Gastrointestinal Stromal Tumor by Edmund Asis 6-24 - Medical ...

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  • GoodMorning doctors! For today’s grand rounds I will be presenting a very interesting case entitled The great masquerader
  • The aim of today’s grand rounds are as follows:
  • Our case is ……………
  • With chief complaint of…
  • History of present illness started 4 months prior to admission, when he started to experience early satiety. He would already get full even with half servings of food. He also had occasional periumbilical pain described as mild discomfort.
  • Then 2 months prior to admission, he then noted an abdominal mass from the right upper quadrant to epigastrium to the periumbilical area. There were persistence of same symptoms. No consult was done
  • Then 5 days prior to admission, still with persistence of the same symptoms. He noted weight loss of 4 kgs in 2 months time. He then sought consult with the company clinic wherein work –ups were done including an abdominal ultrasound which showed large hepatic mass 18 cm, left lobe. CT scan was suggested.
  • The presence ofHBsAgsignfieshepa B infection. IF HBcIgM positive, it is acute. If IgMHBc is absent it is chronic. HBeAg marker for relative infectivity. The hepatitis profile indicates chronic hepatitis B infection
  • Then 1 day prior to admission, with the persistence of the same symptoms he came for follow up. CT scan of the upper and lower abdomen revealed a large intrahepaticmultinodular periphery enhancing mass lesion occupying almost every segment of an enlarged liver, consistent with primary liver malignancy. Hence he opted to seek second opinion to our institution for further work –ups.
  • The review of systems were unremarkable
  • For the past medical history, he has childhood asthma when he was 6 years oldHe was diagnosed with hepatitis B for 9 years.
  • For the personal/social history, he is a non-smoker, drinks alcoholic beverages occasionally. He is presently working in a petroleum company as a supervisor. He regularly goes to the gym for his regular exercise and is a bodybuilder. He denied intake of illicit drugs/ anabolic steroids.
  • For the family history, his brother and sister have an asthma. Both of his parents are diabetic and hypertensive. No one in the family has a cancer.
  • For the Physical examination, vital signs were stable upon admission. The physical examination was unremarkable except in the examination of the abdomen
  • The only significant finding is in the abdomen which showed……
  • To summarize the case: we are presented….
  • Our primary impression was ………….
  • On the day of admission.\\
  • CBC showed mild anemia
  • Blood chem was normal
  • Including the liver function test
  • We requested for AFP to rule out hepatic malignancy which was normal. CEA was also requested to rule out colonic malignancy which turned out to be normal as well. CA 19-9 for pancreatic CA was also negative.
  • He was referred to Interventional radiologist to possible CT guided liver biopsy
  • Bone scan was requested to rule out any metastasis. It showed no definite evidence of osseous metastasis.Bright areas or Hot spot or increasing bone activity. There is also that appear not so bright or cold spot which is a sign of poor blood flow dectruction of tumor. Normal if it appears fairly uniform distribution of radioisotope.
  • MRI of the abdomen was done to compare it with the previous ct scan done outside.
  • Hemangioma is the most common benign tumor affecting the liver.1 Hepatic hemangiomas are mesenchymal in origin and usually are solitary. Some authorities consider them to be benign congenital hamartomas. Hemangiomas are composed of masses of blood vessels that are atypical or irregular in arrangement and size. Etiology remains unknown
  • Surgical intervemtion is warranted if the palpable mass has a risk for exposure to trauma, rapid growth,
  • MRI of the abdomen was done. He was referred to surgery for left hepatectomyvsangio-embolization prior to liver resection.
  • To shrink the mass
  • There is a nodular mass with a seemingly normal looking mucosa at the lesser curvature of the stomach.
  • Since the CT scan done outside showed poor quality images, a repeat CT scan was done
  • CT scan was again done because its easy to do dynamic studies in CT scan as compared to MRI to differentiate between a cavernous hemangioma and hepatocellular mass. Cavernous hemangioma follows a centripetal enhancement whereas HCC the enhancement just stays at the periphery because the center is already necrotic. Ct scan done outside has poor quality images.A large low attenuating mass seen is seen infiltrating the liver.In axial view, there is large mixed attenuation mass with isoattenuation on the periphery that could be originating from the liver.
  • In the coronal view, we can clearly see the mass.
