During this 20-25 minute pre-recorded lecture, Dr. Edward Lung discusses the the topic of lumps/bumps of the GI tract. This in-depth review will provide an update on this topic for your clinical practice as well as supplement your learning for the ABIM Gastroenterology and Hepatology boards. CME pre-requiste of live Q & A webinar, 8th Annual Mount Sinai Intensive Board Review in Gastroenterology & Hepatology on Sept 29.
good afternoon for this session. We will discuss some epithelial lesions of the Gi tract. Sub epithelial lesions are most commonly found in the stomach we will encounter is some epithelial lesion one in every 300 upper endoscopy ease fortunately the majority of benign And less than 15% are malignant at presentation. Men and women appear equally affected And we tend to find these in those over the age of 50 Most lesions are less than two cm in diameter. Therefore cat scan and MRI are not sensitive enough to detect these lesions and this coptic ultrasound has emerged as the gold standard for evaluation. Us affords us the ability to differentiate extra mural compression versus intramural growth Up to 30% of suspected intramural sub epithelial lesions are extra mural in origin. Us allows us to determine the layer of origin which helps make a diagnosis. The US also affords us the ability to provide an accurate estimate of the size. We can also determine if there is a lymph node involvement and most importantly, we can obtain tissue via fine needle biopsy. This diagram just depict various sub epithelial lesions and their locations according to wall layer. An echo level. So for instance, like palmas are typically found in the third layer and are quite bright or hyperlink OIC on the U. S. Gi stromal tumors typically found in the 4th layer. They tend to be hippo quick viruses tend to be located in the third layer where the sub mucosa and or dark or an acoustic. This diagram depicts where most of the suburban theory allegiance that we find will be contained according to wall air. So gi stromal tumors and lie on my Omagh's are typically found where there's muscle in the muscularity mucosal with a deep mucosa or the muscular is appropriate. Like thomas, granular cell tumors. Pancreatic rests, carcinoid, duplication, cysts, viruses and love. NGO mazar typically found in the third layer with the sub mucosa question one. A 65 year old male presents with Melon. You G. D. Reviews a four centimeter by six centimeter sub epithelial lesion with ulceration and the fund. This Us reviews a hippo a quick homogeneous lesion arising from the muscular is appropriate. Or later four FNB varios a spindle cell tumor with Greater than 10 mitosis per high power field. Um You know his, so chemistry staining Is positive for d. o. g. one. The next best step for this patient's management is A S. D. B. Surgical reception alone. See surgery followed by imatinib D. Imaginable. Oh or e altri tied. So the best answer is c surgery followed by McNabb as this is highly characteristic of a Gi stromal tumor. Or just The incidence of just in our country is about 4.3 per million per year. This is typically diagnosed between the ages of 60 and 70. There is no significant gender difference Over 60% are found based on symptoms. While the third of found incidentally and most of these are encountered in the stomach followed by the small bowel duodenum and really direct them Fortunately most of the nine. However up to 30% can be malignant and presentation. However all just have malignant potential. These tumors originally from the interstitial cells of Cathal And they have three cell types spindle cell which are the majority versus epithelial Lloyd versus mixed prognosis. Based on the cell type is limited although the my topic threshold from malignancy is lower for the epithelial type on the photo and the endoscopic photo you see a smooth sub epithelial lesion and on the endoscopic ultrasound view. You see this hippo coke lesion that is arising from the muscular is appropriate. High risk us features include irregular borders, academic fosse within the lesion, a heterogeneous echo pattern and the presence of cystic spaces. The molecular biology of Gi stromal tumors is quite interesting. These tumors involve a gain of function mutation in the C KIT proto oncogene. This is present in 80% of Jess this country. Tyrosine kinase trans membrane receptor Is c. d. 1 17 and tested positive. That is on um you know history chemistry standing CD117 is positive in 95% of cases and this is useful in distinguishing from other sub epithelial tumors. 80% of tumors of just tumors will express cd 34 or D. O. G. One D. O. G. one is the most sensitive. Um you know history chemistry marker. There's a second less common mutation referred to as the Alfred type Platelet derived growth factor receptor and this is found in 10% of Gi stromal tumors. It is however, President 80% of kit negative GIS primarily in the stomach and momentum. It is rare to have both seek it and P. D. G F. R. A mutations simultaneously. In fact 10% of just will be negative for Kit. MPD G F R. G mutations. So typically when A. U. S. Has performed an F. And B. Is done um You know, it's the chemistry standing will hopefully show CD 1 17 If CD 1 17 or Kid is negative. Usually the pathologists will check for CD 34 or d. o. g. one which will also solidify the diagnosis. In addition, we can explore for the presence of the gene mutation of KIT or P. D. GR. For A. For those tumors that are negative for cd 34 D. O. G. One KIT and no D mutation is found other