1	NERRS September, 2009 John Crowe, MD Dan Cornfeld, MD Kevin Chang, MD Maryellen Sun, MD
2	Case 1: John Crowe
3	Case 1
4	Discussion, DDx
5
6	Another case of this…….
7	My other cases of this: Looks similar?
8	Ddx Liposarcoma Myxoid lipoma Myxoma Infiltrating angiolipoma Duplication cyst (N=1)
9	Aggressive Angiomyxoma Soft tissue tumor Pelvis and perineum Adult women Often on the right side Fibroblastic/myoblastic origin Locally infiltrative (aggressive) High risk of local recurrence AAM is regarded as an aggressive neoplasm because of its propensity to recur locally if it is not completely excised Lacks metastatic behavior
10	Case 1: Dan Cornfeld 76 year old female with episode of dyspnea leading to an US showing an echogenic renal mass, 7.5cm in size.
11
12
13
14	Dan, Commence erudite discussion…..
15
16	Disclaimer I trained at BIDMC Neil Rofsky taught me most of what I know about MRI If I bomb on these cases, you know who to blame.
17	Case 1 Summary of findings T2 bright mass above the right kidney Fat, fluid, early hemorrhage
18	Case 1
19
20	Case 1
21
22	"AML (easy choice" AML (easy choice, most common) Fluid signal No connection to the kidney on any image. Can get extra-renal AML . . Cystic AML? Unlikely Mixoid Liposarcomas Rarely peri-renal. Usually in the extremities Don’t enhance in a cystic fashion, appear “pseudo cystic” Can get liposarcomas of the renal capsule Myelolipomas Rarely Extra-adrenal. Rarely Cystic? Teratomas Lymphangiomas Can contain lipidy fluid but should not have bulk fat Hybernomas (brown fat tumors) Dr. Rofsky did a Nuc Medicine Fellowship . . . Can occasionally have myxoid elements Lipoblastomas Can look exactly like this (figure 5c,d in Radiographics article from Jan 2009). Neoplasm of infants and young children. Paragangliomas can rarely have fat Not connected to adrenal.
23	Dx Myxoid retroperitoneal liposarcaoma. Can’t think of clues to lead me elsewhere . . I think I am missing something Needs to be removed.
24	Ddx Where is it coming from? Look for organ& vessel displacement Feeding artery Any defining tissue features? Fat Low SI Fibrous, Muscle, Ca⁺⁺ Fluid? Lymphangiooma, abscess Definition? Necrosis?
25	Ddx Where is it coming from? Any defining tissue features.
26	Different Case
27	Different Case
28	Ddx Fat within Renal AML Adrenal Carcioma Large Clear Cell RCC Retroperitoneal Lipoma/Liposarcoma Teratoma Origin: Retroperitoneum Liposarcoma, Leiomyosarcoma, Malignant fibrous histiocytoma Lipomas of the RP are rare!!
29	Dx: Retroperitoneal Liposarcoma well differentiated, with myxoid stromal change the most common 1° RP tumor 5 subtypes: well differentiated lipomatous, myxoid, round cell, pleomorphic, mixed. Most pts >50 years old > soft tissue suggest de-differentiation Myxoid LPS have high % H₂0 High on T2WI, Low on T1WI Renal defect Þ AML
30	Case 1: Kevin Chang 56 year old female with sharp intermittent LUQ pain
31
32
33
34	2 hour post Gd-BOPTA
35
36
37	Peripheral Cholangiocarcinoma Tumor arising from the bile duct epithelium Most are adenocarcinoma (90%) Intra-hepatic 10% Hilar 25% Extra-hepatic 65% Imaging Appearance Well-defined, single, predominantly homogeneous mass with irregular borders Capsular retraction Often have peripheral thick rim enhancement Dilated bile ducts peripheral to tumor
38
39
40	DDx HCC Mixed Tumor (HCC & Cholangio) Sclerosing HCC Immature abscess Metastases (think of desmoplastic types) Central necrosis more common in mets Hepatic TB Look for layers
41	Maryellen Sun, Case 1 72 year male with history of: Hypertension Thalassemia Renal insufficiency 20 lb. weight loss over recent 6 months
