Dao danh vinh gist imaging jfim hanoi 2015

Journées Francophones d’Imagerie Médical JFIM 2015

Gastro-Intestinal Stroma Tumors - Overview -

Dao Danh Vinh, MD Department of Radiology

Bach Mai Hospital Ha Noi - Viet Nam


Introduction •  1983: Mazur and Clark first

introduced the term GISTs •  < 1998: –  Confused with other tumors in

muscularis propria –  Leiomyomas, leiomyosarcomas,

l e i o m y o b l a s t o m a s , schwannomas,…

•  1998: –  Hirota et al: dicovered mutation

of KIT –  Immunohistochemical staining. –  Studies in ultra-stucture of cells –  GISTs is difinitely difference

w i t h o t h e r m e s e n c h y m a l neoplasms in GI tract

Slide by Cristina Antonescu, M.D


Introduction

•  United - State –  Prevalent: 6,8/1.000.000 –  Incidence: 4.500–6.000/y

•  No association with:

•  Geographic location

•  Ethnicity, race, occupation

•  Age: 50-60 (rarely < 40). •  Sex: M~F •  NF type I: increased prevalence,

multiple

Tran T et al. Am J Gastroenterol. 2005 Jan;100(1):162-8


Introduction Viet Nam: •  > 20 papers about GISTs public in domestic journals •  Case/seri case report; single centre (no national data study)

Authors Year City N GISTs

Pham Minh Hai et al 2004-‐2008 HCM N/A 41

Ngo Quoc Dat et al 2005-‐2011 HCM N/A 130

Nguyen Van Mao et al 2010-‐2011 Ha Noi 84 73

…

Pham Minh Hai et al. Y Hoc TP. Ho Chi Minh, Vol. 13 Supplement of No 1 -2009:65-68 Ngo Quoc Dat et al. Y Hoc TP. Ho Chi Minh, Vol. 15 Supplement of No 3 -2011:129-135 Nguyen Van Mao et al. Y hoc thuc hanh. 2011; 730: 56-60


Introduction Source of GISTs: •  Interstitial cell of Cajal (ICC) •  Pacemaker of smooth muscles

in GI tract Signals to trigger peristalsis: •  Stomach: 3 times/min •  Jejunum: 11-12 times/min •  Ileum: 9-10 times/min •  Colon: 3-4 times/min


Introduction

ICC: Interstitial Cells of Cajal

•  Source of GISTs


Clinical features


Position •  Depend on the normal distribution of ICCs •  From oesophagus to annus •  Extragastrointestinal stroma tumors: mesentery, peritoneum,

retroperitoneum


Symptom

•  Depend on the size and anatomic location of the tumor •  About 20% of case are discovered incidentally •  Presenting:

•  GI bleeding: in stomach, SI, recto-colon. •  Dysphasia: in esophagus •  Anemia •  Nausea, vomiting •  Abdominal pain •  Weight loss •  Abdominal distention, abdominal obstruction (rarely) •  A palpable mass


Pathologic features


Gross features

•  Exophytic: Sub-serous, sub-mucous •  Size: mm - 30 cm •  Border: wel-defined, ill-define •  Centre: degeneration, necrosis, haemorrhage, cavity…

Levy, A. D et al. Radiographics 23, no. 2 (2003): 283-304, 456


Gross features



Microscopic features •  Three major patterns of histologic features:

–  Predominantly spindle cells: 70% –  Predominantly epithelioid cells: 20% –  Other (mixture, angiomatoid, myxoid): 10%

Hematoxylin - Eosin (x40) Levy, A. D et al. Radiographics 23, no. 2 (2003): 283-304, 456


Microscopic features •  Immunohistochemistry (IHC) •  KIT protein (CD117): (+) in 91-95% of cases •  PDGFRA - CD34 (70%), DOG1

Type tế bào hình trụ (spindle) Type tế bào dạng biểu mô (epithelioid)



Microscopic features


Prognosis Mitotic rate (MR): rate of cell division

HE x 400



Prognosis


Benign:MR = 0 Low-grade malignant :MR < 5 Malignant :MR > 5


Prognosis

Factors: size and MR


GISTs of stomach


Features

•  Stomach tumors: 2-3% •  Posistion: 70% in the body,

fundus •  Size: 4-25cm •  Exophytic (sub-mucous, sub-

serous): 80% •  Necrosis, haemorrhage, cavity.

