Neuroendocrine Carcinoma. Lebanon Neuroendocrine Neoplasms of H&N Nov /7/2011. Broad Classification:

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1 H&N Neuroendocrine Neoplasms: Classification and Diagnostic Considerations Adel K. El-Naggar, M.D., Ph.D. The University of Texas MD Anderson Cancer Center, Houston, Texas Broad Classification: A. Epithelial: Neuroendocrine carcinoma (Typical and atypical carcinoid and PDC) Medullary thyroid carcinoma Parathyroid tumors B. Non-Epithelial: Neuroblastoma Paraganglioma Neuroendocrine Carcinoma 1

2 Incidence: <1% of H&N tumors Etiology: Unknown!! Radiation Smoking Histogenesis Neuro-endocrine cell Undifferentiated / basal / stem cell Locations: Larynx Salivary glands (major and minor) Sino-nasal region Middle ear and endolymphatic sac 2

3 Larynx 3

4 Histologic Classification: Well differentiated (typical carcinoid) Moderately differentiated (atypical carcinoid) Poorly differentiated (small, large cell) Typical Carcinoid 4

5 Atypical Carcinoid 5

6 6

7 CAM 5.2 CK5/6 CD56 7

8 Poorly Differentiated Keratin 8

9 Synaptophysin Immuno Markers: Epithelial: Differentiated Undifferentiated Pan cytokeratin (AE1/AE3) ++ + EMA + +/- CK 5/ Neuroendocrine: Chromogranin Synaptophysin Calcitonin ++ - CD Management of Laryngeal NET: Differentiated: surgery Poorly differentiated: chemo + XRT 9

10 Clinical Characteristics of MD-NEC at MD Anderson Differential Diagnosis: A. Differentiated: Medullary thyroid carcinoma Paraganglioma Basaloid salivary adenocarcinoma RCC B. Undifferentiated: Small round cell tumors Undifferentiated carcinoma Basaloid squamous carcinoma Merkel Cell Carcinoma Case Presentation: 42 year old female With left neck mass (4.0 cm) Serum calcitonin: high Excision of neck mass 10

11 11

12 Diagnosis: Neuroendocrine carcinoma favor metastatic MTC Follow-up: Thyroid examination normal Hx of polypoid laryngeal nodule 5 years 12

13 Chromogranin 13

14 Calcitonin Comparative IHC of Laryngeal Primary and Lymph Node Metastasis: Marker Primary L-N Somatostatin - - Serotonin ++ + ACTH - - NSE Chromogranin CEA ++ + Calcitonin Diagnosis: MTC-like carcinoid tumor of the Larynx 14

15 Salivary Glands Primary: rare Differentiated tumors: rare Secondary: more common 15

16 Keratin Chromogranin 16

17 Differential Diagnosis: Merkel cell Ca Melanoma Metastatic, NEC RMS Primary: Surgery ± Chemotherapy/XRT Managment Secondary: Chemotherapy/XRT Middle Ear and Mastoid Neoplasms: 17

18 Tumor types: Adenomatous tumors Paraganglioma Neuromas/ acustic Meningioma (intratemporal) Schwannoma,Labyrinthine Inverted papilloma Hemangiopericytoma Fibromatosis Metastasis Middle Ear Adenomatous Tumors Histogenesis: Surface epithelium Stem / basal progenitor (no evidence for neuroendocrine cells) 18

19 Clinical Features: 2 nd to ninth decades No sex predilection Symptoms: Decreased hearing Ear fullness Tinitus Signs: Mass, middle ear cleft Discharge 19

20 Markers: Mucin: negative Endocrine markers: positive Chromogranin Synaptophysin 20

21 Differential Diagnosis: Ceruminous carcinoma: lateral 2/3 of the external ear canal Salivary choristoma Behavior: Non invasive: adenoma / carcinoid Bone destruction: adenocarcinoma/nec Merkel Cell Carcinoma 21

22 Origin: Metastasis: common Primary:? rare 22

23 Chromogranin Management: Chemo + XRT Conclusions: H&N NET Rare Heterogeneous Exclusion of mimics (D.D) 23

24 Thank you 24

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