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Pathology International 2015; 65: 446–449

doi:10.1111/pin.12289

Letter to the Editor A case of colonic solitary polypoid ganglioneuroma with a feature of inverted hyperplastic polyp To the Editor: We describe herein a case of a solitary polypoid colonic ganglioneuroma, together with a feature of inverted hyperplastic polyp obtained by endoscopic mucosal resection. A 71 year-old-man with occult blood of feces on medical check-up was referred to a digestive medicine specialist at our hospital for investigation. The patient had a past history of nephrotic syndrome and acute ileus. He had no particular family history. Laboratory data did not show anemia, positive systemic inflammation signs or biochemical items. After an upper gastrointestinal endoscopy was performed and confirmed chronic atrophic gastritis alone without esophageal or gastric polyp, a total colonoscopy was performed twice; a total of eight colonic polyps were removed by endoscopic mucosal resection. They were located at the ascending (4), transverse (2), descending (1) and sigmoid colon (1). Among them, a polyp sized approximately 10 mm of the transverse colon was endoscopically removed, and histopathologically examined. The tissue was stained with HE, PAS, alcian blue and elastica von Gieson (EVG). For immunohistochemistry, representative sections were examined by the Envision chem mate kit (Dako, Tokyo, Japan), monoclonal for desmin (Dako, 1:200 dilution), N-CAM (CD56, Novocastra, Tokyo, 1:150 dilution), MUC-2, MUC5AC, MUC1, MUC2, CD10, HIK (Novocastra, 1:100–300 dilutions), CEA (Dako, 1:100 dilution), CAM5.2 (Becton & Dickinson, Tokyo, 1:20 dilution), cytokeratin 20 (Dako, 1:160 dilution), chromogranin A (Dako, 1:1000 dilution), synaptophysin (Dako, 1:20 dilution), CD34 (MBL, Nagoya, Japan, 1:2 dilution), D2-40 (Dako, 1:50 dilution), MIB-1 (Ki-67, Novocastra, 1:200 dilution), and p53 (DO7, Dako, 1:100 dilution), and polyclonal for S100 (Dako, 1:10,000 dilution), neurofilament, NSE, vimentin, and GFAP (Dako, 1:300–800) were respectively used. Microscopically, at the panoramic view of HE staining, a polyp in the transverse colon demonstrated submucosal large mucinous spaces covered with a flat-like mucosal layer. With lengthening of the tubules and an irregular serrated configuration of the tubular lining cells, there appeared to be hyperplastic and partly serrated glands with small cystic change in the submucosa (Fig. 1a,b). There was a long shaped-glands across the muscularis mucosa and continuing partly from mucosa to submucosa (Fig. 2a) With PAS and alcian blue, mucinous substances were positive in the large cystic space of submucosa. Around a large cystic area in the submucosa, tiny bundles of spindle cell proliferation with or without sparse ganglion cells was not sufficiently recognized

on HE, but their spindle cells and cell surfaces of ganglion cells were immunohistochemically strongly positive for S-100 (Fig. 1c) and N-CAM (CD56, Fig. 2c), but negative for GFAP and synaptophysin. The ganglion cells were clearly positive for NSE (Fig. 2d) and neurofilament. The glandular cells of this sessile polyp expressed positive phenotypes of MUC2, CEA, CAM5.2 and CK20, and positive partly for MUC5AC (Fig. 2b), suggesting mixed phenotype of colonic and gastric mucin. But they were negative for MUC1, MUC6, CD10, HIK, and neuroendocrine markers, chromogranin A and synaptophysin. There was almost no venous permeation with EVG and CD34, and also no lymphatic permeation with D2-40. With desmin, muscularis mucosa was slightly interrupted by elongated glands. MIB-1 was localized positively in basal layer of the colonic glands, and p53 immunohistochemically showed no overexpression. As a result, the polyp was judged as an unusual case of colonic ganglioneuroma with a feature of inverted hyperplastic polyp, expressing mixed phenotype of colonic and gastric mucins. In the present patient, seven other polyps were obtained by EMR including five tubular adenomas and two other hyperplastic polyps in the right-sided colon. All these polyps were non-malignant lesions. A ganglioneuroma (GN) of the gastrointestinal tract is rare tumor composed of ganglion cells, nerve fibers and supporting cells.1 GNs of the digestive tract are usually divided into solitary polypoid type and multiple ganglioneuromatosis. The latter multiple lesions may often occur in association with the hamartomas of von Rcklinghausen’s disease (NF-1), multiple endocrine neoplasia (MEN IIb) and Cowden syndrome. On the other hand, the hyperplastic polyp is a benign, nonneoplastic colorectal polyp in which there is lengthening of the tubular glands and an irregular serrated configuration of the tubular surface. Rarely, the deep proliferative zone of a hyperplastic polyp may occur in an endophytic growth toward submucosa, termed inverted hyperplastic polyp or hyperplastic polyp with epithelial misplacement.2,3 Moreover, colonic hamartomatous inverted polyp was described by Allen in 1966, and consisted of elongated crypts with cystic dilatation located mostly in the submucosa of the rectum.4 Thereafter, it has been reported that a case of polypoid colonic hamartomatous inverted polyp at mid-transverse colon showed marked cystic dilatation and proliferation of the smooth muscle in the submucosa.5 Although Yantis et al.3 described 19 cases of hyperplastic polyp with epithelial misplacement (inverted hyperplastic polyp) into the submucosa, and proposed an etiologic consideration such as resulting from local trauma and subsequent protrusion of epithelium into the submucosa through inherently weak areas in the muscularis

