- Email: [email protected]
Mucocele of the appendix caused by endometriosis
Mucocele of the appendix caused by endometriosis ELFRIEDE New
Mu
c OCELE
York,
KOHOUT, New
M.D.*
York
involving
the
flasklike. It was distended in its middle and measured 5.5 by 2.5 cm. The serosa was smooth, glistening, and translucent. The wall of the appendix was thin; at the base, however, it appeared thicker than normal. The lumen contained a large amount of viscid, whitish mucus. Histologic examination of the appendix revealed cylindrical epithelium with numerous goblet cells (Fig. 1) . In the mesenteriolar portion of the appendix was a massive infiltration with endometrial tissue. This was found only in sections from the base of the appendix. The endometrial glands were surrounded by typical endometrial stroma with a loose network of dark-staining fibroblasts (Fig. 2). The glands showed a suggestion of sawtoothing. Some contained numerous erythrocytes. No inflammatory reaction could be found anywhere in the appendix. The lymphoid tissue was almost completely atrophied. The lumen at the base of the appendix was markedly narrowed and compressed by the above-described endometrial implants. There was, however, no complete obstruction.
vermiform
appendix is a relatively common lesion, but a mucocele caused by endometriosis is very rare. An examination of the literature reveals only 3 cases of this kind reported, whereas a case reported by KulikowskaT in 1937 showed endometriosis and focal collections of mucus in the wall of the appendix. Shemiltll described one case in 1952 and Woodruff and MacDonaldzl 2 cases in 1940. These were selected in retrospect and lacked complete surgical examination. Case report This was a 44-year-old housewife with a normal menstrual history. She bore one child at the age of 24. She complained of increasing menorrhagia during the preceding year. There was never a complaint about dysmenorrhea. The only serious disease she could remember having was diphtheria at the age of 2 years. Examination revealed a well-nourished woman in good general condition. Positive findings were limited to the pelvis where an enlarged, nodular, but freely movable uterus was found. The diagnosis of uterine myomas was made, and hysterectomy carried out. The specimen submitted for pathologic examination consisted of a uterus and an appendix. Examination of the uterus confirmed the presence of leiomyomas. The gross appearance of the appendix was
Comment The complete freedom from symptoms was very remarkable in this case; also remarkable was the fact that the infiltration of the appendix was the only proof of ectopic endometrial tissue. There was no adenomyosis of the uterus present, and the symptoms must be attributed solely to the associated myomas of the uterus. The gradual constriction of the base of the appendix by slowly increasing pressure and concurrent gradual distention of the appendix presumably explains the lack of symptoms. A brief review of the literature dealing with mucocele of the vermiform appendix shows that this lesion was first described in
From the Department of Pathology, Misericordia Hospital, Edmonton, Canada, and the Francis Delafield Hospital, Columbia University College of Physicians and Surgeons. *Present address: Pathology, Francis 99 Fort Washington York, New York.
Department Delafield Avenue,
of Hospital, New
1181
118’2
Kohout
Fig. 1. Wall of appendix thelium with numerous duced ?f,.)
Fig.
ing,”
2.
showing
cxAumnar (X150;
goblet cells.
epire-
Endometrial glands suggesting “saw-toothinfiltrating the muscular wall of the ap-
pendix, surrounded by endometrial stroma loose network of dark-staining fibroblasts.
