- Email: [email protected]
Cap polyp of the esophagus caused by cholesterol embolization
J Langhorst, M Stolte, G Dobos, et al.
increased neovascularity in the surrounding capsule; and no remarkable vascularity was recognized within the tumor. The CEUS image also corresponded to the pathologic findings. Although CDFI and CEUS demonstrated that the tumor was vascular only at the periphery, analysis of more cases is necessary to determine whether this finding is characteristic of neurilemoma. EUS-FNA in the present case guided the surgical procedure as a primary pancreatic neoplasm was ruled out and a definitive diagnosis of a neurilemoma was established. To our knowledge, this is the first report of a case of retroperitoneal neurilemoma diagnosed by EUS-FNA. REFERENCES 1. Kim SH, Choi BI, Han MC, Kim YI. Retroperitoneal neurilemoma: CT and MR findings. AJR Am J Roentgenol 1992;159: 1023-6. 2. Hayasaka K, Tanaka Y, Soeda S, Huppert P, Claussen CD. MR findings in primary retroperitoneal schwannoma. Acta Radiol 1999;40:78-82. 3. Das Gupta TK, Brasfield RD, Strong EW, Hajdu SI. Benign solitary schwannomas (neurilemomas). Cancer 1969;24:355-66. 4. Borghese M, Corigliano N, Gabriele R, Antoniozzi A, Izzo L, Barbaro M, et al. Benign schwannoma of the pelvic retroperitoneum. Report of a case and review of the literature. G Chir 2000;21:232-8. 5. Woodruff JM, Selig AM, Crowley K, Allen PW. Schwannoma (neurilemoma) with malignant transformation. A rare, distinctive peripheral nerve tumor. Am J Surg Pathol 1994;18: 882-95.
Cap polyp of the esophagus caused by cholesterol embolization Jost Langhorst, MD, Manfred Stolte, MD, Gustav Dobos, MD, Horst Neuhaus, MD
The “cap polyp” occurs mainly in the rectum and distal descending colon. Microscopically, it consists of elongated, tortuous, and distended crypts covered by a “cap” of inflammatory granulation tissue.1,2 In the few published cases of cap polyposis of the colon, mucosal prolapse, based on abnormal colonic motility, has been postulated as an important etiologic factor.3 Despite some arguments in favor of an infectious oriCurrent affiliations: Department of Medicine, Kliniken EssenMitte, Academic Teaching Hospital of the University of Essen, Department of Pathology, Klinikum Bayreuth, Academic Teaching Hospital of the University of Erlangen, and Department of Medicine, Evangelisches Krankenhaus Düsseldorf, Academic Teaching Hospital of the University of Düsseldorf, Germany. Reprint requests: Jost Langhorst, MD, Department of Internal Medicine, Kliniken Essen-Mitte, Am Deimelsberg 34a, D-45276 Essen, Germany. Copyright © 2003 by the American Society for Gastrointestinal Endoscopy 0016-5107/2003/$30.00 + 0 doi:10.1067/mge.2003.229 792
GASTROINTESTINAL ENDOSCOPY
Cap polyp of the esophagus induced by cholesterol embolization
6. DiMagno EP, Buxton JL, Regan PT, Hattery RR, Wilson DA, Suarez JR, et al. Ultrasonic endoscope. Lancet 1980;22;1:629-31. 7. Nakazawa S, Sugiyama H, Kimoto E, Naito Y. Specifications of endoscopic ultrasonography. Scand J Gastroenterol Suppl 1984;94:1-6. 8. Nakazawa S, Yoshino J, Nakamura T, Yamanaka T, Hase S, Kojima Y, et al. Endoscopic ultrasonography of gastric myogenic tumor. A comparative study between histology and ultrasonography. J Ultrasound Med 1989;8:353-9. 9. Ohashi S, Nakazawa S, Yoshino J. Endoscopic ultrasonography in the assessment of invasive gastric cancer. Scand J Gastroenterol 1989;24:1039-48. 10. Wiersema MJ, Vilmann P, Giovannini M, Chang KJ, Wiersema LM. Endosonography-guided fine-needle aspiration biopsy: diagnostic accuracy and complication assessment. Gastroenterology 1997;112:1087-95. 11. Gress FG, Hawes RH, Savides TJ, Ikenberry SO, Lehman GA. Endoscopic ultrasound-guided fine-needle aspiration biopsy using linear array and radial scanning endosonography. Gastrointest Endosc 1997;45:243-50. 12. Matsui M, Goto H, Niwa Y, Arisawa T, Hirooka Y, Hayakawa T. Preliminary results of fine needle aspiration biopsy histology in upper gastrointestinal submucosal tumors. Endoscopy 1998;30:750-5. 13. McGrath KM, Ballo MS, Jowell PS. Schwannoma of the mediastinum diagnosed by EUS-guided fine needle aspiration. Gastrointest Endosc 2001;53:362-5. 14. O’Toole D, Palazzo L, Arotcarena R, Dancour A, Aubert A, Hammel P, et al. Assessment of complications of EUS-guided fine-needle aspiration. Gastrointest Endosc 2001;53:470-4. 15. Ando N, Goto H, Niwa Y, Hirooka Y, Ohmiya N, Hayakawa T, et al. The diagnosis of GI stromal tumors with EUS-guided fine needle aspiration with immunohistochemical analysis. Gastrointest Endosc 2002;55:37-43.
