J Ped Surg Case Reports 1 (2013) 333e336

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Journal of Pediatric Surgery CASE REPORTS

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Endoscopic-assisted laparoscopic surgical removal of a gastric neurofibroma in a child

Tamarah Westmoreland a, Paul W. Williams b, Kathryn B. Brown c, David E. Sawaya a, Michael J. Nowicki b,* a Division of Pediatric Surgery, University of Mississippi Health Center, 2500 North State Street, Jackson, MS 39216, USA b Division of Pediatric Gastroenterology, University of Mississippi Health Center, 2500 North State Street, Jackson, MS 39216, USA c Department of , University of Mississippi Health Center, 2500 North State Street, Jackson, MS 39216, USA article info abstract

Article history: An 11-year-old boy, who presented with abdominal pain and vomiting was noted to have a gastric Received 14 August 2013 submucosal mass at . Endoscopic ultrasound showed it arising from the fourth ultrasound Received in revised form level of the gastric wall precluding endoscopic removal. Open surgery was avoided by use of endoscopic- 4 September 2013 assisted laparoscopic surgery (EALS) to remove the mass. The mass was found to be an isolated gastric Accepted 7 September 2013 neurofibroma, a rare tumor in children. We show that combined use of intraluminal endoscopy and Available online xxx laparoscopic surgery allows for safe and less-invasive surgery for removal of a submucosal mass in a child. Further, we review the rare finding of gastric neurofibromas. Key words: Ó Endoscopic-assisted laparoscopic surgery 2013 The Authors. Published by Elsevier Inc. Open access under CC BY license. Neurofibroma Gastric submucosal mass

Submucosal gastric tumors present both a diagnostic and 1. Patient report therapeutic challenge for surgeons and gastroenterologists alike. These tumors are most commonly identified as gastrointestinal An 11-year-old male presented with a several week history of stromal tumors (GIST) or leiomyomas; more rare tumors include abdominal pain and vomiting. The pain occurred daily without leiomyosarcomas, schwannomas, and neurofibromas. The thera- identifiable triggering or relieving factors, persisted for hours, and peutic approach to such tumors is dependent on the type of tumor resolved spontaneously. The pain was localized to the epigastrium (benign or malignant), location within the , and depth but was poorly characterized; it awakened him from sleep 5 days a within the gastric wall from which the tumor arises. Historically, week. He had associated vomiting, which was devoid of blood, open resection was the preferred therapeutic option, but advances coffee-ground material, and bile. He had no fever, diarrhea, rectal in laparoscopic surgery and therapeutic endoscopy have led to less bleeding, jaundice, or weight loss. Past medical history was positive invasive options. Laparoscopic surgery and endoscopic resection only for hospitalization for wheezing at 6 months of age, and each offer unique advantages over the other; combining the tech- incision and drainage of a suppurative lymph node at 2 years of age. niques offers the advantages of both procedures, a technique Family history was significant for a hiatal and gastroesoph- referred to as endoscopic-assisted laparoscopic surgery (EALS). This ageal reflux in the father; there was no history of migraines, peptic technique is well-established in adults. To date there have been no ulcer disease, or inflammatory bowel disease. Examination revealed reports of EALS for the removal of submucosal gastric tumors in normal growth parameters, a normal abdominal examination, and children. We report our experience with EALS for removal of a a negative for occult blood. Laboratory studies to gastric neurofibroma in a child and offer a brief review gastric include a complete blood count, erythrocytic sedimentation rate, neurofibromas. and urinalysis were normal. He was placed on a weight-appropriate dose of omeprazole for 2 months, without improvement in the abdominal pain. An upper endoscopy was performed which showed erythema of the antrum without nodularity, erosion, or ulceration; a rapid test was positive within 15 min. A 1-cm submucosal mass with central ulceration was seen on the greater * Corresponding author. Tel.: þ1 601 984 5232; fax: þ1 601 815 1053. curvature of the body of the stomach (Fig. 1); it was non- E-mail address: [email protected] (M.J. Nowicki). compressible with closed forceps. taken from the

2213-5766 Ó 2013 The Authors. Published by Elsevier Inc. Open access under CC BY license. http://dx.doi.org/10.1016/j.epsc.2013.09.003 334 T. Westmoreland et al. / J Ped Surg Case Reports 1 (2013) 333e336

