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Extraperitoneal extension of pseudomyxoma peritonei: A case report
GYNECOLOGIC
ONCOLOGY
13, 101-107 (1982)
CASE REPORT Extraperitoneal Extension of Pseudomyxoma Peritonei: A Case Report JACOB ROTMENSCH,M.D., NEIL ROSENSHEIN, M.D.,’
AND
TIM PARMLEY, M.D.
Department of Gynecology and Obstetrics, Divisions of Gynecologic Oncology and Gynecologic, Pathology, The Johns Hopkins Hospital, Baltimore. Maryland 21205 Accepted June 6. 1981
Pseudomyxoma peritonei, a disseminated intraabdominal neoplasm, which is the result of either metaplasia of the abdominal mesothelium or direct implantation of mutinous epithelium, typically does not metastasize outside the peritoneal cavity. A case of welldocumented pseudomyxoma peritonei with extraperitoneal disease in the form of a colonic polyp is presented. Its etiology may be related to the multiple surgical procedures the patient had undergone.
INTRODUCTION Pseudomyxoma peritonei is a disseminated intraabdominal neoplasm consisting of fused peritoneal implants of a single layer of mature, mucus-secreting epithelium. While mitoses are not seen and there is no nuclear atypia, the epithelium is not entirely normal in the sense that it differs slightly from the mucus-secreting epithelium of the endocervix and the gastrointestinal tract. This disease process is the result of either metaplasia of the abdominal mesothelium or direct implantation of mutinous epithelium which is confined to the abdominal cavity. It has been described in association with a mutinous cystadenoma of the ovary, mucocele of the appendix, primary adenocarcinoma of the ovary, malignant and benign teratoma of the ovary, an ovarian fibroma, uterine carcinoma, mutinous adenocarcinoma of the intestine, mutinous adenocarcinoma in an urachal cyst, mucoid omphalomesentric cyst of the umbilicus, and a carcinoma of the bile duct. Werth in 1884 described the first case of pseudomyxoma peritonei secondary to an ovarian mutinous cystadenoma [l]. He attributed the pseudomyxoma peritonei to peritoneal irritation produced by the leakage of mucin causing a metaplastic reaction to take place in the mesothelial cells [2]. The present case of a pseudomyxoma peritonei was secondary to an ovarian mutinous cystadenoma. It demonstrated extraperitoneal disease as an intraluminal polypoid lesion of the colon. ’ To whom reprint requests should be addressed. 101 0090-8258/82/010101-07$01.00/0 Copyright 0 1982 by Academic Press. Inc. All rights of reproduction in any form reserved.
102
ROTMENSCH,
ROSENSHEIN,
AND PARMLEY
CASE REPORT
The patient is a married, white, female, para 1011 with last normal menstrual period at age 52. She presented in May of 1975 at age 53 with an elevated temperature, vaginal spotting, and a physical examination demonstrating a firm mass in the right adnexa. An eniarged right ovary 10 x 8 cm with a smooth surface was found at laparotomy. A total abdominal hysterectomy, right salpingooophorectomy was performed; no left tube and ovary could be identified. The histological examination of the ovary demonstrated benign, tall columnar epithelium of a mutinous type (Fig. 1). While not frankly atypical this epithelium differs from normal mucus-secreting epithelium in that the nuclei are enlarged and are elevated off the basement membrane. Reexploration in November of 1975 for a right pelvic mass demonstrated a 10 x &cm mass adherent to the right pelvic sidewall. Mutinous material was detected diffusely throughout the abdomen. The mass in the pelvis and 15 cm of small bowel were resected. The histologic findings were of benign mutinous epithelium consistent with pseudomyxoma peritonei and were similar to the original ovarian cyst (Fig. 2). In May of 1976, the patient was referred to the Gynecologic Oncology Service of The Johns Hopkins Hospital for a recurrent right pelvic mass which had extended into the vaginal vault. A pefvic mass adherent to the right pelvic
FIG. I.
