well. Hence, patient in whom SIADH does not resolve after completion of adjuvant treatment should be evaluated for residual or recurrent disease.
#1 Esthesioneuroblastoma: Multimodal management and review of literature
https://www.wjgnet.com/2307-8960/full/v3/i9/774.htm
Esthesioneuroblastoma (ENB) is a rare malignant neoplasm arising from the olfactory neuroepithelium. ENB manifests across all ages, with peaks in the second and sixth decades of life. The most common presentation is unilateral nasal obstruction followed by epistaxis. Other clinical features develops with local spread of tumor like proptosis (infiltration into orbital canal), cranial nerve palsies (infiltration into cranial foramens and brain), swelling in neck (neck nodes) and metastatic symptoms. ENB have varying biological behaviour, ranging from slow indolent growth to a highly aggressive neoplasm with rapid widespread metastasis resulting in poor survival. […] Most of the patients of ENB present in locally advanced stage and the optimal management depends on the cooperation between clinicians, surgeons, radiologists and pathologists from establishing diagnosis to organizing the therapeutic strategy.
#1 Olfactory neuroblastoma | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/olfactory-neuroblastoma?lang=us
Clinical presentation is usually secondary to nasal stuffiness and rhinorrhea or epistaxis. Presentation is often delayed, and symptoms may have been present for many months. Patients often present late with larger tumors, which can extend into the intracranial compartment (25-30% at diagnosis) and usually result in anosmia. […] The tumors are slow-growing and the choice of imaging will depend on the tumor’s size. They begin as masses in the superior olfactory recess and initially involve the anterior and middle ethmoid air-cells unilaterally. As they grow, they tend to destroy surrounding bone and can extend in any direction. Often they are said to form a „dumbbell-shaped” mass with a „waist” as it passes through the cribriform plate. […] Particular attention should be paid to the presence of cervical and retropharyngeal nodal metastases, which are present in 10-44% of cases at diagnosis.
#1 Patterns of Regional Spread for Esthesioneuroblastoma | American Journal of Neuroradiology
http://www.ajnr.org/content/32/5/929
ENB presents clinically with unilateral nasal obstruction or epistaxis. […] While often indolent, it is nonetheless aggressive, characterized by local invasion as well as regional and distant metastases. Local invasion occurs most frequently into the paranasal sinuses, orbits, and anterior cranial fossa. Metastases most frequently involve the cervical lymph nodes, with distant metastasis reported to the lungs, liver, and bone. […] Disease stage on diagnosis, in particular the presence or absence of lymphatic spread, has been shown to be a significant predictor of survival. […] Spread of disease to the cervical lymph nodes was discovered in 14/48 patients (29%). Of the 14 patients, 5 (36%) were discovered to have lymph node involvement at initial staging, while 9 (64%) developed progressive disease in the cervical nodes on radiologic follow-up.
#1 Esthesioneuroblastoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK539694/
Esthesioneuroblastoma, or olfactory neuroblastoma, is a rare malignant neoplasm of the sinonasal tract originating from the olfactory neuroepithelium with neuroblastic differentiation. It most often presents in the superior nasal cavity. Esthesioneuroblastoma is a locally aggressive neoplasm and metastasizes by both hematogenous and lymphatic routes. […] Clinically, esthesioneuroblastoma often has a subtle presentation mimicking benign inflammatory or infectious disease, and delay in diagnosis is not uncommon. Nasal obstruction and epistaxis are typical early manifestations; however, other more specific symptoms can occur depending on the location and extent of the tumor. Anosmia can precede the diagnosis of esthesioneuroblastoma by several years. Other symptoms are related to extension into paranasal sinuses (facial swelling, medial canthus mass), the orbit (orbital pain, proptosis, epiphora, visual field defects), or anterior cranial fossa (headache), and manifestations of inappropriate antidiuretic hormone secretion.