  • There is a mass that is compressing the liver but the border of both the liver and the stomach is clearly delineated. There is no evident invasion by tumor of surrounding tissue or organ
  • 22 x 18 x 15 cm mass abutting the transverse colon, attached to the lesser curvature of the stomachMultiple firm palpable nodes noted along the greather curvature of the stomach.No liver mass noted
  • I would like to present to you a published journal entitled Diagnostic significance of ultrasonography and CT for large upper abdominal mass wherein 43 cases that were pathologically confirmed were analyzed retrospectively. In this study, it doesn’t only include the liver but also the adrenal gland and the kidney that were misdiagnosed.
  • The study concluded that due to the close proximity of upper abdomen organs with each other, correct imaging localization is not easy especially when the organs are compressed or tumor is contiguous with other organs.They also suggested to increase the rate of accurate diagnosis of large abdominal mass, close integration of US and CT and full examination.
  • I would like to show you the recommendation of the american college of radiology in dealing with abdominal mass. This showed to us that the preferred modality is ct scan with or without IV contrast is preferred followed by US then MRI. The other work ups depends on the etiology that you are considering. Investigators found out that US and CT scan are excellent for affirming or excluding a clinically significant abdominal mass with sensitivity and specificity values exceeding 95%.
  • The investigators also determined the success rate in determined…At the time of this recommendation there is no mention in the usefulness of MRI in excluding palpable masses. It is likely comparable to US and CT.
  • What are GIST? Account for < 1% of all GI tumors
  • Before the year 2000, the number of new GIST cases were underestimated and under reported. Many GIST are historically misdiagnosed as either smooth muscle tumor or the neurogenic tumor. But with the advent of molecular definition of GIST, true incidence rises.
  • The metastatic pattern is predominantly intra-abdominal, with spread throughout the peritoneal cavity and to the liver. Lymph nodal invasion is uncommon.
  • As mentioned earlier, CD34 expression is neither sensitive nor specific for GIST because this antigen can also be noted in desmoid tumor and approximately 30% to 40% of GIST lesions are negative for CD34. Kit protein is a protein found in the surface of normal cells. It signals the cells when to proliferate and divide.
  • As with other types of cancer, GIST also has TNM staging based from american joint committee on cancer.
  • Correlate with the patient
  • In the diagnosis of GIST, several diagnostic modalities are available depending on the site of the lesion involved.
  • MRI can be useful in the assessment of liver metastases because some GIST lesions can be fully isodense to normal tissues and thus invisible against surrounding hepatic parenchyma on CT scans.
  • Therefore, many surgeons recommend that preoperative biopsy not be performed if resection is planned in order to minimize risk to the patient. However biopsymust be performed in cases of unresectable GIST in order to mke the diagnosis and justify preoperative administration of imatinib therapy.
  • Efforts of medical oncologists to treat GISTs with conventional cytotoxic chemotherapy were universally futule
  • Complete surgical resection remains the mainstay of treatment
  • As you can see in this graph, there is 54% 5 year survivial rate but it goes down to 35% if the mass is more than 10 cm.
  • The likelihood of GIST recurrence after surgical resection is high.1-4Among patients who undergo complete resection, the likelihood of recurrence of GIST within the abdomen is high.1,3The median time to recurrence following resection of primary GIST is about 2 years, and the probability of recurrence after re-excision approaches is about 90%.4,5 Only two thirds of tumors can be completely resected at presentation.2,3Disease-free survival rate was 80% at 1 year, 67% at 2 years, and 45% at 5-year follow-up 2,4,5Overall, the 5-year survival rate for high-risk patients with GIST varies widely and has been reported to be between 30% and 80%.2,4,5Investigational protocols are under way, with the goal of reducing the rate of recurrence after resection
  • In the management of GIST, a medication was developed initially given for treatment of chronic myelogenous leukemia, which showed a dramatic activity at inhibiting the KIT enzymatic action.With the advent of the molecular targeted therapy
  • The imaging modality are just there to guide us, still a throurough history and pe remains an indispensable tool.
  • I just selected a few schematic diagrams which is applicable to our patient
  • Magnetic resonance image (MRI) of a hemangioma. The lesion appears as a hypointense mass on T1-weighted MRIs (T1WI) and as a hyperintense mass on dual-echo T2-weighted MRIs (T2WI). Note that the signal intensity of the lesion is similar to that of the adjacent cerebrospinal fluid
  • Contrast-enhanced computed tomography (CT) scan. These images reveal the pathognomonic features of a hemangioma, namely, peripheral nodular enhancement and progressive centripetal fill-in (arrow). The smaller, peripheral lesion (circled) shows homogeneous enhancement.
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