gin or contribution, no specific viral or bacterial agent has been identified. Cholesterol embolism in the alimentary tract occurs either spontaneously4,5 or in response to mechanical6 or pharmacologic7,8 factors. To date there is no description of a cap polyp in the esophagus occurring spontaneously or as an effect of sclerotherapy or ligation of esophageal varices. CASE REPORT A 62-year-old man was hospitalized for follow-up EGD. There was a history of chronic excessive ingestion of alcohol and Child-Turcotte grade A cirrhosis, chronic nicotine use (50 pack-years), and chronic obstructive lung disease. Six months earlier he had been hospitalized at another institution because of acute bleeding from esophageal varices (grade III; classification of Paquet and Oberhammer).9 The bleeding varices were treated by 5 endoscopic sessions over 32 days of combined endoscopic variceal ligation and intravariceal injection of cyanoacrylate (Histoacryl, Aesculap-AG, Tuttlingen, Germany). Discharged in stable condition, the patient was treated with propranolol (40 mg 3 times daily). EGD 3 months before the present evaluation disclosed esophageal varices (grade I) with no red color signs. Rigidity of the esophageal wall and a slight fibrous stricture at 33 cm were found. It was possible to pass the VOLUME 57, NO. 6, 2003
Cap polyp of the esophagus induced by cholesterol embolization
Figure 1. Endoscopic view of esophagus showing reddish, 4-mm diameter nodule with eroded surface at 30 cm. endoscope through the narrowing, and no treatment was considered necessary. The patient was stable at admission. His only complaint was occasional upper abdominal pain. Over the 6 months since the index hospitalization he had abstained from the use of alcohol, and there had been no further bleeding. Examination was unremarkable with the exception of adiposity (height 169 cm, weight 76 kg). Endoscopy disclosed findings of reflux esophagitis (grade I, according to criteria of Savary and Miller),10 residual esophageal varices (grade I) without signs of bleeding, and extensive scar tissue with rigidity of the esophageal wall at 33 cm but no significant stenosis. In addition, a reddish nodule 4 mm in diameter with an eroded surface was noted at 30 cm (Fig. 1), from which biopsy specimens were obtained. Histopathologic evaluation of the specimens disclosed signs of cholesterol embolism with foreign-body giant cell granuloma (Figs. 2 and 3) in blood vessels. There was no evidence of cyanoacrylate embolism. On the basis of these findings, the lesion was termed a “cap-polyp” analogous to the lesions described in the colon and rectum. Further evaluation included laboratory tests as follows: total bilirubin, 1.6 mg/dL (normal: 0.2-1.1 mg/dL); γ-glutamyl transferase, 67 U/L (4-28 U/L); alkaline phosphatase, 198 U/L (60-180 U/L); cholesterol, 218 mg/dL (<200 mg/dL); low-density lipoprotein-cholesterol, 155 mg/dL (<150 mg/dL); high-density lipoprotein-cholesterol, 47 mg/dL (>55 mg/dL); and IgA, 460 mg/L (70-400 mg/L). Albumin, cholinesterase, and prothrombin time were within normal ranges. Chest radiograph, electrocardiogram, and a 24-hour Holter monitor study revealed no pathologic findings. Echocardiography disclosed mild aortic regurgitation; the left atrium was high-normal in size. No cardiac embolic source was found. Transabdominal US demonstrated distinct signs of liver cirrhosis with splenomegaly, moderate arteriosclerosis of the abdominal aorta, cholecystolithiasis, a small cyst of the left kidney, and no evidence of ascites. Duplex US demonstrated a 65% stenosis of the left internal carotid artery and a 50% stenosis of the right internal VOLUME 57, NO. 6, 2003
J Langhorst, M Stolte, G Dobos, et al.