reduced
and a (X150:
>G.1
1842 by Kokitansky’” and later studied by Virchow13 and by Fere.” Woodruff and MacDonaldzl stated that mucocele of the appendix was found in 0.3 per cent (146 cases) of 46,000 appendices removed surgically and examined over a period of 24 years (1916-1940); Weave?? found 0.11 per cent in 6,225 appendectomies; Castle,l 0.2 per cent in 13,159 necropsies. The average age of patients with appendiceal mucoccle is quoted’l as 42.3 years in 136 patients ranging from 4 to 70 years of age. Mucocele develops slowly and is the result of hypersecretion in relation to a slowing down of the processes of absorption and normal drainage. Hypersecretion of mucin
is caused by epithelial changes from appendiceal to large bowel epithelium. There is a gradual localized obstruction of the pt,oxirnal end of the appendix with no interference of the blood supply. As the lesion progresses, however, occlusion results, us11ally because of scar formation from previous inflammation. As a consequence: neuromuscular proliferation develops and the appendix may dilate greatly if the lesion persists or is of long standing. Further changes occur in the appendiceal wall itself. The muscle becomes thinner, the lymphoid tissue flattens, a.nd the entire appendix (rraduallv becomes a. mere sac, lacking .a distinctive histologic features.” Bacteria, which are present initially. are gradually washed out before obstruction is complete. Although the common cause of obstruction is postinflammatory scar formation. 1 1 cases of obstruction due to other causes have been reported by Hilsabeck, Woolner, and Judd.” It must be pointed out, however, that not all mucoceles are caused by complete ohstruction.sl I4 Experiments show that ligation of the appendix leads to inflammation, except when prophylactic measures are taken, such as irrigation with normal saline and sulfaguanidine solution.” Wilson”” estimated the degrc,e of anatomical and functional narrowin? necessary for the formation of appendiceal diverticula and found that an internal circumference of 4 mm. was the critical point at which retention of secretion and consequent obstruction occurred. WangenSteen’” recorded resistance to the flow of fluids into the cccum when injection was attempted through an appendicostomy. IIe suggested the possibility of functional obstruction due to a sphincterlike mechanism of the muscle of the appendiceal wall at the base of the appendix. Wellsl’ found that obstruction by ligature of appendices of rabbits with preservation of the blood supply could lead to the formation of the mucoceIe in from 2 weeks to 2 months. Experimental mucoceles show early hypcrtrophy of the muscle and, later on. a very- thin rmtsculal wall: fibrosis. hyalinization, and calcifica-
Volume
79
Xumher
6
Mucocele
tion6 WiIson20 states that actively contractile muscularis is essential to raise the intraappendicular pressure, and passive distention of the appendix is ineffective. StoutI also considers the muscular force an essential factor in raising intraluminal pressure of the appendix, Williams and Boggon point out the importance of fibrous strictures in the submucosa and state that this is possibly the most common cause of mucoce1e.l” Gross examination of the appendix shows it to be globular, conical, or egg-shaped, depending on the site of obstruction.l* The wall may be thin or transparent, sometimes showing diverticula or perforation. The contents are thick and jelly-like mucus. Some show gelatinous, opalescent contents. Hydrops is rare. Endometriosis of the appendix is likewise a relatively common lesion. Romanus,g among others, reviewed a total of 150 cases of endometriosis. He found the average age of the patient to be 37 to 38 years. Of 11 cases of endometrial implants reported in the appendix, 6 occurred in the tip and one in the mid-portion of the appendix. The location of the balance was not mentioned. Ninety cases of external endometriosis were described by Wallace and associates.‘5 In the total series of 90 cases 5 cases of external endometriosis involving the appendix (5.5 per cent) were found. Collins,3 in his review in 1948, stated that the average age incidence of 116 patients
of
appendix
caused
by
endometriosis
1183
with endometriosis in the appendix collected from the literature was 34.85 years. With reference to the 3 cases of mucocele caused by endometriosis, it is of interest to point out that it has occurred in middIeaged women and gives rise to mild colicky pain localized in the right iliac fossa. Nausea and vomiting may be present. There is no pyrexia and no change in bowel habits. Physical examination often reveals tenderness in the right iliac fossa with guarding of the right rectus muscle. X-ray examination of the right iliac fossa may show a filling defect, a mass which will move with the cecum, a cyst which produces deformities of the cecum, or calcific deposits as features of the mucocele.6 Summary
A symptomless case of endometriosis of the appendix producing a mucocele is described. Only 3 other cases were found in the literature. We are indebted to Dr. Edith Sproul, Associate Professor of Pathology, Columbia University College of Physicians and Surgeons, and Director of Pathology, Francis Delafield Hospital, for her valuable suggestions and critical comment in the preparation of this paper. We also thank Dr. J. R. MacDonald, Director of Misericordia Hospital and Head of the Department of Pathology, for permission to report this case. Mr. Karl Liesner, Edmonton, and Mr. Edward Hajjar, New York, prepared the photomicrographs.
