EMR of mucosa-associated lymphoid tissue lymphoma of the rectum

EMR of mucosa-associated lymphoid tissue lymphoma of the rectum Shouichi Tanaka, MD, Takeyuki Ohta, MD, Eisuke Kaji, MD, Tsunenori Kosaka, MD, Ichiro Murakami, MD

There are numerous reports of mucosa-associated lymphoid tissue (MALT) lymphoma involving the large intestine. Several studies have reported that anti-Helicobacter pylori treatment induced regression of colorectal MALT lymphoma, regardless of the presence or absence of H pylori.1-4 Here, a case is reported of rectal MALT lymphoma (negative for H pylori) diagnosed 7 years after surgical resection of MALT lymphoma of the sigmoid colon. The new lesion was treated by EMR.

A

CASE REPORT A 62-year-old man underwent surveillance colonoscopy that revealed a flat, slightly yellowish, elevated lesion about 20 mm in diameter in the rectum. Seven years and 4 months earlier, he had undergone surgical resection for two MALT lymphomas in the sigmoid colon. The latter were protruding lesions with a gyrus-like surface and were, respectively, 90 × 80 mm and 85 × 40 mm in size. Because there was evidence of metastasis in resected lymph nodes, he was treated with 6 cycles of CHOP (cyclophosphamide, adriamycin, vincristine, prednisone) chemotherapy. Follow-up surveillance included yearly colonoscopy. The clinical course had been uneventful with no evidence of recurrence until the most recent colonoscopy. Endoscopic biopsy specimens confirmed that the newly discovered tumor was a MALT lymphoma. Physical examination and laboratory tests on admission revealed no abnormalities. H pylori infection was ruled out by a negative rapid urease test, culture, and histopathologic examination of the biopsy specimens. Colonoscopy, including Indigo carmine spraying, demonstrated a lesion with a smooth and uneven surface, together with telangiectasia (Figs. 1A and B). A 20-MHz catheter US probe revealed the lesion as a hypoechoic thickening of the second sonographic layer (Fig. 2), indicating that it was almost confined to the mucosal layer. Gallium-scintigraphy, whole body CT, contrast radiography of the small intestine, and upper endoscopy did not reveal any abnormality. Although there were several options for treatment, including resection, treatment for H pylori, and irradiation, EMR was chosen based on the wishes of the patient. EMR was performed with a dual accessory channel videocolonoscope. To clearly define the margin, marks were Current affiliations: Departments of Gastroenterology and Pathology, Iwakuni National Hospital, Iwakuni, Japan. Reprint requests: Shouichi Tanaka, MD, Department of Gastroenterology, Iwakuni National Hospital, 2-5-1 Kuroiso-cho, Iwakuni 740-0041, Japan. Copyright © 2003 by the American Society for Gastrointestinal Endoscopy 0016-5107/2003/$30.00 + 0 doi:10.1067/mge.2003.272 956

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B Figure 1. A, Colonoscopic view of lesion, after spraying with Indigo carmine dye, demonstrating smooth but uneven surface and telangiectasia. B, Closer view of lesion.

Figure 2. EUS image showing hypoechoic lesion in thickened second sonographic (arrows), indicating it is almost confined to mucosa. VOLUME 57, NO. 7, 2003

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A Figure 4. Photomicrograph of resection specimen showing diffuse infiltrate of lymphoma cells (centrocyte-like cells) in mucosa. Keratin staining demonstrates formation of lymphoepithelial lesion more clearly (keratin, orig. mag. ×200).

B

Figure 5. Colonoscopic appearance at 28 months after EMR; no local recurrence is evident.

C Figure 3. A, Endoscopic view showing mucosal marks made electrosurgically with pre-cut knife to indicate resection margin around target lesion. B, Endoscopic view after injection of saline solution with epinephrine and indigo carmine dye into submucosa; lesion is lifted easily. C, View after piecemeal-EMR.

made in the mucosa circumferentially around the target lesion by using an electrosurgical pre-cut knife (Fig. 3A). The lesion was then lifted easily from the underlying tissue by injection of a saline solution of epinephrine and indigo carmine dye into the submucosa (Fig. 3B). Finally, piecemeal-EMR was performed, removing the tumor in 3 pieces, and the specimens were recovered (Fig. 3C). Histopathologic evaluation of the resection specimens revealed diffuse infiltration of centrocyte-like cells and the formation of a lymphoepithelial lesion (Fig. 4), findings compatible with MALT lymphoma. Colonoscopy at 3 and 9 months after the EMR and then yearly (Fig. 5) has not revealed evidence of recurrence during a follow-up of 3 years.

DISCUSSION al.5

Shepherd et found that MALT lymphoma accounts for 35.5% of primary colorectal lymphomas VOLUME 57, NO. 7, 2003

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whereas about 70% of primary gastric lymphomas are MALT lymphomas.6 Surgical resection, including laparoscopic surgery, has been the treatment of choice in most cases7-9 of colorectal MALT lymphoma because the tumor tends to remain localized at the site of origin for extremely long periods and responds well to local therapy. In the present case, the less invasive option of EMR was chosen with the informed consent of the patient. To the investigators’ knowledge, only two other cases have been reported in which endoscopic resection alone was performed. In 1994, Schmid et al.10 reported the first case in which a 25-mm MALT lymphoma was removed endoscopically from the sigmoid colon by snare polypectomy. There was no evidence of recurrence during a subsequent 9 months. Tohda et al.11 described a second case in 2001 in which MALT lymphomas in the cecum and rectum, each 5 mm in size, were removed by EMR. There was no evidence of recurrence during 15 months of follow-up. In the present case, piecemeal-EMR was performed for a 20-mm rectal MALT lymphoma with no evidence of recurrence at 3 years’ follow-up. For endoscopic removal of large (>20 mm) flat lesions, systematic piecemeal-EMR is performed, as in this patient. Although the recently developed cutting method with an insulation-tipped electrosurgical knife12 allows safe resections of large, flat lesions en bloc, the risk of perforation is higher in the large intestine because the wall is thinner than that of the stomach. For MALT lymphoma in the stomach, eradication of H pylori infection is the first line therapy. However, anti-H pylori therapy for colorectal MALT lymphoma has not been established. Although there are several reports of regression of colorectal MALT lymphoma after this form of treatment,1-4 MALT lymphoma of the large intestine is not considered to be directly related to H pylori because the bacterium does not exist in the colon and the rectum. Nakase et al.4 described 3 cases of rectal MALT lymphoma that were negative for H pylori. The lesions regressed after anti-H pylori treatment, which possibly suggests that microorganisms in the large intestine other than H pylori are involved in the development of rectal MALT lymphoma. The mechanism whereby colorectal MALT lymphoma responds to anti-H pylori therapy is unknown. Cyclophosphamide administered orally has been used to treat gastric and duodenal MALT lymphomas.13,14 However, a simple chemotherapeutic regimen for colorectal MALT lymphoma has not been established. Irradiation has also been used as a therapeutic modality. However, in the investigators’ patient, the lesion was located in the rectum. 958