immuno. So chemists street staining is helpful including looking for the presence of acting and Desmond which would be suggestive of a leo my oma and S 100 which would be indicative of Schwann Omagh's. Some important prognostic features regarding just uh include size, size graded a two centimeter portends a higher risk of malignancy. In addition, Those tumors with a higher metabolic index that is greater than five per 50 high power food mitosis have a high risk of malignancy and to determine my topic index. We need robust tissue specimens therefore FNB is essential. F. N. A. In and of itself is not adequate. In addition location is very important, tumors located in the small intestine and rectum have a much higher malignant potential than those found in the stomach. Furthermore for allegiance within the stomach. Those found in the fund this and by the G junction or of higher malignancy potential than those found in the Antrim. This diagram depicts rest ratification according to my topic index, size and site. And the important take home points from this slide Are that tumors that are graded in 2cm tumors that Have a higher metabolic rate of great in a 550 high power field and tumors that are found outside of the stomach that is in the small intestine, duodenum direct um are greater risk. The therapeutic approach should be multidisciplinary. That is. We should follow these patients with both medical and surgical oncologists, Surgical excision is recommended for tumors greater than two cm or tumors of any size that are symptomatic. That is patients who present with hemorrhage, operation, pain or obstruction. Finally, any tumors located in the small intestine should undergo surgery regardless of the size. As these are highly malignant. Up to 50% of these are malignant Compared to 20 of tumors which are found in the stomach that will be malignant. For Tumors are less than two cm in diameter. We can follow them with the U. S. Annually or if they're good operative candidates take them to surgery and in the presence of worrisome features on the US. Consider surgery and again to reiterate on the US. If we find irregular borders or internal heterogeneity, if we find an aquatic areas signifying a Croesus academic fosse indicating hemorrhage or regional lymph node enlargement, these patients should be treated more aggressively with surgery. Tyrosine kinase inhibitors have become a important treatment treatment modality for GIS. And in 2000 and two, imagine it was approved by the FDA for Kit positive. Un receptacle or metastatic gist With complete surgical reception. There is a 60% cure rate. Conversely a 40% relapse rates. The first study that showed that imagine it was helpful was a randomized double blinded placebo controlled trial of over 700 patients, patients had surgery underwent surgical reception and then received either one year treatment with the Madison neb versus placebo And there was a significant improvement in recurrence free survival of 98% vs 83%. Subsequently, studies have supported the benefit of imatinib such that currently all guidelines recommend adjuvant therapy with imatinib for three years for high risk or ruptured Gis. This improves relapse free survival and overall survival. The use of vaginal therapy is not recommended for very low risk or low risk. This there's really no consensus for intermediate risk. Just mutation testing is critical in the decision making process. In fact, while type guests are not candidates for adua therapy, these tumors portend a very poor outcome. Regardless if you give them the time we're seeing kinase inhibitors follow up is very important for these patients for very low risk and low risk tumors. CT scans can Be obtained on a yearly basis for five years. High risk tumors treated with adjuvant therapy should undergo cT scan every six months for three years after the completion of the mat snip. The risk of recurrence is highest. So CT scan should be obtained every three months during the first two years after completion of the maximum for three years after that. Uh You can check cat scans every 6-12 months for up to 10 years after surgery. Question two A 35 year old male is referred for an upper endoscopy figured he denies dysplasia. Dina Feige or vomit Endoscopy reviews a smooth sub epithelial lesion in the distal 1/3 of the esophagus. Us reviews a 1.5 centimeter hypo ochoa gleision arising from the muscularity appropriate fine needle biopsy reveals spindle cells. Um You know, his of chemistry standing is most likely positive for which of the following A. c. d. 1 17 b. d. o. g. one see smooth muscle acting and Desmond D. s. or e neuron specific delays. The correct answer is C smooth muscle acting. And Desmond, the description of this is highly suggestive and consistent with a leo myeloma on endoscopy in the distal esophagus, you see this smooth bulge and on endoscopic ultrasound you say hypoxic lesion arising From the 4th layer. The muscular is appropriate. As you know, leo my arms are benign smooth muscle tumors that arise where there is muscle. Typically the muscularity mucosa, Otherwise known as the the Mucosa or the muscular is appropriate layer of four leo. My home is comprised up to 80% of sub epithelial lesions originating from the fourth layer of the esophagus, Over 50% occurring at lower esophagus and 34% in the middle. So typically these lesions on meant to lower esophagus really are. They found an approximately esophagus leo McComas may have calcifications whereas this is a rear finding ingest intron Omagh's amino