This muck epithelium
has moderate sized nuclei elevated above the basement membrane.
EXTENSION
FIG. 2. The nuclei
OF PSEUDOMYXOMA
PERITONEI
103
The epithelium at the bottom is a tangential cut through epithelium like that at the top. are larger than those of a normal mucin epithelium and are not on the basement membrane.
sidewall, ureter, and rectosigmoid was found at laparotomy. A resection of the mass and small bowel was performed. Histologic findings were again consistent with the diagnosis of pseudomyxoma peritonei and identical with all the previous tissue. The patient had a third and fourth laparotomy in January of 1977 and January of 1978 for recurrent masses of the right pelvis. All material was consistent with the diagnosis of pseudomyxoma peritonei and histologically identical to the previous material. The patient was admitted in November 1979 with a history of rectal bleeding and a recurrent vaginal lesion. Sigmoidoscopy demonstrated a large polypoid lesion at 10 cm. Barium enema confirmed the presence of a j-cm polypoid mass in the rectosigmoid (Fig. 3). Multiple biopsies of the mass histologically demonstrated granulation tissue and benign mutinous epithelium. At laparotomy an area of invaginated rectosigmoid serosa was noted at the site of the polypoid lesion. This portion of rectosigmoid containing the pedunculated mass was resected. The resected mass consisted of a 6 x 4 x 2.5-cm multilobular polyp filled with a thick gelatinous material and surrounded by normal appearing colonic mucosa (Fig. 4). The histology of the polyp was identical to material excised at previous laparotomies (Fig. 5). Differential staining of both the polyp and the tissue excised at prior laparotomies was PAS positive and Alcian blue negative.
104
ROTMENSCH, ROSENSHEIN, AND PARMLEY
FIG. 3. Barium enema demonstrating polypoid lesion in the colon.
The adjacent colonic mucosa had no atypicalities and on differential staining was PAS and Alcian blue positive. The patient had an uneventful recovery and is currently without evidence of recurrent tumor. DISCUSSION
Since the initial description of pseudomyxoma peritonei it has been difficult to decide whether this entity is either a metaplastic transformation of the peritoneal mesothelium or metastases from a low-grade malignancy [2]. Werth regarded pseudomyxoma peritonei as an intraabdominal process caused by the local reaction of the peritoneum to extruded mucin [3]. Virchow attributed pseu-
EXTENSION OF PSEUDOMYXOMA PERITONEI
105
FIG. 4. A photo of the excised specimen demonstrating the polypoid lesion with surrounding normal colonic mucosa. The invagination of the colonic mucosa can also be appreciated.
domyxoma peritonei to extravasated mucin into the peritoneal cavity, acting as an irritant, and causing the peritoneum to secrete large amounts of gelatinous material called pseudomucin [ 1,4]. Similarly, Woodruff and Sandenbergh believed that leakage of mutinous material produces the dissemination of an agent which acts as a stimulus for peritoneal mesothelium to become a mucin-secreting epithelium [4]. In contrast to these views, Gibbs and Woodruff believed that pseudomyxoma peritonei was caused by the metastatic dissemination of a mutinous adenocarcinoma within the peritoneal cavity [2,5]. Regardless of the histogenesis, pseudomyxoma peritonei is a clinical entity in which the peritoneal cavity is involved to varying degrees by a mucus-secreting epithelium. The mucin is largely of the acid mucopolysaccharide type and the epithelium in general behaves in a benign manner in that it does not metastasize outside the peritoneal cavity. This case of a colonic polyp is unusual in that a pseudomyxoma peritonei has either invaded into or metastasized to the colon, either of which would be atypical
106
ROTMENSCH, ROSENSHEIN, AND PARMLEY
FIG. 5. This epithelium is identical to that seen in Fig. I.