After discussion with the family and Pediatric Surgery, it was decided to perform endoscopic-assisted laparoscopic removal of the gastric mass. Following informed consent, placement of appropriate monitoring lines, and induction of general anesthesia, a 5-mm trocar was placed. Carbon dioxide was infused into the abdominal cavity to a pressure of 15 mm Hg. A 30-degree laparo- scope was introduced into the abdominal cavity. Next, three 5-mm trocars were placed; one in the right upper quadrant and two in the left to mid upper quadrant. The stomach was examined lapa- roscopically, but the location of the mass could not be identified. The pylorus was then occluded with a grasper, and an upper endoscopy was performed showing a mass arising the on the posterior wall of the stomach (Fig. 3). The stomach was desufflated, and the short gastric arteries were taken down using a harmonic scalpel. The stomach was then turned to expose the posterior wall. Once again, the pylorus was occluded with a grasper, and upper endoscopy was performed. Pressure was applied to the gastric mass from within the stomach with biopsy forceps, and the tented-out stomach was grasped on the serosal surface through the laparo- scope. The stomach was then desufflated, and an Endo GIA60 Fig. 1. Endoscopic features of the gastric tumor. At the initial endoscopy a firm, non- staplerÔ (2.5-mm load) was placed across the stomach containing compressible mass with central ulceration was seen on the posterior wall of the stomach. the gastric mass. Prior to deploying the stapler, the endoscope was advanced back into the stomach to confirm that the mass was successfully captured within the portion of the stomach to be antrum revealed moderate active chronic gastritis with Helicobacter removed. The stapler was then deployed to ligate and transect the pylori (Hp) organisms identified on toluidine blue-stained sections. portion of the stomach containing the mass. The stomach specimen The patient was treated with a 2 week course of amoxicillin, clar- was then removed through the umbilical port and submitted to ithromycin, and lansoprazole for Hp-induced gastritis and repeat pathology. endoscopy scheduled for endoscopic ultrasound and biopsy of the The tumor was a firm nodule (2.0 1.5 1.5 cm) with ulceration mass. at the center (Fig. 4A), composed of interlacing bundles of bland One month later the patient had resolution of his abdominal spindle cells with wavy nuclei admixed with fibroblasts and char- pain, and stool for Hp antigen was negative. At repeat endoscopy acteristic dense bundles of collagen (Fig. 4B and C). Immunohisto- the mass was determined to arise from the fourth ultrasound level chemical studies again showed that the tumor cells were diffusely of the gastric wall suggesting a gastrointestinal stromal tumor positive for S-100 and negative for desmin, myogenin, CD117 and (Fig. 2). Attempts at fine needle biopsy were unsuccessful so a CD34 confirming a diagnosis of neurofibroma (Fig. 4D and E). needle knife was used to open the mucosa followed by pinch bi- opsies of the mass. The mucosal defect was closed with endoclips. The biopsies showed a bland spindle cell neoplasm with lack of nuclear pleomorphism and mitotic activity within the tumor cells. Immunohistochemical studies showed that the tumor cells were diffusely positive for S-100 and negative for desmin, myogenin, CD117 and CD34 supporting a diagnosis of neurofibroma.

Fig. 2. Endoscopic ultrasound of the gastric tumor. Endoscopic ultrasound showed that Fig. 3. Endoscopic findings at the time of surgery. At surgery, light from the laparo- the tumor arose from the fourth ultrasound level of the gastric mucosa representing scopic illuminated the tumor. An endoclip from the previous biopsy is seen attached to the muscularis propria, suggestive for a gastrointestinal stromal tumor. the surface of the tumor. T. Westmoreland et al. / J Ped Surg Case Reports 1 (2013) 333e336 335