has the chemistry stating is positive for Desmond and acting. Surgical resection is only required for those regions associated with symptoms including dysplasia, obstruction, bleeding and perforation. And here you see a smooth sub epithelial lesion on endoscopy on the upper left. Next to that on the us. You see this Hypoxic lesion arising from the 4th layer. When agencies e staining you see uh smooth muscle cells. It almost looks like a swirl of selves and these will again staying positive one Amino. Her cell chemistry for smooth muscle active, this is an endoscopic legion that you can see has almost a yellowish hue. And when you press a biopsy forceps into it is very soft and an almost intense. If you buy up see it, you might see more of a yellowish hue and some fat droplets. This is obviously a light poma commonly found in Antrim and the colon and this just composed of adipose tissue. The Pillow sign is 98% specific for like Palme. On endoscopic ultrasound, you see a homogeneous well defined hyper or bright hyper quick lesion arising from the third layer, the sub mucosal layer, tissue sampling is not required when the endoscopic imaging is suggestive and characteristic. These are generally asymptomatic but on occasion may cause hemorrhage er obstruction when they are large. Again you see a sub epithelial lesion in the stomach here and on endoscopic ultrasound you see this very bright well hypertrophic lesions arising from the sub mucosal layer. This lesion is found in Antrim, the sub epithelial lesion. On endoscopic ultrasound, it arises from the sub mucosal or the third layer on the U. S. Again you can see it arises from the 3rd layer. This is a pancreatic rest an endoscopy These are typically round oval lesions along a greater curve of the interim with a central umbilical nation. The estimated prevalence is anywhere from 2 to 14 generally. These are not associated with symptoms. However, on occasion they can cause ulceration, G. I. Bleeding gastric outlet obstruction of the large or if they're producing pancreatic enzymes you patients can Present with pancreatitis. These typically are very large allegiance over three cm in diameter. On endoscopic ultrasound, there are two types a shallow type which is limited to the third layer. These are typically found in a handsome and the most common pancreatic breasts that we find. There's also a deep type that can extend deeper to the muscular is appropriate or the fourth layer. And these are found in the body and fungus Question three. The patient is a 70 year old female who presents for endoscopy for epic gastric abdominal pain. A five centimeter by two centimeter. Sub epithelial lesion is seen in the body of the stomach On the US the lesion is hypoxic homogeneous and it arises from the 4th layer or the muscular is appropriate. Fine needle biopsy reviews spindle cells And um you know history chemistry staining is positive for s. And is negative for CD 1 17 D. O. G. one Acting in Desmond. What is the optimal treatment strategy for this patient? A surgical reception, be a tired scene kinase inhibitor. See chemotherapy or d external beam radiation. The best choice here is a surgical resection. This is typical of a Schwan Oma and given the size of five cm. Um and the patients abdominal pain it should be removed surgically. Schwan Omagh's uh have very low malignant potential and they are comprised of spindle cells. Schwan omagh's originate from any nerve having a Schwann cell sheath. They can be difficult to differentiate from just and lie on my own mess. They can comprise anywhere from 2-8% of sub epithelial lesions in the stomach. They mainly occur in the body of the stomach, followed by the Antrim and then the fund us again on. Um You know it's the chemistry staining. They are positive for s. Here's a typical US uh image of a swan oma hypoxic OIC and it typically arises from the fourth layer on endoscopy. Again you see a smooth bulge. It's hard to based on this to understand if this is a jazz drummer to malaya myeloma Sonoma. But on Um you know his the chemistry staining, it is positive for s. This lesion is found in the esophagus, it almost has a yellowish hue. And if you push a biopsy forceps it is quite firm and it looks like it has a little rehabilitation on us. This arises from the sub mucosal layer and on um you know, history chemistry standing, It states positive for s. in this lesion is a cannula cell tumor. These are also nerve sheath tumors of Schwann cell origin. That is why it states positive for S- 100. These typically arise from the sub mucosa and grow towards the surface of the mucosa. Therefore diagnosis is quite often possible with a standard biopsy forceps. Most of current admit to distal esophagus. The majority are asymptomatic, those that are greeted in one centimeter may cause dysplasia or retro sternal pain. The rate of malignancy is low. Typically 2- 4%. Those lesions are greater than four cm. Have a much higher risk of malignancy. In fact, lesions over two centimeter. Surgery is recommended for those Lesions between one and two cm and that are limited to the sub mucosal Koza on us. You can consider and endoscopic mucosal resection or dissection for those tumors that a lesson one cm and you'll E G. D. And U. S. Surveillance will suffice. Finally, this is a diagram that have obtained from gastrointestinal endoscopy. It provides an algorithmic approach two sub epithelial allegiance I included for your review, I want to thank you for your time and attention and wish you the best of luck. Thank you.