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Therefore, it would have been difficult to treat by radiotherapy because of the potential for injury to adjacent organs and the development of radiationinduced proctitis. There are reports of rectal MALT lymphoma that regressed spontaneously.15,16 Because treatment for colorectal MALT lymphoma has not been standardized, future studies evaluating the range of therapeutic modalities are necessary. A new MALT lymphoma occurred in the rectum of the investigators’ patient 7 years after surgical resection of MALT lymphomas in the sigmoid colon and chemotherapy. Noninvasive EMR was offered to the patient as a therapeutic modality because the lesion was small and discovered by surveillance colonoscopy. No similar case has been reported. The present case suggests that lifelong follow-up may be necessary in patients with GI MALT lymphoma. Regarding a relationship between the first two MALT lymphomas and the most recent such tumor in the rectum of the investigators’ patient, it is possible that a few tumorous clones remained after treatment of the initial lesions and formed the current lesion in the rectum some years later. Lymphoma cells have a “homing” character and return to their original site. In addition, both lesions had a rare karyotypic abnormality, t(11;18)(q21;q21) (data not shown), which suggests that the current lesion has the same characteristics as the first two lesions. Recent studies have described the t(11;18)(q21;q21) chromosome translocation, which results in the synthesis of a fusion gene and protein “chimeric API2-MLT.”17 This may be a good marker to predict poor responsiveness to H pylori eradication in patients with gastric MALT lymphomas.18 However, its potential clinical significance with regard to the large intestine is, at present, obscure. Because there has been no evidence of recurrence, including recurrence at the initial site, for 3 years after EMR in the present case, EMR could be useful for treatment of recurrent small, localized MALT lymphoma in the colorectum. REFERENCES 1. Matsumoto T, Iida M, Shimizu M. Regression of mucosa-associated lymphoid-tissue lymphoma of rectum after eradication of Helicobacter pylori. Lancet 1997;350:115-6. 2. Inoue F, Chiba T. Regression of MALT lymphoma of the rectum after anti-H. pylori therapy in a patient negative for H pylori. Gastroenterology 1999;117:514-5. 3. Raderer M, Pfeffel F, Pohl G, Mannhalter C, Valencak J, Chott A. Regression of colonic low grade B cell lymphoma of the mucosa associated lymphoid tissue type after eradication of Helicobacter pylori. Gut 2000;46:133-5. 4. Nakase H, Okazaki K, Ohana M, Ikeda K, Uchida K, Uose S, et al. The possible involvement of micro-organisms other than Helicobacter pylori in the development of rectal MALT lymphoma in H. pylori-negative patients. Endoscopy 2002;34:343-6. 5. Shepherd NA, Hall PA, Coates PJ, Levison DA. Primary maligVOLUME 57, NO. 7, 2003

Gastric wall abscess presenting as a submucosal tumor

6.

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nant lymphoma of the colon and rectum. A histopathological and immunohistochemical analysis of 45 cases with clinicopathological correlations. Histopathology 1988;12:235-52. Cogliatti SB, Schmid U, Schumacher U, Eckert F, Hansmann M, Hedderich J, et al. Primary B-cell gastric lymphoma: a clinicopathological study of 145 patients. Gastroenterology 1992;101:1159-70. Orita M, Yamashita K, Okino M, Enoki T, Noshima S, Morita N, et al. A case of MALT (mucosa-associated lymphoid tissue) lymphoma occurring in the rectum. Hepatogastroenterology 1999;46:2352-4. Yasui N, Watanabe M, Iwao Y, Sakaguchi AA, Tahara T, Teramoto T, et al. Laparoscopically assisted bowel resection for primary mucosa-associated lymphoid tissue lymphoma of the cecum. Surg Laparosc Endosc Percutan Tech 1999; 9:156-9. Hasegawa N, Kato K, Yamada K, Morita K, Kuroiwa M, Ito H, et al. Extranodal marginal zone B-cell lymphoma of mucosaassociated lymphoid tissue (MALT) of the sigmoid colon. Gastrointest Endosc 2000;52:802-4. Schmid C, Vazquez JJ, Diss TC, Isaacson PG. Primary B-cell mucosa-associated lymphoid tissue lymphoma presenting as a solitary colorectal polyp. Histopathology 1994;24:357-62. Tohda G, Kato C, Higashi S, Wakahara S, Kosaka S, Sakumoto H, et al. A case of mucosa associated lymphoid tissue (MALT) lymphoma in the colon and rectum. Gastroenterol Endosc 2001;43:2237-44.

Gastric wall abscess presenting as a submucosal tumor: case report Chien-Hua Chen, MD, Chi-Chieh Yang, MD, Yung-Hsiang Yeh, MD, Min-Huo Hwang, MD

Suppurative gastritis is a rare disorder; about 500 cases have been reported, the majority during the preantibiotic era.1 The rarity of suppurative gastritis is thought to be caused by the profuse blood supply to the stomach and the bactericidal effect of gastric acid.2 Pathogenic mechanisms include direct invasion by microorganisms and hematogenous spread from a distant source. The mortality rate ranges from 92% to 37%, specific symptoms are lacking in most cases, and the diagnosis is frequently delayed until laparotomy.1,3 According to the extent of the disorder, suppurative gastritis is classified into diffuse and localized types. The diffuse or phlegmonous type is more common and involves the entire stomCurrent affiliations: Division of Gastroenterology, Department of Internal Medicine, Changhua Show-Chwan Memorial Hospital, Changhua, Taiwan. Reprint requests: Chien-Hua Chen, MD, Division of Gastroenterology, Department of Internal Medicine, Changhua ShowChwan Memorial Hospital, 542, Section 1, Chung-Shang Road, Changhua 500, Taiwan. Copyright © 2003 by the American Society for Gastrointestinal Endoscopy 0016-5107/2003/$30.00 + 0 doi:10.1067/mge.2003.273 VOLUME 57, NO. 7, 2003

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12. Ono H, Kondo H, Gotoda T, Shirao K, Yamaguchi H, Saito D, et al. Endoscopic mucosal resection for treatment of early gastric cancer. Gut 2001;48:225-9. 13. Hammel P, Haioun C, Chaumette MT, Gaulard P, Divine M, Reyes F, et al. Efficacy of single-agent chemotherapy in lowgrade B-cell mucosa-associated lymphoid tissue lymphoma with prominent gastric expression. J Clin Oncol 1995;13:2524-9. 14. Lepicard A, Lamarque D, Levy M, Copie-Bergman C, Chaumette MT, Haioun C, et al. Duodenal mucosa-associated lymphoid tissue lymphoma: treatment with oral cyclophosphamide. Am J Gastroenterol 2000;95:536-9. 15. Takenaka R, Tomoda J, Sakata T, Ichiba T, Motoi M, Mizuno M, et al. Mucosa-associated lymphoid tissue lymphoma of the rectum that regressed spontaneously. J Gastroenterol Hepatol 2000;15:331-5. 16. Okamura S, Horiuchi K, Satoh T. Regression of rectal mucosaassociated lymphoid tissue lymphoma unrelated to Helicobacter pylori [letter]. Ann Intern Med 2000;132:247. 17. Dierlamm J, Baens M, Wlodarska I, Stefanova-Ouzounova M, Hernandez JM, Hossfeld DK, et al. The apoptosis inhibitor gene API2 and a novel 18q gene, MLT, are recurrently rearranged in the t(11;18)(q21;q21) associated with mucosaassociated lymphoid tissue lymphomas. Blood 1999;93:3601-9. 18. Liu H, Ye H, Ruskone-Fourmestraux A, Jong DD, Pileri S, Thiede C, et al. T(11;18) is a marker for all stage gastric MALT lymphomas that will not respond to H pylori eradication. Gastroenterology 2002;122:1286-94.