Figure 2. Photomicrograph of biopsy from nodule showing foreign-body giant cell granuloma with evidence of cholesterol embolism (H&E, orig. mag. ×30).
Figure 3. Photomicrograph showing pathognomonic needleshaped lacunae of cholesterol crystal embolization in lumina of arterioles (caused by dissolving the cholesterol crystals during the histochemical processing procedure) (H&E, orig. mag. ×85). carotid artery because of calcified atherosclerotic plaques. Cranial CT without contrast material (because of known allergy) demonstrated no pathologic findings other than mild cerebral atrophy; there were no embolic or ischemic lesions. The patient was discharged in stable condition and treated with a short course of a proton-pump inhibitor. Endoscopy 6 and 12 months later disclosed residual esophageal varices (grade I) but no other abnormal finding.
DISCUSSION “Cap-polyposis” is a rare clinical condition first described in the large intestine by Williams et al.11 in 1985. Although the pathogenesis is unknown, mucosal prolapse has been postulated as an important etiologic factor, based on certain clinical and histopathoGASTROINTESTINAL ENDOSCOPY
793
J Langhorst, M Stolte, G Dobos, et al.
logic similarities.3 Cap polyposis occurs in association with other inflammatory conditions including pseudomembranous colitis and idiopathic chronic inflammatory bowel disease.1 Despite arguments favoring an infectious etiology or contribution, no specific viral or bacterial agent has been identified. Microscopically, cholesterol embolization with a foreign-body giant cell granuloma were evident in the cap-polyp from the esophagus in the present case. The differential diagnosis included cyanoacrylate embolism,12-15 a well-known cause of embolism after intravariceal injection of cyanoacrylate. However, this was effectively ruled out by the histopathologic findings. The phenomenon of cholesterol crystal embolization is defined as dislodgement of separate cholesterol crystals less than 200 µm in size, with occlusion of downstream arterioles.12 The source is usually an atheromatous plaque with an eroded intimal surface. A thrombus subsequently forms and organizes around the embolus. The blood clot is gradually removed, but the cholesterol crystals remain and become encased by intimal tissue and foreign body giant cells. Recanalization of the thrombus takes place between or beside the crystals to form slit-like vascular spaces. The cholesterol crystals are dissolved during the histopathologic staining process, leaving the pathognomonic needleshaped lacunae in the lumina of arterioles that are readily evident in the histologic sections. The clinical presentation of a patient with cholesterol crystal embolization is highly variable, ranging from transient local ischemia to failure of multiple organs. Embolization occurs either spontaneously4,5 or in response to mechanical6 or pharmacologic7,8 factors (e.g., treatment with coumadin or tissue plasminogen activator). Based on a review of 221 cases in 1987, Fine et al.13 found the skin and the kidneys to be the most common sites of cholesterol crystal embolization, along with the spleen and pancreas. Involvement of the alimentary tract was demonstrated in 9.1% of the necropsy cases in which the diagnosis was made. In a large retrospective analysis of cholesterol crystal embolization to the alimentary tract, 96 patients were collected over a 19-year period with a total of 130 cholesterol crystal embolizations found. In agreement with the pathophysiology noted in earlier studies, most of the patients had a history of atherosclerotic disease, as did our patient. Of the 130 documented cholesterol crystal embolizations, 51.1% were located in the large bowel (colon, 55; rectum, 12). Only 2 were located in the esophagus and resulted in an ulcer in one patient and esophagitis in the other. The endoscopic features of cap-polyposis of the colon and rectum have been divided by Esaki et al.14 794
GASTROINTESTINAL ENDOSCOPY