obtained by fine needle aspiration and Tru-cut needle biopsy, the tissue sample makes up only a small portion of a tumor and may miss malignant cells. Excisional biopsy with adequate tumor margin is the preferred method for gastric submucosal tumors [3,4]. Minimally invasive surgery has revolutionized the approach to gastric tumors, highlighted by rapid advances in laparoscopic sur- gery (LS) and therapeutic endoscopy. Prior to LS, gastric tumors required open . With the advent of LS and improvement in laparoscopic instruments a number of laparoscopic techniques have been employed for the removal of gastric wall tumors including enucleation, transgastric tumor-everting resection, intragastric tumor wedge resection, and extraluminal wedge resection [3]. However, each of these techniques has drawbacks. Enucleation of GISTs is technically successful but there is a high rate of tumor recurrence [5]. Transgastric tumor-everting resection can lead to intra-abdominal contamination with gastric juice, tumor seeding, and injury to the esophago-cardiac junction [3]. Intra- gastric wedge resection requires special equipment that may not be readily available at some centers and there remains a risk of tumor seeding and gastric mucosal injury [3]. A major drawback of extraluminal wedge resection is difficulty in determining the appropriate resection line as it is impossible to ascertain with cer- tainty the margins when the tumor is intraluminal [6]. Endoscopic submucosal dissection (ESD) has become an acceptable modality for removal of benign and malignant gastric tumors with excellent success rates and safety. Initially, it was thought that endoscopic removal of gastric tumors arising from the muscularis propria was contraindicated due to a high risk of perforation and bleeding [1,7]. However, subsequent studies in small numbers of adults have shown that, in experienced hands, gastric tumors arising from the muscularis propria layer can be successfully removed (75%e90%) with low rates of bleeding (0%e 10%) and perforation (0%e10%) [8,9]. An important limitation of ESD is that complete resection relies solely on endoscopic obser- vation; it is difficult to achieve complete histological resection of tumors related to an inability to obtain sufficient margins during endoscopy [8]. In a study assessing endoscopic mucosal resection of early gastric cancers nearly 17% had incomplete resection [10]. EALS for gastric tumors was first introduced in 1999 by Aogi et al. [11]. Initially, endoscopy was used primarily as an “extra set of eyes” allowing better localizing of the tumor [11e21]; recent studies have combined ESD with LS with excellent results [22,23]. Fig. 4. Pathological specimen and histological findings. The tumor was identified as a Benefits of a combined approach include better localization of the firm nodule (2.0 1.5 1.5 cm) with ulceration at the center from a previous biopsy tumor, determining the best laparoscopic approach, verifying (A). Gastric mucosa (top) was seen overlying a well-circumscribed submucosal spindle complete resection, ensuring adequate margins, and assuring leak- cell lesion (bottom) (B, H&E 2 ). The tumor was composed of interlacing bundles of fi bland spindle cells with wavy nuclei admixed with fibroblasts and characteristic dense proof suture lines [22,23]. In the present case, endoscopy con rmed bundles of collagen (C, H&E, 20). The neoplastic cells were strongly positive for S100 that the tumor was localized to the gastric body in a location (D, H&E, 20). CD117 failed to stain the neoplastic cells but highlighted intra-tumoral accessible for laparoscopic removal. Biopsy forceps were then used mast cells (E, H&E, 20). to show the surgeons the location by pressing on the tumor and “tenting” the gastric wall for laparoscopic visualization. Endoscopic 2. Discussion visualization during placement of the gastric stapler confirmed that the entire tumor was captured and that the gastric lumen would Gastric submucosal tumors in children are a rare occurrence. not be impeded once the tumor was removed. Finally, after resec- Studies in adults have shown that these tumors are most commonly tion the stomach was gently insufflated to ensure that the suture gastrointestinal stromal tumors (GIST) or leiomyomas; more rare line was leak-proof. tumors include leiomyosarcomas, schwannomas, and neurofi- The finding of a gastric neurofibroma is exceedingly rare, bromas. Differentiation between these tumors cannot be made particularly in children. Neurofibromas account for 0.1% of gastric solely by endoscopic visualization. Endoscopic ultrasound (EUS) has tumors found at autopsy [24] and 0.06% of surgically removed gastric been used with fair success (89% sensitivity, 86% specificity, 80% tumors [25]. Of individuals with gastrointestinal neurofibromas, overall accuracy) in differentiating GISTs from leiomyomas [1,2]. 15%e17% have neurofibromatosis (von Recklinghausen disease), However, the malignant potential of submucosal tumors cannot be while the remainder (83%e85%) have an isolated neurofibroma made with certainty by EUS, thus a definitive diagnosis requires [26,27]. It has been estimated that 25% of individuals with neurofi- pathological confirmation [1]. Standard forceps biopsy is inadequate bromatosis develop gastrointestinal neurofibromas [28]. The dis- for obtaining tissue from submucosal tumors as the biopsies typi- tribution of neurofibromas differs between individuals with cally do not reach beyond the mucosa. While deeper biopsies can be neurofibromatosis and those with isolated neurofibromas. In 336 T. Westmoreland et al. / J Ped Surg Case Reports 1 (2013) 333e336 neurofibromatosis, the most commonly involved site is the jejunum, [5] Nishimura J, Nakajima K, Omori T, Takahashi T, Nishitani A, Ito T, et al. Surgical strategy for gastric gastrointestinal stromal tumors: laparoscopic vs. open followed by stomach, ileum, duodenum, and colon [27]. For isolated e fi resection. Surg Endosc 2007;21:875 8. neuro bromas the ileum is the most commonly involved site, fol- [6] Kitano S, Shiraishi N. Minimally invasive surgery for gastric tumors. Surg Clin lowed by the jejunum, duodenum, and stomach [29]. Within the North Am 2005;85:151e64. xi. stomach, the antrum is the most commonly involved site (39%), [7] Eda Y, Asaki S, Yamagata L, Ohara S, Shibuya D, Toyota T. Endoscopic treat- ment for submucosal tumors of the : studies in 25 patients. Gas- followed by the posterior wall (16%), lesser curvature (13%), fundus troenterol Jpn 1990;25:411e6. (10%), body (10%), greater curvature (6%), and anterior wall (6%) [26]. [8] Lee IL, Lin PY, Tung SY, Shen CH, Wei KL, Wu CS. Endoscopic submucosal Presentation of gastrointestinal neurofibromas depends on where dissection for the treatment of intraluminal gastric subepithelial tumors originating from the muscularis propria layer. Endoscopy 2006;38:1024e8. the tumors arise. Most arise from the submucosal myogenic (Auer- [9] Park YS, Park SW, Kim TI, Song SY, Choi EH, Chung JB, et al. Endoscopic bach’s) plexus, leading to subserosal lesions, which tend to present enucleation of upper-GI submucosal tumors by using an insulated-tip elec- with obstruction due to external compression or volvulus [30]. trosurgical knife. Gastrointest Endosc 2004;59:409e15. fi ’ [10] Ono H, Kondo H, Gotoda T, Shirao K, Yamaguchi H, Saito D, et al. 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