ach with inflammation spreading to all layers from the submucosa. Emphysematous gastritis is a subtype of phlegmonous gastritis and may be caused by gas-forming organisms. The localized, or wall abscess, type occurs less commonly and frequently involves the antrum or pylorus.4 Because the mucosa appears intact in most cases of gastric wall abscess, it is necessary to differentiate this lesion from other submucosal lesions including tumors.5 EUS clearly delineates the individual layers of the GI wall and adjacent structures and is the standard modality for evaluation of submucosal tumors. However, there was no report that describes the typical EUS features of gastric wall abscess. A case is described of a submucosal tumor in the proximal body of the stomach; the patient also had a co-existing pseudocyst involving the tail of the pancreas. At EUS the lesion was limited to the submucosal layer, and the echogenicity was inhomogeneous. Because of persistent epigastric pain and the unknown nature of the tumor, the patient underwent laparotomy. The surgical histopathologic diagnosis was gastric wall abscess. CASE REPORT A 37-year-old man was admitted for evaluation of vague but persistent epigastric pain and post-prandial abdominal fullness of 2 months’ duration. The pain was worse in the supine position and improved with sitting GASTROINTESTINAL ENDOSCOPY

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Gastric wall abscess presenting as a submucosal tumor

Figure 1. Endoscopic view of submucosal tumor (arrow) in proximal body of stomach.

Figure 3. EUS image (12 MHz, 6-cm range) showing inhomogeneous, hypoechoic (3 × 2 cm) tumor in submucosal layer.

Figure 2. CT image of cystic lesion (arrow) around tail of pancreas.

Figure 4. Power Doppler US image showing increased color vascular signals at periphery of submucosal tumor.

upright. He had lost 5 kg of body weight during the preceding 2 months. On examination there was only minimal tenderness in the epigastrium; there was no fever, jaundice, or abnormal lymph node enlargement. Medical history was remarkable for two episodes of acute pancreatitis about 2 years earlier. He drank ethanol (30 g/day) during the 6 years before the occurrence of pancreatitis but had abstained during the subsequent 2 years after physicians advised him that the pancreatitis was alcoholinduced. He smoked one pack of cigarettes per day for 10 years. Laboratory data were the following: hematocrit, 39.5% (normal: 42%-52%); white blood cell count of 6800/mm3 (4500-10,000/mm3), with a differential count of 82% neutrophils (40%-74%), 12% lymphocytes (19%-48%), 6% monocytes (3.4%-9.0%); and serum amylase, 296 U/L (25-125 U/L). Serum aspartate aminotransferase, alanine aminotransferase, total and direct bilirubin, alkaline phosphatase, cholesterol, and triglyceride levels were

within normal ranges. Chest radiography and transabdominal US were unremarkable. EGD demonstrated a submucosal lesion in the proximal body of the stomach (Fig. 1). Abdominal CT revealed irregular thickening of the gastric wall in the proximal body and a cystic lesion around the tail of the pancreas (Fig. 2). At EUS, the gastric lesion appeared as a relatively hypoechoic and inhomogeneous tumor (3 × 2 cm) within the submucosal layer (Fig. 3). A tentative diagnosis was made of ectopic pancreas in the proximal body of the stomach. After the patient ingested 700 mL of water, transabdominal power Doppler US revealed signals indicative of increased flow at the periphery of the submucosal tumor (Fig. 4). At laparotomy, performed because of the uncertainty as to the nature of the tumor and persistent epigastric pain, a 2.5 × 1.5 × 1.5-cm tumor was found in the proximal body of the stomach. In addition, a cystic lesion of firm consistency measuring 2 × 1.5 cm was also noted

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around the tail of the pancreas. The remaining visceral organs, including the pancreas, looked normal. Subtotal gastrectomy with Billroth II anastomosis and excision of the cyst were performed. The cut surface of the gastric lesion had a yellowish, oily appearance. However, histopathologic evaluation surprisingly revealed that the lesion was an abscess localized in the submucosal layer. Infiltration by neutrophils was evident in all layers of the gastric wall. No bacterial culture or Gram’s stains of the specimens were performed. The cyst lesion involving the tail of the pancreas was a pseudocyst. The postoperative course was uncomplicated. At 2 months after surgery, the patient was asymptomatic.

DISCUSSION Suppurative gastritis can be secondary to local invasion of the gastric mucosa by bacteria; associated conditions include foreign body ingestion, gastric surgery, endoscopic polypectomy, gastric ulcer, carcinoma, leiomyosarcoma, and empyema of the gallbladder.3,4,6-8 There is also evidence for hematogenous seeding as a complication of pneumonia, endocarditis, erysipelas, impetigo, scarlet fever, typhoid fever, and osteomyelitis.9 Alcoholism, diabetes mellitus, decreased gastric acidity, and immunosuppression have been regarded as predisposing factors.10,11 In order of frequency, Streptococci, Staphylococci, Escherichia coli, Haemophilus influenzae, Proteus, and Clostridium are the most common pathogens.12 Chills, fever, nausea, and localized epigastric pain are the most common clinical features of suppurative gastritis. Leukocytosis with a left shift is usual. Hemodynamic compromise and diffuse guarding-type pain may develop if the diagnosis is delayed and peritonitis occurs. The presence of pus in vomitus and Deininger’s sign (decreased abdominal pain on changing from supine to sitting position) are diagnostic signs of suppurative gastritis, but these rarely are seen.4 As in the present case, suppurative gastritis may rarely present as prolonged epigastric pain without fever or signs of systemic infection. The patient had a history of alcoholism, a predisposing factor for suppurative gastritis, but he had abstained from drinking alcohol during the 2 years before presentation. Gastric wall abscess may occur as a result of pancreatitis with transmural erosion of the gastric wall or as a consequence of inflammation in ectopic pancreatic tissue.3,13 However, the latter abscess appears only as a solitary submucosal tumor without evidence of transmural erosion; there was no ectopic pancreatic tissue in the present case. The diagnosis of suppurative gastritis is easy only when pus is seen to flow from the small opening in the mucosa created when a biopsy specimen is obtained. Thickened, reddened gastric folds, or, as VOLUME 57, NO. 7, 2003