#1 Esthesioneuroblastoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK539694/
The prognosis of esthesioneuroblastoma is both stage- and grade-dependent. The 5-year survival of stage A tumors is 75% to 90% whereas stage C has a 45% survival. Likewise, high-grade tumors have a 25% 5-year survival whereas low-grade tumors have an 80% survival. Poor prognostic indicators of esthesioneuroblastoma include metastases, high stage, high-grade tumor, above 50 or below 20 years of age at presentation, intracranial spread, high proliferation, females, and polyploidy/aneuploidy. […] Recurrence of esthesioneuroblastoma is common, usually occurs within 2 years of the initial presentation (about 30%). Metastasis develops in 35% of cases. The cervical lymph nodes are the most common site of spread. Distant metastasis occurs in approximately 10% of patients (lung, bone, liver, skin).
#1 Childhood Esthesioneuroblastoma – NCI
https://www.cancer.gov/types/head-and-neck/patient/child/esthesioneuroblastoma-treatment-pdq
Children may not have symptoms of esthesioneuroblastoma until the tumor has grown bigger. It’s important to check with your child’s doctor if your child has: […] a mass in the nose […] trouble breathing through the nose […] a change in or loss of the sense of smell […] nosebleeds […] a headache […] bulging of the eye […] trouble seeing […] eye pain. […] Most children with esthesioneuroblastoma are at an advanced stage at the time of diagnosis. Esthesioneuroblastoma spreads most often to nearby tissue, including the olfactory bulb, nasal sinuses, or brain. It can also spread to the lymph nodes, lungs, liver, bone or bone marrow.
#1 Recurrent Esthesioneuroblastoma: Long-Term Outcomes of Salvage Therapy
https://www.mdpi.com/2072-6694/15/5/1506
Esthesioneuroblastoma (ENB) recurrence is common; however, data regarding patient outcomes after recurrence is still lacking. This study examines patient outcomes after ENB recurrence using 143 patients with ENB and describes the patterns of salvage therapeutic interventions. Our study found the 5-year overall survival after ENB recurrence to be 63% and the 5-year progression-free survival to be 56%. The mean time to develop a secondary recurrence was significantly shorter than the mean time to develop the first recurrence. Additionally, patients who developed secondary recurrences were, on average, significantly older at the time of their primary tumor diagnosis. […] Esthesioneuroblastoma (ENB) recurrence has been estimated to be around 30 to 60% after successful treatment of the primary tumor. Recurrent disease is usually locoregional and tends to have a long interval to relapse with a mean of 6 years.
#1 Esthesioneuroblastoma | Neupsy Key
https://neupsykey.com/esthesioneuroblastoma-3/
The most common presenting symptoms are unilateral nasal obstruction and epistaxis; however, less common presenting symptoms include headache, facial pain, rhinorrhea, anosmia, diplopia, decreased visual acuity, proptosis, epiphora, and the syndrome of inappropriate antidiuretic hormone secretion. […] Esthesioneuroblastoma arises from the olfactory epithelium of the superior nasal cavity and is most commonly a rapidly growing, aggressive neoplasm. […] After treatment, recurrence is most often local. However, the tumor also exhibits a propensity for nodal and distant recurrence irrespective of the initial tumors grade, with distant recurrence most commonly occurring in the lungs and bone. […] Local recurrence has been reported in up to 60% of patients treated with surgery and radiation, with the majority of recurrences being seen within the first 12 to 24 months. […] Cervical nodal metastases are present in 5% to 10% of patients at presentation and confer a significantly worse prognosis. […] Distant metastases have been reported in up to 7% of patients at the time of initial presentation and occur in up to 25% of patients over the course of the disease.
#1 Recurrent Esthesioneuroblastoma: Long-Term Outcomes of Salvage Therapy
https://www.mdpi.com/2072-6694/15/5/1506
Although the overall survival (OS) of ENB patients is generally favorable compared to other sinonasal malignancies, recurrence is quite common and tends to occur in a delayed fashion, often 5 years or more after initial treatment. When recurrence occurs, the treatment guidelines are not well established but mainly consist of salvage surgery, targeted radiotherapy, and/or chemotherapy. […] Following a recurrence, the subsequent 5-year OS was 63%, and the PFS was 56%. Recurrent ENB shares a similar 5-year OS and PFS to that of primary ENB reported by prior studies. […] Secondary recurrence has a shorter time to recurrence compared to primary recurrence in our cohort. The mean time to secondary recurrence after treatment of the primary recurrence was 32 months, which is significantly shorter than the time to primary recurrence (32 months vs. 57 months, p = 0.048). […] Following ENB recurrence, salvage therapy appears to be an effective therapeutic option with a subsequent 5-year OS of 63%. However, subsequent recurrences are not infrequent and may require additional therapy.
