Transcript GIST
Carcinoma, GIST & Neuroendocrine Tumor in the Gastrointestinal Tract – Radiopathologic Correlations 성균관의대 삼성서울병원 영상의학과 최동일 Carcinoma Histological Classification • WHO international classification (1997) - Papillary - Tubular - Mucinous - Signet ring cell • Lauren classification - Intestinal type - Diffuse type • Ming’s classification - Expanding type - Intestinal type Gross Classification of EGC Elevated Superficial Excavated * most predominant patterns listed first Advanced Gastric Cancer Gross Classification of AGC B II B II B III B IV T-staging of Gastric Cancer LN metastases • • • • EGC ; ~10%, AGC ; ~80% Size criteria ; > 6-8 mm Round shape, enhancement on CT Accuracy ; ~60% Peritoneal seeding • About 25-40% • Rectovesical space, SB mesentery(RLQ), Sigmoid mesocolon, paracolic gutter • “drop” metastases Krukenberg’s tumors (especially signet-ring cell ca) • Omental cake – irregular, beaded thickening and stranding – Nodules • Loculated fluid collections Hematogeneous Metastasis • Liver (m/c), lungs, adrenal gland, bone, brain, 다른 GI tract (rectum, small intestine) 위암 만성간염환자에서 생긴 위암 동맥기 – enhancing 문맥기 – wash-out 지연기 – wash-out 동맥기 문맥기 지연기 T2 MR : 고신호강도 MR 동맥기영상 : 조영증강 문맥기와 지연기 : 테두리 있는 저신호강도 wash-out ?? T2 동맥기 문맥기 지연기 Cancer Healing ulcer Papillary adenocarcinoma Stomach Liver Gastric Cancer: CT T-staging Detectability of Tumor • Helical CT 77-100% (63-81% for EGC) • MDCT ~ 100% T-staging • Helical CT 48-82% • MDCT 77% with trans. CT vs. 84% with vol. CT N staging (more important than T- staging for prog.) • Helical CT 51%-56% • MDCT 62% with trans. CT vs. 64% with vol. CT Gastrointest Endosc. 2004; 59:619 Radiology 2005;236:879-885 T1 (EGC) T2 T3 T4 ? T4 on transverse CT T3 on MPR image Pathologic T3 cancer. ? T1 on transverse CT T2 on MPR image Pathologic T2 cancer ? T2 on transverse CT T3 on MPR image Pathologic T3 cancer Irregular perigastric fat infiltration Pathologic T2 stage !!! Irregular and nodular strands eAGC vs. eEGC – Samsung study • The tumor detection - 61% (64 of 105) for 3 orthogonal MPR imaging by at least 2 radiologists. • In 30 eAGCs, the accuracies for all T staging - 3 MPR imaging > transverse imaging • However, in 34 eEGCs, the only accuracy of muscular invasion (T2 or higher) 3 MPR imaging > transverse imaging - In eEGC, it may be enough to evaluate the preoperative staging and make a treatment plan with transverse CT imaging only. MPR images including coronal or sagittal reconstruction may have little impact on the diagnostic accuracy for tumor that is impressed as EGC in the gastric endoscopy. Endoscopic submucosal dissection (ESD) using IT knife > 650 μm Long performance time, High rate of Cx High level of technical skills Hepatic mets after EMR for EGC (M/82) - SM2 (+), surgery refused 28 months after EMR Mucinous adenocarcinoma Park MS, et al. Radiology 2002;223:540 The most common type of gross appearance in both carcinomas was fungating: It occurred in 71% of patients with mucinous carcinomas and in 59% of patients with nonmucinous carcinomas. The next most common gross appearance type was ulcerative (24% of patients) in nonmucinous carcinomas and diffusely infiltrative (29% of patients) in mucinous carcinomas (P = .009). The most common contrast enhancement pattern was homogeneous (61% of patients) in nonmucinous carcinomas and layered (62% of patients) in mucinous carcinomas (P = .001). These findings were significantly different. The predominantly affected thickened layer was the high-attenuating inner layer or the entire layer (88% of patients) in nonmucinous carcinomas and the lowattenuating middle or outer layer (57% of patients) in mucinous carcinomas. Only two mucinous tumors showed miliary punctate calcifications in infiltrative lesions. Gastric Submucosal Diseases • Mesenchymal tumor (mc) - 50% of gastric benign tumor - 1-5% of gastric malignant tumor Gastric Submucosal Diseases • • • • • • • • • • • • • Gastrointestinal Tumor (GIST) Leiomyoma/sarcoma Lymphoma Neural Tumor Lipoma Hemangioma Lymphangioma Neuroendocrine tumor Glomus Tumor Ectopic pancreas Duplication cyst Inflammatory fibroid polys Metastasis GIST • Age: > 50 yr (75%), median, 58 yr • Asx. ------- Sx. (palpable mass, pain, GI bleeding) • Size: 1-35 cm, median, 5 cm • Most common mesenchymal tumor in GIT - Stomach; 50-60% (2-3% of gastric tumor) - Small bowel; 20-30% - Anorectum, colon; 10% - Esophagus; 5% - mesentery, omentum; 5% UGIS of gastric GIST - Well-defined smooth-surfaced mass - Right or obtuse angle to the lumen - Central ulcer - Overlying normal mucosal fold (bridging fold and fading folds) CT of gastric GIST • Well defined enhanced mass • Malignant GIST large size, direct organ invasion, metastasis (liver, lung, bone) • Cystic degeneration, ulceration, mesenteric fat infiltration, • Necrosis, hemorrhage • LN metastasis, Ca++: rare Gastric