Some uncommon causes of appendiceal mucocele

SOME UNCOMMON CAUSES OF APPENDICEAL MUCOCELE JOHN R . HILSABECK, M .D ., * Fellow in Surgery, Mayo Foundation, University of Minnesota

LEWIS B . WOOLNER, M .D .

AND

EDWARD S . JUDD, JR ., M .D .

Division of Surgical Pathology, Mayo Clinic

Division of Surgery, Mayo Clinic

Rochester, Minnesota N vivo studies" -13 of human beings and animals have established the appendix as a secretory organ which will continue to secrete despite obstruction . The fate of the obstructed appendix in animals is known to be acute appendicitis in the presence of, or mucocele in the absence of, infection . As a matter of fact, mucoccles can be produced consistently in most instances in dogs and rabbits by merely ligating the base of the appendix provided the appendiceal lumen is washed prior to ligation . Available data indicate that mucocele occurs in human beings when the prerequisites found necessary for experimental production of mucocele in animals are fulfilled . Thus a mucocele is likely to be produced in human beings or animals whenever secretion into the appendiceal lumen exceeds loss from it by drainage and by absorption, provided marked intraluminal contamination is absent. Presumably in human beings when proximal blockage is gradual the appendix is able to rid itselFof luminal infection by washing out the bacteria, much as isolated segments of bowel sterilize themselves .' In such a case reintroduction of bacteria would be prevented since the increasing intra-appendiceal pressure effected by continued secretion and progressive obstruction would he higher than the intracecal pressure . That the contents are relatively free of infection is well known and is borne out clinically by the sterile peritonitis resulting from intraperitoneal rupture of the mucocele . The most common factor responsible for formation of mucocele in human beings is obstruction of the appendiceal lumen by cicatricial contraction . Most of the 146 mucoceles reported by Woodruff and McDonald developed distal to a luminal stenosis, which was usually the result of chronic inflammation . Appendices] drainage need not cease altogether to achieve

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formation of mucocelc, nor must intraluminal obstruction be the result of fibrosis . There are reports of mucoccles developing after obstruction from other causes besides inflammatory stricture and Dodge has reported five cases in which obstruction was not present, the lumen being patent in each case . It is the intent of this communication to present and to consider briefly eleven cases of mucocele of the appendix removed surgically at the Mayo Clinic in which the mucocelc was not the result of inflammatory stricture . During the forty-year period which was reviewed appendiceal mucocele had been removed surgically in 258 cases . FORMATION OF MUCOCELE DESPITE PATENT APPENDICEAL LUMEN

Dodge found reports of five eases of appendiceal mucocele in the literature which developed although the lumina were patent . Since then Vorhaus and Latimer have each reported a case of mucocele with an unobstructed lumen and, reportedly, Lifvendahl and Rie3 have observed a similar instance . Since none of the observers mentioned the presence of any cecal lesion, it is not clear why a mucocele was formed in these cases . Dodge was unable to find a cause for the mucoceles in any of the five cases which lie reviewed . Ile suggested that the fault might have been a defective nwscularis propria not strong enough to empty the appendix of secretion . Seven cases of mucoceles which developed despite patent appendiceal fuminaa were encountered at the clinic . In all seven cases the mucoceles were associated with carcinoma in the right portion of the colon . In one case, Case vii, the mucocele was most likely the result of incomplete evacuation of appendiceal secretion subsequent to back pressure from the obstruc-

* Now of Santa Ana, California .

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Case i . Patent proximal appendiceal lumen . (a) Gross section, hematoxylin and eosin, X section, hematoxylin and eosin, X 1g . . i. ; Pi

tion in the ascending colon . In each of the others mucocele was produced because the patent appendiceal orifice was partially obstructed by the growth in the cecum . Evidently drainage was impeded just enough so that continued appendiceal secretion could not be entirely expelled and, in the absence of marked infection, mucoceles were formed . CASE REPORTS CASE t . A man fifty-nine years of age was operated upon because of roentgenographic demonstration of a large polypoid carcinoma of the ascending colon in the region of the hepatic flexure and a pedunculated polypoid lesion 2 cm . in diameter in the descending colon above the level of the iliac crest . The terminal ileum, cecum, appendix, ascending colon and a portion of the transverse colon were resected . The sigmoidal polyp was removed transcolonically . Pathologic examination of the surgical specimen revealed an annular, ulcerative, mucous adenocarcinoma of grade in, to by 6 by 2 cm . The serosa was involved but there was no nodal involvement . The appendix was the site of an unruptured mucocele 8 by 2 by 2 cm . which communicated freely with the cecum . The carcinoma had partially obstructed the base of the appendix to produce an unruptured mucocele . (Fig . la and b .) CASE II . A woman sixty-eight years of age was operated upon because of a large mass in the right portion of the abdomen . A radical right hemicolectomy with removal of a portion of the terminal ileum was performed . Pathologic examination of the surgical specimen revealed an ulcerated adenocarcinoma of the cecum,