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in the present case, a submucosal tumor with intact mucosa, are the most common endoscopic features of gastric wall abscess.14,15 The present case represents the first EUS demonstration of a gastric wall abscess that appeared as an inhomogeneous but predominantly hypoechoic tumor in the muscularis propria or submucosa. Diffuse thickening of the gastric wall with a hypoechoic tumor is sometimes a clue to the diagnosis of gastric wall abscess.1 It is difficult to conclusively differentiate a gastric wall abscess from other submucosal lesions, including tumors, by EUS. However, EUS is valuable for demonstrating the presence of the submucosal lesion, evaluating extension of the lesion, defining the layers involved, and also after the response to pharmacotherapy of a gastric wall abscess. The differential diagnosis of gastric submucosal tumor usually includes GI stromal tumor, hematoma, intramural varices, neurofibroma, and lipoma.16 For most of these tumors, the power Doppler vascular signals are either intratumoral or hypovascular. Kang et al.17 have suggested that, as with our patient, the presence of a hypoechoic tumor with increased peritumoral vascular signals by power Doppler US may be characteristic of a gastric wall abscess. Treatment of suppurative gastritis should be prompt and includes systemic administration of antibiotics, drainage, and/or resection. There are a few case reports of successful treatment of suppurative gastritis with antibiotics alone, but the mortality rate is much higher than that for patients treated with gastrectomy.12 Surgery, either resection or drainage, is currently the standard treatment modality for suppurative gastritis. Resection is indicated in cases where there is uncertainty as to the nature of the inhomogeneous submucosal tumor or clinical evidence of peritonitis. Subtotal or total gastrectomy should be carried out according to the extent of gastric involvement. Surgery is less effective in cases of diffuse suppurative gastritis, although it may reduce the mortality rate.18 Combined therapy with systemically administered antibiotics and endoscopic drainage of pus is reported to be an alternative to aggressive surgery, but adequate drainage must be assured by incision of the mucosa and submucosa with a needle knife.5,9 In conclusion, gastric wall abscess is a rare clinical problem. It may simulate other causes of the acute abdomen, but the clinical symptoms can be nonspecific. It does not have typical endoscopic or EUS features. However, a gastric wall abscess should be included in the differential diagnosis when EUS reveals an inhomogeneous, hypoechoic, submucosal tumor and/or disruption of the normal wall structure of the stomach. GASTROINTESTINAL ENDOSCOPY

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Endoscopic clip application for esophagomediastinal-tracheal fistula

REFERENCES 1. Iwakiri Y, Kabemura T, Yasuda D, Okabe H, Soejima A, Miyagahara T, et al. A case of acute phlegmonous gastritis successfully treated with antibiotics. J Clin Gastroenterol 1999;28:175-7. 2. Farman J, Dallmand S, Rosen Y. Gastric abscess, a complication of pancreatitis. Am J Dig Dis 1974;19:751-8. 3. Seidel RH, Burdick JS. Gastric leiomyosarcoma presenting as a gastric wall abscess. Am J Gastroenterol 1998;93:2241-4. 4. Murphy JF, Graham DY, Frankel NB, Spjut H. Intramural gastric abscess. Am J Surg 1976;131:618-21. 5. Will U, Masri R, Bosseckert H, Knopke A, Schonlebe J, Justus J. Gastric wall abscess, a rare endosonographic differential diagnosis of intramural tumors: successful endoscopic treatment. Endoscopy 1998;30:432-5. 6. Berk RN, Reit RJ. Intra-abdominal chicken bone abscess. Radiology 1971;101:311-3. 7. Lifton LJ, Schlossberg D. Phlegmonous gastritis after endoscopic polypectomy. Ann Intern Med 1982;97:373-4. 8. Vandyk K, German J. Empyema of the gallbladder causing gastroduoenal intramural abscess and pyloric obstruction. Am J Surg 1967;113:295-7. 9. Lantz PE, Westerman EL, Seifert RW. Gastric wall abscess drained at endoscopy. Gastrointest Endosc 1989;35:272-4. 10. Aviles JF, Fernandez-Seara J, Barcena R, Dominguez F,

Endoscopic clip application for closure of an esophagomediastinal-tracheal fistula after surgery for esophageal cancer Shunji Mizobuchi, MD, Kenshi Kuge, MD, Hironori Maeda, MD, Yasuhisa Matsumoto, MD, Morio Yamamoto, MD, Shiro Sasaguri, MD

Benign anastomotic stricture develops in 22% to 48% of patients after cervical esophagogastrostomy.1-5 Esophageal dilation continues to be an important method for treating patients with dysphagia caused by stricture of these cervical anastomoses. Dilation is safe, with a less than 0.5% chance of perforation or bleeding and a 0.01% risk of death.6 Therefore, perforation of a cervical anastomosis during esophageal dilation is a rare occurrence. A case is reported of an esophagomediastinal-tracheal fistula after balloon dilation and self-bougienage for a cervical anastomotic stricture, which was then closed by an endoscopic clip application. To the investigators’ knowledge, there is no report of endoscopic clip placement for closure of an intractable fistula from a cervical esophagogastric anastomosis. Current affiliations: Department of Surgery II, Kochi Medical School, Kohasu, Okohcho, Nankoku, Japan. Reprint requests: Shunji Mizobuchi, MD, Kochi Medical School, Kohasu, Okohcho, Nankoku, Kochi 783-8505, Japan. Copyright © 2003 by the American Society for Gastrointestinal Endoscopy 0016-5107/2003/$30.00 + 0 doi:10.1067/mge.2003.225 962

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12. 13.

14. 15.

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Fernandez C, Ledo L. Localized phlegmonous gastritis: endoscopic view. Endoscopy 1988;20:38-9. Zazzo JF, Troche G, Bertand M, Aubert A, Bedossa P, Keros L. Phlegmonous gastritis associated with HIV-I seroconversion: endoscopic and microscopic evolution. Dig Dis Sci 1992;37: 1454-9. Miller AI, Smith B, Rogers AI. Phlegmonous gastritis. Gastroenterology 1975;68:231-8. Flejou JF, Potet F, Molas G, Bernades P, Amouyal P, Fekete F. Cystic dystrophy of the gastric and duodenal wall developing in heterotopic pancreas: an unrecognized entity. Gut 1993;34:343-7. Schultz MJ, Van der Hulst RWM, Tytgat GNJ. Acute phlegmonous gastritis. Gastrointest Endosc 1996;44:80-3. Bron BA, Deyhle P, Pelloni S, Krejs GJ, Siebenmann RE, Blum AL. Phlegmonous gastritis diagnosed by endoscopic snare biopsy. Dig Dis Sci 1977;22:729-33. Brogdon GB, Davies JP, Billing PJ, Jones MR. Intramural gastric abscess mimicking leiomyoma: clinical, radiologic, and pathologic features of an unusual gastric lesion. Invest Radiol 1993;28:175-6. Kang BC, Kim KW, Lee SW, Kim JH. Gastric wall abscess: imaging diagnosis and endoscopic treatment. J Comput Assist Tomogr 1998;22;673-5. Stephenson SE, Yasrebi H, Rhatogan R, Woodward ER. Acute phlegmasia of the stomach. Am Surg 1970;36:225-31.