Cap polyp of the esophagus induced by cholesterol embolization
into a semipedunculated type and flat-topped protruding type. In the former, the polyp is characterized by a reddish protrusion with an eroded surface, which is comparable with the polyp in the esophagus of our patient. Microscopically, the colonic cap-polyp consists of elongated, tortuous, and distended crypts covered by a “cap” of mucoid, inflammatory, and fibrinoid granulation tissue.1,2 Histochemical examination, electron microscopy, and mRNA in situ hybridization have demonstrated that the colonic mucus in the large intestine of patients with cap polyposis differs in 3 respects from that of the normal colon in adults: abnormal ultrastructure of the mucus in the goblet cells, predominance of nonsulphated mucins, and abnormal expression of the MUC4, MUC3, and MUC5AC genes.15 Most of these abnormalities have been described in other pathologic conditions, suggesting that the abnormalities observed in the mucus of the patient with cap polyposis are probably secondary rather than primary phenomena. The cap-polyp in the present case appeared 6 months after variceal ligation and intravariceal injection of cyanoacrylate. Risk factors for embolic or ischemic disease included long-standing use of nicotine, a slightly elevated serum cholesterol, and calcified atheromatosis. Despite extensive evaluation, no embolic or ischemic lesion was diagnosed, other than the cholesterol embolism in the esophagus. Several “cap-polyps” have been found in the sigmoid colon that were caused by cholesterol embolism (unpublished observation). In addition to mucosal prolapse and possibly an infectious etiology or contribution, cholesterol embolism should be considered a possible etiologic factor in cap-polyposis. REFERENCES 1. Oriuchi T, Kinouchi Y, Kimura M, Hiwatashi N, Hayakawa T, Watanabe H. Successful treatment of cap polyposis by avoidance of intraluminal trauma: clues to pathogenesis. Am J Gastroenterol 2000;95:2095-8. 2. Peny MO, Noel JC, Haot J, Sokolow Y, Zalcman M, Houben JJ. Cap polyposis: a rare syndrome. Gastroenterol Clin Biol 1998;22:349-52. 3. Campbell AP, Cobb CA, Chapman RW, Kettlewell M, Hoang P, Haot BJ, et al. Cap polyposis—an unusual cause of diarrhoea Gut 1993;34:562-4. 4. Gore I, Collins DP. Spontaneous atheromatous embolization. Am J Clin Pathol 1960;33:416-26. 5. Moolenaar W, Kreunung J, Elderink, Lamers CBHW. Ischemic colitis and acalculous cholecystitis as rare manifestations of cholesterol emboli in the same patient. Am J Gastroenterol 1989;84:1421-2. 6. Gaines PA, Kennedy A, Moorhead P, Cumberland CD, Welsh CL, Rutley MS. Cholesterol embolization: a lethal complication of vascular catheterization. Lancet 1988;1:168-70. 7. Feder W, Auerbach R. “Purple toes”: an uncommon sequela of oral coumarin drug therapy. Ann Intern Med 1961;55:911-17. 8. Shapiro LS. Cholesterol embolization after treatment with tissue plasminogen activator. N Engl J Med 1989;321:1270. VOLUME 57, NO. 6, 2003
Cap polyp of the esophagus induced by cholesterol embolization
J Langhorst, M Stolte, G Dobos, et al.
9. Paquet KJ, Oberhammer E. Sclerotherapy of bleeding esophageal varices by means of endoscopy. Endoscopy 1978; 10:7-11. 10. Savary M, Miller G. Der ösophagus. Lehrbuch und endoskopischer atlas [German]. Solothurn: Gassmann; 1977. 11. Williams GT, Bussey HJR, Morson BC. Inflammatory “cap” polyps of the large intestine. Br J Surg 1985;72(Suppl):S133. 12. Moolenaar W, Lamers CBHW. Cholesterol crystal embolisation to the alimentary tract. Gut 1996;38:196-200.
13. Fine MK, Kapoor W, Falanga V. Cholesterol crystal embolization: a review of 221 cases in the English literature. Angiology 1987;38:769-84. 14. Esaki M, Matsumoto T, Kobayashi H, Yao T, Nakamura S, Mizuno M, et al. Cap polyposis of the colon and rectum: an analysis of endoscopic findings. Endoscopy 2001;33:262-6. 15. Buisine MP, Colombel JF, Lecomte-Houcke M, Gower P, Aubert JP, Porchet N, et al. Abnormal mucus in cap polyposis. Gut 1998;42:135-8.
