Disclosures The Thin Red Line Between I have nothing to disclose - PowerPoint PPT Presentation

� Current Issues 2015 - Tihan � 5/21/2015 Disclosures The Thin Red Line Between I have nothing to disclose Neuropathology and Head & Neck Pathology Tarik Tihan, MD, PhD UCSF, Department of Pathology Neuropathology Division Introduction CASE 1 Dear Doctor I had the pleasure of evaluating this patient, a very pleasant � Three cases that straddle the boundary 73-year-old male who has a history of nasal congestion for between Neuropathology and Head & Neck years. In November 2007, he developed some epistaxis for Pathology which he went to the emergency room and a workup � Importance of recognizing different revealed a suggestion of sinusitis on CT scan. He was perspectives that are often complementary in referred to Dr. from reaching the correct diagnosis Otolaryngology who found an intranasal mass and performed a biopsy on February 2008. The biopsy was � The importance of thinking out of the “box” of consistent with esthesioneuroblastoma. He was referred to a specific subspecialty UCSF for surgical resection with a plan for postoperative � Recognition of the differences in the literature radiation therapy. Past medical history includes diabetes and abnormal electrocardiogram. from different subspecialties, and the need to reconcile these differences in real life � 1

� Current Issues 2015 - Tihan � 5/21/2015 AXIAL T1-gad AXIAL T1-gad Smear Frozen � 2

� Current Issues 2015 - Tihan � 5/21/2015 Frozen � 3

� Current Issues 2015 - Tihan � 5/21/2015 Synaptophysin Chromogranin MIB-1 BUT WAIT!!! ISN’T THERE ANYTHING UNUSUAL HERE? � 4

� Current Issues 2015 - Tihan � 5/21/2015 Cytokeratin ACTH Answer Case 1= Pituitary Adenoma Olfactory Olfactory Sinonasal Pituitary FEATURE Neuroblastoma Neuroblastoma Undifferentiated Adenoma Clinical: Typical visual field defect and � Low Grade High Grade Carcinoma endocrinological symptoms are helpful if present. Often a long-standing clinical history Lobular Common Common Focal or Rare Rare pattern Radiological: Involvement of the sella turcica and � Uniform sphenoid prior to nasal or ethmoid involvement Typical Typical Focal or Absent Absent nuclei Histological: Ample, sometimes clear cytoplasm, � Mitotic Rare Rare Frequent Frequent Figures rare mitoses. Otherwise similar to carcinoid tumors Necrosis Absent Absent Rare Frequent Immunohistochemistry: CHR, SYN, Pituitary � Rosettes Absent Present Rare/Absent Absent Transcription Factors or Hormones � 5

� Current Issues 2015 - Tihan � 5/21/2015 Follow-up 7 years later Olfactory Olfactory Sinonasal Pituitary FEATURE Neuroblastoma Neuroblastoma Undifferentiated Adenoma Low Grade High Grade Carcinoma Dear Doctor I am delighted to report that the MRI showed no evidence Mostly Cytokeratins Negative Negative Positive whatsoever of a recurrent pituitary tumor. This is excellent Positive news! I would recommend that you repeat the MRI again in S100 protein Negative Positive Positive/focal Negative/Rare two years. You could work with at NSE Positive Positive Positive Positive (50%) to make the arrangements for the follow-up MRI and the PIT1/SF- appointment. 1/TPIT Positive Negative Negative Negative Or Pit Hormones Synaptophysin Positive Positive Positive/Focal Negative Often Occasionally Chromogranin Often positive Rare positive cells positive positive 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. � 6

� Current Issues 2015 - Tihan � 5/21/2015 SAGITTAL T2 AXIAL T1-gad � 7

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� Current Issues 2015 - Tihan � 5/21/2015 AE1-AE3 CAM5.2 Brachyury EMA � 9

� Current Issues 2015 - Tihan � 5/21/2015 Brachyury Answer 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 CHONDROSARCOMA Localization Midline Clivus Lateralized, Temporal bone Physalliphorous YES NO cells Cytokeratin Positive Negative S100 protein Positive Positive EMA Positive Negative Brachyury Positive Negative IDH1 or IDH2 Absent Present mutations � 10

� Current Issues 2015 - Tihan � 5/21/2015 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. � 11

� Current Issues 2015 - Tihan � 5/21/2015 Trichrome Type IV Collagen EMA � 12

� Current Issues 2015 - Tihan � 5/21/2015 CD34 BCL-2 STAT6 BUT WAIT!!! ISN’T THERE ANYTHING UNUSUAL HERE? � 13

� Current Issues 2015 - Tihan � 5/21/2015 Unification Answer Case 2 = Solitary Fibrous Tumor FEATURE Solitary Fibrous Tumor Hemangiopericytoma Collagen-rich YES NO HPC-like YES YES vasculature Focal positive & vascular Reticulin Stain Strongly positive pattern CD34 staining Diffuse Strong Focal or Negative BCL-2 staining Diffuse Strong Diffuse Strong Diffuse Strong STAT-6 staining Diffuse Strong (nuclear) (nuclear) Biphasic Common Uncommon architecture Local Recurrence Rare Common (~60%) Extracranial Exceptional Common (~30%) metastasis NAB2/STAT6 YES (ex4-ex6 fusion)* YES (ex6-ex16 fusion)* fusion � 14

� Current Issues 2015 - Tihan � 5/21/2015 THANK YOU � 15