CASE REPORT A 63-year-old man underwent blunt dissection of the esophagus with retrosternal reconstruction by gastric tube for esophageal carcinoma. There was no complication, and he was discharged. During an outpatient followup, dysphagia, caused by stricturing of the cervical anastomosis, was detected. Endoscopic balloon dilation was performed several times; however, the stenosis was refractory to therapy, resulting in readmission for treatment approximately 10 weeks after surgery. Endoscopic dilation was performed several times, but improvement in dysphagia was temporary. Thus, self-bougienage with a 42F Maloney-type tungsten-filled silicone bougie (Pilling Weck, Washington, Pa.) was initiated. The patient was taught self-dilation by swallowing the dilator at the bedside. He responded well to treatment, with significant relief of dysphagia and was discharged. Eight weeks later, a cough developed and he was treated for a “common cold.” However, after a further 8 weeks he developed severe coughing with bloody sputum in response to swallowing, and he returned to the investigators’ institution. Esophagography with a water-soluble contrast agent confirmed the presence of an esophagomediastinal-tracheal fistula (Fig. 1), and the patient was hospitalized. On admission, the patient’s temperature was 36.3°C, the white blood cell count was 5300/mm3 (normal: 40008000/mm3) with a normal differential, and the C-reactive protein level was 0.7 mg/dL (0-0.6 mg/dL). A chest radiograph was interpreted as unremarkable. Endoscopy demonstrated the orifice of a fistula at the cervical anastomosis (Fig. 2). Fiberoptic bronchoscopy revealed an elevated lesion near the bifurcation of the trachea, which was composed of granulation tissue and contained the orifice VOLUME 57, NO. 7, 2003

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Figure 3. Bronchoscopic view showing elevated lesion near bifurcation of trachea. The lesion was composed of granulation tissue and included the orifice of the fistula.

Figure 1. Esophagogram made with water soluble contrast showing esophagomediastinal-tracheal fistula. Long arrow indicates esophagomediastinal fistula; short arrow indicates mediastinal-tracheal fistula.

Figure 4. Endoscopic view with transparent plastic cap attached to tip of endoscope showing clip at orifice of fistula.

Figure 2. Endoscopic view showing orifice of fistula at cervical anastomosis to be small and not edematous. of the fistula (Fig. 3). On the chest CT, there was a solid density posterior to the trachea, suggesting mediastinitis. The patient was treated by intravenous hyperalimentation and not permitted to eat or drink. An attempt, 8 days after admission, to occlude the fistula with a fibrin sealant was unsuccessful. Endoscopic clip placement was attempted 17 days after admission but was also unsuccessful because a suitable endoscopic view could not be obtained. However, on hospital day 24, endoscopic clip application (HX-5 LR-1, Olympus Optical Co., Ltd., Tokyo, VOLUME 57, NO. 7, 2003

Japan) was easily performed by attaching a transparent plastic cap to the tip of the endoscope (Fig. 4). Fluoroscopy with a water soluble contrast agent 7 days later demonstrated good flow through the anastomosis with no evidence of leakage from the esophagus. Chest CT did not demonstrate inflammation in the mediastinum. At endoscopy before discharge, the fistula had healed completely with scar formation (Fig. 5). Moreover, at bronchoscopy, the granulation tissue in the trachea in the area of the fistula orifice from mediastinum to trachea had become flatter. The patient did not experience dysphagia, cough, or bloody sputum on ingestion of food and was discharged asymptomatic.

DISCUSSION al.3

found that 30% of 81 patients who Pierie et underwent cervical esophagogastrostomy developed benign anastomotic stricture. Poor vascularization of the gastric tube and postoperative anastomotic GASTROINTESTINAL ENDOSCOPY

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Figure 5. Endoscopic view of anastomosis demonstrating complete closure of fistula with scar formation.

leakage were significant risk factors for stricture formation. In the study of Honkoop et al.,4 bougie dilation was successful in 78% of patients with cervical anastomotic strictures, similar to the success rate of 83% noted by Pierie et al.3 In the present case, there was no leakage from the anastomosis during the immediate postoperative period. The anastomotic fistula developed after balloon dilation and self-bougienage, undoubtedly caused by trauma as a result of these procedures. When an esophagomediastinal fistula develops, surgical intervention is indicated for mediastinitis and to establish external drainage. Conservative treatment, by contrast, with nutritional support (enteral and/or parenteral) and administration of antibiotics, is not always sufficient to stop leakage, but even when successful such an approach takes a long time. The lack of abscess formation in the present case may have been because of good drainage from the posterior mediastinum. At admission, the white blood cell count and the C-reactive protein were normal, and the patient did not have a high fever. In view of these findings, it was decided to attempt to close the orifice of the fistula at the cervical anastomosis. There are a growing number of reports of conservative endoscopic therapy for intractable esophagomediastinal fistula, including descriptions of the use of fibrin glue to seal the fistula,7,8 injection of ethyl2-cyanoacrylate (a bioadhesive substance),9 placement of a flexible tube,10 endoscopic laser photocoagulation using an Nd-YAG laser,11 and other methods. Because fibrin glue is biocompatible and spontaneously absorbed, its use for closure of an acquired esophageal fistula is safe, relatively noninvasive, and easily repeated. In the present case, this 964

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Endoscopic clip application for esophagomediastinal-tracheal fistula

procedure failed because it was impossible to maintain the seal because of the patient’s cough reflex. The adhesion strength of fibrin glue is relatively poor, and several minutes are required after application for tissue adhesion to develop. Therefore, it seems unlikely that treatment of an esophagobronchial fistula with fibrin glue can be successful. There is one report in which placement of an esophageal stent was found to be useful in the treatment of a cervical anastomotic leak.10 However, because the cervical esophagus is usually curved after reconstruction, insertion and removal of a stent involves risk. Moreover, patients experience a foreign body sensation when stents are placed in the proximal esophagus near the hypopharynx.12 Therefore, the rationale for stent placement in the treatment of cervical anastomotic leakage must be evaluated carefully. The esophageal fistula in the present case was closed using endoscopically applied metallic clips developed by Hachisu.13 Metallic clips were first developed by Hayashi et al.14 and Kuramata et al.15 and subsequently adapted for treatment of GI bleeding and for marking lesions in the GI tract. Endoscopic closure of a perforation with metallic clips after snare excision of a gastric leiomyoma was first reported by Binmoeller et al.16 in 1993. In 1995, Wewalka et al.17 described closure of an esophageal perforation after pneumatic dilation for achalasia. In 1998, Rodella et al.18 reported experience with treatment of anastomotic leaks in 7 patients after gastric surgery by endoclip placement. The time required for closure was clearly shorter than for conservative treatment alone. There is no previous report of successful closure of a cervical esophageal anastomotic leak or fistula by endoscopic application of clips. Seemingly, such a procedure should be difficult because the anastomosis is near the cricopharyngeus, and the cervical esophagus is curved after reconstructive surgery. However, with the attachment of a transparent plastic cap to the endoscope, a good working space was provided, and clips were easily applied to the fistula orifice. A transparent cap that can be attached and later removed from the tip of a forward-viewing GI fiberscope was first devised by Ozaki et al.19 It is possible to observe a lesion en face by using such a transparent cap and to withdraw it into the cap even when it is situated tangential to the endoscope. Moreover, the transparent cap maintains a certain distance between the endoscope lens and the lesion, enabling more effective clip placement. In summary, endoscopic clip application in the present case was a safe and effective method for closure of an acquired esophageal fistula. An aggresVOLUME 57, NO. 7, 2003

