ARIB FARRAS WAHDANCase Presentation

I. IdentityName: NAAge: 13 years oldGender: WomenTribe: JaveneseOccupation: -Address: KedawungIn hospital since: Mei 19th 2015II. Anamnesis- Main Grievance: Lump in abdomen since 2 months- Additional Grievance: Pain in abdomen, nausea

III. Historical of Present Disease

A girl with her mother came to the Arjawinangun hospital because she felt unwell in her stomach. There is a lump in the abdomen since 2 months ago. The patient also feel the pain at abdomen. The patient feels nauseous. The stool was black indicated that there was blood in her stool. There was palpable mass in her abdomen. And She had not historical of hipertension.

IV. Historical of Past Disease

Hipertension (-)

V. Historical of Family Disease

Hipertension (-)

VI. Physical Examination

General Status

Present StatusGeneral Condition: ModerateAwareness: ComposmantisBlood Pressure: 110/70Pulse: 84 x/minuteBreathing: 20 x/minuteTemperature: 37,5 C

HeadForm : Normal, SimetricalHair: Black Colour, No hair fallEye : Anemic Conjungtival -/-, Icteric Schlera -/-, Light Refleks (+), Isocor pupil right = leftEar: Normal form, cerumen (-), tympani membrane intacNose: Normal form, No septum deviation, epitaction -/-Mouth: Normal

NeckEnlargement lymph nodes (-)Trachea in the middleNo mass

ThoraksLungs - pulmonaryInspection : The chest shape is symmetrical both of left and rightPalpation : Fremitus tactile and vocal symmetrical right and left, crepitus (-), tenderness (-), rebound tenderness (-)Percussion : Sound of resonant in both lung fieldsAuscultation: Sound of vesicular and bronchial the entire lung field, ronkhi -/-, wheezing -/-

AbdomenInspection : Lump in abdomenPalpation : Tenderness (-), rebound tenderness (-)Percussion : There was a deaf/somber in abdomenAuscultation : Bowel (+)

ExtremityUpper: Muscle Tone: normal Movement: aktif / aktif Mass: - / - Strenght: 5/5 Edema: - / -Lower: Muscle Tone: normal Movement: aktif / aktif Mass: - / - Strenght: 5/5 Edema: - / -Genitalia : No abnormalities

Laboratory ExaminationRoutine BloodLeukocytes : 7790/mm3Hb : 8,8 gr/dL Platelets : 253.000/mm3 Hematocrite : 25,1 %

DiagnosisGIST

Differential Diagnosis Two main differential diagnosis:1. True gastric leimyoma or leimyosarkoma2. Schwannoma General differential diagnostic features of GIST reactive antibodies: CD117 stains very few other spindled lesions but stains many carcinomas and melanomas CD34 stains many spindled lesions but stains almost no carcinomas or melanomas DOG1 stains very few spindled lesions or melanomas or carcinomas Spindled, bland GIST DDx Leiomyoma Schwannoma Fibromatosis Sclerosing mesenteritis Inflammatory fibroid polyp Gastric plexiform fibromyxoma Solitary fibrous tumor Inflammatory myofibroblastic tumor Endometrial stromal sarcoma Calcifying fibrous pseudotumor Spindled, malignant GIST DDx Leiomyosarcoma Malignant fibrous histiocytoma Dedifferentiated liposarcoma Epithelioid GIST Poorly differentiated carcinoma Melanoma/clear cell sarcoma Glomus tumor Gangliocytic paraganglioma GI endocrine carcinoma Extramedullary myeloid tumor GI mucosal benign epithelioid nerve sheath tumoManagementOperative: Laparotomy, Omentectomy, Drainase

PrognosisQuo ad vitam: Ad bonamQuo ad fungsionam: Dubia ad bonamQuo ad sanationam: Ad BonamVII. Literature Review

The Gastrointestinal System

The gastrointestinal (GI) system (or digestive system) processes food for energy and rids the body of solid waste. After food is chewed and swallowed, it enters the esophagus, a tube that carries food through the neck and chest to the stomach. The esophagus joins the stomach just beneath the diaphragm (the thin band of muscle below the lungs).

