Pathology Notes

Practice question answers Upper respiratory tumors © Jun Wang, MD, PhD 1. A. The microscopic features of fibrotic stroma with irregular dilated vessels are suggestive of nasopharyngeal angiofibroma . Angiosarcoma is characterized by pack vascular proliferation with endothelial atypia. Inverted papilloma is characterized by inward growth of benign squamous epithelium. Nasopharyngeal carcinoma is more common in certain areas, including Middle East. However, it is a type of squamous cell carcinoma that is associated with EBV infection. Olfactory neuroblastoma is characterized by sheets or nests of tumor cells with round to oval nuclei, scant cytoplasm, and expression of keratin and neuroendocrine markers including chromogranin and synaptophysin. 2. A. Nasopharyngeal angiofibroma is positive for androgen receptor. Chromogranin is a neuroendocrine marker that is expressed in neuroendocrine tumors, including carcinoid , small cell carcinoma , and olfactory neuroblastoma .

Practice questions I Upper respiratory tumors © Jun Wang, MD, PhD 1. Use this case for next two questions . A 17-year-old boy from middle east presents with nasal congestion and epistaxis for 2 months. He denies history of fever, nasal discharge, or trauma. He has a history of infectious mononucleosis 2 years ago. His history is otherwise unremarkable. Physical examination identified a 1.2 cm pink mass at the upper lateral wall of his left nasal cavity. The mass has smooth surface. Biopsy of the mass reveal a lesion with benign squamous epithelial lining, and a fibrotic containing irregularly dilated vessels. There are scattered lymphocytic and plasmacytic infiltrate. No cytological atypia is noted in either the endothelium or stroma. What is most likely the diagnosis? A. Angiofibroma B. Angiosarcoma C. Inverted papilloma D. Nasopharyngeal carcinoma E. Olfactory neuroblastoma 2. A 17-year-old boy from middle east presents with nasal congestion and epistaxis for

Nasopharyngeal carcinoma   Updated: 10/13/2020 © Jun Wang, MD, PhD General features Arising from the epithelium of the nasopharynx Common in South Asia, North Africa, Middle East and the Arctic More common in men Rare in the states, but incident increasing in black teenagers Bimodal age distribution, a small peak in late childhood, and second peak around 50-59 Risk factors Associated with Epstein-Barr virus infection Other risk factors: Consumption of salt-preserved fish containing carcinogenic nitrosamines , family history, specific HLA class I genotypes, tobacco smoking , chronic respiratory tract conditions and low consumption of fresh fruits and vegetables Pathogenesis EBV genome products (LMP1, LMP2, etc) associated telomerase dysregulation, bcl2 overexpression, etc. Loss of function mutation of NF-kappa B inhibitors (CYLD, TRAF3, etc) Multiple chromosomal abnormalities p16 deletion LTBR (lymphotoxin-beta receptor) amplification Clinical presentations

Vocal cord polyp   Updated: 10/13/2020 © Jun Wang, MD, PhD General features AKA laryngeal nodule or singer’s nodule Noninflammatory response to chronic irritation More common in heavy smokers or singers Almost never transforms to malignancy Etiology Chronic irritation including phonotrauma, allergies, smoking, etc Clinical presentations Generalized and persistent hoarseness Key morphological features Round growth with smooth surface on true vocal cords Fibrotic edematous core with reactive squamous epithelium Treatment Surgery Vocal rest Back to respiratory tract neoplasms Back to contents

Sinonasal papilloma   Updated: 10/12/2020 © Jun Wang, MD, PhD General features AKA Schneiderian papilloma Benign Usually adults, 2/3 men Three subtypes Inverted (Most common): Risk for malignant transformation Fungiform (exophytic) : No known risk for malignant transformation Oncocytic : Risk for malignant transformation Clinical presentations Nasal obstruction, stuffiness or epistaxis Key pathogenesis Inverted: High and low risk HPV, EGFR mutation Fungiform : Low risk HPV Oncocytic : KRAS mutation HPV associated tumorigenesis Key morphological features Soft to moderately firm, with granular or finely clefted surface May have malignant foci Inverted papilloma : Endophytic growth , non keratinizing squamous, transitional or respiratory epithelium Fungiform papilloma : Exophytic growth , non keratinizing squamous, transitional or respiratory epithelium Oncocytic papilloma : Oncocytic epithelium, intraepithelial mucin-filled cysts and microabscesses Trea

Olfactory neuroblastoma   Updated: 10/12/2020 © Jun Wang, MD, PhD General features AKA esthesioneuroblastoma Rare, malignant neuroectodermal tumor Probably arise from olfactory membrane or olfactory placode NOT related to neuroblastomas elsewhere in body Mean age 53 years, range 3-79 years Slightly more common in male Late recurrence common Metastasis: Cervical lymph nodes, lungs Clinical presentations Usually upper nasal vault; rarely in nasopharynx, maxillary or ethmoid sinus Nasal obstruction, epistaxis Neurologic presentations: headache, nausea, diplopia, etc Rarely paraneoplastic syndromes : Hypercalcemia, ectopic adrenocorticotropic hormone syndrome, etc Presentations associated with local invasion Radiologic findings Homogeneous soft tissue mass with uniform and moderate contrast enhancement Key morphological features Nests or sheets of uniform small cells with round nuclei Markers Positive for cytokeratin, neuron-specific enolase, synap