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(b) sagittal

grade in, which also had involved the base of the appendix, partially obstructing it and producing a mucocele 2 by 1 .5 cm . Three regional lymph nodes were involved by the carcinoma . CASE m . A woman sixty-two years of age was operated upon because of roentgenographic demonstration of a polypoid lesion in the cecum . An exteriorization procedure was performed removing a portion of the terminal ileum, cecum, appendix and half of the right portion of the colon . Pathologic examination of the surgical specimen revealed an ulcerated, colloid adenocarcinoma of grade u, g by g by 3 cm ., which had been situated in the cecum just over the opening of the appendix . This had partially obstructed the appendiceal lumen to produce a large unruptured mucocele . CASE 1v . A man fifty-four years of age was operated upon because of a palpable, large, mobile mass in the right mid-portion and lower portion of the abdomen which roentgenographically appeared to be a polypoid cecal lesion . Radical right hemicolectomy was performed . Pathologic examination of the surgical specimen revealed a polypoid, intussuscepting, mucous adenocarcinoma of high grade 1, 8 by 6 by 4 cm . There was no nodal or scrosal involvement . The lesion partially obstructed the lumen of the appendix producing an appendiceal mucocele 12 by 2 by 2 cm . CASE v . A male sixty-two years of age, who fourteen years before had undergone a twostage combined abdominoperineal resection at the clinic because of adenocarcinoma, grade u, of the rectum, again underwent operation because of roentgenographic demonstration of a lesion in the cecum . Radical right hemicolec-

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Case VIII . (a) Gross specimen obtained at operation ; note the bulge of the obstructing carcinoid in the proximal portion of the appendix ; (h) sagittal .section demonstrating the ctu'cinoid completely blocking the appendiceal lumen with the production of a mucoeele distally ; (c) henrisection of appendix taken under low power at the point of obstruction showing the carcinoid intraluminally, hematoxylin and eosin, X 8 ; (d) portion of specimen shown in (c) under high power, hematoxylin and eosin, X 20, FIG . 2 .

tomy was performed . Pathologic examination of the surgical specimen revealed an ulcerative, perforating adenocarcinoma, grade t, 7 by 6 by 4 cm . It had partially obstructed the orifice of the appendix to produce a mucoeele g by 2 by 2 cm . which was intact . The carcinoma had involved the serosa and regional lymph nodes . CASE vi . A woman fifty-nine years of age was operated upon because of roentgenographic demonstration of a lesion considered to be carcinoma of the cecum . A two-stage radical right henticolectomy was performed . Pathologic examination of the surgical specimen revealed adenocarcinoma of the cecum, grade ii, which

also had involved the base of the appendix and had partially obstructed it to produce an unruptured mucocele 6 by 2 cm . The regional nodes were not involved . CASE vii . A man was operated upon because of an obstructing lesion in the mid-portion of the ascending colon . A non-resectable, annular adenocarcinoma was found in this location with metastasis to the liver and to the regional lymph nodes . The appendix was removed prophylactically since it " . . . was markedly distended and appeared ready to rupture because of the obstruction in the colon ." Pathologic examination of the appendix reAmerican Journal of Surgery

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vealed a mucoccle 4 .5 by 1 .5 cm . and a diverticulum . The appendiceal lumen was patent . MUCOCELE PRODUCED BY CARCINOID

According to Weiner and Sala theirs was the first case reported in the literature of a mucoccle caused by an obstructing carcinoid . Although not recognized as such Harte and Le Conte apparently observed mucoceles produced by a proximally obstructing carcinoid . Flarte's description of the gross specimen removed at appendectomy on a forty-one year old man was definitely that of a mucocele, and the histologic description of the yellow material occluding the appendix proximally was that of a carcinoid . Le Conte noted the same thing in the case of a twenty-one year old woman . Garrow and Keenan reported a mucoccle that developed distal to an occluding nodule of "cubical cell carcinoma" involving all coats of the appendix . Topping reported a mucocele and a yellowish mass made up of solid groups of cancer cells in the appendix of a twenty year old man, but from his description it is not certain that the probable carcinoid produced the mucocele . Three such cases have been encountered at the clinic . CASE vin . A man twenty-three years of age was operated upon for acute appendicitis. Pathologic examination of the surgical specimen revealed an acutely inflamed mucoccle of the appendix 7 by 3 cm . in size . Complete luminal obstruction was produced proximally by a carcinoid tumor which measured 8 mm . in diameter . (Fig . 2a to d .) CASE Ix . Athirty-seven yearold man underwent appendectomy . Pathologic examination of the surgical specimen revealed a mucocele 5 mm . in diameter at the tip of the appendix . Proximal luninal obstruction of the appendix had been produced by a carcinoid 3 mm . in diameter. CASE x . A woman forty-one years of age underwent appendectomy . Pathologic examination of the surgical specimen revealed a carcinoid 6 by 8 by 5 mm . situated 2 cm . from the tip of the appendix . Distal to the constriction produced by the carcinoid was a mucocele 2 c on . i n diameter. (Fig . 3 .) MUCOCELE PRODUCED BY A POLYP

In the literature reviewed no cases of mucocele of this nature were reported . One case has been encountered at the clinic in which a polyp December, 1952

Fie . 3 . Case x . Carcinoid tumor under low power showing occlusion of the lumen, hematoxylin and eosin, X 22 .