for this lesion. The polypoid lesion is felt to be secondary to and consistent with a pseudomyxoma peritonei and not a primary colonic tumor; as the adjacent colonic mucosa is normal and the epithelium in the stroma of the polyp and on its surface is consistent with the previous lesions removed from this patient. Furthermore, colonic polyps and colonic carcinoma are PAS and Alcian blue positive whereas this polyp was PAS positive and Alcian blue negative [6]. The previously excised tissue diagnosed as pseudomyxoma peritonei also stained PAS positive and Alcian blue negative. The formation of the colonic polyp can be explained by the gradual invagination of an implant of pseudomyxoma peritonei on the serosal surface of the rectosigmoid. This process is plausible if access to the peritoneal cavity was restricted by adhesions from previous laparotomies. The serosal lesion upon expanding may protrude into the lumen of the colon with subsequent polyp formation. The continuing improvement in both operative techniques and postoperative
EXTENSION OF PSEUDOMYXOMA PERITONEI
107
care may permit patients with pseudomyxoma peritonei to live for long periods of time with the secondary consequence of atypical recurrences. REFERENCES 1. Early, K., Stephenson, D., and Davis, W. Giant retroperitoneal mucocele simulating pseudomyxoma peritonei and mutinous adenocarcinoma, Amer. J. Surg. 116, 439-443 (1968). 2. Gibbs, N. Mutinous cystadenoma and cystadenocarcinoma of the veriform appendix with particular reference to mucocele and pseudomyxoma peritonei, J. C/in. Pafhol. 26,413-421 (1973). 3. Qizilbash, A. Mucoceles of the appendix, Arch. Parho/. 99, 548-555 (1975). 4. Sandenbergh, H. A., and Woodruff, J. D. Histogenesis of pseudomyxoma peritonei. A review of 9 cases, Obstet. Gynecol. 49, 339-345 (1977). 5. Woodruff, R., and McDonald, J. R. Benign and malignant cystic tumors of the appendix, Surg. Gynecol. Obstet. 71, 750-755 (1940). 6. Goldman, H., and Si-Chun, M. Mucins in normal and neoplastic gastrointestinal epithelium, Arch. Parho/. 85, 580-586 (1968).
ONCOLOGY
13, 101-107 (1982)
CASE REPORT Extraperitoneal Extension of Pseudomyxoma Peritonei: A Case Report JACOB ROTMENSCH,M.D., NEIL ROSENSHEIN, M.D.,’
AND
TIM PARMLEY, M.D.
Department of Gynecology and Obstetrics, Divisions of Gynecologic Oncology and Gynecologic, Pathology, The Johns Hopkins Hospital, Baltimore. Maryland 21205 Accepted June 6. 1981
Pseudomyxoma peritonei, a disseminated intraabdominal neoplasm, which is the result of either metaplasia of the abdominal mesothelium or direct implantation of mutinous epithelium, typically does not metastasize outside the peritoneal cavity. A case of welldocumented pseudomyxoma peritonei with extraperitoneal disease in the form of a colonic polyp is presented. Its etiology may be related to the multiple surgical procedures the patient had undergone.
INTRODUCTION Pseudomyxoma peritonei is a disseminated intraabdominal neoplasm consisting of fused peritoneal implants of a single layer of mature, mucus-secreting epithelium. While mitoses are not seen and there is no nuclear atypia, the epithelium is not entirely normal in the sense that it differs slightly from the mucus-secreting epithelium of the endocervix and the gastrointestinal tract. This disease process is the result of either metaplasia of the abdominal mesothelium or direct implantation of mutinous epithelium which is confined to the abdominal cavity. It has been described in association with a mutinous cystadenoma of the ovary, mucocele of the appendix, primary adenocarcinoma of the ovary, malignant and benign teratoma of the ovary, an ovarian fibroma, uterine carcinoma, mutinous adenocarcinoma of the intestine, mutinous adenocarcinoma in an urachal cyst, mucoid omphalomesentric cyst of the umbilicus, and a carcinoma of the bile duct. Werth in 1884 described the first case of pseudomyxoma peritonei secondary to an ovarian mutinous cystadenoma [l]. He attributed the pseudomyxoma peritonei to peritoneal irritation produced by the leakage of mucin causing a metaplastic reaction to take place in the mesothelial cells [2]. The present case of a pseudomyxoma peritonei was secondary to an ovarian mutinous cystadenoma. It demonstrated extraperitoneal disease as an intraluminal polypoid lesion of the colon. ’ To whom reprint requests should be addressed. 101 0090-8258/82/010101-07$01.00/0 Copyright 0 1982 by Academic Press. Inc. All rights of reproduction in any form reserved.