Access to Gastrointestinal Endoscopy Online is reserved for all subscribers! Full-text access to Gastrointestinal Endoscopy Online is available for all subscribers. ASGE MEMBER SUBSCRIBERS: To activate your individual online subscription, please visit http://www.asge.org and follow the instructions. NONMEMBER SUBSCRIBERS: To activate your individual online subscription, please visit http://www.mosby.com/gie and follow the prompts to activate your online access. To activate your account, you will need your subscriber account/ membership number, which you can find on your mailing label (note: the number of digits in your subscriber account number varies from 6 to 10 digits). See the example below in which the subscriber account number has been circled: Sample mailing label This is your Nonmember subscriber account number
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VOLUME 57, NO. 6, 2003
GASTROINTESTINAL ENDOSCOPY
795
increased neovascularity in the surrounding capsule; and no remarkable vascularity was recognized within the tumor. The CEUS image also corresponded to the pathologic findings. Although CDFI and CEUS demonstrated that the tumor was vascular only at the periphery, analysis of more cases is necessary to determine whether this finding is characteristic of neurilemoma. EUS-FNA in the present case guided the surgical procedure as a primary pancreatic neoplasm was ruled out and a definitive diagnosis of a neurilemoma was established. To our knowledge, this is the first report of a case of retroperitoneal neurilemoma diagnosed by EUS-FNA. REFERENCES 1. Kim SH, Choi BI, Han MC, Kim YI. Retroperitoneal neurilemoma: CT and MR findings. AJR Am J Roentgenol 1992;159: 1023-6. 2. Hayasaka K, Tanaka Y, Soeda S, Huppert P, Claussen CD. MR findings in primary retroperitoneal schwannoma. Acta Radiol 1999;40:78-82. 3. Das Gupta TK, Brasfield RD, Strong EW, Hajdu SI. Benign solitary schwannomas (neurilemomas). Cancer 1969;24:355-66. 4. Borghese M, Corigliano N, Gabriele R, Antoniozzi A, Izzo L, Barbaro M, et al. Benign schwannoma of the pelvic retroperitoneum. Report of a case and review of the literature. G Chir 2000;21:232-8. 5. Woodruff JM, Selig AM, Crowley K, Allen PW. Schwannoma (neurilemoma) with malignant transformation. A rare, distinctive peripheral nerve tumor. Am J Surg Pathol 1994;18: 882-95.
Cap polyp of the esophagus caused by cholesterol embolization Jost Langhorst, MD, Manfred Stolte, MD, Gustav Dobos, MD, Horst Neuhaus, MD
The “cap polyp” occurs mainly in the rectum and distal descending colon. Microscopically, it consists of elongated, tortuous, and distended crypts covered by a “cap” of inflammatory granulation tissue.1,2 In the few published cases of cap polyposis of the colon, mucosal prolapse, based on abnormal colonic motility, has been postulated as an important etiologic factor.3 Despite some arguments in favor of an infectious oriCurrent affiliations: Department of Medicine, Kliniken EssenMitte, Academic Teaching Hospital of the University of Essen, Department of Pathology, Klinikum Bayreuth, Academic Teaching Hospital of the University of Erlangen, and Department of Medicine, Evangelisches Krankenhaus Düsseldorf, Academic Teaching Hospital of the University of Düsseldorf, Germany. Reprint requests: Jost Langhorst, MD, Department of Internal Medicine, Kliniken Essen-Mitte, Am Deimelsberg 34a, D-45276 Essen, Germany. Copyright © 2003 by the American Society for Gastrointestinal Endoscopy 0016-5107/2003/$30.00 + 0 doi:10.1067/mge.2003.229 792
GASTROINTESTINAL ENDOSCOPY
Cap polyp of the esophagus induced by cholesterol embolization
6. DiMagno EP, Buxton JL, Regan PT, Hattery RR, Wilson DA, Suarez JR, et al. Ultrasonic endoscope. Lancet 1980;22;1:629-31. 7. Nakazawa S, Sugiyama H, Kimoto E, Naito Y. Specifications of endoscopic ultrasonography. Scand J Gastroenterol Suppl 1984;94:1-6. 8. Nakazawa S, Yoshino J, Nakamura T, Yamanaka T, Hase S, Kojima Y, et al. Endoscopic ultrasonography of gastric myogenic tumor. A comparative study between histology and ultrasonography. J Ultrasound Med 1989;8:353-9. 9. Ohashi S, Nakazawa S, Yoshino J. Endoscopic ultrasonography in the assessment of invasive gastric cancer. Scand J Gastroenterol 1989;24:1039-48. 10. Wiersema MJ, Vilmann P, Giovannini M, Chang KJ, Wiersema LM. Endosonography-guided fine-needle aspiration biopsy: diagnostic accuracy and complication assessment. Gastroenterology 1997;112:1087-95. 11. Gress FG, Hawes RH, Savides TJ, Ikenberry SO, Lehman GA. Endoscopic ultrasound-guided fine-needle aspiration biopsy using linear array and radial scanning endosonography. Gastrointest Endosc 1997;45:243-50. 