#1 Patterns of recurrence and disease progression in patients with positive-margin olfactory neuroblastoma following primary resection in: Journal of Neurosurgery Volume 141 Issue 3 (2024) Journals
https://thejns.org/view/journals/j-neurosurg/141/3/article-p711.xml
The majority of patients with local recurrence had intracranial tumor extension (n = 9; 90%). […] Disease recurrence may develop in up to 46% of patients, with a median time to recurrence of approximately 7 years. […] In our margin-positive ONB cohort, we found patients to have a disease recurrence/progression rate of 56%. […] Therefore, margin-positive ONBs may have slightly higher recurrence rates and shorter times to recurrence compared to margin-negative ONBs.
#1 Olfactory Neuroblastoma – NCI
https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/other-rare-tumors/olfactory-neuroblastoma
Olfactory neuroblastoma can cause several symptoms as the tumor grows, including: […] Nose bleeds […] Loss of the sense of smell […] Difficulty breathing through the nose […] Pain. […] Doctors estimate olfactory neuroblastoma survival rates by how groups of people with olfactory neuroblastoma have done in the past. The survival rate varies depending on the stage of disease but is thought to range from 50-90%.
#1 Olfactory neuroblastoma | Radiology Reference Article | Radiopaedia.org
https://radiopaedia.org/articles/olfactory-neuroblastoma?lang=us
Treatment usually involves combined chemotherapy and/or radiotherapy with surgical excision. Prognosis is significantly affected by the presence of distant metastases: no distant metastases: 60% 5-year survival; distant metastases: 0% 5-year survival. Small localized tumors have a high cure rate, up to 85-90%.
#1 New Onset SIADH Masquerading Disease Progression during Treatment of Esthesioneuroblastoma
https://www.cpho.or.kr/journal/view.html?uid=885&pn=lastest
Furthermore, SIADH in follow up period can indicate late recurrences hence, any incidence of hyponatremia should be followed up with workup for recurrence. […] This case is an unusual case of ENB in a 16 year old where SIADH onset occurred during treatment and it reflected disease progression that was confirmed by radiological imaging. […] ENB is a unique malignancy with reports of long standing SIADH prior to its diagnosis. SIADH can occur months to years before the diagnosis of ENB, can infrequently also be at the time of presentation or years later prior to recurrence. However, any new onset SIADH during treatment can represent disease progression as seen in this case has not been reported before.
#1 Childhood Esthesioneuroblastoma (PDQÂ®): Treatment – Patient Information [NCI] – Sol Wellness and Psychiatry
https://solwellnessandpsychiatry.com/patient-education/healthwise?DOCHWID=ncicdr0000789047
Children may not have symptoms of esthesioneuroblastoma until the tumor has grown bigger. It’s important to check with your child’s doctor if your child has: […] a mass in the nose […] trouble breathing through the nose […] a change in or loss of the sense of smell […] nosebleeds […] a headache […] bulging of the eye […] trouble seeing […] eye pain. These symptoms may be caused by problems other than esthesioneuroblastoma. The only way to know is to see your child’s doctor. […] Most children with esthesioneuroblastoma are at an advanced stage at the time of diagnosis. Esthesioneuroblastoma spreads most often to nearby tissue, including the olfactory bulb, nasal sinuses, or brain. It can also spread to the lymph nodes, lungs, liver, bone or bone marrow.
#1 Exploration of the optimal treatment regimes for Esthesioneuroblastoma: a single center experience in China
https://www.jcancer.org/v09p0174.htm
Esthesioneuroblastoma (ENB) is an uncommon neoplasm arising from the olfactory mucosa. […] The age of onset of ENB showed a bimodal distribution, with peaks at 10~20 and 50~60 years. […] The 5-year overall and progression free survival rates were 42.7% and 39.1%, respectively, with 10-year rates of 28.9% and 21.7% respectively. Overall, 19 patients developed recurrent disease. […] No-Skin-involved ENB was associated with markedly better 5-year overall survival (45.5% vs. 0 %, P=0.038) and progress-free survival (31.3% vs. 0 %, P=0.001) compared with skin-involved tumor. […] ENB is a rarely malignant tumor with high probability of locoregional recurrence and poor survival. […] Patients with skin-involved ENB demonstrated significantly poorer 5-year overall survival (0% vs. 45.5%, P=0.038) and 5-year progress-free survival (0% vs. 31.3%, P=0.001) than those without skin involvement.
