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A Year Old Male Presenting with Fever and Malaise

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A Year Old Male Presenting with Fever and Malaise Powered By Docstoc
					   Metastatic GIST: Correlating
Disparate Radiologic Findings on CT

          Joe McQuaid, HMS III
          Gillian Lieberman, MD




              November, 2007
Our Patient: CH

  Clinical Presentation
          History of Present Illness
• 68 year-old male
• One week history – malaise, chills, low-grade temps
• Four days PTA – high grade temperatures to 103.0 °F
• Morning of admission – three to four BM’s that were
  loose and explosive but not tarry, sticky, or bloody
• ROS upon admission – denies chest pain, cough,
  shortness of breath, sputum, dysuria, hematuria,
  hematochezia.
• No recent travel history
           Past Medical History
• Hypertension
• Hypercholesterolemia
• “Benign esophageal growth” with previous
  history of bleeds
• Prostate cancer with surgical resection
• No previous imaging studies on file
                    Physical Findings
• Vital signs upon admission:
   – Tc=100.6 HR=72 BP=98/68 O2=97% on RA
• Physical examination notable for:
   – General: NAD. Mucus membranes clear.
   – Pulmonary: CTA bilaterally. No wheezes, ronchi, rales.
   – Cardiac: Normal S1 and S2. No extra heart sounds.
   – Abdomen: Positive BS. Soft. NTND. No abnormal masses or
     organomegaly noted.
   – Lymphatics: No cervical, supraclavicular, axillary, inguinal
     lymphadenopathy.
• Laboratory Studies:
   – WBC=17.1 RBC=3.64 Hgb=10.1 Hct=31.2
   – Plt Ct=418
Our Patient: Chest CT

“Please evaluate for infectious etiologies”
Our Patient: Chest CT with Contrast
   11 mm solid nodular density                Left upper lobe apex




            Irregular, “spiculated” margins



  BIDMC, PACS
Our Patient: Chest CT with Contrast
                                12x6 mm solid, nodular density
                                with irregular, spiculated margins




                Adjacent to the pleura
  BIDMC, PACS
Chest CT: Differential Diagnoses
     Type of Cause                     Disease Entity
Neoplasm                Metastases
                        BAC
                        Lymphoma
Infectious           X TB
                     X Fungal (Histoplasmosis, Aspergillus)
                       Bacterial (Staph, Klebsiella, Strep)
Noninfectious          Sarcoid
                     X Rheumatoid
                     X Silicosis
                       Wegener’s
                       Histocytosis
                       Necrotizing granulomatous vasculitis
Other                X Drug Toxicity

                                                  X = Eliminated
Our Patient: Abdominal CT

  “Please evaluate for infectious etiologies”
    Our Patient: Abdominal CT with Contrast
8.0 x 9.6 x 8.0 cm (AP, transverse,   multiple loculations…
craniocaudal) heterogeneous lesion,
hypoenhancing compared to the liver                           …and septations
parenchyma




                                                        BIDMC, PACS
Our Patient: Abdominal CT with Contrast




         BIDMC, PACS

  Multiple hypoattenuating satellite lesions
Abdominal CT: Differential Diagnoses
  Type of Cause               Disease Entity

Congenital Cystic   Simple cysts
Lesions             ADPLD
                    Bile duct harmartomas
                    Caroli’s disease


Infective Cystic    Pyogenic liver abscess
Lesions             Amebic liver abcess
                    Hydatid cysts


Neoplastic Cystic   Cystic liver metastases
Lesions             Cystic HCC
                    Billiary cystadenoma                 BIDMC, PACS
                    Undifferentiated embryonal sarcoma
      Companion Patients

Some common radiologic findings of congenital cystic
                  lesions…
                         Companion Patient #1:
                    Simple Hepatic Cysts on CT


                                              • Simple Hepatic Cysts
                                                 – Develop from
                                                   harmartomatous tissue
                                                 – Hypoattenuating lesion on
                                                   nonenhanced CT scans
                                                 – No enhancement of wall or
                                                   contents with contrast
                                                 – Round or ovoid, well
                                                   defined, with a thin wall