12. Matsui M, Goto H, Niwa Y, Arisawa T, Hirooka Y, Hayakawa T. Preliminary results of fine needle aspiration biopsy histology in upper gastrointestinal submucosal tumors. Endoscopy 1998;30:750-5. 13. McGrath KM, Ballo MS, Jowell PS. Schwannoma of the mediastinum diagnosed by EUS-guided fine needle aspiration. Gastrointest Endosc 2001;53:362-5. 14. O’Toole D, Palazzo L, Arotcarena R, Dancour A, Aubert A, Hammel P, et al. Assessment of complications of EUS-guided fine-needle aspiration. Gastrointest Endosc 2001;53:470-4. 15. Ando N, Goto H, Niwa Y, Hirooka Y, Ohmiya N, Hayakawa T, et al. The diagnosis of GI stromal tumors with EUS-guided fine needle aspiration with immunohistochemical analysis. Gastrointest Endosc 2002;55:37-43.
gin or contribution, no specific viral or bacterial agent has been identified. Cholesterol embolism in the alimentary tract occurs either spontaneously4,5 or in response to mechanical6 or pharmacologic7,8 factors. To date there is no description of a cap polyp in the esophagus occurring spontaneously or as an effect of sclerotherapy or ligation of esophageal varices. CASE REPORT A 62-year-old man was hospitalized for follow-up EGD. There was a history of chronic excessive ingestion of alcohol and Child-Turcotte grade A cirrhosis, chronic nicotine use (50 pack-years), and chronic obstructive lung disease. Six months earlier he had been hospitalized at another institution because of acute bleeding from esophageal varices (grade III; classification of Paquet and Oberhammer).9 The bleeding varices were treated by 5 endoscopic sessions over 32 days of combined endoscopic variceal ligation and intravariceal injection of cyanoacrylate (Histoacryl, Aesculap-AG, Tuttlingen, Germany). Discharged in stable condition, the patient was treated with propranolol (40 mg 3 times daily). EGD 3 months before the present evaluation disclosed esophageal varices (grade I) with no red color signs. Rigidity of the esophageal wall and a slight fibrous stricture at 33 cm were found. It was possible to pass the VOLUME 57, NO. 6, 2003
Cap polyp of the esophagus induced by cholesterol embolization
Figure 1. Endoscopic view of esophagus showing reddish, 4-mm diameter nodule with eroded surface at 30 cm. endoscope through the narrowing, and no treatment was considered necessary. The patient was stable at admission. His only complaint was occasional upper abdominal pain. Over the 6 months since the index hospitalization he had abstained from the use of alcohol, and there had been no further bleeding. Examination was unremarkable with the exception of adiposity (height 169 cm, weight 76 kg). Endoscopy disclosed findings of reflux esophagitis (grade I, according to criteria of Savary and Miller),10 residual esophageal varices (grade I) without signs of bleeding, and extensive scar tissue with rigidity of the esophageal wall at 33 cm but no significant stenosis. In addition, a reddish nodule 4 mm in diameter with an eroded surface was noted at 30 cm (Fig. 1), from which biopsy specimens were obtained. Histopathologic evaluation of the specimens disclosed signs of cholesterol embolism with foreign-body giant cell granuloma (Figs. 2 and 3) in blood vessels. There was no evidence of cyanoacrylate embolism. On the basis of these findings, the lesion was termed a “cap-polyp” analogous to the lesions described in the colon and rectum. Further evaluation included laboratory tests as follows: total bilirubin, 1.6 mg/dL (normal: 0.2-1.1 mg/dL); γ-glutamyl transferase, 67 U/L (4-28 U/L); alkaline phosphatase, 198 U/L (60-180 U/L); cholesterol, 218 mg/dL (<200 mg/dL); low-density lipoprotein-cholesterol, 155 mg/dL (<150 mg/dL); high-density lipoprotein-cholesterol, 47 mg/dL (>55 mg/dL); and IgA, 460 mg/L (70-400 mg/L). Albumin, cholinesterase, and prothrombin time were within normal ranges. Chest radiograph, electrocardiogram, and a 24-hour Holter monitor study revealed no pathologic findings. Echocardiography disclosed mild aortic regurgitation; the left atrium was high-normal in size. No cardiac embolic source was found. Transabdominal US demonstrated distinct signs of liver cirrhosis with splenomegaly, moderate arteriosclerosis of the abdominal aorta, cholecystolithiasis, a small cyst of the left kidney, and no evidence of ascites. Duplex US demonstrated a 65% stenosis of the left internal carotid artery and a 50% stenosis of the right internal VOLUME 57, NO. 6, 2003
J Langhorst, M Stolte, G Dobos, et al.
