Esthesioneuroblastoma is an uncommon neoplasm
of the nasal cavity which is curable if diagnosed early but
fatal if neglected. We describe a case of Stage B esthesioneuroblastoma
excised by using the lateral radical rhinotomy surgical technique
followed by radiation therapy. The patient was free of the tumor
and asymptomatic 13 years after treatment. Our diagnosis and
treatment of this uncommon nasal tumor serves as an example
of how family physicians and nurse practitioners must be alert
to the possibility of such serious tumors and when to assertively
seek consultation for their management.
Esthesioneuroblastoma (EN) is an uncommon neoplasm
which arises from the olfactory epithelium in the cribriform
region of the nasal septum. Of neuroectodermal origin, EN accounts
for 3% of all nasal neoplasms1,2 and mainly affects
white persons. Mean age at diagnosis is 48 years (range, 9 to
83 years).3 EN occurs 1.6 times more often in women
than in men.4
Differentiation of EN from other tumors is difficult
because it resembles other neuroendocrine tumors of the head
and neck. The cause of EN is unknown. Smokers are at increased
risk for EN.4 EN also is not as aggressive as undifferentiated
carcinoma; however, if not treated early in its course, EN spreads
rapidly and is fatal.
We describe our treatment of a case of EN and
review its diagnosis and treatment.
Case Report
A 50-year-old white man was seen by an otolaryngologist for
nasal obstruction, chronic sinusitis, a feeling of fullness
in the head, and numbness of the left cheek and left maxillary
teeth. He was given antihistamines but returned the next month
with continued nasal obstruction as well as rhinorrhea and dizziness.
Because he was noncompliant, the patient was not seen until
three months later, when he was seen for epistaxis. Nasal biopsy
was done. Computed tomography (CT) scans and x-ray films of
the sinuses showed a soft-tissue mass in the nasopharynx which
crossed the midline, left maxillary sinus, ethmoid, inferior
orbit, and cribriform plate. The left paranasal sinus was eroded
up to the orbit. The patient was sent to an urban medical center
for surgical and adjunctive treatment. Initial biopsy results
suggested small cell neoplasm or a metastatic lesion from occult
lung cancer. Bronchoscopy, sputum cytology, and chest x-ray
results were negative. Chemistry panel and liver function test
results were normal. The patient had mild anemia. Expert review
of pathology slides showed EN.
The patient, raised on a farm, had smoked for
35 years but had no history of alcohol abuse. One of the patient's
two brothers had died of pancreatic cancer at age 65 years;
and the other, a heavy smoker, died of a nasopharyngeal neoplasm.
Because the tumor was located in the postnasal
cavity and extended superiorly and posteriorly to the ethmoid
labyrinth and into the maxillary sinus, the patient had subtotal
maxillectomy and complete ethmoid and sphenoid exenteration
through the lateral rhinotomy approach on the left side. The
sphenoid bone was partly eroded anteriorly. The left nasal bone
was removed with the maxillary mucosa. The orbital floor was
eroded. The tumor was peeled off the petriorbital fascia, which
was intact. The eroded sphenoid sinus was excised with the sphenoid
mucosa. Three days after having maxillectomy, radiation treatment
was begun.
The tentative diagnosis of undifferent, small
cell, undifferentiated cancer was changed to EN at further review.
The tumor weighed 25 g; consisted of soft, tan-to-yellow, friable
tissue; and measured 2 X 2 X 3.2 cm. As is consistent with EN,
no true rosette was seen.
Careful evaluation by his physician and oncologist
every six months showed that the patient remained disease-free
13years after surgery.
Discussion
Clinical Diagnosing EN
Knowing the clinical manifestations of EN enhances the role
of the primary care physician, the nurse practitioner, the ophthalmologist,
the surgeon, and the oncologist in the vital early detection
and prompt treatment of the disease. The most common initial
signs and symptoms of EN are nasal obstruction, epistaxis, exophthalmos
with visual field defects, blurred vision, eye pain, conjunctival
erythema, eyelid edema, headache, blindness, excessive tearing,
anosmia, diplopia, facial numbness and sweating, a polypoid
intranasal mass with a granular red appearance, shortness of
breath, fatigue, weight loss, and chest pain.
Our patient's smoking history and family history
of neoplasms made us suspect that the etiology of EN has a hereditary
component.
Treating EN
Timely surgery with radiation therapy yields a good prognosis
in Stage A disease, which is defined5 as a tumor
confined to the nasal cavity. Stage B disease is defined5
as a tumor confined to the nasal cavity and one or more paranasal
sinuses. A craniofacial approach followed by radiation therapy
is required. In Stage C disease (defined as a tumor extended
beyond the nasal cavity or paranasal sinuses into the orbit,
base of skull or intracranial cavity, cervical lymph nodes,
or distant sites5), chemotherapy with such agents
as cyclophosphamide is required in addition to surgery and radiation
therapy.
The importance of salvage therapy to prolonging
survival in patients with advanced EN has been recognized: a
retrospective review of EN treatment4 reported survival
rate of 88% in patients with Stage C disease after treatment
with surgery, radiation therapy, and chemotherapy, in contrast
to a survival rate of 50% after treatment with only combined
craniofacial resection and radiation.
Conclusion
EN is an uncommon, complex olfactory tumor which is curable
if diagnosed early but which may be fatal if misdiagnosed and
neglected. To identify possible EN, the treating family physician,
oncologist, surgeon, nurse practitioner, and pathologist should
be alert for any initial complaints of nasal obstruction lasting
longer than one month or of persistent rhinorrhea or epistaxis
with neurologic deficits (eg, anosmia, focal numbness of the
face or cheek, or visual defects) especially in patients who
have a history of smoking and a family history of neoplasm.
X-ray films of the sinuses and CT scans should immediately be
obtained when the family physician or nurse practitioner is
in doubt about the differential diagnosis, and the patient must
be promptly referred for head and neck or neurosurgery consultation
for possible tumor resection. As in other conditions, patients
should be instructed that noncompliance could affect prognosis.
Although esthesioneuroblastoma is still seen uncommonly,
it is being observed with increasing frequency. Therefore, as
for other such increasingly observed conditions, family practitioners
must increase their awareness of this tumor.
Acknowledgments: The Medical Editing
Department, Kaiser Foundation Research Institute, provided editorial
assistance.
References
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