Ryan B. O'Malley, MD Assistant Professor, Body Imaging Department of Radiology, University of Washington School of Medicine, 1959 NE Pacific St, Seattle, WA 98195. History: 65-year-old female with right lower extremity melanoma What is the most likely diagnosis? a) Mesenteric hemorrhage b) Mesenteric panniculitis c) Lymphoma d) SMV thrombosis with mesenteric edema 1 Findings: Mesenteric fat stranding (“misty mesentery”) surrounding non-enlarged LN Preserved fat halo around the vasculature Tumoral pseudocapsule Benign idiopathic inflammatory process involving the mesenteric fat Commonly incidentally detected in patients being imaged for other reasons (including malignancy) Autopsy series reported up to 1% Does not progress or evolve into malignancy Subtype of sclerosing mesenteritis – spectrum based on the predominant imaging findings and tissue type Mesenteric panniculitis – ill-defined inflammation without discrete soft tissue or mass Histology: Heterogeneous chronic inflammatory infiltrate Mesenteric lipodystrophy – fat necrosis Histology: Lipid-laden macrophages Retractile mesenteritis – fibrotic soft tissue mass with retraction and distortion Histology: Collagen deposition Abdominal manifestation of IgG4-related sclerosing disease RPF IPT AIP Sclerosing cholangitis 2 Imaging: “Misty mesentery” – nonspecific term describing increased attenuation within the mesenteric fat Fluid Fibrosis Neoplastic infiltration Fat-ring sign preserved fat halo surrounding the mesenteric vasculature Tumoral pseudocapsule border outlining the affected mesentery Lymphoma DDx: Lymphoma (after treatment, may be identical to mesenteric panniculitis) Edema (e.g. thrombosis, CHF, pancreatitis) Hemorrhage Carcinomatosis {separate slide with images of each?} History: 53-year-old female with fatigue What is the most likely diagnosis? a) Lymphoma b) Sclerosing mesenteritis c) Carcinoid d) Metastatic GI stromal tumor 3 History: 53-year-old female with fatigue Findings: Clusters of mesenteric and retroperitoneal LN Confluent soft tissue encasing mesenteric vasculature with lost fat halo Most common malignancy affecting the mesentery Primarily spreads via lymphatics Most common malignant retroperitoneal tumor Mesenteric LAD more common in NHL than HD ~50% of NHL has enlarged mesenteric LN at presentation compared to 5% for HD Coexistent retroperitoneal LAD helps suggest the diagnosis 4 Imaging: Can present with LN with a wide range of size and morphology Characteristically confluent soft tissue surrounding and encasing vessels (especially SMA and SMV) with lost fat halo, but no narrowing Sandwich sign – confluent soft tissue on both sides of mesenteric vessels and perivascular fat Following response to therapy, may only see infiltration of the mesenteric fat +/Ca++ FDG PET is most accurate modality, 90-95% sensitive DDx: Sclerosing mesenteritis Misty mesentery and Ca+ usually only seen in treated lymphoma) PTLD Can also see sandwich sign Metastatic disease Typically localized rather than diffuse Infectious/inflammatory adenopathy (e.g. TB, Whipple disease) LN may be rim-enhancing with central necrosis History: 44-year-old man with enlarging palpable L testicular mass Which of the following is considered regional LN in the setting of testicular CA? a) b) c) d) Paraaortic Common iliac Internal iliac Inguinal 5 History: 44-year-old man with enlarging palpable R testicular mass Findings: Confluent retroperitoneal LN at the level of the renal veins No pelvic LN Right testicular mass Metastases from testicular CA most commonly occur in paraaortic region Lymphatics follow gonadal vessels to level of the renal hilae Typically ipsilateral to the primary tumor May spread inferiorly toward the aortic bifurcation Frequently skip the pelvic LN (unless there has been prior inguinal or scrotal surgery; e.g. post-orchiectomy) Always consider testicular CA in DDx for male patients with retroperitoneal LN Testicular germ cell tumors (95% of testicular CA) Seminoma (60%) LN usually soft tissue attenuation Nonseminomatous LN usually more cystic Stage II Pure seminoma + regional LN Staged based on maximum diameter of Paño B et al. RadioGraphics. 2011 Jan-Feb;31(1):135-60. involved LN Stage IIA: ≤2 cm RT Stage IIB: >2 and ≤5 cm RT or chemo Stage IIC: >5 cm chemo (cisplatin- based) 6 DDx – retroperitoneal soft tissue mass(es): Lymphoma Metastatic LN Most commonly pelvic source (e.g. ovary, testis, prostate, uterus) Mesenchymal tumor Leiomyosarcoma Liposarcoma Retroperitoneal fibrosis Hematoma Paño B et al. RadioGraphics. 