Figure 2. Photomicrograph of biopsy from nodule showing foreign-body giant cell granuloma with evidence of cholesterol embolism (H&E, orig. mag. ×30).
Figure 3. Photomicrograph showing pathognomonic needleshaped lacunae of cholesterol crystal embolization in lumina of arterioles (caused by dissolving the cholesterol crystals during the histochemical processing procedure) (H&E, orig. mag. ×85). carotid artery because of calcified atherosclerotic plaques. Cranial CT without contrast material (because of known allergy) demonstrated no pathologic findings other than mild cerebral atrophy; there were no embolic or ischemic lesions. The patient was discharged in stable condition and treated with a short course of a proton-pump inhibitor. Endoscopy 6 and 12 months later disclosed residual esophageal varices (grade I) but no other abnormal finding.
DISCUSSION “Cap-polyposis” is a rare clinical condition first described in the large intestine by Williams et al.11 in 1985. Although the pathogenesis is unknown, mucosal prolapse has been postulated as an important etiologic factor, based on certain clinical and histopathoGASTROINTESTINAL ENDOSCOPY
793
J Langhorst, M Stolte, G Dobos, et al.
logic similarities.3 Cap polyposis occurs in association with other inflammatory conditions including pseudomembranous colitis and idiopathic chronic inflammatory bowel disease.1 Despite arguments favoring an infectious etiology or contribution, no specific viral or bacterial agent has been identified. Microscopically, cholesterol embolization with a foreign-body giant cell granuloma were evident in the cap-polyp from the esophagus in the present case. The differential diagnosis included cyanoacrylate embolism,12-15 a well-known cause of embolism after intravariceal injection of cyanoacrylate. However, this was effectively ruled out by the histopathologic findings. The phenomenon of cholesterol crystal embolization is defined as dislodgement of separate cholesterol crystals less than 200 µm in size, with occlusion of downstream arterioles.12 The source is usually an atheromatous plaque with an eroded intimal surface. A thrombus subsequently forms and organizes around the embolus. The blood clot is gradually removed, but the cholesterol crystals remain and become encased by intimal tissue and foreign body giant cells. Recanalization of the thrombus takes place between or beside the crystals to form slit-like vascular spaces. The cholesterol crystals are dissolved during the histopathologic staining process, leaving the pathognomonic needleshaped lacunae in the lumina of arterioles that are readily evident in the histologic sections. The clinical presentation of a patient with cholesterol crystal embolization is highly variable, ranging from transient local ischemia to failure of multiple organs. Embolization occurs either spontaneously4,5 or in response to mechanical6 or pharmacologic7,8 factors (e.g., treatment with coumadin or tissue plasminogen activator). Based on a review of 221 cases in 1987, Fine et al.13 found the skin and the kidneys to be the most common sites of cholesterol crystal embolization, along with the spleen and pancreas. Involvement of the alimentary tract was demonstrated in 9.1% of the necropsy cases in which the diagnosis was made. In a large retrospective analysis of cholesterol crystal embolization to the alimentary tract, 96 patients were collected over a 19-year period with a total of 130 cholesterol crystal embolizations found. In agreement with the pathophysiology noted in earlier studies, most of the patients had a history of atherosclerotic disease, as did our patient. Of the 130 documented cholesterol crystal embolizations, 51.1% were located in the large bowel (colon, 55; rectum, 12). Only 2 were located in the esophagus and resulted in an ulcer in one patient and esophagitis in the other. The endoscopic features of cap-polyposis of the colon and rectum have been divided by Esaki et al.14 794
GASTROINTESTINAL ENDOSCOPY
Cap polyp of the esophagus induced by cholesterol embolization