#2 Esthesioneuroblastoma – Prognosis and Treatmentphone iconphone iconGroup 49
https://www.barrowneuro.org/condition/esthesioneuroblastoma/
Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare type of cancer that starts in the upper part of the nasal cavity, specifically in the olfactory epithelium, which is responsible for your sense of smell. Because of its location and rarity, the symptoms can be mistaken for other, more common nasal conditions, making it somewhat challenging to diagnose early on. Hereâs a straightforward explanation of the symptoms you might experience if you have esthesioneuroblastoma: […] One of the most common symptoms is difficulty breathing through one side of the nose. This happens because the tumor can block one of the nasal passages. […] Since the tumor originates in the olfactory region, which controls your sense of smell, you might notice a decreased or complete loss of smell.
#2 Esthesioneuroblastoma Clinical Presentation: History, Physical Examination
https://emedicine.medscape.com/article/278047-clinical
The signs and symptoms of esthesioneuroblastoma (ENB) can be divided into nasal, neurologic, oral, facial, cervical, and ophthalmologic and are as follows: […] The average delay between the appearance of the initial symptom and the diagnosis is 6 months, but diagnosis is delayed for years in some cases. Delay is understandable because initial symptoms tend to be subtle and are frequently banal, occurring also in common nasal diseases, including long-term rhinosinusitis or allergic polypoid sinus disease. […] For the most part, malignancy is not considered until secondary symptoms such as facial pain and deformity or cranial nerve impairment are observed. However, early referral for an intranasal biopsy is essential to early diagnosis. […] Nasal examination, particularly if aided by endoscopy, reveals a reddish-gray tumor arising in the upper nasal fossa, which bleeds easily with instrumentation. Although this aspect is strictly different from the white, glistening appearance of benign nasal polyps, little differentiates esthesioneuroblastoma (ENB) from other nasal malignancies. Late findings may include signs related to extensive disease such as orbital, cranial, and cervical involvement.
#2 Esthesioneuroblastoma Symptoms | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD
https://www.aaroncohen-gadol.com/en/patients/esthesioneuroblastoma/symptoms
Esthesioneuroblastoma, also called olfactory neuroblastoma, is a rare cancer that begins in the upper part of the nasal cavity, near the nerves responsible for our sense of smell. This tumor can be difficult to detect early because its symptoms often resemble those of more common conditions like sinus infections or allergies. […] However, recognizing the signs and symptoms of esthesioneuroblastoma is crucial for early diagnosis and effective treatment. […] One of the common early signs of esthesioneuroblastoma starts in the nasal passages. Since the tumor grows in the upper part of the nasal cavity, it often causes symptoms like those seen in sinusitis or other nasal conditions. […] Persistent congestion, especially if it affects only one side of the nose, can be an early sign. […] Recurrent nosebleeds can occur as the tumor irritates the delicate tissues inside the nasal cavity.
#2 Esthesioneuroblastoma Symptoms | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD
https://www.aaroncohen-gadol.com/en/patients/esthesioneuroblastoma/symptoms
A gradual or sudden loss of smell is often one of the first symptoms. […] As the tumor grows, it can put pressure on surrounding areas in the head and face, leading to pain or discomfort. […] Ongoing headaches, particularly those around the forehead, eyes, or upper nose, can be a sign of esthesioneuroblastoma. […] Esthesioneuroblastoma can grow in a way that affects nearby structures, including the eyes, resulting in vision-related problems. […] The tumor may press on nerves controlling eye movement, leading to double vision. […] In some cases, the tumor can push the eye outward, a condition known as proptosis. […] As esthesioneuroblastoma progresses, it may extend into the brain. This can lead to more severe symptoms that affect neurological function. […] A large tumor can obstruct the nasal passages, making it difficult for patients to breathe through their nose.
