CASE DISCUSSION

1. CASE DISCUSSION Aarti Sardana 2nd year Resident Dept of Radiodiagnosis, SSGH Date: 06-02-08

2. CASE 1

3. HISTORY • 8 mths old female infant presented with • Epigastric lump. • P/A: marked hepatomegaly

5. USG FINDINGS • Marked hepatomegaly noted with multiple hypoechoic lesions involving whole of the liver. • Proximal aortic dilatation noted.

8. CT FINDINGS • Marked hepatomegaly with multiple hypodense lesions involving whole of the liver. • On CECT, marked peripheral enhancement noted. • Proximal aorta appears dilated and distal aortic narrowing noted.

9. DIAGNOSIS INFANTILE HAEMANGIOENDOTHELIOMA

10. DISCUSSION • Infantile hemangioendothelioma is a benign tumor of the liver composed of anastomosing vascular channel lined by plump endothelial cells. • Most patient are 6 months of age or younger & have hepatomegaly or abdominal mass. • CCF, bleeding, anemia, jaundice & cutaneous or visceral hemangioma may also be present. • Malignant transformation of the lesion into the angiosarcoma may occur.

11. CCF has been reported to occur on 8%-68% of patient. • Patient may also have anemia & obstructive jaundice. • Hemoperitoneum may develop due to rupture of the tumor. • Cutaneous hemangioma have been reported in 10-90% of cases.

12. Finely speckled calcification may also be present in about 16% of the cases on plain radiograph • CT is more sensitive in detecting calcification. • May be ass with Kasabach Merritt Syndrome ( thrombocytopenia, DIC)

13. PRENATAL US: • Cardiomegaly, anasarca, ascites & liver mass. • Fetal Hydrops USG ABD: • A single lesion or multiple nodules seen throughout the liver. • The margin of the lesion are usually well defined consistent with pseudocapsule .

14. The lesions are complex or predominantly hypoechoic. • Hyperehoic lesions, which are more typical of adult hemangioma. • On Doppler US, hepatic artery and proximal aorta may appear enlarged with marked tapering of the aorta distal to the celiac artery. • Hepatic vein may also be enlarged due to marked blood flow through the lesion. • Venous flow can be demonstrated by the doppler US in some of the anechoic spaces in the lesion.

15. 1 month old girl with CCF: CXR shows marked cardiomegaly with pulm edema. USG shows diffusely abnormal echotexture & 2 hypoechoiec lesions. Aorta at this level is enlarged. Multiple cutaneous hemangiomas also noted.

16. ANGIOGRAPHY: • Highly vascular tumor. • An abrupt decrease in the caliber of the aorta is noted in caseswith arteriovenous shunting. • The hepatic artery and extrahepatie feeding vessels are typically enlarged and tortuous. Image from arterial phase of selective celiac angiogram shows prominent Rt hepatic A & hypervascular mass in inferior Rt lobe of liver. Venous phase shows continued tumor blush. Central avascular area is noted in both the images.

17. NECT : low attenuation mass+/- Calcification. Multifocal lesions are less likely to calcify. • CECT: Peripheral enhancement - most common CT enhancement pattern. • Delayed scan : filling-in of the central attenuation area , center remains hypoattenuating on delayed scan particularly with large solitary lesions. • Multifocal lesions are usually small with lack of hemorrhage or necrosis & frequently enhance completely.

18. MRIMAGING • Appearance varies due to presence or absence of hemorrhage or infarction. • Generally lesions are heterogenous & of low signal intensity on T1W images, although high signal intensity in the area of hemorrhage may be seen • On T2W image, the lesion usually have high signal intensity. • In this , the appearance of infantile HET is similar to that of adult hemangioma.

19. A 2-month-old boy presented with a palpable mass in the left side of the abdomen. (a) Contrast- enhanced CT scan reveals a large mass in the left lobe of the liver with marked peripheral enhancement. (b) Axial T2-weighted MR image demonstrates high signal intensity of the lesion, particularly in its periphery.

20. Localized infantile hemangioma in a newborn with hepatomegaly and CHF.(a) Transverse US image of the liver depicts a localized heterogeneous mass in the right hepatic lobe (arrowheads) with coarse calcifications (arrow). (b) Color Doppler US image demonstrates the highly vascular nature of the mass.