Mortele KJ et al. Radiographics 2001; 21(4)
                        Companion Patient #2:
                          ADPLD on CT


                                              • Autosomal Dominant Polycystic
                                                Liver Disease
                                                 – Often asymptomatic
                                                 – Coexists with renal cysts
                                                 – Multiple homogenous,
                                                    hypoattenuating lesions
                                                 – Regular outline
                                                 – No wall enhancement with
                                                    contrast

Mortele KJ et al. Radiographics 2001; 21(4)
                   Companion Patient #3:
               Bile Duct Harmartomas on CT

                                              • Bile Duct Harmartomas
                                                 – Scattered throughout biliary
                                                    tree
                                                 – 0.5-1.5 cm in diameter
                                                 – More irregular outline
                                                 – Do not exhibit a
                                                    characteristic pattern of
                                                    enhancement with contrast
                                                    on CT
Mortele KJ et al. Radiographics 2001; 21(4)
                       Companion Patient #4:
                        Caroli Disease on CT

                                              • Caroli Disease
                                                 – Right upper quadrant pain,
                                                   fever, jaundince
                                                 – Hypoattenuated dilater cystic
                                                   structures
                                                 – Various sizes
                                                 – Communicate with bile duct
                                                   system
                                                 – “Central dot sign” –
                                                   enhancement within dilated
                                                   bile duct on CT
Mortele KJ et al. Radiographics 2001; 21(4)
Abdominal CT: Differential Diagnoses
  Type of Cause                   Disease Entity

Congenital Cystic   X   Simple cysts
Lesions             X   ADPLD
                    X   Bile duct harmartomas
                    X   Caroli’s disease

Infective Cystic        Pyogenic liver abscess
Lesions                 Amebic liver abcess
                        Hydatid cysts


Neoplastic Cystic       Cystic liver metastases
Lesions                 Cystic HCC
                        Billiary cystadenoma                             BIDMC, PACS
                        Undifferentiated embryonal sarcoma



                                                             X = Eliminated
      Companion Patients

Some common radiologic findings of infective cystic
                 lesions…
                       Companion Patient #5:
                       Pyogenic Abscess on CT

                                              • Pyogenic Abscess
                                                 – Often infection by e coli or
                                                   clostridium
                                                 – Thick-walled
                                                 – Low attenuation on CT
                                                 – Rim enhancement with
                                                   contrast
                                                 – Air pockets are diagnostic of
                                                   a gas-forming organism


Mortele KJ et al. Radiographics 2001; 21(4)
                   Companion Patient #6:
                 Amebic Liver Abscess on CT

                                              • Amebic Liver Abscess
                                                 – Caused by entamoeba
                                                   histolytica
                                                 – In addition to above features
                                                   show perilesion edema
                                                 – Peripheral rim enhancement
                                                   or “double target sign”




Mortele KJ et al. Radiographics 2001; 21(4)
                 Companion Patient #7:
            Intrahepatic Hydatid Cyst on CT

                                              •   Intrahepatic Hydatid Cysts
                                                   – Endemic to Mediterranean and
                                                       sheep-raising contries
                                                   – Contact with dog feces or
                                                       contaminated food
                                                   – On CT appears as
                                                       hypoattenuating lesion with a
                                                       distinguishable wall
                                                   – Coarse calcifications of wall in
                                                       50% of cases
                                                   – Daughter cysts nearby in 75% of
Mortele KJ et al. Radiographics 2001; 21(4)            patients
Abdominal CT: Differential Diagnoses
  Type of Cause                   Disease Entity

Congenital Cystic   X   Simple cysts
Lesions             X   ADPLD
                    X   Bile duct harmartomas
                    X   Caroli’s disease


Infective Cystic        Pyogenic liver abscess
Lesions                 Amebic liver abcess
                    X   Hydatid cysts


Neoplastic Cystic       Cystic liver metastases
Lesions                 Cystic HCC
                        Billiary cystadenoma                           BIDMC, PACS
                        Undifferentiated embryonal sarcoma