#2 Esthesioneuroblastoma – Prognosis and Treatmentphone iconphone iconGroup 49
https://www.barrowneuro.org/condition/esthesioneuroblastoma/
Frequent and unexplained nosebleeds can occur as the tumor grows and affects the nasal tissues. […] You might experience a discharge from one side of your nose, which can be clear, bloody, or resemble pus. […] As the tumor grows, it can press on surrounding structures and cause headaches. […] In advanced cases, if the tumor grows upwards into the brain or towards the eyes, it may cause changes in vision, such as double vision or a decrease in vision. […] The tumorâs growth can also lead to swelling or pain in the face, especially around the eyes or in the forehead. […] If the tumor affects the nerves or muscles around the eyes, it may cause difficulty moving the eyes, leading to double vision or a noticeable difference in eye positioning. […] Other, more benign conditions can also cause these symptoms. However, if you notice these symptoms persisting, especially if theyâre only on one side or if they donât improve with standard treatments, itâs crucial to see a healthcare provider for further evaluation. Early detection and treatment of esthesioneuroblastoma can significantly affect the outcome.
#2 Esthesioneuroblastoma Symptoms | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD
https://www.aaroncohen-gadol.com/en/patients/esthesioneuroblastoma/symptoms
In rare cases where the tumor invades the brain, symptoms such as confusion, memory loss, or changes in personality can occur. […] Esthesioneuroblastoma can spread to neck lymph nodes, causing noticeable swelling. […] Though less common, esthesioneuroblastoma can sometimes affect the teeth and gums, leading to dental symptoms. […] Early detection of esthesioneuroblastoma greatly improves the chances of successful treatment. […] If symptoms persist or seem unusual, especially when they dont respond to typical treatments, should prompt further medical evaluation. […] Addressing symptoms early not only helps with early diagnosis but also can provide better outcomes and potentially reduce the risk of recurrence after treatment. […] Nasal symptoms such as persistent congestion, nosebleeds, and loss of smell are common early signs of esthesioneuroblastoma.
#2 Esthesioneuroblastoma history and symptoms – wikidoc
https://www.wikidoc.org/index.php/Esthesioneuroblastoma_history_and_symptoms
Symptoms of esthesioneuroblastoma include nasal obstruction, epistaxis, ear pain, otitis media, hyposmia, nasal discharge, facial pain, changes in vision, lacrimation, facial numbness, and anosmia. […] The presenting symptoms of esthesioneuroblastoma depend on the location and extent of the tumor. […] The most common symptoms are nasal bleeding, unilateral nasal obstruction, headaches, decreased sense of smell, and facial pain. […] Location Features Nasal: Epistaxis, Unilateral nasal obstruction, Headaches, Hyposmia, Facial pain. […] Orbital extension: Proptosis, Diplopia, Decreased visual acuity. […] Intracranial extension: Altered mental status, Nausea, Vomiting, Headaches, Seizures, Coma and death. […] Obstruction of the eustachian tube: Ear pain, Serous otitis media. […] Oral: Mobile tooth, Non-healing tooth extraction site, Ill-fitting dental prosthesis, Ulceration.
#2 Esthesioneuroblastoma | Oncohema Key
https://oncohemakey.com/esthesioneuroblastoma/
Up to 20% of patients will present with orbital symptoms, including visual loss, diplopia, epiphora with nasolacrimal obstruction, and proptosis. Headaches, nausea, and vomiting can be indicative of dural or intracranial involvement. Rarely, patients will present with frontal lobe symptoms, seizures, or symptoms of syndrome of inappropriate antidiuretic hormone secretion.
