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 pat tern 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.

Repor t 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 bar ium 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 bar imn enema revealed a defect with a max imum 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 sur rounding this peculiar lesion, a seg- mental resection was performed. I t 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.

359

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.

Discuss ion

T h e gross a p p e a r a n c e of the p o l y p a n d the mic roscop ic p a t t e r n of much of the mucosa was tha t of a typica l a d e n o m a t o u s po lyp . T h e submucosa l ex tens ion of g lands

in such a p o l y p is u n i q u e in my exper ience . T h e d i l a t ed , mucus-f i l led glan.ds r e m i n d e d me of r e t e n t i o n po lyps ( juveni le polyps) . Such po lyps may be p e d u n c u l a t e d a n d can occur in pa t i en t s of this age group.7 How- ever, the re are two chief differences. Char- acter is t ical ly , r e t e n t i o n po lyps are u l c e r a t e d over mos t or al l of the surface a n d the s t roma resembles g r a n u l a t i o n tissue con- t a in ing m a n y n e u t r o p h i l s a n d consp icuous eos inophi l s . By contrast , the po lyp in my

p a t i e n t h a d an in tac t mucosa a n d the s t roma was f ibrous a n d compact . A well- def ined muscu la r i s mucosae is no t seen in a r e t e n t i o n polyp .

H a m a r t o m a t o u s po lyps have a c o m p l e x g l a n d u l a r p a t t e r n which may a p p e a r to involve the stalk. In them, there are bands of smoo th muscle wi th g l a n d u l a r compo-

nen ts p r o d u c i n g a n " a r b o r i z a t i o n " effect.5 T h e muscu la r i s mucosae c o n t r i b u t e s to .this a rbo r i zed smooth musc le a n d loses its iden- tity. T h e e p i t h e l i u m of h a m a r t o m a t o u s po lyps of ten inc ludes P a n e t h cells. N o n e of t h e s e fea tures was p re sen t in m y case.

O'ne disease in wh ich there are wide- sp read submucosa l g lands has been desig- n a t e d as col i t is cystica p r o f u n d a . I t inc ludes m u l t i p l e lesions, cover ing ex tens ive areas of colon, 2-4 a n d also so l i t a ry b u l k y lesions. Bo th d i s t r i bu t i ons have been i n c l u d e d u n d e r this .term a n d the mucus-secre t ing g lands in the submucosa have the same character is t ics , w h e t h e r one or m a n y lesions are present . Cyto logica l ly , there are ben ign eos inoph i l i c c o l u m n a r cells a n d g o b l e t cells. F l a t t e n i n g of the cells is a p r o m i n e n t fea- ture in large sectors of i n d i v i d u a l g lands a n d m a n y areas a re devo id of l i n i n g cells.

N o specific e t io logy has been iden t i f i ed a n d i t is poss ib le tha t t he re are d i f ferent causes of the submucosa l lesions. G o o d a l l a n d S inc la i r 4 m e n t i o n dysen te ry a n d ulcer- a t ive coli t is as poss ib le precursors . One of the .cases r e p o r t e d by E ps t e in a n d associates 3 h a d dysen te ry 40 years before surgery. I n view of the f r equency of dysen te ry a n d u lce ra t ive colit is, the poss ib i l i ty tha t they

m i g h t c u l m i n a t e as coli t is cystica p r o f u n d a mus t be ex t r eme ly remote . A n o t h e r inter- p r e t a t i o n was sugges ted by Al lenA H e de- sc r ibed three so l i t a ry lesions, two of wh ich were h i s to log ica l ly s imi l a r to col i t is cystica p r o f u n d a . I n his second case the re were c o m p l e x g l a n d u l a r p a t t e r n s in the sub- mucosa , which, h is to logica l ly , were some- w h a t d i f ferent f rom o t h e r cases. H e be l i eved the lesions wh ich he saw p r o b a b l y repre- sen ted a congen i t a l m a l f o r m a t i o n of the mucosa l g lands and he des igna t ed them as h a m a r t o m a t o u s i n v e r t e d polyps . W h e t h e r these so l i t a ry lesions have a n y t h i n g in com- m o n wi th the more diffuse p a t t e r n r e m a i n s to be seen. Diffuse submuc osa l i n v o l v e m e n t wi th g lands in the s tomach has been de- scr ibed. T h e s e cases seem ana logous to the