© 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd

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Figure 1 (a) The panoramic view of the polyp demonstrated submucosal large cystic glands in the sections of endoscopic mucosal resection. (H&E). (b) The polyp revealed focally serrated cystic glands. (H&E). (c) Immunohistochemically, numerous stromal cells in the submucosa demonstrated diffusely positive findings for S100.

mucosae, they did not refer to hamartomatous inverted polyp at all. Concerning two categories of the inverted polyp, it would be still controversial at present whether they are almost the same lesions in the colon. However, two characteristic points could be pointed out, as follows: (i) inverted hyperplastic polyps demonstrate the serrated structure in mucosal surface of the polyp or in the sumucosal glands; and (ii) those lesions might express immunophenotype of gastric mucin or mixed colonic and gastric mucin. In the present case, we consider a ganglioneuroma together with a feature of inverted hyperplastic polyp, which revealed both serrated glands in the submucosa and mixed immunophenotype of colonic and gastric mucin, rather than that of hamartomatous inverted polyp. In our case, the stromal cells in the polyp were demonstrated with strongly positive immunostainings for S100 and N-CAM (CD56), followed by clearly positive results for NSE and neurofilament in the ganglion cells. According to Shekitka and Sobin1 who have reported on 43 cases of GNs from the AFIP (Armed Forces Institute of Pathology) registry from 1940–1990, 28 polypoid GNs grossly resemble juvenile polyps, adenomas or

hyperplastic polyps. In their report, the GNs were microscopically divided into three patterns: (i) a juvenile polyp-like; (ii) a neurofibroma-like as nodular mucosal and submucosal ganglion and spindle cell proliferation; and (iii) a combination of the juvenile polyp-like and the neurofibroma-like patterns. Our case resembled the third combination pattern, in the rare morphology, mimicking the inverted hyperplastic polyp. It is unclear whether GNs represent hamartomas or benign neoplasms.1,6,7 It is not usual that a solitary GN in the colon might occur associated with neurofibromatosis (NF-1) and multiple endocrine neoplasia type 2b(MEN2B) syndromes. Moreover, polypoidal GNs have been described in association with Cowden syndrome, which has hamartomatous polyps, usually multiple, in the colon as a minor criteria, with a germ line mutation in PTEN gene. In our patient’s case, although genetic analysis of PTEN was not examined in detail, skin lesions of NF-1 were not found clinically. Consequently, the present polyp might be judged as a GN with inverted hyperplastic polyp, with abundant S100- and N-CAM-positive nerve fibers around NSE-positive ganglion cells, but not in normal morphology of Meissner’s plexuses.

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Figure 2 (a) The mucosal glands are connecting with submucosal glands and seemed to show focally serrated glands. (H&E). (b) Immunohistochemically, the mucosal glands partly showed positive findings for MUC5AC. (c) Immunohistochemically, the short-spindle cells around the ganglion cells demonstrated positive for CD56. (d) Immunohistochemically, the ganglion cells were clearly positive for NSE.

However, to our referential investigation, GNs with inverted hyperplastic polyp have not yet been found in the English literature. At the histologic diagnosis of hyperplastic or hamartomatous of colonic polyp, pathologists might be needed carefully to examine both glandular lesion and stromal proliferation, bewaring of unusual morphology.

ACKNOWLEDGMENTS We thank Dr. Huai-Pao Lee and the pathologists of Veterans General Hospital-Kaohsiung in Taiwan, and Dr. Masaru Takase of Dept. of Pathology, Juntendo University, for their pathologic advice in the 3rd Taiwan–Japan conjoint slide conference of IAP in 2010.

DISCLOSURE STATEMENT None declared. Hiroshi Maruyama,1 Yoshio Torii,2 Yasunori Enomoto,3 Akitaka Nonomura4 and Chiho Ohbayashi4 1

Department of Pathology and 2Digestive Medicine, Hoshigaoka Medical Center, JCHO, Hirakata, 3 Department of Diagnostic Pathology, Toho University Medical Center, Ohashi Hospital, Tokyo and 4 Department of Diagnostic Pathology, Nara Medical University, Nara, Kashihara, Japan REFERENCES 1 Shekitka KM, Sobin LH. Ganglioneuromas of the gastrointestinal tract. Am J Surg Pathol 1994; 18: 250–57.

© 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd

Letter to the Editor

2 Sobin LH. Inverted hyperplastic polyps of the colon. Am J Surg Pathol 1985; 9: 265–72. 3 Yantiss RK, Goldman H, Odze RD. Hyperplastic polyp with epithelial misplacement (inverted hyperplastic polyp): A clinicopathologic and immunohistochemical study of 19 cases. Mod Pathol 2001; 14: 869–76. 4 Allen MS Jr. Hamartomatous inverted polyps of the rectum. Cancer 1966; 19: 257–65.

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5 Tanaka S, Iwamuro M, Kubota J et al. Polypoid colonic hamartomatous inverted polyp. Dig Endosc 2007; 19: 40–42. 6 Riddell RH, Petras RE, Wiiliams GT, Sobin LH. Ganglioneuroma. In: Rosai J, Sobin LH, eds. Tumors of the Intestines, 3rd, Fasc 32, Atlas of Tumor Pathology. Washington, DC: AFIP, 2003; 49. 7 Al-Daraji WI, Abdelaoui A, Salman WD. Solitary polypoidal rectal ganglioneuroma: A rare presentation of a rare tumor. J Gastroenterol Hepatol 2005; 20: 961–63.

© 2015 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd

A case of colonic solitary polypoid ganglioneuroma with a feature of inverted hyperplastic polyp.

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