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Kadish System Grading of Olfactory Neuroblastoma

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I'm really not one for memorizing

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very many grading systems of tumors.

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However, it is useful to look at the Kadish

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system grading of olfactory neuroblastoma in

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order to better inform us of what we should be

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looking for with regard to spread of olfactory

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neuroblastoma, i.e., esthesioneuroblastoma.

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So Group A are those confined to the nasal cavity.

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Group B include not only the nasal

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cavity, but also the paranasal sinuses.

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Group C grow into the skull base and the

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intracranial cavity, and Group D, distant metastases.

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So the important factors here is, is just in

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the nasal cavity, has it spread to the maxillary

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sinus, the ethmoid sinus, is it going into

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the skull base, perineural spread, intracranial

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compartment, and then this is a malignancy,

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do they have distant metastases?

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I mentioned that there was a recent

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upgrade in the World Health Organization

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with regard to sinonasal tumors.

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I've mentioned some of these in passing, but

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you should understand that there are certain

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tumors that are called sinonasal epithelial

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hamartomas, which are benign lesions that

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contain portions of ciliated respiratory

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epithelium, and I mentioned the REAH as a

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respiratory epithelial adenomatoid hamartoma

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as one of these that is the more common.

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These are usually along the olfactory

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cleft and associated, as I mentioned, with

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polyposis, and they may have a little bit of a

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crescent sign around them, reflecting their curved

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linear border.

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I mentioned also the NUT carcinoma.

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This is nuclear protein in testis.

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This is generally a midline tumor

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that can affect the nasal septum.

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They're locally aggressive.

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They may have some mineralization, but

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it's not the same mineralization as that

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chondroid matrix of a chondrosarcoma.

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Now there are other tumors including

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biphenotypic sinonasal sarcomas.

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These are tumors that are characterized

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by avid enhancement and hyperostosis.

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They can occur anywhere in the sinonasal cavity.

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I also mentioned HPV sinonasal carcinomas,

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and the HPV subtype is different than

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the 16, 32 that we typically see in tonsil

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carcinomas and base of the tongue carcinomas.

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These are usually subtype 34, but they may be unique

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tumors, and they by and large have a better prognosis.

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Additionally, I mentioned in talking about

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the sinonasal undifferentiated carcinomas,

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that some of these have been reclassified as

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the SWI/SNF, or SWItch/Sucrose Non-Fermentable related

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matrix-associated actin-dependent

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regulator of chromatin subfamily B tumors.

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So these are replacing some of the

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SMARCB1 tumors, which you see here.

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And these are aggressive tumors,

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may have a calcified matrix, and they may invade the dura.

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And then finally, there are those

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chondro-mesenchymal hamartomas.

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These are what we would've previously called enchondromas

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or chondromas but have been reclassified as well.

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So that about takes us through the

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primary tumors of the sinonasal cavity.

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You should understand that there are

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some tumors that have a predilection for

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metastasizing to the sinonasal cavity.

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The most common of this is kidney carcinoma.

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Kidney carcinoma is often hypervascular.

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It may be hemorrhagic, and that might be one of

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the indicators that this is a metastasis to the

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sinonasal cavity, as opposed to a primary tumor.

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So kidney primaries more common

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than lung and breast and prostate.

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Sometimes we'll have a melanoma

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in the sinonasal cavity, and the patient also has a skin

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cancer, and we question, is that metastatic melanoma

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disease, or is that primary sinonasal melanoma?

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Same thing true with lymphoma.

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You can have primary sinonasal lymphomas,

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or you can have systemic lymphoma with

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metastatic disease to the sinonasal cavity.

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In addition, the sinonasal cavity may be a site where

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myeloma with lytic bone lesions may occur as well.

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Here, for example, is a patient who has a mass

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in the sinonasal cavity associated with bright

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signal on T1-weighted scan that was hemorrhagic.

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This was indeed a renal cell

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carcinoma to the sinonasal cavity.

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This is a patient who has a tumor in

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the maxillary antrum, which is showing

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very avid FDG uptake on the PET scan.

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This was a lymphoma

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of the maxillary antrum, and

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was primary sinonasal lymphoma.

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From the sinonasal cavity, as I mentioned, it's

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relatively unusual to see lymph node spread.

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When we do see lymph node spread, you may see it in

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the level II high jugular chain above the hyoid bone.

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You may see it in the retropharyngeal

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lymphadenopathy, the so-called nodes of Rouvière, or you

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may see submental or submandibular spread of tumor,

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the so-called level I lymphadenopathy.

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With regard to sinonasal lymphatic cancer or

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lymphoma, this is usually diffuse large B-cell

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lymphoma, although you can have your NKT or T-cell

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lymphomas. These tend to occur more commonly

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in the Asian and South American population.

Report

Faculty

David M Yousem, MD, MBA

Professor of Radiology, Vice Chairman and Associate Dean

Johns Hopkins University

Mahla Radmard, MD

Postdoctoral Research Fellow

Johns Hopkins University School of Medicine

Tags

Sinus

Sinonasal Cavity

Oncologic Imaging

Neuroradiology

Neoplastic

MRI

CT

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