the thin red line between neuropathology and head & neck

�Current Issues 2015 - Tihan �5/21/2015

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Disclosures

I have nothing to disclose The Thin Red Line Between Neuropathology and Head &

Neck Pathology

Tarik Tihan, MD, PhDUCSF, Department of Pathology

Neuropathology Division

Introduction

� Three cases that straddle the boundary between Neuropathology and Head & Neck Pathology

� Importance of recognizing different perspectives that are often complementary in reaching the correct diagnosis

� The importance of thinking out of the “box” of a specific subspecialty

� Recognition of the differences in the literature from different subspecialties, and the need to reconcile these differences in real life

CASE 1Dear Doctor

I had the pleasure of evaluating this patient, a very pleasant 73-year-old male who has a history of nasal congestion for years. In November 2007, he developed some epistaxis for which he went to the emergency room and a workup revealed a suggestion of sinusitis on CT scan. He was referred to Dr. from Otolaryngology who found an intranasal mass and performed a biopsy on February 2008. The biopsy was consistent with esthesioneuroblastoma. He was referred to UCSF for surgical resection with a plan for postoperative radiation therapy. Past medical history includes diabetes and abnormal electrocardiogram.


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AXIAL T1-gad AXIAL T1-gad

Smear Frozen


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Frozen


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Synaptophysin Chromogranin

BUT WAIT!!!

ISN’T THERE ANYTHING UNUSUAL HERE?

MIB-1


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Cytokeratin ACTH

Answer Case 1= Pituitary Adenoma

� Clinical: Typical visual field defect and endocrinological symptoms are helpful if present. Often a long-standing clinical history

� Radiological: Involvement of the sella turcica and sphenoid prior to nasal or ethmoid involvement

� Histological: Ample, sometimes clear cytoplasm, rare mitoses. Otherwise similar to carcinoid tumors

� Immunohistochemistry: CHR, SYN, Pituitary Transcription Factors or Hormones

FEATURE Pituitary Adenoma

OlfactoryNeuroblastomaLow Grade

Olfactory Neuroblastoma High Grade

Sinonasal Undifferentiated

Carcinoma

Lobular pattern Common Common Focal or Rare Rare

Uniform nuclei Typical Typical Focal or Absent Absent

Mitotic Figures Rare Rare Frequent Frequent

Necrosis Absent Absent Rare Frequent

Rosettes Absent Present Rare/Absent Absent


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FEATURE Pituitary Adenoma

OlfactoryNeuroblastomaLow Grade

Olfactory Neuroblastoma High Grade

Sinonasal Undifferentiated

Carcinoma

Cytokeratins Mostly Positive Negative Negative Positive

S100 protein Negative Positive Positive/focal Negative/RareNSE Positive Positive Positive Positive (50%)

PIT1/SF-1/TPITOr Pit Hormones

Positive Negative Negative Negative

Synaptophysin Positive Positive Positive/Focal Negative

Chromogranin Often positive Often positive Occasionally

positive Rare positive cells

Follow-up 7 years later

Dear Doctor

I am delighted to report that the MRI showed no evidence whatsoever of a recurrent pituitary tumor. This is excellent news! I would recommend that you repeat the MRI again in two years. You could work with at to make the arrangements for the follow-up MRI and the appointment.

CASE 2

� A 21 year old man presented with dysphagia and a change in his voice. He has also lost 15 lb over the last few months. An MRI revealed a cervical mass. He underwent a biopsy of the lesion, followed by a radical resection. The tumor appeared to have encased the vertebral artery and involved the neural foramen and partially compressed the cervical spinal cord.


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SAGITTAL T2 AXIAL T1-gad


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AE1-AE3 CAM5.2

EMA Brachyury


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BrachyuryAnswer Case 2 = Chordoma

� Most common location sacrum, followed by skull base/clivus

� Midline with contrast enhancement� Epithelial differentiation, typically EMA positive, and also cytokeratin positive

� S100 protein often strongly positive along with Vimentin

� Brachyury is the marker of choice for the diagnosis of Chordomas

FEATURES CHORDOMA CHONDROSARCOMALocalization Midline Clivus Lateralized, Temporal

bonePhysalliphorous cells

YES NO

Cytokeratin Positive NegativeS100 protein Positive PositiveEMA Positive NegativeBrachyury Positive NegativeIDH1 or IDH2 mutations

Absent Present


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CASE 3

� A 43-year-old man presented with significant weight loss, postural instability and difficulty in walking. He also suffered from occasional nausea and vomiting. A recent audiogram demonstrated left severe mixed hearing loss. An MRI revealed a mass that distorted the fourth ventricle with significant hydrocephalus.


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Trichrome

Type IV Collagen EMA


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CD34 BCL-2

BUT WAIT!!!

ISN’T THERE ANYTHING UNUSUAL HERE?

STAT6


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Answer Case 2 = Solitary Fibrous Tumor

Unification

FEATURE Solitary Fibrous Tumor HemangiopericytomaCollagen-rich YES NOHPC-like vasculature YES YES

Reticulin Stain Focal positive & vascular pattern Strongly positive

CD34 staining Diffuse Strong Focal or NegativeBCL-2 staining Diffuse Strong Diffuse StrongSTAT-6 staining Diffuse Strong (nuclear) Diffuse Strong

(nuclear)Biphasic architecture Common UncommonLocal Recurrence Rare Common (~60%)Extracranial metastasis Exceptional Common (~30%)

NAB2/STAT6 fusion YES (ex4-ex6 fusion)* YES (ex6-ex16 fusion)*


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THANK YOU

the thin red line between neuropathology and head & neck

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