REFERENCES
1. 2.
3.
Castle, Cheng, Collins,
0. L.: Ann. Surg. 61: 582, 1915. K. K.: J. Path. & Bact. 61: 217, 1949. D. C.: A. M. A. Arch. Surg. 63:
11. 12. 13.
617, 1951.
4. 5. 6. 7. 8.
Fere, M.: Prog. Med., Paris 5: 73, 1877. Hilsabeck, J. R., Woolner, L. B., and Judd, E. S., Jr.: Am. J. Surg. 84: 670, 1952. Kalmon, E. H., and Winningham, E. V.: Am. J. Roentgenol. 72: 432, 1954. Kulikowska, L.: Gyntc. et obst. 36: 48, 1937. Lifvendahl, R. A., and Ries, E.: Am. J. Surg.
17: 270, 1932.
9. 10.
Romanus, 1952. Rokitansky, Anatomie, p. 288.
R.:
A. M.
A. Arch.
Surg.
65: 682,
C.: Handbuch der pathologischen Wien, 1842, Braumueller, Aufl.
1,
14.
15. 16. 17. 18. 19. 20. 21.
Shemilt, P.: Brit. J. Surg. 37: 118, 1949. Stout, A. P.: Arch. Surg. 6: 793, 1923. Virchow, R.: Pathologie des tumeurs traduit de l’allemand par Paul Aronssohn, 1867, Ed. 1, p. 247. Vorhaus, M. G.: J. A. M. A. 94: 165, 1930. Wallace, J. T., Nelms, W. F., Nilsson, D. E.: Brooklyn HOSD. T. 10: 63. 1952. Wangensteeq-0: H., et al.: Ann. Surg. 166: 910, 1937. Weaver, C. H.: Am. J. Surg. 36: 523, 1937. Wells, A. Q.: Brit. J. Surg. 24: 766, 1937. Williams, B. W., and Boggon, R. H.: Lancet 1: 9, 1934. Wilson, R. R.: Brit. J. Surg. 38: 65, 1950. Woodruff, R., and MacDonald, J. R.: Surg. Gynec. & Obst. 71: 750, 1940.
Mu
c OCELE
York,
KOHOUT, New
M.D.*
York
involving
the
flasklike. It was distended in its middle and measured 5.5 by 2.5 cm. The serosa was smooth, glistening, and translucent. The wall of the appendix was thin; at the base, however, it appeared thicker than normal. The lumen contained a large amount of viscid, whitish mucus. Histologic examination of the appendix revealed cylindrical epithelium with numerous goblet cells (Fig. 1) . In the mesenteriolar portion of the appendix was a massive infiltration with endometrial tissue. This was found only in sections from the base of the appendix. The endometrial glands were surrounded by typical endometrial stroma with a loose network of dark-staining fibroblasts (Fig. 2). The glands showed a suggestion of sawtoothing. Some contained numerous erythrocytes. No inflammatory reaction could be found anywhere in the appendix. The lymphoid tissue was almost completely atrophied. The lumen at the base of the appendix was markedly narrowed and compressed by the above-described endometrial implants. There was, however, no complete obstruction.
vermiform
appendix is a relatively common lesion, but a mucocele caused by endometriosis is very rare. An examination of the literature reveals only 3 cases of this kind reported, whereas a case reported by KulikowskaT in 1937 showed endometriosis and focal collections of mucus in the wall of the appendix. Shemiltll described one case in 1952 and Woodruff and MacDonaldzl 2 cases in 1940. These were selected in retrospect and lacked complete surgical examination. Case report This was a 44-year-old housewife with a normal menstrual history. She bore one child at the age of 24. She complained of increasing menorrhagia during the preceding year. There was never a complaint about dysmenorrhea. The only serious disease she could remember having was diphtheria at the age of 2 years. Examination revealed a well-nourished woman in good general condition. Positive findings were limited to the pelvis where an enlarged, nodular, but freely movable uterus was found. The diagnosis of uterine myomas was made, and hysterectomy carried out. The specimen submitted for pathologic examination consisted of a uterus and an appendix. Examination of the uterus confirmed the presence of leiomyomas. The gross appearance of the appendix was
Comment The complete freedom from symptoms was very remarkable in this case; also remarkable was the fact that the infiltration of the appendix was the only proof of ectopic endometrial tissue. There was no adenomyosis of the uterus present, and the symptoms must be attributed solely to the associated myomas of the uterus. The gradual constriction of the base of the appendix by slowly increasing pressure and concurrent gradual distention of the appendix presumably explains the lack of symptoms. A brief review of the literature dealing with mucocele of the vermiform appendix shows that this lesion was first described in
From the Department of Pathology, Misericordia Hospital, Edmonton, Canada, and the Francis Delafield Hospital, Columbia University College of Physicians and Surgeons. *Present address: Pathology, Francis 99 Fort Washington York, New York.