GIST Gastric GIST Hepatic mets after gastrectomy of gastric GIST Tx Gastric lymphoma Gastric CA (Adenocarcinomas) Gastric CA (Adenocarcinomas) EGC type I+IIc : W/D tubular adenocarcinoma (0.5 cm) in the herniated gastric mucosa (2 cm) Gastric Schwannomas Duodenal GISTs Ileal GIST Ileal GIST Jejunal GIST Mesenteric GIST Colonic GIST Multiple rectal GISTs Managements of GISTs • Complete resection • Imatinib mesylate (Gleevec) – Phenylaminopyrimidine derivative – Selective inhibits protein tyrosine kinases Cystic change - Idx: Incomplete resection, metastatic tumor - Cx: Rupture Mets 3 years after Gastrectomy 1 years after Imatinib Tx. Choi H, et al. J Clin Oncol 2007;25: 시험 6월15일 5시20분부터 영상의학과 의사가 아닌 분들은 풀 필요없는 영상의학과 의사 전용 문제들도 있음 과제물 복부영상의학 관련 2011년 이후 발간된 SCI논문 하나에 대한 감상문 (A4 한페이지 이내) 메일로 제출 – 감상문과 논문 pdf 4월10일까지 논문 제목 (잡지명과 페이지포함) 제출 후 OK 받은 후 5월20일까지감상문과 논문 pdf 제출 Overall survival according to KIT mutation 42 HU 30 HU (29%감소) Hepatic Mets from Colon CA treated with targeted agents 35 HU 25 HU (29%감소) FOLFRI/SUTENE 77 HU 44 HU (43%감소) XELOX/avastin Inoperable HCCs treated with Sorafenib 57 HU 31 HU (56%감소) Gastric GIST 50 HU 25 HU (50%감소) 2년후 PET • Sensitive in early tumor response, but given its cost and availability, it is not easy to include it in basic imaging tests. • The use of PET is considered in cases of: (1) suspected metastatic lesions not clearly delineated by CT (2) exploration of an undetectable primary lesion (3) inconclusive CT findings (4) when early confirmation of tumor response to imatinib is required (for example, when surgery is considered after tumor regression) 2008 Japanese guideline on GIST (Nishida T, Hirota S, Yanagisawa A, et al. Clinical practice guidelines for gastrointestinal stromal tumor in Japan: English version. Int J Clin Oncol 2008; 13:416–430 Heaptic mets after Ileal GIST resection Suspicious lesion after Rt. hemihepatectomy Responses of Imatinib F/62 Exon 11 deletion Size decrease & cystic change Before Tx 3M F/U M/62 Exon 9 insertion Cystic change Before Tx 3M F/U F/66 Exon 11 insertion Size decrease Before Tx 3M F/U Therapeutic Efficacy of Malignant GISTs with c-KIT Mutations: CT with Imatinib Mesylate Size decrease & cystic change Number Exon 11 deletion 93% (14 of 15) Other mutations 50% (4 of 8) (p=0.032, Fisher’s exact test) Choi D, et al. AJR 2009 Aug. Recurrence after initial response CT findings suggesting relapse or resistance after initial response to imatinib: (1) nodules in necrotic or degenerated masses (2) new lesions (3) growth of tumors that previously had decreased in size 2008 Japanese guideline on GIST (Nishida T, Hirota S, Yanagisawa A, et al. Clinical practice guidelines for gastrointestinal stromal tumor in Japan: English version. Int J Clin Oncol 2008; 13:416–430) Neoadjuvant Imatinib Tx. to downsize GIST 14 months with Imatinib Tx. GIST- Summary • KIT (+) • Well defined enhanced mass • Necrosis, hemorrhage • LN metastasis rare, Ca++: rare • Cystic degeneration after imatinib Tx. Classification of NET 1. Well differentiated endocrine tumor carcinoid 2. Well-differentiated endocrine carcinoma – malignant carcinoid 3. Poorly differentiated endocrine carcinoma • • • • Fig. 4. WHO classification of endocrine tumors. (Hematoxylin & Eosin stain, x200). A. Well-differentiated endocrine tumor shows round, regular, isomorphic cells. B. Well-differentiated endocrine carcinoma shows characteristic well-formed rosettes. C. Poorly-differentiated endocrine carcinoma shows densely packed, small cells with scanty cytoplasm and finely granular nuclear chromatin. The fusiform shape is prominent in this microphotograph. 200 patients with NETs in SMC, Rectum (51.9%) > stomach (21.9%) > duodenum (11.2%) > colon (5.9%) > appendix (3.2%) > esophagus (3.2%) > small intestine (2.1%). The majority of NETs occur sporadically, that is, nonfamilial. However, they may sometimes occur as part of complex familial endocrine cancer syndromes such as multiple endocrine neoplasia type I (MEN-I) (Fig. 1) [5] and neurofibromatosis type I (NF-1) [6]. General Neuroendocrine Markers • Chromogranin A • Synaptophysin • Neuron-specific enolase (NSE) Specific Neuroendocrine Markers • Serotonin, glucagon,…. Electron Microscopic Findings • Dilated mitochondria, rough endoplasmic reticulum, free ribosomes • Membrane-bound secretory granules MEN type I (Multiple endocrine neoplasia) Synaptophysin Esophaseal cancer and carcinoid Chromogranin-A Malignant carcinoid in the stomach Gastric P/D endocrine carcinoma (Small cell carcinoma) Gastric P/D endocrine carcinoma (Large cell NE carcinoma) Like Borrmann type II AGC Duodenal carcinoid B Ileal carcinoid Appendiceal carcinoid Sx. : Acute appendicitis Cecal NE carcinoma + adenocarcinoma Rectal carcinoid Rectal malignant carcinoid Radiologic Findings of Neuroendocrine Neoplasms (GIT) •2008 삼성서울병원 소화기영상의학과 워크샵 (용인 에버랜드)