2011 Jan-Feb;31(1):135-60. History: 60-year-old female with intermittent left flank pain Which of the following is an abdominal manifestation of IgG4related sclerosing disease? a) b) c) d) Primary biliary cirrhosis Whipple syndrome Castleman’s disease Retroperitoneal fibrosis 7 History: 60-year-old female with intermittent left flank pain Findings: Infiltrative retroperitoneal soft tissue encasing aorta and IVC, tethering toward the spine Left renal obstruction with delayed nephrogram and medial deviation of the left ureter Confluent peri-pancreatic soft tissue Inflammatory and fibrotic plaquelike retroperitoneal mass or soft tissue, which encases aorta, IVC, and ureters Presents in middle age (40-60 y/o), men 2-3x more common than women Non-specific presentation – flank or back pain, fever, fatigue, decreased urination, malaise Majority (>70%) are idiopathic, exact mechanism uncertain Increasingly recognized as systemic inflammatory condition Abdominal component of IgG4-related sclerosing disease along with other fibroinflammatory conditions Some suggest an immune response to leaking antigens from atheromatous plaque within the aorta Secondary causes Ergot derivatives (e.g. mehysergide) are most common Coexistent malignancy in up to 8%, possible a desmoplastic reaction to underlying retroperitoneal metastases 8 Imaging: Infiltrative retroperitoneal soft tissue, most commonly along the aorta and common iliac arteries (centered at the aortic bifurcation) Can be more extensive Anteriorly pancreas and duodenum Craniocaudally mediastinum or pelvis Bilateral or unilateral ureteral encasement results in medial deviation and obstruction Enhancement and T2 SI reflects the degree of inflammation Acute phase – avid enhancement, T2 SI Chronic/fibrotic – little to no enhancement, low SI on T1 and T2 DDx: Lymphoma Metastatic disease (e.g. colon, prostate, testicular) Hematoma Postirradiation Imaging cannot reliably distinguish benign from malignant RPF! Benign/idiopathic RPF is classically plaquelike and tethers the aorta and IVC toward the spine, reflecting underlying fibrosis Malignancy tends to be bulkier and lobulated, displacing aorta and IVC anteriorly and ureters laterally Poor sensitivity and specificity of these (and other) features and many exceptions exist Biopsy may be required (often surgical) 9 Management Treat underlying cause and/or remove causative agent Some drug-induced causes also respond to immunosuppresion Glucocorticoids (usually prednisone) – high-dose initially, followed by taper and maintenance therapy If renal impairment, PCN and ureteral stent History: 54-year-old female with increasing abdominal girth over 1 year What is the most likely diagnosis? a) b) c) d) Dermoid Angiomyolipoma Myelolipoma Liposarcoma 10 History: 54-year-old female with increasing abdominal girth over 1 year Findings: Large retroperitoneal mass containing fat, soft tissue nodules, and septations Mass effect upon, but separate from, retroperitoneal structures Coarse Ca++ Most common primary retroperitoneal malignant tumor 80% of all retroperitoneal tumors are malignant (lymphoma most common, followed by sarcoma) >90% of retroperitoneal sarcomas are liposarcoma or leiomyosarcoma 2nd most common adult soft tissue sarcoma (after MFH) Malignant from inception almost never arise from lipomas No fat-containing retroperitoneal lesion should be dismissed as a lipoma retroperitoneal lipomas are exceedingly rare, more likely represents lipomalike area of a well-differentiated liposarcoma Grow slowly and are very large at detection Results in longer time to diagnosis more likely to dedifferentiate 15% of well-differentiated liposarcomas dedifferentiate (median 8 years) 5 histologic types Well-differentiated Myxoid Dedifferentiated Pleomorphic NOS 11 Imaging: Large heterogeneous mass with varying components of fat, fibrous strands, & soft tissue Mixed (most common) – fat (< -20 HU) and non-lipomatous components (soft tissue, fluid) Solid (myxoid tissue) – T2 hyperintensity with enhancement Pseudocystic (-20-20 HU) – mimics fluid collection Displaces retroperitoneal structures Identify kidneys and adrenals separately to distinguish from AML or myelolipoma Regional LN involvement is rare Mets most commonly lung and liver Well-differentiated Predominantly fat and fibrous septations Myxoid Craig WD et al. RadioGraphics. 