21. Coronal single-shot fast spin-echo MR image of the abdomen shows a hyperintense mass in the right hepatic lobe (arrows) with flow voids within and around the lesion (arrowheads). Axial contrast material-enhanced gradient-echo T1-weighted MR image of the liver (arterial phase) depicts intense peripheral uptake with early filling of the draining veins (arrows), a finding that indicates the presence of arteriovenous shunting in the lesion.

22. DIFFERENTIAL DIAGNOSIS • The d/d of infantile HET are • Hepatoblastoma • Metastatic neuroblastoma • Mesenchymal hamartoma

23. Hepatoblastoma: • Most common primary liver tumor in children and is usually seen in infants and children less than 3 years old • Alpha-fetoprotein are markedly elevated, whereas it is normal or mild elevated in infantile HET. • Patients present with a palpable mass, but jaundice, pain, anorexia, or weight loss may be present as well. • The mass is typically large, solid, and well defined, with variable echogenicity and often including calcifications • On CT, hepatoblastoma is more heterogenous than infantile HET especially after contrast injection.

24. Hepatoblastoma in a 7-month-old boy with an abdominal mass. Transverse sonogram demonstrates a heterogeneous solid mass in the right hepatic lobe (arrows) that contains shadowing calcifications (arrowhead).

25. 2) Metastatic neuroblastoma: • Urinary level of catecholamine is almost always elevated with this cond. • Marked enhancement is usually noted in infantile HET with enhancing areas represent tumor. In metastatic neuroblastoma areas that enhance represent normal residual liver

26. 3) Mesenchymal hamartoma: • Occur in infant. • Alpha fetoprotein is normal. • Typically appear as multilocular cystic mass on US & CT, and rarely as a solid lesion. • It will appear avascular on angiography, scintigraphy and doppler US.

27. Large mesenchymalhamartoma in a young child. Contrast-enhanced CT scan (a), T1-weighted MR image (b), and T2-weighted MR image (c) show that the tumor is predominantly cystic but has a thick wall and some enhancing internal stromal components

28. CASE2

29. A 69 years old lady with recurrent severe abdominal pain for the last four months.

30. Contrast enhanced CT shows marked ileal wall thickening (arrow) with a mass projecting into the adjacent mesentery. Contrast enhanced CT shows an ill-defined soft tissue mass projecting in the mesentery (arrow) with a characteristic wheel and spoke appearance

31. CT abdomen shows presence of multiple metastases.

32. Differential diagnosis • Desmoid Tumor • Sclerosing Mesenteritis • Lymphoma • Carcinoid tumour

33. Desmoid Tumor • Desmoid tumors -rare, locally aggressive, nonencapsulated masses resulting from a benign proliferation of fibrous tissue. • Primary neoplasm of mesentery • Desmoids forming in the mesentery are especially common in patients with familial adenomatous polyposis.

34. Mesenteric tumors appear as soft-tissue masses with well-demarcated or poorly defined borders, strands radiating into the adjacent mesenteric fat, or a "whorled appearance" of fibrosis growing into the mesenteric fat. Infiltration into adjacent organs or growth into the abdominal wall musculature or the psoas muscle is not uncommon. Vessels are displaced but not encased by the mass.

35. Axial CECT image of the lower abdomen shows a 5cm soft-tissue mass in the mesentery (arrow). The attenuation of the mass is similar to that of the psoas muscles. Coronal reformatted image demonstrates that the mesenteric vessels are displaced but not encased by the mass.

36. Sclerosing Mesenteritis • Sclerosing mesenteritis - rare inflammatory condition characterized by fibrofatty infiltration of the root of the mesentery. • CT - mesenteric mass containing a variable proportion of fat and soft tissue with radiating linear strands reflecting the fibrous reaction of the mesentery. Occasionally, calcifications may be present.

37. The mass frequently engulfs mesenteric vessels and is not locally invasive. Additional findings include: Well-defined, small (<5mm) nodules of soft tissue density with a hypodense fatty halo, a hyperattenuated stripe surrounding the mass, and displacement of adjacent bowel loops. The latter is associated with kinking and tethering of adjacent bowel loops which may also have serrated contours. Enlarged mesenteric lymphnodes.