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sive endoscopic approach appears to be feasible for treatment of an esophagomediastinal fistula with external drainage when the orifice of the fistula is small and not edematous. REFERENCES 1. Zieren HU, Muller JM, Pichlmaier H. Prospective randomized study of one- or two-layer anastomosis following oesophageal resection and cervical oesophagogastrectomy. Br J Surg 1993;80:608-11. 2. Dewar L, Gelfand G, Finley RJ, Evans K, Inculet R, Nelems B. Factors affecting cervical anastomotic leak and stricture formation following esophagogastrectomy and gastric tube interposition. Am J Surg 1992;163:484-9. 3. Pierie JP, de Graaf PW, Poen H, Van der Tweel I, Obertrop H. Incidence and management of benign anastomotic stricture after cervical oesophagogastrostomy. Br J Surg 1993;80:471-4. 4. Honkoop P, Siersema PD, Tilanus HW, Stassen LPS, Hop WCJ, van Blankenstein M. Benign anastomotic strictures after transhiatal esophagectomy and cervical esophagogastrectomy: risk factors and management. J Thorac Cardiovasc Surg 1996;111:1141-8. 5. Heitmiller RF, Fischer A, Liddicoat JR. Cervical esophagogastric anastomosis: results following esophagectomy for carcinoma. Dis Esophagus 1999;12:264-9. 6. Nostrant TT, Nandi PS. Esophageal dilation. Gastroenterologist 1998;6:5-15. 7. Hirao M, Shimada M, Kaneko T, Mizunoya S, Okagawa K. Two cases of esophageal fistula, in which a fibrin glue preparation was effective. J Jpn Assn Thorac Surg 1994;42:2144-9. 8. Kohler B, Kohler G, Riemann JF. Spontaneous esophagotracheal fistula resulting from ulcer in heterotopic gastric mucosa. Gastroenterology 1988;95:828-30.

Diagnosis of solid-pseudopapillary neoplasm of the pancreas by EUSguided FNA Sonali S. Master, MD, Thomas J. Savides, MD

Solid-pseudopapillary tumors (SPT) of the pancreas account for approximately 1% of all pancreatic neoplasms. They occur predominantly in young women, often do not cause symptoms, have low malignant potential, and are most commonly located in the body or tail of the pancreas. They generally are treated by surgical resection. Two cases of SPT of the pancreas diagnosed by EUS-FNA are described. CASE REPORTS Case 1 A 51-year-old woman presented with a 1-year history of abdominal pain, maximal in the epigastrium, associated Current affiliations: Division of Gastroenterology, University of California, San Diego, California. Reprint requests: Thomas J. Savides, MD, UCSD Division of Gastroenterology, 200 W. Arbor Dr., San Diego, CA 92103-8413. Copyright © 2003 by the American Society for Gastrointestinal Endoscopy 0016-5107/2003/$30.00 + 0 doi:10.1067/mge.2003.228 VOLUME 57, NO. 7, 2003

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9. Takagi Y, Sato S, Kuroda N, Osaka Y, Takagi M, Hayashi M, et al. A case esophagobronchial fistula effectively treated with cyanoacrylate. Jpn J Gastroenterol Surg 1999;32:888-91. 10. Kagawa Y, Takiyama W, Saeki T, Takashima S. Successful treatment of leakage following esophagectomy for an esophageal cancer by intubating tube: report of a case. J Jpn Surg Assoc 1996;57:2193-7. 11. Miyaike J, Okada H, Mizuno M, Uesu T, Takahashi A, Ariyoshi M, et al. A case of an acquired esophago-bronchial fistula successfully treated by endoscopic laser coagulation therapy. Gastroenterol Endosc 2001;43:1152-6. 12. Moses FM, Wong RK. Stents for esophageal disease. Curr Treat Options Gastroenterol 2002;5:63-71. 13. Hachisu T. Evaluation of endoscopic hemostasis using an improved clipping apparatus. Surg Endosc 1988;2:13-7. 14. Hayashi T, Yonezawa M, Kuwabara T, Kudoh I. The study on stanch clip for the treatment by endoscopy. Gastroenterol Endosc 1975;17:92-101. 15. Kuramata H, Eto S, Horiguchi K, Unayama F, Kazato K, Tsuboi A. Evaluation of gastrofiberscope for treatment (TGF proto-type by Olympus Co.). Stomach Intestine 1974; 9:355-64. 16. Binmoeller KF, Grimm H, Soehendra N. Endoscopic closure of a perforation using metallic clips after snare excision of a gastric leiomyoma. Gastrointest Endosc 1993;39:172-4. 17. Wewalka FW, Clodi PH, Haidnger D. Endoscopic clipping of esophageal perforation after pneumatic dilation for achalasia. Endoscopy 1995;27:608-11. 18. Rodella L, Laterza E, de Manzoni G, Kind R, Lombardo F, Catalano F, et al. Endoscopic clipping of anastomotic leakages in esophagogastric surgery. Endoscopy 1998;30:453-6. 19. Ozaki M, Urita Y, Otsuka S, Watanabe Y. Improved endoscopic therapies with a new transparent tip-hood. Endoscopia Digestiva 1990;2:659-66.

with nausea and 2 episodes of vomiting. The symptoms were thought to be most consistent with functional dyspepsia. She noted no association of the symptoms with ingestion of food, and there had been no weight loss. There was no personal or family history of pancreatic disease. Examination and laboratory studies, including serum lipase, were unremarkable. Transabdominal US revealed a 1.8 × 1.6 × 1.2-cm hypoechoic mass in the body of the pancreas. CT confirmed the presence of an illdefined, nonenhancing 1.5-cm soft tissue mass in the body of the pancreas, with decreased attenuation compared with the adjacent pancreatic tissue. EUS with a radial echoendoscope revealed a 16 × 11mm hypoechoic mass in the superior portion of the pancreatic genu. The mass was located within the pancreas and did not involve the pancreatic duct or any adjacent structure. At the request of the referring physicians, EUSFNA was performed to document malignancy before subjecting the patient to a major pancreatic resection. The procedure was performed with a linear array echoendoscope (GF-UC30P, Olympus America Corp., Melville, N.Y.). A total of 2 passes were made with a 22-gauge fine needle (Wilson-Cook Medical Inc., Winston-Salem, N.C.). Evaluation of the cytologic specimens revealed a monomorphic population of small cells with scant to moderate cytoplasm and irregular nuclear membranes. Papillary fronds with fibrovascular stalks, trabecular GASTROINTESTINAL ENDOSCOPY