The stomach is a sac-like organ that holds food and helps the digestive process by secreting gastric juice. The food and gastric juices are mixed into a thick fluid calledchymethat is then emptied into the small intestine. The small intestine continues breaking down the food and absorbs most of the nutrients into the bloodstream. This is the longest section of the GI tract, measuring more than 20 feet.The small intestine joins the large intestine, the first part of which is the colon, a muscular tube about 5 feet long. The colon absorbs water and mineral nutrients from the remaining food matter. The waste left after this process goes into the rectum as stool (feces), where it is stored until it passes out of the body through the anus.

Anatomy

Stomach The stomach a thick walled organ that lies between the esophagus and the first part of the small intestine (the duodenum). It is on the left side of the abdominal cavity; the fundus of the stomach lying against the diaphragm. Lying beneath the stomach is the pancreas. The greater omentum hangs from the greater curvature. A mucous membrane lines the stomach which contains glands (with chief cells) that secrete gastric juices, up to three quarts of this digestive fluid is produced daily. The gastric glands begin secreting before food enters the stomach due to the parasympathetic impulses of the vagus nerve, making the stomach also a storage vat for that acid. The secretion of gastric juices occurs in three phases: cephalic, gastric, and intestinal. The cephalic phase is activated by the smell and taste of food and swallowing. The gastric phase is activated by the chemical effects of food and the distension of the stomach. The intestinal phase blocks the effect of the cephalic and gastric phases.

Gastric juice also contains an enzyme named pepsin, which digests proteins, hydrochloric acid and mucus. Hydrochloric acid causes the stomach to maintain a pH of about 2, which helps kill off bacteria that comes into the digestive system via food. The gastric juice is highly acidic with a pH of 1-3. It may cause or compound damage to the stomach wall or its layer of mucus, causing a peptic ulcer. On the inside of the stomach there are folds of skin call the gastric rugae. Gastric rugae make the stomach very extendable, especially after a very big meal. The stomach is divided into four sections, each of which has different cells and functions. The sections are: 1) Cardiac region, where the contents of the esophagus empty into the stomach, 2) Fundus, formed by the upper curvature of the organ, 3) Body, the main central region, and 4) Pylorus or atrium, the lower section of the organ that facilitates emptying the contents into the small intestine. Two smooth muscle valves, or sphincters, keep the contents of the stomach contained. They are the: 1) Cardiac or esophageal sphincter, dividing the tract above, and 2) Pyloric sphincter, dividing the stomach from the small intestine. After receiving the bolus(chewed food) the process of peristalsis is started; mixed and churned with gastric juices the bolus is transformed into a semi-liquid substance called chyme.

Stomach muscles mix up the food with enzymes and acids to make smaller digestible pieces. The pyloric sphincter, a walnut shaped muscular tube at the stomach outlet, keeps chyme in the stomach until it reaches the right consistency to pass into the small intestine. The food leaves the stomach in small squirts rather than all at once. Water, alcohol, salt, and simple sugars can be absorbed directly through the stomach wall. However, most substances in our food need a little more digestion and must travel into the intestines before they can be absorbed. When the stomach is empty it is about the size of one fifth of a cup of fluid. When stretched and expanded, it can hold up to eight cups of food after a big meal.

Gastric Glands There are many different gastric glands and they secret many different chemicals. Parietal cells secrete hydrochloric acid; chief cells secrete pepsinogen; goblet cells secrete mucus; argentaffin cells secrete serotonin and histamine; and G cells secrete the hormone gastrin.

Vessels and nerves

Arteries: The arteries supplying the stomach are the left gastric, the right gastric and right gastroepiploic branches of the hepatic, and the left gastroepiploic and short gastric branches of the lineal. They supply the muscular coat, ramify in the submucous coat, and are finally distributed to the mucous membrane.

Capillaries: The arteries break up at the base of the gastric tubules into a plexus of fine capillaries, which run upward between the tubules, anatomizing with each other, and ending in a plexus of larger capillaries, which surround the mouths of the tubes, and also form hexagonal meshes around the ducts.

Veins: From these the veins arise, and pursue a straight course downward, between the tubules, to the submucous tissue; they end either in the lineal and superior mesenteric veins, or directly in the portal vein.

Lymphatics: The lymphatics are numerous: They consist of a superficial and a deep set, and pass to the lymph glands found along the two curvatures of the organ. Nerves: The nerves are the terminal branches of the right and left urethra and other parts, the former being distributed upon the back, and the latter upon the front part of the organ. A great number of branches from the celiac plexus of the sympathetic are also distributed to it. Nerve plexuses are found in the submucous coat and between the layers of the muscular coat as in the intestine. From these plexuses fibrils are distributed to the muscular tissue and the mucous membrane.