102
ROTMENSCH,
ROSENSHEIN,
AND PARMLEY
CASE REPORT
The patient is a married, white, female, para 1011 with last normal menstrual period at age 52. She presented in May of 1975 at age 53 with an elevated temperature, vaginal spotting, and a physical examination demonstrating a firm mass in the right adnexa. An eniarged right ovary 10 x 8 cm with a smooth surface was found at laparotomy. A total abdominal hysterectomy, right salpingooophorectomy was performed; no left tube and ovary could be identified. The histological examination of the ovary demonstrated benign, tall columnar epithelium of a mutinous type (Fig. 1). While not frankly atypical this epithelium differs from normal mucus-secreting epithelium in that the nuclei are enlarged and are elevated off the basement membrane. Reexploration in November of 1975 for a right pelvic mass demonstrated a 10 x &cm mass adherent to the right pelvic sidewall. Mutinous material was detected diffusely throughout the abdomen. The mass in the pelvis and 15 cm of small bowel were resected. The histologic findings were of benign mutinous epithelium consistent with pseudomyxoma peritonei and were similar to the original ovarian cyst (Fig. 2). In May of 1976, the patient was referred to the Gynecologic Oncology Service of The Johns Hopkins Hospital for a recurrent right pelvic mass which had extended into the vaginal vault. A pefvic mass adherent to the right pelvic
FIG. I.
This muck epithelium
has moderate sized nuclei elevated above the basement membrane.
EXTENSION
FIG. 2. The nuclei
OF PSEUDOMYXOMA
PERITONEI
103
The epithelium at the bottom is a tangential cut through epithelium like that at the top. are larger than those of a normal mucin epithelium and are not on the basement membrane.
sidewall, ureter, and rectosigmoid was found at laparotomy. A resection of the mass and small bowel was performed. Histologic findings were again consistent with the diagnosis of pseudomyxoma peritonei and identical with all the previous tissue. The patient had a third and fourth laparotomy in January of 1977 and January of 1978 for recurrent masses of the right pelvis. All material was consistent with the diagnosis of pseudomyxoma peritonei and histologically identical to the previous material. The patient was admitted in November 1979 with a history of rectal bleeding and a recurrent vaginal lesion. Sigmoidoscopy demonstrated a large polypoid lesion at 10 cm. Barium enema confirmed the presence of a j-cm polypoid mass in the rectosigmoid (Fig. 3). Multiple biopsies of the mass histologically demonstrated granulation tissue and benign mutinous epithelium. At laparotomy an area of invaginated rectosigmoid serosa was noted at the site of the polypoid lesion. This portion of rectosigmoid containing the pedunculated mass was resected. The resected mass consisted of a 6 x 4 x 2.5-cm multilobular polyp filled with a thick gelatinous material and surrounded by normal appearing colonic mucosa (Fig. 4). The histology of the polyp was identical to material excised at previous laparotomies (Fig. 5). Differential staining of both the polyp and the tissue excised at prior laparotomies was PAS positive and Alcian blue negative.
104
ROTMENSCH, ROSENSHEIN, AND PARMLEY
FIG. 3. Barium enema demonstrating polypoid lesion in the colon.
