Polyp of the colon possessing features of colitis cystica profunda

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  • Polyp of the Colon Possessing Features of Colitis Cystica Profunda* ROBEa'r E. F~C~INER, M.D.

    From the Depctrtn~ent o[ Pathology, Baylor University College of Medicine and Met.hodist Hospital, Houston, Texas

    RECENT articles have directed attention to a lesion characterized by mucus-secreting glands in the colonic wall, which has been named colitis cystica profunda3 -4 In some instances, the lesion has been misdiagnosed clinically and pathologically as adenocar- cinoma.a, 4 Because of the rarity of colitis wstica profunda and its possible confusion with carcinoma, the following case report is presented.

    Gross Pathology: The polyp measured 3.6 cm. in length and averaged 1.3 cm. in diameter. It tapered to a tip I cm. in diameter. The mucosa was intact and had a finely nodular pattern similar to that of the usual adenomatous polyp (Fig. 2). The stalk of the polyp removed in the original polypectomy was a 2.4-cm. segment of normal colonic mucosa. In the cut surface it was observed that the distal third of the stalk was filled with clear mucus. The entire polyp was prepared for microscopic examina- tion.

    Report of a Case

    A 62-year-old man, a school teacher, entered the hospital with a history of bright red blood appear- ing on the surface of formed stools. This had oc- curred intermittently for several months, and was not accompanied by pain or diarrhea. He had been treated for duodenal ulcer in 1939, 1953, and 1959. Roentgenologic examination after a barium enema, in 1959, showed a defect which could not be verified on films taken three days later.

    On admission, physical examination was negative. There were no masses in the abdomen, the rectum was normal on digital examinatidn, and proctoscop~ revealed a normal mucosa. Hemogram and urinaty- sis were normal.

    Roentgenologic examination after a barimn enema revealed a defect with a maximum dimension of 3.5 cm. in the descending colon (Fig. I ) . Lapa- rotomy was performed. A healed ulcer was pal- pated in the pylorus but, with the exception of the colon, the remainder of the abdominal exploration was negative. Situated in the left colon was a freely movable polyp which was removed through a colotomy.

    Microscopic examination of a frozen section was reported as "adenomatous polyp with submucosal mucus retention." The surgeon was informed of the unusual nature of this polyp and, because of uncertainty surrounding this peculiar lesion, a seg- mental resection was performed. It was believed that the added risk of the resection would be less than that of a second procedure, should malignancy be recognized ultimately as the cause of the sub- mucosal glands.

    * Received for publication April 10, 1967.

    Fro. i. RoentgenogTam showing defect in descend- ing colon near sig'moid flexure.

    Fro. 2. Gross specimen showing lobulated exter- nal surface with intact mucosa and broad pedicle.


  • 360 FECHNER

    The segment of colon removed during the second operation was 23 cm. in length and it contained a polyp, 0.5 cm. in diameter, attached to a stalk 0.8 cm. long, situated 7 cm. proximal to the site of polypectomy. Microscopically it was a typical ade- nomatous polyp. Dissection of the removed portion of the mesentery, which was 10 cm. long, revealed that the lymph nodes were normal. Multiple sec- tions of the remainder of the intestine failed to disclose other submucosal abnormalities.

    Microscopic Pathology: The mucosa covering the polyp was thicker than normal. The cytologic structure of many hyperplastic glands was normal. In others, there was a decrease in the goblet cells and the irregular glands were those of an adeno- matous polyp. There were several small defects in the muscularis mucosae and there was continuity between glands of the mucosa and the submucosa (Fig. 4). Some glands, lying in the submucosa, had a pattern similar to that of the surface glands. In other areas, the epithelium was more flattened and many parts of the trapped glands were devoid of epithelium (Fig. 5). A few neutrophils and lymph- ocytes floated in the mucin, but inflammation was negligible. Multiple sections failed to disclose epi- thelial cells within the mucin. All epithelium was confined to the rim of the mucin lakes. There were large deposits of hemosiderin in the submucosal stroma.


    The gross appearance of the po lyp and the microscopic pat tern of much of the mucosa was that of a typical adenomatous polyp. The submucosal extension of glands in such a polyp is un ique in my experience. The di lated, mucus-fi l led glan.ds reminded me of retent ion polyps ( juveni le polyps). Such polyps may be peduncu lated and can occur in pat ients of this age group.7 How- ever, there are two chief differences. Char- acteristically, retent ion polyps are u lcerated over most or all of the surface and the stroma resembles granu lat ion tissue con- ta in ing many neutrophi ls and conspicuous eosinophils. By contrast, the polyp in my pat ient had an intact mucosa and the stroma was fibrous and compact. A well- defined muscular is mucosae is not seen in a retent ion polyp.