Small Intestine The small intestine is the site where most of the chemical and mechanical digestion is carried out. Tiny projections called villi line the small intestine which absorbs digested food into the capillaries. Most of the food absorption takes place in the jejunum and the ileum. The functions of a small intestine is, the digestion of proteins into peptides and amino acids principally occurs in the stomach but some also occurs in the small intestine. Peptides are degraded into amino acids; lipids (fats) are degraded into fatty acids and glycerol; and carbohydrates are degraded into simple sugars. The three main sections of the small intestine is The Duodenum, The Jejunum, The Ileum.

The Duodenum In anatomy of the digestive system, the duodenum is a hollow jointed tube connecting the stomach to the jejunum. It is the first and shortest part of the small intestine. It begins with the duodenal bulb and ends at the ligament of Treitz. The duodenum is almost entirely retro peritoneal. The duodenum is also where the bile and pancreatic juices enter the intestine.

The Jejunum The Jejunum is a part of the small bowel, located between the distal end of duodenum and the proximal part of ileum. The jejunum and the ileum are suspended by an extensive mesentery giving the bowel great mobility within the abdomen. The inner surface of the jejunum, its mucous membrane, is covered in projections called villi, which increase the surface area of tissue available to absorb nutrients from the gut contents. It is different from the ileum due to fewer goblet cells and generally lacks Preyer's patches.

The Ileum Its function is to absorb vitamin B12 and bile salts. The wall itself is made up of folds, each of which has many tiny finger-like projections known as villi, on its surface. In turn, the epithelial cells which line these villi possess even larger numbers of micro villi. The cells that line the ileum contain the protease and carbohydrate enzymes responsible for the final stages of protein and carbohydrate digestion. These enzymes are present in the cytoplasm of the epithelial cells. The villi contain large numbers of capillaries which take the amino acids and glucose produced by digestion to the hepatic portal vein and the liver. The terminal ileum continues to absorb bile salts, and is also crucial in the absorption of fat-soluble vitamins (Vitamin A, D, E and K). For fat-soluble vitamin absorption to occur, bile acids must be present.

Large Intestine The large intestine (colon) extends from the end of the ileum to the anus. It is about 5 feet long, being one-fifth of the whole extent of the intestinal canal. It's caliber is largest at the commencement at the cecum, and gradually diminishes as far as the rectum, where there is a dilatation of considerable size just above the anal canal. It differs from the small intestine in by the greater caliber, more fixed position, sacculated form, and in possessing certain appendages to its external coat, the appendices epiploic. Further, its longitudinal muscular fibers do not form a continuous layer around the gut, but are arranged in three longitudinal bands or tni.

The large intestine is divided into the cecum, colon, rectum, and anal canal. In its course, describes an arch which surrounds the convolutions of the small intestine. It commences in the right iliac region, in a dilated part, the cecum. It ascends through the right lumbar and hypochondriac regions to the under surface of the liver; here it takes a bend, the right colic flexure, to the left and passes transversely across the abdomen on the confines of the epigastric and umbilical regions, to the left hypochondriac region; it then bends again, the left colic flexure, and descends through the left lumbar and iliac regions to the pelvis, where it forms a bend called the sigmoid flexure; from this it is continued along the posterior wall of the pelvis to the anus.

There are trillions of bacteria, yeasts, and parasites living in our intestines, mostly in the colon. Over 400 species of organisms live in the colon. Most of these are very helpful to our health, while the minority are harmful. Helpful organisms synthesize vitamins, like B12, biotin, and vitamin K. They breakdown toxins and stop proliferation of harmful organisms. They stimulate the immune system and produce short chain fatty acids (SCFAs) that are required for the health of colon cells and help prevent colon cancer. There are many beneficial bacteria but some of the most common and important are Lactobacillus Acidophilus and various species of Bifidobacterium. These are available as "probiotics" from many sources.

Epidemiology

GISTs represent the most common mesenchymal neoplasms of the GIT. With an annual incidence of 11-14 per 106 , they form 0.1%-3.0% of gastrointestinal malignant tumors[5,6]. The median age at diagnosis is 60 years. There is usually no predilection for either gender but some series suggest a slight male predominance. GIST occurring in the familial form is autosomal dominant[5-7]. 5% of GISTs occur in patients with neurofibromatosis type 1 syndrome, occurring mostly in the small intestine and without KIT mutations. GIST also occurs as a part of Carney triad along with paraganglioma and pulmonary chordoma in young females[6-9].