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ous FNA-cytologic diagnosis of solid-pseudopapillary neoplasm of the pancreas. Postoperative recovery was uneventful. At an 18-month follow-up, the patient continued to experience similar abdominal symptoms thought to be caused by nonulcer dyspepsia. There was no evidence of endocrine or exocrine pancreatic insufficiency and no evidence of tumor recurrence or metastatic disease. Case 2

A

B Figure 1. A, CT showing 4.6 × 4.1-cm well-circumscribed pancreatic mass with cystic component. B, Radial EUS image (7.5 MHz, range 12 cm) showing 34 × 37-mm cystic pancreatic lesion with debris/solid component. areas, and microacinar structures were noted, as well as metachromatic hyaline globules. Immunohistochemical stains for chromogranin and synaptophysin were negative. These findings were consistent with a diagnosis of solid-pseudopapillary neoplasm. The patient underwent a central pancreatectomy with Roux-en-Y pancreaticojejunostomy. Gross examination revealed a well-circumscribed, gelatinous-appearing, heterogeneous mass measuring 1.8 × 1.4 × 1.3 cm. Histopathologic evaluation demonstrated pseudopapillary areas, fibrosis, hyalinization, slit-like cystic spaces, and hemorrhage. There were no mitoses or nuclear pleomorphism. No perineural invasion or vascular invasion was identified. The surgical resection margins were free of tumor. The histopathologic findings confirmed the previ966

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A 32-year-old woman with no GI symptoms or history of pancreatic disorder underwent magnetic resonance imaging to further evaluate an enlarged right ovary. This revealed a large, complex, cystic mass posterior to the gastric body. CT revealed a 4.6 × 4.1-cm well-circumscribed mass with a cystic portion originating within the junction of the body and neck of the pancreas (Fig. 1A). EUS was performed with a radial echoendoscope with imaging at both 7.5 and 12 MHz. This revealed a 45 × 35mm mixed cystic and solid lesion at the pancreatic genu (Fig. 1B). On imaging, it was unclear whether this lesion represented a benign pseudocyst with debris versus a solid mass with a cystic component. Transgastric FNA was performed with removal of all of the fluid from the cystic portion of the lesion. FNA was then performed of the solid/debris portion of the lesion, a total of 5 passes being made with a 22-gauge fine needle (Wilson-Cook). Fluid analysis of the cystic portion revealed an amylase of 51 U/L, lipase of 32 U/L, CA 19-9 of 5 U/mL (0-37 U/mL), and CEA of less than 0.2 ng/mL (0-3.0 ng/mL). Examination of the cellular aspirate from the mass lesion revealed loosely cohesive aggregates and single monomorphic cells with moderately enlarged nuclei and a moderately increased nuclear/cytoplasmic ratio, as well as characteristic papillary structures (Fig. 2A). The chromatin was fine and evenly distributed. Immunostains showed a strong positive reaction with neuron specific enolase and a weak reaction with chromogranin in only rare cells. The tumor cells were also positive for vimentin (diffuse strong reaction) and alpha-1-antitrypsin. Synaptophysin and cytokeratin were negative. These findings were consistent with a diagnosis of solid-pseudopapillary neoplasm of the pancreas. The patient subsequently underwent a segmental resection of this lesion. Histopathologic evaluation demonstrated both solid and pseudopapillary areas (Fig. 2B). Immunocytochemical stains confirmed the findings for the aspirate, and, in addition, the tumor cells exhibited a positive reaction for progesterone receptor and a negative reaction for estrogen receptor. At a 6-month follow-up, the patient was asymptomatic from a GI standpoint, with no signs of tumor recurrence, metastases, or signs of either endocrine or exocrine insufficiency of the pancreas.

DISCUSSION SPT of the pancreas was first described in 1959.1 There have since been many synonyms for SPT used in various publications; some of these are solid and papillary epithelial neoplasm, solid and cystic VOLUME 57, NO. 7, 2003

Diagnosis of solid-pseudopapillary neoplasm of the pancreas by EUS-guided FNA

tumor, papillary-cystic neoplasm, papillary cystic tumor, and Franz’s tumor. In 1996, this tumor was renamed solid-pseudopapillary tumor by the World Health Organization for the histologic classification of tumors of the exocrine pancreas.2 Although these tumors have characteristic cytologic and histologic features, their cell of origin remains unclear. During embryogenesis, the genital ridges are in close proximity to the pancreatic anlage. Some have speculated that SPTs may come from genital ridge/ovarian anlage-related cells, which were attached to the pancreatic tissue during early embryogenesis.3 Yet others have considered ductal, acinar, and endocrine cell origins without convincing evidence. SPTs comprise approximately 1% of all pancreatic neoplasms, and in one center they accounted for 12% of 134 resected, clinically cystic pancreatic tumors.4 These tumors most commonly are identified in young women. In one series of 59 SPTs, 88% of the patients were women.3 Mean age at diagnosis is approximately 26 years, with a reported range of patient’s age from 8 to 70 years.3,5-7 Patients with SPTs are frequently asymptomatic, and the tumor is often found incidentally on imaging studies performed for other indications. When present, symptoms are nonspecific and may include nausea, vomiting, abdominal pain, and fullness. These lesions can present at any size, with reported dimensions ranging from 1.5 to 30 cm (mean 10.5 cm).8 They most commonly are located in the pancreatic body and tail. On CT, SPTs may appear as well-circumscribed hypodense lesions with or without obvious cystic change.9 Bondeson et al.10 were the first to correctly diagnose SPT by preoperative transabdominal FNA. Subsequently, SPTs have also been studied by preoperative, sonographically guided, percutaneous aspiration.11 However, SPT has not been diagnosed previously by EUS-FNA, as in the 2 cases presented. The cytologic features of SPT of the pancreas are distinctive and allow differentiation from other lesions.11 These include highly cellular aspirates composed of small, uniform epithelial cells with oval nuclei, fine chromatin, and small nucleoli. The cells are arranged as papillary structures with delicate fibrovascular cores, and there may be a layer of myxoid material between the core and lining epithelial cells. Cytologic features were diagnostic in each of these 2 cases and were confirmed by surgical resection. By immunohistochemistry, SPTs are usually positive for vimentin, alpha-1-antitrypsin, alpha-1antichymostrypsin, and neuron specific enolase. Approximately one third are keratin positive, whereas some express other neuroendocrine markVOLUME 57, NO. 7, 2003

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A

B Figure 2. A, Photomicrograph of EUS-FNA specimen showing characteristic papillary structures (Papanicolau stain, orig. mag. ×100). B, Photomicrograph of tissue section demonstrating solid and papillary areas (H&E, orig. mag. ×100).