Rarely calcification •  Surface ulceration: 60% •  Hyper-vascular: peripheral 90%. •  Mitoses rate: hight


Submucous GISTs

M, 67Y F, 67Y

AP view Oblique view



58YO female Chronic epigastric pain Sub-‐mucous mass


CLVT

56YO Female Sub-‐mucous mass


54YO female Chronic epigastric pain Sub-‐serous GISTs


69YO Female, epigastric pain

Schwanoma


Difference diagnosis


Schwanoma


Difference diagnosis: AGC •  Most common tumor in stomach, especially in the antrum •  Large base •  Rarely with exophytic (sub-serous, sub-mucous) •  Regional adenopathies •  Endoscopy with biopsy


GISTs of small bowel


Features

•  Jejunum, Ilium > Duodenum •  Size: 2-20cm •  Exophytic: sub-mucous, sub-serous •  Hypervascular with arterial feeders •  GI obstruction, sub-obstruction •  Cavity, necrosis, fistula •  Rarely local invasion: malignant •  Atypic GIST (infiltration): lymphoma,

adenocarcinoma…


•  Nam, 57 tuổi •  Đau bụng âm ỉ, diễn biến 4 tháng


D T

GISTs: gastrointescnal stroma tumors (IHC -‐ CD117)


43YO Female Epigastric distencon Duodenum mass


52YO Male Epigastric distencon Duodenum mass



Ilium GIST: sub-serous feature with cavity and communication with lumen bowel


Atypic GISTs: adenocarcinoma, lymphoma, metastasis…


Difference diagnosis


Colo-rectum GISTs


Features

•  Uncommon •  Rectum >> Colon •  Often diffuse development

(intramural). •  Surrounding infiltration •  Difficult to differentiate to other

colorectal tumors: adenocarcinoma, leiomyosarcoma…


47YO Female Sub-‐obstruccon Hepacc flexure colon mass



69YO Female Hypogastric pain, hematochezia GIST đoạn cuối trực tràng

nhiều ổ chảy máu



Difference diagnosis: adenocarcinoma


Metastasis GISTs


Features

•  GISTs < 2cm are often benign. •  Some GISTs are malignant, metastasis by many ways •  Target organs

–  Most often: live, peritoneum (95%). –  Uncommon: soft-tissue, lung, pleural membrance –  Rare: regional adenopathies, cerebral, bone…

•  Characteristic: –  Similar to original GISTs –  Hyper-attenuation –  Hypervascular –  Heterogenous: necrosis, hemorrhage, cavity…


GIST hỗng tràng di căn gan

Nam 55 tuổi Chướng bụng thượng vị


Nam 68 tuổi, GIST dạ dày di căn gan, phúc mạc

Nam 67 tuổi, GIST dạ dày di căn gan

Hong, X. Radiographics 26, no. 2 (2006): 481-95.


Nam 56 tuổi, GIST hỗng tràng di căn gan Nữ 64 tuổi, GIST hồi tràng di căn phần mềm, phúc mạc

Hong, X. Radiographics 26, no. 2 (2006): 481-95.


Nữ, 71 tuổi, đau hạ vị, sờ thấy khối khu trú


IHC: CD117 (+)

Multiple malignant GISTs


Treatment

Curative: •  Surgical resection •  Open/Endoscopy Palliative: •  Imatinib therapy: inhibitor the receptor of KIT-tyrorine kinase •  Un-operation, metastasis, recurence •  Imatinib dose: 400 mg/day •  Imatinid resistance: •  Sunitinib 37.5 mg/ngày •  Sunitinib 50 mg/ngày


Conclusion

•  GISTs is the most common of the in muscularis propria in GI tract •  Course of GIST comes frome ICC (interstitial cell of Cajal) •  Imaging features:

–  Sub-serous type –  Sub-mucous type –  Heterogeneous –  Necrosis, haemorrhage, cavity, –  Hype-rvascularity

•  Definitely diagnosis –  IHC: CD117, CD34, DOG1 –  Prognosis: size, mitoses rate


Thanks so much for your a/en1on!

Dao danh vinh gist imaging jfim hanoi 2015

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