#2 Esthesioneuroblastoma overview – wikidoc
https://www.wikidoc.org/index.php/Esthesioneuroblastoma_overview
Esthesioneuroblastoma can cause loss of vision, and taste. […] Symptoms of esthesioneuroblastoma include nasal obstruction, epistaxis, ear pain, otitis media, hyposmia, nasal discharge, facial pain, changes in vision, lacrimation, facial numbness, and anosmia. […] If left untreated, 10%-62% of patients with esthesioneuroblastoma may progress to develop metastasis. Common complications of esthesioneuroblastoma include tumor recurrence and metastasis. These tumors often display varying biologic activity ranging from indolent growth, with patient survival exceeding 20 years, to a highly aggressive neoplasm capable of rapid widespread metastasis, with survival limited to a few months. The five-year mortality rate of patients with esthesioneuroblastoma is approximately 56% and 20% for grade I and II and grade III and IV tumors, respectively. The ten-year mortality rate of patients with esthesioneuroblastoma is approximately 67% and 34% for grade I and II and grade III and IV tumors, respectively.
#2 Childhood Esthesioneuroblastoma – NCI
https://www.cancer.gov/types/head-and-neck/patient/child/esthesioneuroblastoma-treatment-pdq
Children may not have symptoms of esthesioneuroblastoma until the tumor has grown bigger. It’s important to check with your child’s doctor if your child has: […] a mass in the nose […] trouble breathing through the nose […] a change in or loss of the sense of smell […] nosebleeds […] a headache […] bulging of the eye […] trouble seeing […] eye pain. […] Most children with esthesioneuroblastoma are at an advanced stage at the time of diagnosis. Esthesioneuroblastoma spreads most often to nearby tissue, including the olfactory bulb, nasal sinuses, or brain. It can also spread to the lymph nodes, lungs, liver, bone or bone marrow.
#2 Esthesioneuroblastoma (Olfactory Neuroblastoma) – MD Searchlight
https://mdsearchlight.com/cancer/esthesioneuroblastoma-olfactory-neuroblastoma/
Esthesioneuroblastoma commonly returns within two years of first appearing, affecting about 30% of patients. Additionally, the disease spreads in 35% of cases, frequently targeting the cervical lymph nodes. About 10% of patients experience distant metastasis, in which the cancer travels to distant parts of the body such as the lungs, bone, liver, or skin.
#2 Patterns of Regional Spread for Esthesioneuroblastoma | American Journal of Neuroradiology
http://www.ajnr.org/content/32/5/929
ENB presents clinically with unilateral nasal obstruction or epistaxis. […] While often indolent, it is nonetheless aggressive, characterized by local invasion as well as regional and distant metastases. Local invasion occurs most frequently into the paranasal sinuses, orbits, and anterior cranial fossa. Metastases most frequently involve the cervical lymph nodes, with distant metastasis reported to the lungs, liver, and bone. […] Disease stage on diagnosis, in particular the presence or absence of lymphatic spread, has been shown to be a significant predictor of survival. […] Spread of disease to the cervical lymph nodes was discovered in 14/48 patients (29%). Of the 14 patients, 5 (36%) were discovered to have lymph node involvement at initial staging, while 9 (64%) developed progressive disease in the cervical nodes on radiologic follow-up.
#2 Esthesioneuroblastoma – StatPearls – NCBI Bookshelf
https://www.ncbi.nlm.nih.gov/books/NBK539694/
The prognosis of esthesioneuroblastoma is both stage- and grade-dependent. The 5-year survival of stage A tumors is 75% to 90% whereas stage C has a 45% survival. Likewise, high-grade tumors have a 25% 5-year survival whereas low-grade tumors have an 80% survival. Poor prognostic indicators of esthesioneuroblastoma include metastases, high stage, high-grade tumor, above 50 or below 20 years of age at presentation, intracranial spread, high proliferation, females, and polyploidy/aneuploidy. […] Recurrence of esthesioneuroblastoma is common, usually occurs within 2 years of the initial presentation (about 30%). Metastasis develops in 35% of cases. The cervical lymph nodes are the most common site of spread. Distant metastasis occurs in approximately 10% of patients (lung, bone, liver, skin).