ers such as synaptophysin. Chromogranin has been consistently reported as negative. Recently, SPTs have been described that also stain positive for beta catenin.12 In both cases, there was a pattern consistent with SPT, although in the second case, there were reportedly rare cells positive for chromogranin. There is no known correlation between tumor size and metastatic potential. However, in tissue sections, the presence of venous invasion and large necrotic clusters may be indicative of an increased risk of malignancy.13 SPTs have an excellent prognosis with a low potential for local invasion or metastasis. At presentation, approximately 85% are limited to the pancreas with metastases in only 10% to 15% of cases. 8,14 Over 95% of SPTs limited to the pancreas may be cured by complete surgical resection.15 Longterm survival with a 15-year follow-up has been reported after surgical resection of both primary tumors and metastatic disease that is primarily GASTROINTESTINAL ENDOSCOPY

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found in the liver.16 Long-term survival has also been noted for patients with residual disease.13,17 A common reason for performing EUS-FNA of small or incidentally found pancreatic lesions is that most patients with such lesions are reluctant to undergo major pancreatic surgery, and most community surgeons hesitate to consider such an operation without a preoperative diagnosis of a potentially malignant lesion, as was the situation in Case 1. This is especially the case for patients who are poor candidates for surgery. In addition, EUS-FNA can guide surgical management, because a potentially benign tumor (e.g., neuroendocrine, SPT) may be treated by local excision, in contrast to an adenocarcinoma, which would require a more extensive resection. This was also the result in Case 1, as the patient underwent a central pancreatectomy. EUS features alone cannot reliably distinguish between benign and malignant cystic lesions of the pancreas, making preoperative FNA desirable.18 There is increasing evidence that EUS-FNA may help to distinguish benign from potentially malignant pancreatic cysts through analysis of cyst fluid, not only cytologic evaluation, but also for amylase, lipase, and CEA.19 This was the situation in Case 2 in which there was a question whether the lesion might be a pseudocyst from previous clinically silent pancreatitis. EUS-FNA is not always needed for evaluation of potentially resectable pancreatic masses. When there is an obvious large mass lesion, enlargement of a lesion demonstrated by serial imaging studies, a lesion that is clearly the cause of symptoms, or when the patient and the surgeon are comfortable with resection based on imaging characteristics alone, the diagnostic information provided by EUSFNA ultimately may not change the need for surgical resection. Also, EUS-FNA has potential complications (1% complication rate for solid lesions, 14% for cystic lesions), which could make subsequent surgery more difficult or even impossible.20 In summary, solid-pseudopapillary neoplasm of the pancreas should be considered in young, asymptomatic women with incidentally detected pancreatic body masses. EUS-FNA can accurately diagnose SPTs of the pancreas and may help guide surgical management. ACKNOWLEDGMENT Cynthia Behling, MD, PhD, for pathology and manuscript review. REFERENCES 1. Frantz VK. Papillary tumors of the pancreas: benign or malignant? In: Frantz VK, editor. Tumors of the pancreas. Atlas of tumor pathology. 1st Series. Section VII. Fascicle 27. Washington DC: U.S. Armed Forces Institute of Pathology; 1959. p. 32-3. 968

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2. Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. Histological typing of tumours of the exocrine pancreas. In: World Health Organization international classification of tumours. New York: Springer; 1996. p. 8452. 3. Kosmahl M, Seada LS, Janig U, Harms D, Kloppel G. Solidpseudopapillary tumor of the pancreas: its origin revisited. Virchows Arch 2000;436:473-80. 4. Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol 2000;17:66-80. 5. Pettinato G, Manivel C, Ravetto C, Terracciano L, Gould E, Di Tuoro A, et al. Papillary cystic tumor of the pancreas. Am J Clin Pathol 1992;98:478-88. 6. Matsunou H, Konishi F. Papillary-cystic neoplasm of the pancreas. A clinicopathologic study concerning the tumor aging and malignancy of nine cases. Cancer 1990;65:283-91. 7. Cubilla AL, Fitzgerald PJ. Tumors of the exocrine pancreas. In: Hartmann WH, Sobin LH, editors. Atlas of tumor pathology. Series 2. Fascicle 19. Washington DC: Armed Forces Institute of Pathology; 1984. p. 201-7. 8. Mao C, Guvendi M, Domenico DR, Kim K, Thomford NR, Howard JM. Papillary cystic and solid tumors of the pancreas: a pancreatic embryonic tumor? Studies of three cases and cumulative review of the world’s literature. Surgery 1995;118:821-8. 9. Dong PR, Lu DS, Degregario F, Fell SC, Au A, Kadell BM. Solid and papillary neoplasm of the pancreas: radiologicalpathological study of five cases and review of the literature. Clin Radiol 1996;51:702-5. 10. Bondeson L, Bondeson AG, Genell S, Lindholm K, Thorstenson S. Aspiration cytology of the rare solid and papillary epithelial neoplasm of the pancreas: light and electron microscopic study of a case. Acta Cytol 1984;28:605-9. 11. Pelosi G, Iannucci A, Zamboni G, Bresaola E, Iacono C, Giovanni S. Solid and cystic papillary neoplasm of the pancreas: a clinico-cytopathologic and immunocytochemical study of five new cases diagnosed by fine-needle aspiration cytology and a review of the literature. Diagn Cytopathol 1995;13:233-46. 12. Tanaka Y, Notohara K, Kato K, Ijiri R, Nishimata S, Miyake T, et al. Usefulness of beta-catenin immunostaining for the differential diagnosis of solid-pseudopapillary neoplasm of the pancreas. Am J Surg Pathol 2002;26:818-20. 13. Nishihara K, Nagoshi M, Tsuneyoshi M, Yamaguchi K, Hayasshi I. Papillary cystic tumors of the pancreas: assessment of their malignant potential. Cancer 1993;71:82-92. 14. Adair CF, Wenig BM, Heffess CS. Solid and papillary cystic carcinoma of the pancreas: a tumor of low malignant potential [abstract]. Int J Surg Pathol 1995;2:326. 15. Jeng LB, Chen MF, Tang RP. Solid and papillary neoplasm of the pancreas. Emphasis on surgical treatment. Arch Surg 1993;128:433-6. 16. Martin RCG, Klimstra DS, Brennan MF, Conlon KC. Solidpseudopapillary tumor of the pancreas: a surgical enigma? Ann Surg Oncol 2002;9:35-40. 17. Zinner MJ, Shurbaji MS, Cameron JL. Solid and papillary epithelial neoplasms of the pancreas. Surgery 1990;108:475-80. 18. Ahmad NA, Kochman ML, Lewis JD, Ginsberg GG. Can EUS alone differentiate between malignant and benign cystic lesions of the pancreas? Am J Gastroenterol 2001;96:3229-30. 19. Brugge WR, Saltzman JR, Scheiman JM, Wallace MB, Jowell PS, Pochapin MB, et al. Diagnosis of cystic neoplasms of the pancreas by EUS; The report of the cooperative pancreatic cyst study [abstract]. Gastrointest Endosc 2001;53:AB71. 20. Wiersema MJ, Vilmann P, Giovannini M, Chang KJ, Wiersema LM. Enodsonography-guided fine-needle aspiration biopsy: diagnostic accuracy and complication assessment. Gastroenterology 1997;112:1087-95. VOLUME 57, NO. 7, 2003