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NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 COLLECTING CANCER DATA: GIST/SARCOMA 2017‐2018 NAACCR WEBINAR SERIES Q&A • Please submit all questions concerning webinar content through the Q&A panel. • Reminder: • If you have participants watching this webinar at your site, please collect their names and emails. • We will be distributing a Q&A document in about one week. This document will fully answer questions asked during the webinar and will contain any corrections that we may discover after the webinar. 2 Sarc o ma : Bo ne , So ft T issue , GI ST 1

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 Fabulous Prizes 3 AGENDA • Overview • Epi Moment • Quiz 1 • Staging • Bone • Soft Tissue • GIST • Treatment • Quiz 2 • Case Scenarios 4 Sarc o ma : Bo ne , So ft T issue , GI ST 2

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 SARCOMA ANATOMY 5 SOFT TISSUE SARCOMA • Soft tissues include: • Muscles • Tendons • Fat • Blood vessels • Lymph vessels • Nerves • Tissues around joints 6 Sarc o ma : Bo ne , So ft T issue , GI ST 3

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 SOFT TISSUE SARCOMA • Over 50 different types • Angiosarcoma – blood vessels or lymph vessels; following radiation • Undifferentiated pleomorphic sarcoma – most often in arms or legs (previously malignant fibrous histiocytoma MFH) • Spindle cell sarcoma – descriptive name based on the appearance of the cells • Liposarcoma – fat cells; most frequently seen 7 SOFT TISSUE SARCOMA • Pleomorphic dermal sarcoma • Atypical Fibroxanthoma (AFX) • Arise in sun‐damaged skin • Arise in sun‐damaged skin • Head, Neck, Scalp • Ear, Nose, Forehead, Cheek • Negative: S100, Multiple CK, • Negative: S100, multiple CK, Desmin, CD34 Desmin, CD34 • Behavior likely more aggressive • Behavior is almost invariably a but limited follow up due to benign behavior with only rare patient advanced age at local recurrence presentation 8 Sarc o ma : Bo ne , So ft T issue , GI ST 4

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 SOFT TISSUE SARCOMA • Question • What is the appropriate histology code for a final diagnosis or undifferentiated pleomorphic sarcoma and/or pleomorphic sarcoma, undifferentiated? Does the Other Sites MP/H Rule H17 apply in this case, which results in coding higher histology 8805/3? Or does the “undifferentiated” statement only refer to grade, which results in coding histology to 8802/3 (pleomorphic sarcoma)? 9 ANSWER • SINQ 20160044 • Assign 8802/34 to pleomorphic cell sarcoma/undifferentiated pleomorphic sarcoma. Pleomorphic is more important than undifferentiated when choosing the histology code in this case. Undifferentiated can be captured in the grade code. 10 Sarc o ma : Bo ne , So ft T issue , GI ST 5

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 SARCOMA • 2018 Histology – New Terms • 8571/3 Carcinoma with chondroid differentiation (C50._) Carcinoma with osseous differentiation (C50._) Metaplastic carcinoma with chondroid differentiation (C50._) Metaplastic carcinoma with osseous differentiation (C50._) 11 SARCOMA • 2018 Histology – New Terms • 8801/3 Undifferentiated spindle cell sarcoma • 8802/3 Undifferentiated pleomorphic sarcoma • 8803/3 Undifferentiated round cell sarcoma • 8804/3 Undifferentiated epithelioid sarcoma • 8805/3 Undifferentiated uterine sarcoma • 8830/3 Undifferentiated high‐grade pleomorphic sarcoma 12 Sarc o ma : Bo ne , So ft T issue , GI ST 6

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 OSTEOSARCOMA • Most common malignant bone tumor • Arise from osteoblasts • Typically occurs in long bones • Mutation in TP53 are most common • Distant metastasis occur in ~20% • Lung is most common site 13 OSTEOSARCOMA • Periosteum • 2 layers • Fibrous connective tissue • Inner osteogenic • Medullary cavity • Bone marrow https://seer.cancer.gov/tools/ssm/musculoskel.pdf 14 Sarc o ma : Bo ne , So ft T issue , GI ST 7

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 SOFT TISSUE SARCOMA GRADE • FNCLCC grading system • Differentiation • Necrosis • Mitotic rate • Histologic Grade: GX Grade cannot be assessed G1 FNCLCC grade score of 2 or 3 G2 FNCLCC grade score of 4 or 5 G3 FNCLCC grade score of 6, 7, or 8 15 GIST ANATOMY 16 Sarc o ma : Bo ne , So ft T issue , GI ST 8