#2 Recurrent Esthesioneuroblastoma: Long-Term Outcomes of Salvage Therapy
https://www.mdpi.com/2072-6694/15/5/1506
Esthesioneuroblastoma (ENB) recurrence is common; however, data regarding patient outcomes after recurrence is still lacking. This study examines patient outcomes after ENB recurrence using 143 patients with ENB and describes the patterns of salvage therapeutic interventions. Our study found the 5-year overall survival after ENB recurrence to be 63% and the 5-year progression-free survival to be 56%. The mean time to develop a secondary recurrence was significantly shorter than the mean time to develop the first recurrence. Additionally, patients who developed secondary recurrences were, on average, significantly older at the time of their primary tumor diagnosis. […] Esthesioneuroblastoma (ENB) recurrence has been estimated to be around 30 to 60% after successful treatment of the primary tumor. Recurrent disease is usually locoregional and tends to have a long interval to relapse with a mean of 6 years.
#2 Esthesioneuroblastoma – Wikipedia
https://en.wikipedia.org/wiki/Esthesioneuroblastoma
Esthesioneuroblastoma frequently first presents as a nasal mass. The most common signs and symptoms of esthesioneuroblastoma are nasal obstruction (70%) and epistaxis (50%). 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 status. 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 diplopia. As the tumor metastasizes to the oral cavity, there can be tooth pain and tooth mobility. […] Esthesioneuroblastoma is a slow developing but malignant tumor with high recurrence rates because of its anatomical position. 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 classification. 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 percent. 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 recurrence. […] 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%).
#2 Esthesioneuroblastoma – EyeWiki
https://eyewiki.org/Esthesioneuroblastoma
Esthesioneuroblastoma (ENB) is a rare malignant tumor of the upper nasal cavity and anterior skull base first described in 1924 by Berger et al. Local invasion into the orbit, sinuses and central nervous system is common. Signs and symptoms vary and depend on location and degree of invasion. […] Diagnosis begins with a thorough history as most patients will have chronic nasal obstruction and congestion refractory to standard medical treatment. Epistaxis is also common. The average delay between appearance of initial symptoms and diagnosis is 6 months as initial symptoms tend to be subtle and frequently found in common nasal diseases such as rhinosinusitis and allergic polypoid sinus disease. […] Clinically, symptoms depend on location and extent of the tumor. Initially, most symptoms tend to be those found in common nasal diseases such as congestion and obstruction. As the tumor grows, symptoms worsen depending on location and extent of disease. It is common to have local invasion into the orbit, sinuses and intracranial cavity through erosion of the cribriform plate. Worsening sinus invasion commonly causes facial pain and epistaxis, a symptom often indicating a malignant process. Extension into the orbit can cause injection, ptosis, diplopia, proptosis, decreased vision and epiphora. Intracranial extension can present with headaches, seizures and neurologic complications. […] Esthesioneuroblastoma requires lifelong surveillance for recurrence and metastasis. Most surgeons obtain regular imaging such as CT/MRI and PET scan to help monitor disease progression.
#2 Esthesioneuroblastoma | Texas Children’s
https://www.texaschildrens.org/content/conditions/esthesioneuroblastoma
Esthesioneuroblastoma is a rare cancerous tumor that arises in the roof of the nasal cavity. Common presenting symptoms include nasal obstruction, nose bleeds, disturbance in smell, visual symptoms and headache. This tumor has a tendency to spread to the brain cavity and to the lymph glands in the neck. […] Small tumors are treated with surgery and radiation therapy. Advanced tumors are treated with combination of chemotherapy, surgery and radiation. Children are at risk for developing long term side effects due to therapy.
#2 Esthesioneuroblastoma – Vejthani Hospital | JCI Accredited International Hospital in Bangkok, Thailand.
https://www.vejthani.com/diseases-conditions/esthesioneuroblastoma/
Esthesioneuroblastoma can severely affect ones sense of smell and breathing. […] People with esthesioneuroblastoma may lose their sense of smell, have frequent nosebleeds, and have trouble breathing through their nostrils. […] The symptoms of esthesioneuroblastoma may change as the condition progresses. Common symptoms include: Decreased or loss of sense of smell, Recurrent nosebleeds, Trouble breathing via the nose, Pain in the area surrounding the eyes, Vision loss or alteration, Pain or pressure in the ear, Frequent headaches, Nasal obstruction, Congestion or stuffiness that worsens or does not improve, Pus in the nose, Neck lymph nodes enlargement, Having difficulty opening the mouth. […] If any of the signs and symptoms of esthesioneuroblastoma persists, consult a doctor right away for proper diagnosis and treatment.