Department Delafield Avenue,
of Hospital, New
1181
118’2
Kohout
Fig. 1. Wall of appendix thelium with numerous duced ?f,.)
Fig.
ing,”
2.
showing
cxAumnar (X150;
goblet cells.
epire-
Endometrial glands suggesting “saw-toothinfiltrating the muscular wall of the ap-
pendix, surrounded by endometrial stroma loose network of dark-staining fibroblasts.
reduced
and a (X150:
>G.1
1842 by Kokitansky’” and later studied by Virchow13 and by Fere.” Woodruff and MacDonaldzl stated that mucocele of the appendix was found in 0.3 per cent (146 cases) of 46,000 appendices removed surgically and examined over a period of 24 years (1916-1940); Weave?? found 0.11 per cent in 6,225 appendectomies; Castle,l 0.2 per cent in 13,159 necropsies. The average age of patients with appendiceal mucoccle is quoted’l as 42.3 years in 136 patients ranging from 4 to 70 years of age. Mucocele develops slowly and is the result of hypersecretion in relation to a slowing down of the processes of absorption and normal drainage. Hypersecretion of mucin
is caused by epithelial changes from appendiceal to large bowel epithelium. There is a gradual localized obstruction of the pt,oxirnal end of the appendix with no interference of the blood supply. As the lesion progresses, however, occlusion results, us11ally because of scar formation from previous inflammation. As a consequence: neuromuscular proliferation develops and the appendix may dilate greatly if the lesion persists or is of long standing. Further changes occur in the appendiceal wall itself. The muscle becomes thinner, the lymphoid tissue flattens, a.nd the entire appendix (rraduallv becomes a. mere sac, lacking .a distinctive histologic features.” Bacteria, which are present initially. are gradually washed out before obstruction is complete. Although the common cause of obstruction is postinflammatory scar formation. 1 1 cases of obstruction due to other causes have been reported by Hilsabeck, Woolner, and Judd.” It must be pointed out, however, that not all mucoceles are caused by complete ohstruction.sl I4 Experiments show that ligation of the appendix leads to inflammation, except when prophylactic measures are taken, such as irrigation with normal saline and sulfaguanidine solution.” Wilson”” estimated the degrc,e of anatomical and functional narrowin? necessary for the formation of appendiceal diverticula and found that an internal circumference of 4 mm. was the critical point at which retention of secretion and consequent obstruction occurred. WangenSteen’” recorded resistance to the flow of fluids into the cccum when injection was attempted through an appendicostomy. IIe suggested the possibility of functional obstruction due to a sphincterlike mechanism of the muscle of the appendiceal wall at the base of the appendix. Wellsl’ found that obstruction by ligature of appendices of rabbits with preservation of the blood supply could lead to the formation of the mucoceIe in from 2 weeks to 2 months. Experimental mucoceles show early hypcrtrophy of the muscle and, later on. a very- thin rmtsculal wall: fibrosis. hyalinization, and calcifica-
Volume
79
Xumher
6
Mucocele