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 WHAT IS GIST? • Rare type of soft tissue sarcoma • Develop in muscle layer of gut rather than mucosa • Grow outward (exophytic) • Described as a distinct entity in 1998 • Umbrella term for most mesenchymal tumors of stomach and intestine • Most tumors historically called leiomyosarcoma are now classified as GISTs 17 GIST • Interstitial cells of Cajal • “Pacemaker cells” • Sends signals to move food and liquid through system (peristalsis) http://www.gistsupport.org/media/Understanding%20Pathology%20Report/Fig‐1‐ 400pix.jpg 18 Sarc o ma : Bo ne , So ft T issue , GI ST 9

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 ONCOGENIC MUTATIONS • ~85% of GIST contain oncogenic mutations in one of two receptor tyrosine kinases • KIT‐Mutant GIST • PDGFRA (Platelet‐derived Growth Factor Receptor Alpha) • Wild Type GIST • ~12‐15% GIST contain no genetic mutation of KIT or PDGFRA 19 TUMOR LOCATION Stomach 60% Small Intestine 30% Rectum 3% Colon 1‐2% Esophagus <1% Omentum/Mese Rare ntery 20 Sarc o ma : Bo ne , So ft T issue , GI ST 10

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 GIST • How do you determine if a GIST is malignant i.e. reportable? • GIST, NOS is a borderline tumor (/1) • If your state or facility requires collection of these GISTs, you should follow their requirements • Don’t determine reportability based on staging • AJCC staging forms are used on all GISTs 21 Theme Song: The Skeleton Dance COLLECTING CANCER DATA: SARCOMA EPI MOMENT: RECINDA SHERMAN JANUARY 11, 2018 Sarc o ma : Bo ne , So ft T issue , GI ST 11

NAACCR We b ina r Se rie s 2017-2018 1/ 11/ 17 EPIDEMIOLOGY OF SARCOMAS • Large grouping of distinct cancers • 50+ distinct histologies; putative mesenchymal origin • Combined & studied as group • Rare in adults (<2%); Top 5 for Pedi (21%) • Majority soft tissue (87%); malignant bone (13%) • Soft: muscles, joints, fat, nerves, deep skin, blood vessels • Bone: commonly in cartilage • Prognosis generally poor; esp soft • Delayed diagnosis: arise anywhere, lack of specific symptoms • No population based screening • Poor survival adults; better for pedi Figures from: Burningham, Zachary, Mia Hashibe, Logan Spector, and Joshua D. Schiffman. "The Epidemiology of Sarcoma." Clinical Sarcoma Research (2012). BioMed Central Ltd. Web. 10 Feb. 2016. 23 Sarcoma (Soft), 0‐19, CiNA 2014 PEDIATRIC SARCOMAS (SOFT) Rhabdomyosarcomas 12% 36% • Rhabdomyosarcoma – most common soft tissue for peds Fibrosarcomas, peripheral nerve & other fibrous • Skeletal muscle Other specified soft tissue sarcomas 40% • 50% occur <10; slightly more common in males Unspecified soft tissue 12% sarcomas • Often presents as painless mass; risk factor Li‐Fraumeni syndrome • 5 year survival 70%; dependent upon location, stage, and histology—often lymph node involvement • Embryonal better prognosis than alveolar subtype; 20% present metastatic with 5 year survival 30‐40% vs 80% for local • Other Sarcomas (Soft), Rate per 1,000,000, CiNA 2014 • Fibrosarcoma – historically 2/3rds of sarcomas 1.4 1.2 1.2 • now 12% due to better classification (proportion changed but not risk) 1 0.8 • Liposarcoma – often large tumors; common among adults but <5% of ped sarcomas 0.5 0.6 0.4 0.4 Synovial sarcoma –4 th most common; 2x more common in males • 0.1 0.1 0.2 0 • origin mesenchymal not synovium; largely genetic • Malignant peripheral nerve sheath tumors – grouped into Brain CNS category for Epi • Alveolar Soft Part Sarcoma (ASPS) – rare, slow growing; generally mets at dx • Mesenchymoma – rare but highly aggressive 24 Sarc o ma : Bo ne , So ft T issue , GI ST 12