POLYP OF T H E COLON 3 6 1

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

T h e niceties of terminology or classifi- cation are of less consequence than biologic behavior. In this regard, no pat ient 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

T h e pat ient in this report has had no symptoms dur ing eight months after sur- gery. Growth of the lesion was verified by review of a roentgenogram after ba r ium enema, taken seven years prior to surgery. At that t ime there was a defect in the lower port ion 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. T h e 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- t ioning of the patient. By measuring the defect in the present roentgenogram, the size was calculated at approximately 14 cm. 3 in diameter. Therefore, the doubl ing 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. T w o of ten patients reported in medical l i terature 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 epi thel ial cells within the mucus. This is in contrast to mucinous carcinoma of the colon in which tumor cells float throughout the mucin.3

T h e origin of this lesion is speculative. T h e 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 examinat ion of some published photo- graphs shows apparent thickening of the mucosa and perhaps hyperplasia of the colonic glands. 1, 3 If one were to agree that some degree of hyperplasia of the mucosa is a part of colitis cystica profunda, then perhaps our case might be viewed as a variant of that disease (or diseases). A solitary lesion such as was encountered in this case would favor the concept of a localized developmental abnormal i ty or hamartoma.

Summary

Submucosal glands have been observed in a polyp of the colon. Histologically, the submucosal pat tern was similar to that of colitis cystica profunda and inverted hamar- tomatous polyps. T h e major problem in this type of lesion is differentiating it from adenocarcinoma. Histologic criteria are re- viewed which provide the correct diagnosis.

Acknowledgment T h e au tho r wishes to t hank Dr. J o h n W. Over-

street for permiss ion to pub l i sh the case report , and Dr. Har lan J. Spjut for his he lp fu l criticism.

R e f e r e n c e s

1. Allen, M. S., Jr.: Hamar tomatous inverted polyps of the rectum. Cancer. t9: 257, 1966.

2. Castleman, B. (editor): Case Records of the Massachusetts General Hospital (case 41- 1966). New Eng. J. Med. 275: 608, 1966.

3. Epstein, S. E., ~vV. Q. Ascari, R. C. Ablow, W. B. Seaman, and R. Lattes: Colitis cystica pro- funda. Am. J. Clin. Path. 45: 186, 1966.

362 FECHNER

FIG, 3. Mucosa with epithelial changes characteristic of adenomatous polyp (hematoxylin and eosin; x64).

FIG. 4. Glands connect mucosa and submucosa through defect in muscularis mucosae (hematoxylin and eosin; x345).

POLYP OF THE COLON 3 6 3

FIG. 5. Pools of mucin within submucosa (hematoxylin.and eosin; x64).

k :: ?

° 1 X $$

3~

Fro, 6. Benign epithelium and pool of mucin without epithelial l ining (hematoxylin and eosin; x345).

364 FECHNER

4. Goodall, H. B., and I. S. R. Sinclair: Colitis cystica profunda. J. Path. Bact. 73: 33, 1957.

5. Morson, B. C.: Some peculiarities in the histol- ogy of intestinal polyps. Dis. Colon & Rec- tum. 5: 337, 1962.

6. Oberman, H. A., J. G. Lodmell, and N. D. Sower: Diffuse heterotopic cystic malforma- tion of the stomach. New Eng. Med. 269: 909, 1963.

7. Roth, S. I., and E. B. Helwig: Juvenile polyps of the colon and rectum. Cancer. 16:468, 1963.

8. Scott, H. W., Jr., and T. P. B. Payne: Diffuse congenital cystic hyperplasia of stomach clin- ically simulating carcinoma: Repor t of a case. Bull. Johns Hopkins Hosp. 81" 448, 1947.

9. Welin, S., J. Youker, Jr., J. s. Spratt, Jr., F. Linnell , H. J. Spjut, R. E. Johnson, and L. V., Ackerman: T h e rates and pat terns of growth of 375 tumors of the large intest ine and rectum observed serially by double con- trast enema study (Malm6 technique). Am. J. Roentgenol. 90: 673, 1963.