Year : 2003 | Volume
: 13 | Issue : 2 | Page : 219--222
Esthesioneuroblastoma with hepatic and splenic metastases
S Shekhar, Rajesh, S Madhavan
Department of Radiodiagnosis, St. John's Medical College Hospital, Bangalore, 560034, Karnataka, India
Kanchanganga, Kadri Temple Road, Kadri, Mangalore 575 002, Karnataka
|How to cite this article:|
Shekhar S, Rajesh, Madhavan S. Esthesioneuroblastoma with hepatic and splenic metastases.Indian J Radiol Imaging 2003;13:219-222
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Shekhar S, Rajesh, Madhavan S. Esthesioneuroblastoma with hepatic and splenic metastases. Indian J Radiol Imaging [serial online] 2003 [cited 2018 Nov 18 ];13:219-222
Available from: http://www.ijri.org/text.asp?2003/13/2/219/28665
Olfactory neuroblastoma (ON) is an uncommon malignant tumor which arises from the olfactory epithelium found in the cribriform region, the upper third of the nasal septum, and the superior and supreme nasal turbinates. ON shows a wide range of age distribution (3 to 79 years), the median age about 50 years .The clinical course is characterized by local aggressive and less commonly distant metastases. 
We would like to report a patient diagnosed to have an olfactory neuroblastoma in the right maxillary sinus, presenting with extensive local spread and with distant metastases to the liver and spleen.
A 52 year old man with multiple symptoms of pain on the right side of the face, right nasal block, disturbed vision on the right side, drooping of the right eye lid, toothache, epistaxis, abdominal pain and distension for two months was admitted to the Oncology center. The ENT examination revealed a fleshy growth in the turbinate on the right side and bloody discharge. On clinical examination, the patient had pallor, icterus, abdominal distension, shifting dullness and hepatomegaly with a liver palpable for seven centimeters below the right costal margin. The patient was delirious with features of encephalopathy. The liver function tests were abnormal with elevated serum bilirubin level of 9.8 mg, elevated liver enzymes and decreased total protein levels of 5.2 mg. A diagnosis of hepatic encephalopathy was made based on these investigations.
The axial and coronal CT scan sections [Figure 1][Figure 2] of the paranasal sinuses, showed a heterogeneously enhancing soft tissue mass lesion with few specks of calcification and cystic areas in the right maxillary sinus with expansion of the sinus and osseous destruction of the medial and lateral walls, roof and floor. There was extra antral extension into the ipsilateral nasal cavity with involvement of all three turbinates and deviation of the nasal septum to the left side. Extension was also seen into the infratemporal fossa, pterygoid fossa , sphenoid and anterior and posterior ethmoid sinuses and medial portion of the orbit on the right side. There was associated destruction of the greater wing of the sphenoid and cribriform plate on the right side with intracranial extension into the right frontal lobe. The entire right nasal cavity was occupied by the mass. There was opacification with a fluid level in the left maxillary sinus and minimal opacity in the left posterior ethmoid sinus, possibly due to retained secretions. The biopsy was reported as that of a small round cell tumor -suggestive of olfactory neuroblastoma.
On USG of the abdomen [Figure 3][Figure 4], there was moderate ascites, mild splenomegaly measuring 12.7cm and hepatomegaly measuring 17cm. There were multiple hyperechoic lesions of varying sizes in the right and left lobes of the liver. In the lower pole of the spleen, there was a solitary isoechoic lesion measuring 1.7 x 1.6 cm with a peripheral hypoechoic halo. These were reported as olfactory neuroblastoma metastases to the liver and spleen.
ON is a tumor of neural crest origin arising from the olfactory epithelium. Synonyms include olfactory esthesioneuroma, neuroesthesioma, olfactory neurocytoma and olfactory esthesioneuroblastoma.
ON occurs with equal frequency in men and women. There is a bimodal age distribution with peaks at two age groups between, 11-20 years and second in 51-60 year olds. The most common presenting symptoms are epistaxis and nasal obstruction ,. The other symptoms can be headache, nasal pain , excessive lacrimation, anosmia and visual disturbances  .
The tumor can spread submucosally in all directions, thereby involving the nasal sinuses, nasal cavity and surrounding structures.
These lesions can cross the cribriform plate and invade the brain or seed the cerebrospinal fluid. This rare neoplasm is locally aggressive and can metastasize by lymphatic and hematogenous routes .
Local recurrence has been reported in upto 57% of patients. A metastatic rate of 20% to 62% is reported, with the most common site being the cervical lymph nodes. Other sites include the parotid glands, skin, lung, bone, liver, orbit, spinal cord and spinal canal ,.
There are isolated reports of metastasis to the scalp, aorta, spleen, adrenal gland and ovary .
CT and MR imaging are complementary examinations done preoperatively to define the tumor extent , staging and surgical approach. The advantage of CT is that it allows evaluation of osseous involvement around the nasal septum, orbit and anterior skull base.
The protocols for CT scanning include axial and coronal scans of 1 to 5mm thick slices with intravenous contrast. ONs are solid and enhancing nasal cavity masses that may manifest erosion into nearby osseous structures of the orbital plate, ethmoid bone ,cribriform plate, and fovea ethmoidalis. Intra lesional calcification and presence of cysts along the intracranial margins in case of intra cranial extension yield a definitive diagnosis , .
On TI- weighted MR images, ON present as homogenously enhancing tumors with intermediate signal intensity, whereas on T2 - weighted images, the original intensity is increased. MR imaging can delineate intraorbital and intracerebral extension. The tumor appears hypointense to gray matter on T1- weighted images and isointense or hyperintense to gray matter on T2 weighted images .
Gadolinium enhanced MR images help to differentiate tumors from obstructed secretions in paranasal sinuses, determining meningeal and extradural spread and to detect peri neural spread .
Metastatic neuroblastoma to the liver may be seen as discrete focal lesions, simulating a primary liver tumor of high or low reflectivity or as diffuse multifocal tumor infiltration. The liver is enlarged, heterogeneous in appearance with multifocal areas of low reflectivity with or without calcification, giving it an extremely coarse speckled appearance .
The Kadish esthesioneuroblastoma staging system .
The main shortcoming of the Kadish classification is that the language of defining stage C is too broad. In 1992, Dulguerov and Calcaterra proposed the modified TNM staging based on CT and / MR findings. Modified TNM staging system .
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