38. Sclerosing mesenteritis in a 70-year-old man with recurrent rectal bleeding. Axial contrast-enhanced CT image of the midabdomen shows soft-tissue mass at the root of the mesentery (solid arrows). The lesion contains coarse calcifications (open arrow). Mesenteric varices are present (arrowheads). Coronal oblique reformatted image displays the full extent of the lesion encasing the superior mesenteric artery.

39. Lymphoma: • Lymphoma is the most common malignant neoplasm affecting the mesentery. • Bulky retroperitoneal adenopathy commonly accompanies the mesenteric disease and should be a clue to the diagnosis.

40. Patterns of mesenteric lymphoma at CT include: • Multiple, rounded, mildly enhancing, homogeneous masses that often encase the mesenteric vessels and produce the "sandwich sign"; • A large, lobulated, "cakelike," heterogeneous mass with low-attenuation areas of necrosis displacing small bowel loops; or • An ill-defined infiltration of the mesenteric fat, particularly after successful chemotherapy.

41. Axial CECT image of the lower abdomen shows a large soft-tissue mass in the mesentery (arrows) that displaces the small intestine. The mass has low attenuation, indicating extensive necrosis & is also encasing the superior mesenteric vein (arrowhead). Large retroperitoneal nodes are also present (open arrow).

42. Gastrointestinal carcinoid • Gastrointestinal carcinoid, also called carcinoid tumor, is MC primary tumor of the small bowel and appendix. • Most carcinoid occur in patients > 50 years • They arise from neuroendocrine cells in the intestinal mucosa or submucosa. • Approximately 40%–80% of gastrointestinal carcinoid spread to the mesentery, either by direct extension or through the local lymphatic.

43. Locations of gastrointestinal carcinoid tumors: • Appendix - 30-45% (most benign) • Small bowel - 25-35% • Rectum, colon, stomach, pancreas & duodenum. • Gastrointestinal carcinoid metastasize to the lymph nodes and liver. Liver metastases may produce carcinoid syndrome.

44. Plain abdominal radiographs may reveal curvilinear calcification within the abdomen. These are usually smaller than 15 mm in diameter and result from calcification within the tumor.

45. On barium studies: Well-defined, round, intraluminal, bowel-filling defects. These may be associated with thickening of the valvulae conniventes Rigidity, displacement/stretching, and fixation of small-bowel loops. Desmoplastic reaction from mesenteric invasion causes sharp angulation of a bowel loop or a stellate or spokelike wheel arrangement of adjacent bowel loops. Kinking of the small bowel loops is considered the hallmark of carcinoid tumor that has been called a hairpin turn .

46. Upper gi barium series shows a smooth submucosal mass in the mid jejunum eccentrically placed and associated with thickened valvulae conniventes resulting from bowel edema and proximal small-bowel dilatation. Note the angulation of the bowel and kinking of the jejunum at the site of the submucosal mass.

47. The primary tumor is often small, sometimes occult, and only occasionally diagnosed at CT. • CT reveals a mass with soft-tissue attenuation and variable size, with spiculated borders and radiating surrounding strands. • Calcification may be noted in the tumor. • Linear strands within the mesenteric fat probably are thickened and retracted vascular bundles and represent peritumoral desmoplastic reaction.

48. The triad of calcification within a mesentric mass, radiating soft tissue strands due to reactive desmoplasia around the mass, and mural thickening of an adjacent bowel loop is highly suggestive of this diagnosis. Lymphadenopathy and liver metastases may be visualized on CT. Somatostatin-receptor scintigraphy performed with indium In 111 octreotide and 111In pentetreotide is a sensitive and noninvasive technique for imaging primary carcinoid tumors and carcinoid metastatic spread.

49. Axial CT scan through the mid abdomen shows a mesenteric mass (long arrow) with shaggy borders and probable intratumoral punctate calcification (short arrow).

50. The angiographic appearances: • Foreshortening of the bowel occurring with desmoplastic reaction makes mesenteric arteries tortuous and frequently narrowed and draws the arteries into a stellate pattern. • The areas involved appear hypervascular, but in reality, the number of arteries in the area does not increase. Instead, the arteries contract into a smaller area as a result of fibrosis.