                                                             X = Eliminated
      Companion Patients

Some common radiologic findings of neoplastic cystic
                  lesions…
                        Companion Patient #8:
                        Cytic Liver Mets on CT

                                              • Cystic Liver Mets
                                                 – Most are solid but may have
                                                    partial cyst-like appearance
                                                 – Mechanisms of seeding:
                                                    hematogenous or peritoneal
                                                 – Often with enhancement of
                                                    peripheral viable irregular
                                                    tissue

Mortele KJ et al. Radiographics 2001; 21(4)
                        Companion Patient #9:
                         Cystic HCC on CT

                                              • Cystic HCC
                                                 – 70% of patients will also
                                                    show signs or complications
                                                    of liver cirrhosis
                                                 – Often with a capsule or
                                                    hypervascular solid parts
                                                 – May show vascular or biliary
                                                    invasion

Mortele KJ et al. Radiographics 2001; 21(4)
                     Companion Patient #10:
                    Biliary Cystadenoma on CT

                                              • Biliary Cystadenoma
                                                 – Large range in size (1.5cm to
                                                     35 cm)
                                                 – Multilocular slow growing
                                                     lesions
                                                 – 55% in the right liver lobe
                                                 – On CT appears a solitary,
                                                     well-defined cystic mass with
                                                     a capsule and internal septa
Mortele KJ et al. Radiographics 2001; 21(4)
         Companion Patient #11:
Undifferentiated Embryonal Sarcoma on CT

                                              • Undifferentiaed Embryonal
                                                Sarcoma
                                                 – Predominates in children or
                                                    young adults
                                                 – Pseudocapsule
                                                 – Heterogeneous enhancement
                                                    of the peripheral borders of
                                                    the mass
                                                 – Usually very large (>10 cm)
Mortele KJ et al. Radiographics 2001; 21(4)
Abdominal CT: Differential Diagnoses
  Type of Cause                   Disease Entity

Congenital Cystic   X   Simple cysts
Lesions             X   ADPLD
                    X   Bile duct harmartomas
                    X   Caroli’s disease


Infective Cystic        Pyogenic liver abscess
Lesions                 Amebic liver abcess
                    X   Hydatid cysts


Neoplastic Cystic       Cystic liver metastases
Lesions             X   Cystic HCC
                                                                 BIDMC, PACS
                        Billiary cystadenoma
                        Undifferentiated embryonal
                        sarcoma

                                                     X = Eliminated
Our Patient: Pelvic CT

“Please evaluate for infectious etiologies”
         Our Patient:
Coronal Pelvic CT with Contrast




             BIDMC, PACS
                 Our Patient:
        Coronal Pelvic CT with Contrast




                            BIDMC, PACS


• Within the LLQ and centered in the mesenteric fat
• 4.4 x 5.4 x 5.5 cm (AP, transverse, and craniocaudal)
• Lesion with thick, irregular, enhancing rim with a low density
  center. Well-defined borders.
• Supply by several feeding vessels
                    Our Patient:
         Sagittal Pelvic CT with Contrast




                        Courtesy of Dr. Mortiz Kircher

• No pathologically enlarged lymph nodes in pelvis
• No free fluid
• Mass is inseparable from the jejunum?
                          Our Patient:
               Sagittal Pelvic CT with Contrast




CT images: courtesy of Dr. Moritz Kircher
Small bowel cartoon: http://concise.britannica.com/ebc/art-53188/The-walls-of-the-hollow-organs-of-the-digestive-tract
Pelvic CT: Differential Diagnoses

              Disease Entity

     Adenocarcinoma of the small bowel

                 Lymphoma

   Mesenteric fibromatosis (desmoid tumor)

         Inflammatory pseudotumor

           Sclerosing mesenteritis

                 Carcinoid


                   GIST

                                             Courtesy of Dr. Mortiz Kircher
     Companion Patients

Let’s consider some common radiologic findings of
             these disease entities…
        Companion Patient #12:
Adenocarcinoma of the Small Bowel on CT