2009 Jan-Feb;29(1):261-90 Uniform low attenuation with increased SI on T2W Dedifferentiated Discrete nodular soft tissue > 1 cm Discrete soft tissue components are the hallmark of dedifferentiation 12 Craig WD et al. RadioGraphics. 2009 Jan-Feb;29(1):261-90 DDx: AML Large internal vessels, renal notch, hemorrhage History of tuberous sclerosis Myelolipoma Most (90%) arise from adrenal, but extra-adrenal type can mimic sarcoma Hematoma May be secondary to AAA rupture or tumor (e.g. RCC, ACC, AML) Management: Surgical resection Very difficult to obtain clear margins Frequently recurs and becomes more aggressive Recurrence most commonly as soft tissue rather than fat 13 History: 60-year-old male with weight loss and facial flushing Fused CT and OctreoScan In what percentage of patients does carcinoid syndrome occur? a) b) c) d) >90% 50% 30% <10% History: 60-year-old male with weight loss and facial flushing Fused CT and OctreoScan Findings: Spiculated mesenteric mass in the RLQ with encasement of vasculature and tethering of small bowel loops Mesenteric mass and liver lesions have uptake on OcreoScan 14 Uncommon neuroendocrine tumor arising from the endocrine system outside the pancreas and thyroid ~2% of all GI tract tumors Most common malignant neoplasm of the small bowel Most commonly distal ileum Primary tumor is a small (< 3.5 cm) submucosal mass that is usually occult by imaging 40-80% spread to mesentery (directly or via lymphatics) Typically the first or only imaging manifestation (unless there are hepatic metastases) Imaging findings are a result of extramural spread and resultant desmoplastic reaction in the mesenteric fat retraction and tethering Imaging: Enhancing calcified, soft tissue mass with spiculation of the mesenteric fat, tethering of adjacent bowel and vasculature Metastatic disease becomes more common with larger tumors (> 1 cm) Regional LN Liver (usually hypervascular) 5-year survival 79% for <5 lesions vs. 47% for ≥5 Peritoneum Carcinoid syndrome (<10%) Refers to a constellation of symptoms – flushing, diarrhea, wheezing, right heart failure 90% have metastatic disease, most commonly patients with liver metastases tumor products (e.g. histamine, serotonin) released into systemic circulation Majority originate in midgut (jejunum, ileum, cecum), bronchial in 10%, rarely distal colon and rectum 111In pentetreotide helps distinguish from other etiologies and predict response to octreotide therapy (80% have somatostatin receptors) DDx: Desmoid Hematoma Retractile mesenteritis Treated lymphoma {more detail for these? Separate slide?} 15 Management: Localized, non-metastatic resection Extent depends on origin and size Post-treatment surveillance often continues for up to 10 years due to late recurrence Carcinoid syndrome Somatostatin analogs (e.g. ocreotide) – bind to somatostatin receptors and block release of active tumor products Hepatic resection Considered for select patients with localized disease, preserved liver function, and no extrahepatic disease Symptomatic relief in most patients, disease-free survival in up to 20% Liver-directed therapy Bland or chemoembolization, ablation, and 90Y have been shown to improve symptoms in unresectable cases Prophylactic octreotide required to prevent carcinoid crisis History: 29-year-old female status post total proctocolectomy with abd pain Which of the following describes Gardner syndrome? Ectopic gallstone with gastric outlet obstruction RCC, pancreatic cysts, hemangioblastoma, pheochromocytoma c) Lung cysts and RCC d) Colorectal polyps, skull osteomas, desmoid tumors, epidermoid cysts a) b) 16 History: 29-year-old female status post total proctocolectomy with abd pain Findings: Infiltrative mesenteric soft tissue in RLQ Tethering and spiculation of surrounding structures AKA aggressive or deep fibromatosis Fibromatoses – benign, but locally aggressive proliferation of fibrous or connective tissue Superficial – smaller, slow-growing, rarely involve deeper structures Deep – larger, more aggressive, grow rapidly, infiltrate surrounding structures Abdominal wall – classically young women during pregnancy or within 1 year postpartum Intraabdominal – mesenteric, retroperitoneal, and pelvic Can be familial or