42	Coronal HASTE
43
44
45	DCE- VIBE Imaging
46	Multiplanar Reformations
47	Comments?
48
49	History as it occurred Presented with weight loss, fatigue and decreased appetite. Renal insufficiency discovered MRA done to assess for RAS Bilateral adrenal masses discovered @MRA Labs: Dexamethosone suppression; VMAs C/w adrenal insufficiency CT Core bx: Non-Hodgkin's lymphoma, large B cell type
50	Primary Adrenal Lymphoma - Imaging Complex masses variable density (necrosis and/or hemorrhage) Most bilateral FDG avid
51	Adrenal Lymphoma Usually due to contiguous spread from RP 4% of patients with widespread non-Hodgkin's on CT 24% with widespread non-Hodgkin's have adrenal involvement at autopsy 46% bilateral Adrenal insufficiency seen 50% of pts with bilateral involvement Primary lymphoma is rare! Approximately 80 cases in English literature Most common type is diffuse large B-cell lymphoma May respond to CHOP regimen Prognosis is poor Older pts with co-morbidities; adrenal insufficiency
52	Case 2: John Crowe 77 y.o. male with smoking history CT of chest extends to liver demonstrating a ‘lesion’
53	Case 2
54	Case 2
55	Case 2
56	Case 2
57	Thoughts??
58
59	T2-Weighted Imaging
60	Macro fat containing liver lesions
61	Intracellular lipid liver lesions
62	Hepatic Angiomyolipoma Rare, benign mesenchymal neoplasm Varying amts of 1) smooth muscle cells, 2) thick-walled blood vessels, and 3)mature adipose tissue. Sporadically or with tuberous sclerosis TS in only 6% if cases Often Asymptomatic and incidental Diverse imaging features various proportions of its 3 components 50% of hepatic AMLs lack considerable fat content It should not be treated surgically because of its benign nature.
63	Case 2 Dan Cornfeld 19 year old female with intermittent abdominal pain WBC, C-reactive protein, EST all WNL Elevated lipase (250IU/L [nl 0-60]) and amylase (158 IU/L [nl 0-100])
64
65
66
67
68	Well???
69	Case 2 Mass in the mesentery / bowel – it moves Edema surrounding the mass ?? Tubular structure in the mass with fluid and ?? Filling defects or stones Symptoms and laboratory values suggesting pancreatitis Normal pancreas
70	Diagnosis Pancreatitis in ectopic pancreatic tissue either located in the small bowel or in the mesentery. There may be stones in the duct.
71	Ectopic Pancreatic Tissue Gastric Antrum Duodenum Jejunum Reported in small bowel mesentery fallpoian tubes, gall bladder, esophagus, Meckels Diverticulm, mediastinum, ileum, umbilicus, ileum
72
73	Diagnosis Pancreatitis in ectopic pancreatic tissue either located in the small bowel or in the mesentery. There may be stones in the duct.
74
75	Ectopic Pancreas (heterotopic, accessory or aberrant) DDx Jejunal diverticulitis Perforated bowel Lymphoma Foreign body GIST
76	Ectopic Pancreas Congential anomaly of GI tract 0.55 – 14% of autopsy series Common sites Duodenum 28%, Stomach 26%, Jejunum 16% Typically submucosal Most pts are assymptomatic Typically found as microscopic, incidental deposits of panc tissue Signs & Symptoms Pain, Obstruction, elevated panc enzymes Serum panc enzymes may be elevated
77	Case 2: Kevin Chang
78
79
80
81
82
83
84	Retropertioneal Leimyosarcoma Leiomyosarcoma represents ~ 9% of all soft tissue sarcomas Primarily affects middle-aged & older adults F>M Signs & Sxs abdominal mass, pain, swelling, weight loss or lower extremity edema Poor Prognosis 5 yr survival ~35%
85	Retropertioneal Leimyosarcoma Ddx GIST Necrotic lymphoma PNET (esp if vascular) Renal Capsular Tumor
86	Retropertioneal Leimyosarcoma
87	Case 2: Sun
88
89	A brain teaser??
90
91	Tubulovillous adenoma of the CHD Intraductal tumors w/ innumerable minute, frondlike papillary projections Friable, and can break off & obstruct Imaging Partial obstructed by intraluminal masses Look for inconsistency and changeability Look for enhancement Vs. Mucin and Stones Other dxs Biliary IPMN Cholangiocarcinoma
92	Tubulovillous adenoma of the CHD Rare in biliary tract; much more common in enteric system Signs & Sxs jaundice, right upper quadrant abdominal pain, dyspepsia, nausea and vomiting Clinical and radiologic dx is often bile duct stone with cholangitis Thought to follow the adenoma ® carcinoma progression; therefore, resect!
93
94
95	Comments?