    Hamartomatous polyps have a complex g landu lar pat tern which may appear to involve the stalk. In them, there are bands of smooth muscle with g landu lar compo-

    nents produc ing an "arbor izat ion" effect.5 The muscular is mucosae contr ibutes to .this arbor ized smooth muscle and loses its iden- tity. The ep i the l ium of hamartomatous polyps often includes Paneth cells. None of these features was present in my case.

    O'ne disease in which there are wide- spread submucosal glands has been desig- nated as colit is cystica profunda. It includes mul t ip le lesions, covering extensive areas of colon, 2-4 and also sol i tary bu lky lesions. Both d istr ibut ions have been inc luded under this .term and the mucus-secret ing glands in the submucosa have the same characteristics, whether one or many lesions are present. Cytological ly, there are benign eosinophi l ic co lumnar cells and goblet cells. F la t ten ing of the cells is a prominent fea- ture in large sectors of ind iv idua l glands and many areas are devoid of l in ing cells.

    No specific et iology has been ident i f ied and it is possible that there are different causes of the submucosal lesions. Goodal l and Sinclair 4 ment ion dysentery and ulcer- ative colitis as possible precursors. One of the .cases repor ted by Epste in and associates 3 had dysentery 40 years before surgery. In view of the frequency of dysentery and ulcerat ive colitis, the possibi l i ty that they might cu lminate as colitis cystica pro funda must be extremely remote. Another inter- p retat ion was suggested by Al lenA He de- scr ibed three sol i tary lesions, two of which were histological ly s imi lar to colit is cystica profunda. In his second case there were complex g landu lar patterns in the sub- mucosa, which, histological ly, were some- what different f rom other cases. He bel ieved the lesions which he saw probab ly repre- sented a congenital ma l fo rmat ion of the mucosal glands and he designated them as hamartomatous inverted polyps. Whether these sol i tary lesions have anyth ing in com- mon with the more diffuse pat tern remains to be seen. Diffuse submucosal invo lvement with glands in the stomach has been de- scribed. These cases seem analogous to the


    diffuse lesions of the colon and have been interpreted as congenital heterotopia or hamartoma.6, s

    The niceties of terminology or classifi- cation are of less consequence than biologic behavior. In this regard, no patient has had any evidence of malignancy. Periods of follow up have varied from 2 to 10 years in seven cases in which information was available3m

    The patient in this report has had no symptoms during eight months after sur- gery. Growth of the lesion was verified by review of a roentgenogram after bar ium enema, taken seven years prior to surgery. At that time there was a defect in the lower portion of the descending colon, measuring 3.0 by 1.5 by 1.5 cm. (6.753). This was situated at the exact site of the resected polyp. The significance of this lesion was not appreciated seven years ago because a repeat roentgenogram, done three days after the first one, failed to show it. Appar- ently this was due to differences in posi- tioning of the patient. By measuring the defect in the present roentgenogram, the size was calculated at approximately 14 cm. 3 in diameter. Therefore, the doubling time must have been about 2,400 days. This relatively long doubling time is character- istic of most adenomatous polyps2

    Of greatest importance is the incorrect diagnosis of such a lesion as adenocarci- noma. Two of ten patients reported in medical literature have undergone abdom- inoperineal resection because of a clinical diagnosis of carcinoma. 1, 3 One had a pre- operative biopsy in which the possibility of carcinoma was suggested by the pathologist. In three others, carcinoma was seriously considered prior to biopsy or surgery. 3

    Biopsy of such a lesion should not lead to confusion with carcinoma. Cytologically benign cells are present in the submucosal component. Unlike carcinoma, the mucus lakes are lined by a single cell layer. ,Per- haps the most conspicuous feature is the

    lack of epithelial cells within the mucus. This is in contrast to mucinous carcinoma of the colon in which tumor cells float throughout the mucin.3

    The origin of this lesion is speculative. The mucosal changes have some features of an adenomatous polyp, but submucosal glands are not at all characteristic. By con- trast, in cases of colitis cystica profunda, no emphasis has been placed on the appear- ance of the overlying mucosa. However, close examinati