sporadic Mesenteric desmoids are associated with patients with FAP (Gardner syndrome) 18-20% of patients with Gardner syndrome develop desmoids 75% develop in patients with prior abdominal surgery 17 Variable imaging appearance, frequently non-specific Masslike or ill-defined (and both forms can coexist in same patient) Infiltrative types may encase bowel loops and vasculature Masslike types tend to displace rather than encase Spiculated with radiating strands of soft tissue and retraction of surrounding structures Similar attenuation/SI to muscle, but can become necrotic when large Usually low SI on T1; If myxoid high SI on T2 Imaging primarily used to plan surgical resection Size and number of lesions Local extent – vasculature, bowel, solid organs DDx: Carcinoid Sclerosing mesenteritis Metastasis Lymphoma History: 64-year-old male with LLE numbness, new hemorrhagic brain lesions What is the most likely diagnosis? a) b) c) d) Desmoid Metastatic melanoma Metastatic colorectal cancer GIST 18 History: 64-year-old male with LLE numbness, new hemorrhagic brain lesions Findings: Dominant lobulated sigmoid mass with eccentric wall thickening Multiple round, circumscribed mesenteric masses Numerous tiny subcutaneous nodules Skin cancer arising from melanocytes Most in sun-exposed areas, but can develop elsewhere Mucosal melanoma 1% of all melanoma Primarily head & neck, anorectal, vulvovaginal Worse prognosis than cutaneous primary LN disease starts regionally and progresses contiguously through the lymphatic chain Can metastasize hematogeneously to any organ More common in advanced regional disease Distant metastases can occur even without regional LN spread 60% of metastatic melanoma have abd/pelvis mets Most common sites of metastases: Regional LN (75%) Lung (70%) Subcutaneous/soft tissues (67%) – in transit, between primary and regional LN Liver (58%) – most common visceral site CNS (49-73%) GI tract (40%) Spleen (5%) 19 Small bowel and mesentery are most common site of GI metastases GI tract Can present anywhere in the GI tract, but usually multiple Small bowel more common (75%) than colon (25%) Intramural, serosal, or polypoid intraluminal masses Target appearance with central necrosis or ulceration Aneurysmal SB dilation Eccentric wall thickening Mesentery and LN Mesentery and omentum usually both involved Lobulated, confluent, or infiltrative patterns (overlap with lymphoma, carcinoid, and desmoid) Rarely result in desmoplastic response (contrast to carcinoid) Peritoneal or omental carcinomatosis LN may bleed Abdominal LN in 30% PET/CT (with diagnostic CT) most accurate for staging and assessing for recurrence Sensitivity 98%, specificity 94% Detects unsuspected metastases in 15% Demonstrates blind spots better than CT alone Management Metastatectomy – isolated or limited metastatic disease Immunotherapy (e.g. IL-2, ipilimumab, anti-PD1 monoclonal Abs) Ipilimumab preferred for most patients, produces durable response in 20% IL-2 in selected patients can be curative, but severe associated toxicities Anti-PD1 monoclonal Abs 2nd line after PD with ipilimumab Radiation therapy Mostly for brain metastases Palliative for symptomatic localized disease 20 History: 47-year-old male, fall from 27 feet What is the most likely diagnosis? a) b) c) d) Desmoid Mesenteric hematoma Carcinoid Sclerosing mesenteritis History: 47-year-old male, fall from 27 feet Findings: High attenuation mesenteric fluid collection displacing adjacent bowel loops Active extravasation centrally Pelvic fractures 21 Bowel and mesenteric injuries found in ~5% of laparotomies for blunt trauma CT is the test of choice with sensitivity 69-95% and specificity 94-100% Mesenteric hematoma should prompt search for associated bowel injury Imaging: Ill-defined mesenteric fat stranding May reflect isolated mesenteric injury or bowel injury Highest sensitivity, but nonspecific Hematoma Specific to mesenteric injury, but does not always require surgery Irregularity of mesenteric vasculature Usually indicates underlying vascular injury Extravasation of IV contrast – reportedly 100% specific for significant mesenteric injury Usually indicates need for laparotomy History: 49-year-old dock worker with increased abdominal girth What is the most likely diagnosis? a) b) c) d) Malignant peritoneal mesothelioma Pseudomyxoma peritonei TB peritonitis Mesenteric hemorrhage 