EtiologyThe exact cause of GIST is unknown. However, 95% of patients with GIST have a protein called Kit (CD 117) that has become abnormal, which then causes normal cells to grow faster and become cancerous.PathophysiologyGISTs can develop anywhere along the GI tract from the esophagus to the rectum; however, stomach (60%) and small intestine (30%) are the most common locations for GIST. Only 10% of GISTs are found in the esophagus, mesentery, omentum, colon or rectum. Up to 30% of GISTs exhibit high-risk (malignant) behavior such as metastasis and infiltration[8,9,13,14]. The metastatic pattern is predominantly intra-abdominal, with spread throughout the peritoneal cavity and to the liver. Lymph nodal invasion is uncommon. GISTs with indolent (low-risk) behavior are typically found as small submucosal lesions. True smooth muscle tumors/leiomyomas also occur throughout the GI tract but are now thought to be rare in comparison to GISTs, except in the esophagus where they are more commonSymptoms and SignsSome patients have no symptoms and their tumors are found accidentally. For example, some tumors may be found when having testing or surgery for another reason. However, some people may experience symptoms such as abdominal pain and/or bloating, bleeding from the bowel, decreased appetite, and/or tiredness.Only 70% of the patients with GIST are symptomatic. While 20% are asymptomatic and the tumors are detected incidentally, 10% of the lesions are detected only at autopsy. Symptoms and signs are not disease specific, they are related more to the site of the tumor[6,7,16]. Bleeding (30%-40%) comprises the most common symptom after WJGO|www.wjgnet.com 104 June 15, 2013|Volume 5|Issue 6| vague abdominal discomfort (60%-70%). Bleeding is attributed to the erosion into the GIT lumen. Bleeding occurring into the peritoneal cavity due to a ruptured GIST can lead to acute abdominal pain presenting as a surgical emergency. Bleeding into the GI tract lumen, causing hematemesis, melena or anemia, is usually more chronic on presentation. Most of the patients present with vague symptoms, such as nausea, vomiting, abdominal discomfort, weight loss or early satiety. Symptoms are usually site specific. These include dysphagia in the esophagus, biliary obstruction around the ampulla of Vater or even intussusception of the small bowel[6,7]. Lymph node metastases are uncommon in GIST. Distant metastases most commonly occur in GISTs of the peritoneum, omentum, mesentery and the liver. GISTs have a high tendency to seed and hence intraperitoneal or even scar metastases are known to occur.

