Interesting case. Vikas Kundra, M.D., Ph.D. October Vikas Kundra, M.D., Ph.D.

Transcription

1 Interesting case October 2012

2 Disclosure Information Vikas Kundra, M.D, Ph.D. I have no financial relationships to disclose. I WILL NOT include discussion of investigational or off-label use of a product in my presentation.

3 History Breast tumor 60 yo

4 Left Mastectomy and Lymph node dissection

5 Status Post Partial Hepatectomy

6 Low Attenuation Lesion

7 Low Attenuation Lesion

8 Low Attenuation Lesion

9 Low Attenuation Lesion

10 Low Attenuation Lesion

11 Differential Diagnosis Metastatic Disease from Breast Cancer Metastatic Disease from Breast Cancer Infection? Second Primary?, Met from Second Primary

12 6 Months Prior

13 1 Year Prior

14 2 Years Prior

15 Finally made cancer into a chronic, managable disease like diabetes!

16 4 Years Prior

17 4 Year Prior

18 3.5 Years Prior

19 3.5 Years Prior

20 3.5 Years ago

21 Ductal Carcinoma

22 8 Years Prior

23 Differential Diagnosis Metastatic Disease from Breast Cancer Metastatic Disease from Breast Cancer Infection? echinococcal cyst Second Primary?, Met from Second Primary Slow growing metastases from second primary Treated metastases from second primary like GIST Hepatocellular carcinoma Cholangiocarcinoma Benign lesion such as adenomas, inflammatory pseudotumor (Patnana et al. AJR 198, 2012, W217) Epithelioid Hemangioendothelioma

24 Epithelioid Hemangioendothelioma

25 Epithelioid Hemangioendothelioma Rare vascular primary tumor of the liver Incidence 1 per million (Hertl M, Cosimi AB Oncologist 10, 2005, 269) May involve soft tissues, lung, bone Age, 42; females>males 3:2 (Mehrabi A. et al. Cancer 107, 2006, 2108) Variable clinical course Rapidly progressing Stable for several years

26 Epithelioid Hemangioendothelioma Path: Multifocal nodules (87%) mm to cm s in size (Mehrabi A. et al. Cancer 107, 2006, 2108) Extra-hepatic involvement at time of Dx (37%) Lungs (8.5%) Lymph nodes (7.7%) Peritoneum (6.1%) Bone (4.9%) Spleen (3.2%) Diaphragm (1.6%) Other

27 Epithelioid Hemangioendothelioma Histology: dendritic spindle shaped cells with epithelioid round cells and a matrix of myxoid and fibrous stroma Factor VIII related antigen +, CD31 and 34+ potential new markers: VEGF, Podoplanin, WWTR1- CAMTA1 gene fusion product - Mistry AM, J GastroIntestin CA 2012 The neoplastic endothelial cells may invade sinusoids and terminal hepatic and portal veins, thereby cutting off the blood supply to the tumor

28 Epithelioid Hemangioendothelioma Symptoms: Abdominal pain Weakness Anorexia Jaundice None Lab: Abnormal LFT s

29 Epithelioid Hemangioendothelioma Imaging findings: Focal/multifocal type (most common by imaging) Diffuse type Likely due to coalescing focal lesions Furui S, Radiology , 63

30 Epithelioid Hemangioendothelioma Imaging findings: look like mets CT Low-attenuation masses that may become isoattenuating with contrast Target sign and delayed enhancement Chen Y et al. World J Gastroenterol ,3544 Calcifications and capsular retraction ~10% Ultrasound hypoechoic

31 Epithelioid Hemangioendothelioma Imaging findings: look like mets MR T1-low, 10% with low intensity rim T2, high or mixed, 35% with low intensity rim Enhance, centrally or peripherally Larger lesions can have central necrosis (Mehrabi A. et al. Cancer 107, 2006, 2108)

32 Epithelioid Hemangioendothelioma Prognosis: 20% die within 2 years of diagnosis 20% live 2-28 years + treatment Therapy 1yr 5 yr %survival No treatment 40 5 Liver transplantation Chemo- or radiation therapy Liver resection (Mehrabi A. et al. Cancer 107, 2006, 2108)

33 Thank you