                                              • Adenocarcinoma of the small
                                                bowel
                                                 – 50% are in the duodenum
                                                 – On CT manifests as:
                                                     • annular narrowing with
                                                       abrupt edges
                                                     • a discrete tumor mass
                                                       (papillary or polypoid
                                                       often)
Buckley JA et al. Radiographics 1998; 18(2)          • an ulceration
                      Companion Patient #13:
                        Lymphoma on CT

                                              •   Lymphoma
                                                   – Primary tumor mass centered on
                                                     small bowel with appropriate
                                                     lymph node involvement
                                                   – Absence of hepatic and splenic
                                                     lesions
                                                   – Presents on CT:
                                                       • Discrete polyp within the
                                                          bowel
Buckley JA et al. Radiographics 1998; 18(2)
                                                       • Nodular filling defect within
                                                          the bowel
                                                       • Exocentric mass extending
                                                          to adjacent tissue
             Companion Patient #14:
           Mesenteric Fibromatosis on CT

                                        •   Mesenteric Fibromatosis
                                             – Present with abdominal mass or
                                               abdominal pain
                                             – Looks very similar to GIST
                                               histologically and radiologically
                                             – May present as discrete mass or
                                               infiltrate small bowel
                                             – CT findings vary from
                                               homogenous soft-tissue density
Levy et al. Radiographics 2006; 26(1)          to masses with hypoattenuation
                                               (based on histologic
                                               composition)
                                             – Typically no contrast
                                               enhancement
           Companion Patient #15:
      Inflammatory Pseudotumor on CT

                                        •   Inflammatory Pseudotumor
                                             – Often occuring in young adults
                                             – Presenting symptoms of malaise,
                                                 weight loss, or fever
                                             – Nonencapsulated, nodular mass
                                             – CT findings are nonspecific:
                                                  • Heterogeneous attenuation
                                                  • Often with well-defined
Levy et al. Radiographics 2006; 26(1)
                                                     margins
                                                  • Variable enhancement
                                                     pattern
                                                  • Rarely can involve small
                                                     bowel
                Companion Patient #16:
             Sclerosing Mesenteritis on CT

                                        •   Sclerosing Mesenteritis
                                             – Tumor-like masses in the
                                                 mesentery composed of fibrosis
                                                 and chronic inflammation
                                             – Retracts and shortens the
                                                 mesentery
                                             – On CT:
                                                   • Well-defined or ill-defined
                                                     (variable)
Levy et al. Radiographics 2006; 26(1)
                                                   • Mixed fat and soft-tissue
                                                     density
                                                   • Many with radiating strands
                                                     of fibrosis
Companion Patient #17:
     Carcinoid




     Levy et al. Radiographics 2006; 26(1)
Pelvic CT: Differential Diagnoses

              Disease Entity

     Adenocarcinoma of the small bowel

                 Lymphoma

   Mesenteric fibromatosis (desmoid tumor)

         Inflammatory pseudotumor

           Sclerosing mesenteritis

                 Carcinoid


                   GIST



                                             Courtesy of Dr. Mortiz Kircher
    Putting it all together…
 An Ockham’s Razor Differential

Can we find a way to relate these disparate radiologic
                     findings?
Our Ockham’s Razor Differentials


                       Disease Entity

                        Lymphoma

                         Desmoid

                         Carcinoid

                           GIST




                         Courtesy of Dr. Mortiz Kircher
Our patient underwent a right hepatic
lobectomy and a partial small bowel
              resection
        Our Patient: Surgical Pathology
• Right liver lobe
   – Metastatic gastrointestinal
     stromal tumor
   – Multiple organizing abscesses
• Small bowel
   – Malignant gastronintestinal
                                       Above: Multiple mitotic centers
     stromal tumor                     Below: C-KIT positive staining
       • Invasion through muscle to
         mucosa
       • Rare mitotic figures
       • Necrosis with blood clot
   – C-KIT positive