Fic . ¢. Case xi . Cross section through the proximal end of the appendix showing the benign polyp which occluded the lumen . The appendiceal wall has been unfolded .

in the proximal portion of the appendiceal lumen produced a mucocele distal to it . CASE xi . A girl eight years of age underwent ileosigmoidostomy preparatory to undergoing subtotal colectomy for multiple polyposis . The appendix was removed because it was dilated . Pathologic examination of the appendix revealed an adenomatous polyp 2 .5 by 8 by 8 mm . which completely occluded the lumen of the appendix in its proximal portion . An onruptured mucoccle 9 by i cm . was found distal to the polyp . (Fig . 4 .) MUCOCELE PRODUCED BY ENDOMETRIOSIS

Shemilt was of the opinion that his was the only case on record of an appendiceal mucocele produced by a proximally obstructing endometrial implant . There were two cases in the clinic files of appendices with endometriomal implants associated with a mucocele but the



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surgical specimens were so mutilated from previous sectioning that it could not be proved that the endometriomas produced the obstruction,

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CARROW, A . E_ and KEENAN, C . B . Primary carcinoma of the appendix : report of two cases . Ann . Surg ., 48 : 560-562, u)o8. I IARTF, R . I I . Primary carcinoma and sarcoma of the appendix vermifbun . Ann . Sung„ 47 : 968-

LAPI31ER, E . O . Mucocele of the appendix . Am . J . Surg ., 47 : 124 -1 27, 1940 . 6 . LE CONTE, R. G . Carcinoma of the appendix with metastasis to the ileocolic glands . Ann . Surg ., 5.

In the absence of marked intraluminal contamination a mucocele of the appendix may be formed whenever the rate of appendiceal secretion exceeds the rate of absorption and proximal drainage . The majority of mucoceles develop distal to an inflammatory stricture . During a forty-year period eleven cases in which the mucocele was not the result of inflammatory stricture were encountered at the clinic . In seven cases the appendiceal lumen was patent, the mucocele being produced by partially obstructing carcinoma of the cecum ; in three cases obstruction was caused by a carcinoid, and in one case obstruction resulted from a polyp . Two mucoceles were associated with appendiceal endometriomas but it was impossible to prove that the endometrial tissue had caused obstruction .

47 : 10001-05, [908 . 7.

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DODGE, G . E. Cystic dilatation of the vermiform appendix : report of a case of hydropic cyst, with a study of 142 cases . Ann . Surg ., 63 : 334-348,

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DRAGSTEDI, L . It ., DACK, G. M . and KansNER,J . B . Chronic ulcerative colitis : a summary of evidence implicating bacterium necrophorum as an etiologic agent . Ann . Surg ., 1,4 :653-662, 1941 .

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LIFVENDAHL, R . A . and RIES, F . Open communication between appendiceal mueocele and cecum . Am . J . Slurs ., 17 : 270-274, 1932 . SHE.MILr, P . Endometrimna of the caceum causing mueocele of the appendix . Brit . J. Surg ., 37 :

9 . TOPPING, M . H . Carcinoma with mueocele of appendix : report of a case . California cx West,

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Appendiceal Mucocele

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VORHAUS, M . G . Recognition of some of the less common diseases . Duodenal-jejuna) diverticula ; mucocele of the appendix and the cecum . J . A_ M. A ., 94 : 165-169, 1930 . WANGENSTEEN, O . H . and BowERs, W . F . Significance of the obstructive factor in the genesis of acute appendicitis ; an experimental study . Arcb . Surg ., 34 :496-526, 1937 . WANGENSTEEN, O . fl . and DENNIS, C . Experimental proof of the obstructive origin of appendicitis in man . Ann . Sung ., 110 : 629-647, 1939 . WANORNSTEEN, ll, H- and DraxIs, C . The, production of experimental acute appendicitis (with rupture) in higher apes by luminal obstruction . Sum, Cynec . e+ Ohct ., 70 : 799-806, 1940 . WEINER, J . J . and SALA, A . M . Carcinoid tumor of the appendix producing a mucocele . Ain . J. Surg., 71 :420-422, 1946 .

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WOODRUFF, R . and MCDONALD, J . R . Benign and malignant cystic tumors of the appendix . Surg., Gynec . N Obst ., 71 : 750 -755, 1940

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