tion6 WiIson20 states that actively contractile muscularis is essential to raise the intraappendicular pressure, and passive distention of the appendix is ineffective. StoutI also considers the muscular force an essential factor in raising intraluminal pressure of the appendix, Williams and Boggon point out the importance of fibrous strictures in the submucosa and state that this is possibly the most common cause of mucoce1e.l” Gross examination of the appendix shows it to be globular, conical, or egg-shaped, depending on the site of obstruction.l* The wall may be thin or transparent, sometimes showing diverticula or perforation. The contents are thick and jelly-like mucus. Some show gelatinous, opalescent contents. Hydrops is rare. Endometriosis of the appendix is likewise a relatively common lesion. Romanus,g among others, reviewed a total of 150 cases of endometriosis. He found the average age of the patient to be 37 to 38 years. Of 11 cases of endometrial implants reported in the appendix, 6 occurred in the tip and one in the mid-portion of the appendix. The location of the balance was not mentioned. Ninety cases of external endometriosis were described by Wallace and associates.‘5 In the total series of 90 cases 5 cases of external endometriosis involving the appendix (5.5 per cent) were found. Collins,3 in his review in 1948, stated that the average age incidence of 116 patients
of
appendix
caused
by
endometriosis
1183
with endometriosis in the appendix collected from the literature was 34.85 years. With reference to the 3 cases of mucocele caused by endometriosis, it is of interest to point out that it has occurred in middIeaged women and gives rise to mild colicky pain localized in the right iliac fossa. Nausea and vomiting may be present. There is no pyrexia and no change in bowel habits. Physical examination often reveals tenderness in the right iliac fossa with guarding of the right rectus muscle. X-ray examination of the right iliac fossa may show a filling defect, a mass which will move with the cecum, a cyst which produces deformities of the cecum, or calcific deposits as features of the mucocele.6 Summary
A symptomless case of endometriosis of the appendix producing a mucocele is described. Only 3 other cases were found in the literature. We are indebted to Dr. Edith Sproul, Associate Professor of Pathology, Columbia University College of Physicians and Surgeons, and Director of Pathology, Francis Delafield Hospital, for her valuable suggestions and critical comment in the preparation of this paper. We also thank Dr. J. R. MacDonald, Director of Misericordia Hospital and Head of the Department of Pathology, for permission to report this case. Mr. Karl Liesner, Edmonton, and Mr. Edward Hajjar, New York, prepared the photomicrographs.
REFERENCES
1. 2.
3.
Castle, Cheng, Collins,
0. L.: Ann. Surg. 61: 582, 1915. K. K.: J. Path. & Bact. 61: 217, 1949. D. C.: A. M. A. Arch. Surg. 63:
11. 12. 13.
617, 1951.
4. 5. 6. 7. 8.
Fere, M.: Prog. Med., Paris 5: 73, 1877. Hilsabeck, J. R., Woolner, L. B., and Judd, E. S., Jr.: Am. J. Surg. 84: 670, 1952. Kalmon, E. H., and Winningham, E. V.: Am. J. Roentgenol. 72: 432, 1954. Kulikowska, L.: Gyntc. et obst. 36: 48, 1937. Lifvendahl, R. A., and Ries, E.: Am. J. Surg.
17: 270, 1932.
9. 10.
Romanus, 1952. Rokitansky, Anatomie, p. 288.
R.:
A. M.
A. Arch.
Surg.
65: 682,
C.: Handbuch der pathologischen Wien, 1842, Braumueller, Aufl.
1,
14.
15. 16. 17. 18. 19. 20. 21.
Shemilt, P.: Brit. J. Surg. 37: 118, 1949. Stout, A. P.: Arch. Surg. 6: 793, 1923. Virchow, R.: Pathologie des tumeurs traduit de l’allemand par Paul Aronssohn, 1867, Ed. 1, p. 247. Vorhaus, M. G.: J. A. M. A. 94: 165, 1930. Wallace, J. T., Nelms, W. F., Nilsson, D. E.: Brooklyn HOSD. T. 10: 63. 1952. Wangensteeq-0: H., et al.: Ann. Surg. 166: 910, 1937. Weaver, C. H.: Am. J. Surg. 36: 523, 1937. Wells, A. Q.: Brit. J. Surg. 24: 766, 1937. Williams, B. W., and Boggon, R. H.: Lancet 1: 9, 1934. Wilson, R. R.: Brit. J. Surg. 38: 65, 1950. Woodruff, R., and MacDonald, J. R.: Surg. Gynec. & Obst. 71: 750, 1940.