into a semipedunculated type and flat-topped protruding type. In the former, the polyp is characterized by a reddish protrusion with an eroded surface, which is comparable with the polyp in the esophagus of our patient. Microscopically, the colonic cap-polyp consists of elongated, tortuous, and distended crypts covered by a “cap” of mucoid, inflammatory, and fibrinoid granulation tissue.1,2 Histochemical examination, electron microscopy, and mRNA in situ hybridization have demonstrated that the colonic mucus in the large intestine of patients with cap polyposis differs in 3 respects from that of the normal colon in adults: abnormal ultrastructure of the mucus in the goblet cells, predominance of nonsulphated mucins, and abnormal expression of the MUC4, MUC3, and MUC5AC genes.15 Most of these abnormalities have been described in other pathologic conditions, suggesting that the abnormalities observed in the mucus of the patient with cap polyposis are probably secondary rather than primary phenomena. The cap-polyp in the present case appeared 6 months after variceal ligation and intravariceal injection of cyanoacrylate. Risk factors for embolic or ischemic disease included long-standing use of nicotine, a slightly elevated serum cholesterol, and calcified atheromatosis. Despite extensive evaluation, no embolic or ischemic lesion was diagnosed, other than the cholesterol embolism in the esophagus. Several “cap-polyps” have been found in the sigmoid colon that were caused by cholesterol embolism (unpublished observation). In addition to mucosal prolapse and possibly an infectious etiology or contribution, cholesterol embolism should be considered a possible etiologic factor in cap-polyposis. REFERENCES 1. Oriuchi T, Kinouchi Y, Kimura M, Hiwatashi N, Hayakawa T, Watanabe H. Successful treatment of cap polyposis by avoidance of intraluminal trauma: clues to pathogenesis. Am J Gastroenterol 2000;95:2095-8. 2. Peny MO, Noel JC, Haot J, Sokolow Y, Zalcman M, Houben JJ. Cap polyposis: a rare syndrome. Gastroenterol Clin Biol 1998;22:349-52. 3. Campbell AP, Cobb CA, Chapman RW, Kettlewell M, Hoang P, Haot BJ, et al. Cap polyposis—an unusual cause of diarrhoea Gut 1993;34:562-4. 4. Gore I, Collins DP. Spontaneous atheromatous embolization. Am J Clin Pathol 1960;33:416-26. 5. Moolenaar W, Kreunung J, Elderink, Lamers CBHW. Ischemic colitis and acalculous cholecystitis as rare manifestations of cholesterol emboli in the same patient. Am J Gastroenterol 1989;84:1421-2. 6. Gaines PA, Kennedy A, Moorhead P, Cumberland CD, Welsh CL, Rutley MS. Cholesterol embolization: a lethal complication of vascular catheterization. Lancet 1988;1:168-70. 7. Feder W, Auerbach R. “Purple toes”: an uncommon sequela of oral coumarin drug therapy. Ann Intern Med 1961;55:911-17. 8. Shapiro LS. Cholesterol embolization after treatment with tissue plasminogen activator. N Engl J Med 1989;321:1270. VOLUME 57, NO. 6, 2003
Cap polyp of the esophagus induced by cholesterol embolization
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9. Paquet KJ, Oberhammer E. Sclerotherapy of bleeding esophageal varices by means of endoscopy. Endoscopy 1978; 10:7-11. 10. Savary M, Miller G. Der ösophagus. Lehrbuch und endoskopischer atlas [German]. Solothurn: Gassmann; 1977. 11. Williams GT, Bussey HJR, Morson BC. Inflammatory “cap” polyps of the large intestine. Br J Surg 1985;72(Suppl):S133. 12. Moolenaar W, Lamers CBHW. Cholesterol crystal embolisation to the alimentary tract. Gut 1996;38:196-200.
13. Fine MK, Kapoor W, Falanga V. Cholesterol crystal embolization: a review of 221 cases in the English literature. Angiology 1987;38:769-84. 14. Esaki M, Matsumoto T, Kobayashi H, Yao T, Nakamura S, Mizuno M, et al. Cap polyposis of the colon and rectum: an analysis of endoscopic findings. Endoscopy 2001;33:262-6. 15. Buisine MP, Colombel JF, Lecomte-Houcke M, Gower P, Aubert JP, Porchet N, et al. Abnormal mucus in cap polyposis. Gut 1998;42:135-8.
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