The adjacent colonic mucosa had no atypicalities and on differential staining was PAS and Alcian blue positive. The patient had an uneventful recovery and is currently without evidence of recurrent tumor. DISCUSSION
Since the initial description of pseudomyxoma peritonei it has been difficult to decide whether this entity is either a metaplastic transformation of the peritoneal mesothelium or metastases from a low-grade malignancy [2]. Werth regarded pseudomyxoma peritonei as an intraabdominal process caused by the local reaction of the peritoneum to extruded mucin [3]. Virchow attributed pseu-
EXTENSION OF PSEUDOMYXOMA PERITONEI
105
FIG. 4. A photo of the excised specimen demonstrating the polypoid lesion with surrounding normal colonic mucosa. The invagination of the colonic mucosa can also be appreciated.
domyxoma peritonei to extravasated mucin into the peritoneal cavity, acting as an irritant, and causing the peritoneum to secrete large amounts of gelatinous material called pseudomucin [ 1,4]. Similarly, Woodruff and Sandenbergh believed that leakage of mutinous material produces the dissemination of an agent which acts as a stimulus for peritoneal mesothelium to become a mucin-secreting epithelium [4]. In contrast to these views, Gibbs and Woodruff believed that pseudomyxoma peritonei was caused by the metastatic dissemination of a mutinous adenocarcinoma within the peritoneal cavity [2,5]. Regardless of the histogenesis, pseudomyxoma peritonei is a clinical entity in which the peritoneal cavity is involved to varying degrees by a mucus-secreting epithelium. The mucin is largely of the acid mucopolysaccharide type and the epithelium in general behaves in a benign manner in that it does not metastasize outside the peritoneal cavity. This case of a colonic polyp is unusual in that a pseudomyxoma peritonei has either invaded into or metastasized to the colon, either of which would be atypical
106
ROTMENSCH, ROSENSHEIN, AND PARMLEY
FIG. 5. This epithelium is identical to that seen in Fig. I.
for this lesion. The polypoid lesion is felt to be secondary to and consistent with a pseudomyxoma peritonei and not a primary colonic tumor; as the adjacent colonic mucosa is normal and the epithelium in the stroma of the polyp and on its surface is consistent with the previous lesions removed from this patient. Furthermore, colonic polyps and colonic carcinoma are PAS and Alcian blue positive whereas this polyp was PAS positive and Alcian blue negative [6]. The previously excised tissue diagnosed as pseudomyxoma peritonei also stained PAS positive and Alcian blue negative. The formation of the colonic polyp can be explained by the gradual invagination of an implant of pseudomyxoma peritonei on the serosal surface of the rectosigmoid. This process is plausible if access to the peritoneal cavity was restricted by adhesions from previous laparotomies. The serosal lesion upon expanding may protrude into the lumen of the colon with subsequent polyp formation. The continuing improvement in both operative techniques and postoperative
EXTENSION OF PSEUDOMYXOMA PERITONEI
107
care may permit patients with pseudomyxoma peritonei to live for long periods of time with the secondary consequence of atypical recurrences. REFERENCES 1. Early, K., Stephenson, D., and Davis, W. Giant retroperitoneal mucocele simulating pseudomyxoma peritonei and mutinous adenocarcinoma, Amer. J. Surg. 116, 439-443 (1968). 2. Gibbs, N. Mutinous cystadenoma and cystadenocarcinoma of the veriform appendix with particular reference to mucocele and pseudomyxoma peritonei, J. C/in. Pafhol. 26,413-421 (1973). 3. Qizilbash, A. Mucoceles of the appendix, Arch. Parho/. 99, 548-555 (1975). 4. Sandenbergh, H. A., and Woodruff, J. D. Histogenesis of pseudomyxoma peritonei. A review of 9 cases, Obstet. Gynecol. 49, 339-345 (1977). 5. Woodruff, R., and McDonald, J. R. Benign and malignant cystic tumors of the appendix, Surg. Gynecol. Obstet. 71, 750-755 (1940). 6. Goldman, H., and Si-Chun, M. Mucins in normal and neoplastic gastrointestinal epithelium, Arch. Parho/. 85, 580-586 (1968).