DiagnosisNo laboratory test can specifically confirm or rule out the presence of a GIST. The following tests are generally ordered in the workup of patients who present with nonspecific abdominal symptoms; abdominal pain; or complications of a GIST-like hemorrhage, obstruction, or perforation: Complete blood cell count Coagulation profile Serum chemistry studies BUN and creatinine Liver function tests, amylase and lipase values Type, screen, and crossmatch Serum albuminImaging studiesPlain abdominal radiography: Nonspecific May be part of an emergent workup Abnormal gas patterns, including dilated loops of bowel or free extraluminal air, may be seen with bowel obstruction or perforationBarium and air (double-contrast) series: Frequently provides only limited information Can usually detect GISTs that have grown to a size sufficient to produce symptoms Barium swallow for patients with dysphagia Barium enema for patients with constipation, decreased stool caliber, or colonic manifestations GISTs appear as an elevated, sharply demarcated filling defect[1] The overlying mucosa typically has a smooth contour unless ulceration has developedComputed tomography scans of the abdomen and pelvis: Important in the diagnosis and staging of GISTs Provides comprehensive information regarding the size and location of the tumor and its relationship to adjacent structuresCan also be used to detect the presence of multiple tumors and of metastatic spread. CT characteristics of small GISTs (< 5 cm) are as follows: Sharply demarcated Homogeneous density Mainly exhibit intraluminal growth patternsCT characteristics of intermediate GISTs (5-10 cm) are as follows: Irregular shape Heterogeneous density An intraluminal and extraluminal growth pattern Signs of biological aggression, sometimes including adjacent organ infiltrationCT characteristics of large GISTs (>10 cm) are as follows: Irregular margins Heterogeneous densities Locally aggressive behavior Distant and peritoneal metastasesCT criteria associated with high-grade histology and increased mortality: Tumor larger than 11.1 cm Irregular surface contours Indistinct margins Adjacent organ invasion Heterogeneous enhancement Hepatic or peritoneal metastasisMagnetic resonance imaging: Like CT scanning, MRI can depict tumors and yield information about surrounding structures Can also be used to detect the presence of multiple tumors and metastases Less well studied than CT for diagnosing GISTs, but appears equally sensitive GISTs may appear hypointense on T2-weighted imagesPositron emission tomography scanning with 2-[F-18]-fluoro-2-deoxy-D-glucose has the following uses: Detection of metastatic disease Monitoring of response to adjuvant therapy (eg, imatinib mesylate)Endoscopy: Frequently performed early in the workup of patients with GI bleeding, abdominal pain, or GI obstructive symptoms from GISTs Endoscopic features of GISTs include the suggestion of a smooth submucosal mass displacing the overlying mucosa Ulceration or bleeding of the overlying mucosa from pressure necrosis may be present Problematic for biopsy specimen collection because of the submucosal location of GISTs Endoscopic biopsy results yield a diagnosis in less than 50% of cases Obtaining a repeat biopsy in the same site as a prior biopsy may increase the diagnostic yieldEndoscopic ultrasonography (EUS): Allows localization of lesions and their characterization by ultrasonography Fine-needle aspiration biopsy specimens may be obtained under sonographic guidance GISTs typically appear as a hypoechoic mass in the layer corresponding to the muscularis propria Complementary with CT More accurate than CT in differentiating benign from malignant lesions Allows a more comprehensive evaluation of the mass and the surrounding structures than CTEUS characteristics of malignant GISTs include the following: Size larger than 4 cm (the only independent predictor) Heterogeneous echogenicity Internal cystic areas Irregular borders on the extraluminal surfacesEUS features that may help differentiate gastric GISTs from leiomyomas are as follows: Inhomogenicity Hyperechogenic spots A marginal halo Higher echogenicity than the surrounding muscle layerAspects of EUS-guided biopsy are as follows: Biopsy provides definitive diagnosis Biopsy may be required when preoperative therapy is needed in cases where the tumor is unresectable or only marginally resectable Biopsy may not be necessary if the tumor is surgically resectable and preoperative medical therapy is not requiredManagementSurgery is the definitive therapy for patients with GISTs, as follows: Radical and complete surgical extirpation offers the only chance for cure Surgery is also indicated in symptomatic patients with locally advanced or metastatic disease Debulking large lesions is helpful when adjuvant therapy is contemplated Laparoscopic resection has improved and is a more frequently considered optionImatinib mesylate is used in GIST as follows: Adjuvant therapy post complete surgical resection in patients with high-risk tumors Neoadjuvant therapy with the goal of tumor shrinkage prior to surgical resectionOther tyrosine kinase inhibitors are used when imatinib is not tolerated or is not effective are as follows: Sunitinib: Less specific than imatinib; approved as a second-line agent for advanced GIST Sorafenib: Investigational second-generation agent Dasatinib: Investigational second-generation agent Nilotinib: Investigational second-generation agentComplicationGIST can complicate unusual locations such as colonic interposition and should be kept in the differential diagnosis of such unusual presentations.PrognosisGIST can transform into malignant on 10 30 % cases. The manifestation of malignant can be high selularity, local invansion, distant metastases, that normally on liver and peritoneum. The metastases rarely happens on regional lymph node, lungs, or bones.Prognosis becomes worse if there is rupture on tumor, location in distal part, high selularity, necrosis on tumor, an invansion or other organs metastases and c-kit gen mutation.

REFFERENCEAmerican Cancer Society. 2014. Gastrointestinal Stromal Tumor (GIST). Retrieved from http://www.cancer.org/acs/groups/cid/documents/webcontent/003103-pdf.pdfLumongga F. 2008. Gastro Intestinal Stromal Tumor. Departemen Patologi Anatomi Universitas Sumatera Utara : MedanRammohan A., Sathyanesan J., Rajendran K., Pitchaimuthu A., Perumal S., Srinivasan UP, Ramasamy R., Palaniappan R., Govindan M. A gist of gastrointestinal stromal tumors: A review. World J Gastrointest Oncol, 5(6), 102-112Robbins & Cotran. 2009. Dasar Patologis Penyakit edisi 7. EGC : JakartaWengert A., Andybee, Atkin A., Angieatkin, Brentwaldrop, Lenford B., dkk. 2010. Human Physiology/The gastrointestinal system.

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