                                      Levy et al. Radiographics 2003; 23(2)
What is Gastrointestinal Stromal
        Tumor (GIST)?
            GIST: An Overview
• Most common GI mesenchymal neoplasm
• Frequency between 10-20 cases per million
• Most inidividuals over 50 years old at
  presentation
• Slight male predilection
• Neuro-fibromatosis type I with increased
  prevalance
• 20% are malignant
               GIST: An Overview
• Occurs through GI tract,
  mesentery, omentum, and
                                    Anatomic Location
  retroperitoneum
• Defined by the expression             Stomach (70%)

  of KIT (CD117) – a tyrosine       Small Intestine (20-30%)
  kinase growth factor
  receptor                             Anorectum (7%)

• Usually arises in muscularis   Colon and Esophagus (Small %)
  propria of stomach or
  intestinal wall
   – Often presents with an
     exophytic growth pattern
         GIST: Menu of Radiologic Tests
•   CT
     – excellent for visualizing the anatomy as seen in this case study
•   MR
     – Can be used to visualize necrosis and hemorrhage (but these factors also affect
       the signal-intensity pattern)
     – In general, a useful adjunct to CT as multiplanar capability can help to
       determine the organ of origin in large tumors and its relation to blood vessels
•   US
     – Excellent for biopsy
     – Often used intraoperatively
• Abdominal Radiography
     – Commonly used in patients presenting with signs of small intestinal
       obstruction from GIST
     – Reveals small intestinal dilation or soft-tissue masses
            GIST: Radiologic Features
• Generally characterized by well-
  circumscribed enhancing masses with central
  areas of low attenuation (hemorrhage,
  necrosis, or cyst formation)
• Small intestine
   – Barium studies demonstrate intraluminal and
     submucosal masses with sharp margins
   – CT reveal intramural or intraluminal polyps.
   – 22% with extraserosal locations such that small
     bowel not readily evident as origin on CT.
   – Often enhancing masses with centers of low        BIDMC, PACS
     attenuation
        GIST: Radiologic Features




                           BIDMC, PACS
• Stomach
  – 75% occur in body of stomach
  – On barium swallow have a smooth mucosal surface with features of
    a submucosal mass
  – On CT usually show an intramural component. Meanwhile 86%
    show extragastric extension with peripheral enhancement.
                           Summary
In this presentation we…
• Generated a long list of differential diagnosis based upon the
   various CT findings of metastatic GIST
• Used our knowledge of the anatomy of the gut wall to
   recognize a mesenteric mass as extending from the small
   intestine
• Further paired down our differential diagnoses by considering
   and relating our radiologic findings together as a whole
• Reviewed the features of GIST, including the menu of
   radiologic tests used in its diagnosis and the its typical imaging
   patterns
                                References
Buckley JA and EK Fishman. CT Evaluation of Small Bowel Neoplasms: Spectrum of
   Disease. 1998; 18:379-392.
Burkill GJC et al. Malignant Gastrointestinal Stromal Tumor: Distribution, Imaging
   Features, and Pattern of Metastatic Spread. Radiology. 2003; 226(2):527-532.
Levy AD et al. From the Archives of the AFIP - Gastrointestinal Stromal Tumors:
   Radiologic Features with Pathologic Correlation. Radiographics. 2003; 23(2):283-304.
Levy AD et al. From the Archives of the AFIP – Benign Fibrous Tumors and Tumorlike
   Lesions of the Mesentery: Radiologic-Pathologic Correlation. Radiographics. 2006;
   26:245-264
Mortele KJ et al. Cystic Focal Liver Lesions in the Adult: Differential CT and MR
   imaging findings. Radiographics. 2001; 21(4):895-910.
O’Sullivan PJ et al. The Imaging Features of Gastrointestingal Stromal Tumours.
   European Journal of Radiology. 2006; 60:431-438.
Sandrasegaran K et al. Gastrointestinal Stromal Tumors: CT and MRI Findings.
   European Radiology. 2005; 15:1407-1414.
           Acknowledgements
My sincerest thanks goes to Dr. Moritz Kircher,
 who provided this case and helped to guide me
                   through it.

            Many thanks also to:
           Gillian Lieberman, MD
           Jonathan Kruskal, MD
            Andrew Bennett, MD
              Maria Levantakis

				
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