22 History: 49-year-old dock worker with increased abdominal girth Findings: Diffuse infiltrative soft tissue throughout the peritoneal cavity Scalloping of the liver surface Small amount of ascites No enlarged LN Rare aggressive neoplasm arising from mesothelial cells lining serosal surface of peritoneal cavity, spreads rapidly throughout the abdomen and pelvis Peritoneum is 2nd most common site of mesothelioma (after pleura), accounting for 10-15% cases annually Most have h/o asbestos exposure, but the link is less strong than for pleural mesothelioma Incidence rises sharply at higher levels of exposure 20-40 year latency 23 Imaging: Most commonly, diffuse infiltrative peritoneal soft tissue, including scalloping of liver or diaphragmatic surface Ascites frequently present (60-100%), but rarely large volume Pleural Ca++ (50%), thickening, and/or effusion Less commonly presents as a dominant mass with peritoneal nodularity Distant metastases are rare (consider alternate diagnosis) DDx: TB peritonitis High attenuation ascites, LN, peritoneal thickening Exposure history Carcinomatosis Cannot be reliably distinguished by imaging Lymphomatosis Usually with LN Pseudomyxoma peritonei Serous carcinoma of the peritoneum Primary arising from ovarian epithelial rests Management No consensus, poor prognosis Median survival historically < 1 year, but up to 5 years at certain centers If good performance status and no extraperitoneal disease cytoreductive surgery + hyperthermic intraoperative peritoneal perfusion with chemotherapy (HIPEC) Systemic chemotherapy for patients who cannot tolerate surgery + HIPEC or have extraperitoneal disease 24 History: 66-year-old female with abdominal bloating and fatigue What is the most likely diagnosis? a) b) c) d) Pseudomyxoma peritonei Malignant peritoneal mesothelioma Simple ascites Perforated appendicitis History: 66-year-old female with abdominal bloating and fatigue Findings: Dilated tubular structure in the RLQ with punctate Ca++ Mucinous implants displacing bowel loops, scalloping liver surface Cystic left ovarian lesion 25 Clinical entity with accumulation of gelatinous ascites and mucinous implants along peritoneal surfaces secondary to mucinous epithelial neoplasia Histologically bland or low-grade mucinous epithelium on the peritoneal surface Most common presenting symptom is increased abdominal girth Terminology is confusing and variable, but the current consensus is that PMP should be used to describe peritoneal spread in association with appendiceal mucinous neoplasm AKA disseminated peritoneal adenomucinosis (DPAM) Allows more uniform pathologic and prognostic classification Appendiceal tumor obstructs lumen ruptures with tumor cells disseminated throughout peritoneal cavity Appendiceal neoplasm itself is histologically lowgrade, but can result in appendiceal rupture and peritoneal dissemination, resulting in a more aggressive clinical scenario ~20% of mucinous tumors of the appendix results in PMP Found incidentally in 2/10,000 laparotomies Disseminated mucin-producing appendiceal adenocarcinoma (peritoneal mucinous carcinomatosis) is an aggressive subset with mucinous epithelium demonstrating features of carcinoma Ovarian mucinous tumors are presumed to be metastatic (present in 44%) Rare metastases to LN or liver parenchyma Imaging findings: Loculated/mucinous ascites accumulates throughout the peritoneal cavity in characteristic locations (corresponding to areas of fluid reabsorption and dependent accumulation) Greater omentum Right subphrenic space Pouch of Douglas Right retrohepatic space Liver and spleen surface Left para-colic gutter Ligament of Treitz Centrally displaced bowel loops Scalloping of liver and spleen surface Mucinous appendiceal +/- ovarian tumors May have curvilinear Ca++ in wall or lumen Left ovary 26 TB Peritonitis Peritoneal carcinomatosis DDx: – Peritoneal carcinomatosis Burrill et al. RadioGraphics 2007 27:5, 1255-1273 – Peritonitis (e.g. TB, bacterial) – Peritoneal mesothelioma Pseudomyxoma peritonei Malignant peritoneal mesothelioma Management Palliative, intended to manage symptoms by reducing accumulation of mucus Repeated surgical debulking Most aggressive approach with curative intent – complete cytoreduction followed by intraperitoneal heated chemotherapy Best results at select expert centers with extensive experience (Sugarbaker) 27