Successful treatment of ileal pouch desmoids using multimodal chemotherapy with low-dose vinblastine and methotrexate in a patient with familial adenomatous polyposis

We describe a case of multiple desmoid tumors (DT) that developed in an ileal J pouch, which were successfully treated by low-dose combination chemotherapy using vinblastine (VBL) and methotrexate (MTX). Serial changes in the serum d-dimer levels reflected the treatment response. DT involving both the abdominal wall and the ileal pouch developed after total proctocolectomy with ileoanal anastomosis for familial adenomatous polyposis in a 26-year-old female who was treated in an outpatient unit with low-dose VBL and MTX biweekly for 12 months. The treatment response was assessed at routine intervals by physical examination and abdominal computer tomography (CT) imaging. We assessed serial changes in plasma d-dimers, a potential marker for angiogenic activity, during the low-dose VBL and MTX treatment. DT were successfully treated with low-dose VBL and MTX chemotherapy without any significant side effects or pouch sacrifice. Abdominal CT imaging revealed a decrease in tumor size, and the plasma d-dimer levels decreased in association with tumor regression. This case report shows the efficacy of a low-dose combination chemotherapeutic regimen of VBL and MTX for the treatment of ileoanal pouch mesenteric DT in an outpatient setting. In addition, plasma d-dimers may be a marker for desmoid tumor treatment efficacy.     

Successful resection of an ileoanal pouch mesenteric desmoid without pouch sacrifice

PURPOSE: Intra-abdominal desmoid tumors are associated with familial adenomatous polyposis and may compromise ileoanal pouch function after restorative proctocolectomy, particularly if the pouch mesentery is involved. This usually necessitates pouch excision. The aim of this report was to describe the first known case of pouch salvage after surgery for a desmoid tumor arising from the pouch mesentery. METHODS: The management of a desmoid tumor involving an ileoanal pouch in a 21-year-old female is described and the literature reviewed. RESULTS: The tumor was successfully excised completely after failed medical therapy, and pouch function was fully preserved. CONCLUSIONS: This is a unique case highlighting the possibility of pouch salvage when affected by desmoid tumor.     

A Multimodality Approach to Successful Ileal Pouch Desmoid Excision with Pouch Salvage: Report of a Case

Desmoid tumors occur infrequently in patients who undergo proctocolectomy for familial adenomatous polyposis but may result in significant morbidity and mortality depending on the sight of desmoid location. A case of successful ileal pouch salvage using a multimodality approach for treatment of a large ileal pouch associated desmoid tumor is presented. This approach used neoadjuvant chemotherapy to induce a partial response, followed by complete surgical excision with pouch preservation. This is the first reported case of combined chemotherapy and surgical treatment of a desmoid tumor involving an ileal pouch, and the second reported successful attempt at surgical excision with pouch salvage.     

Management of desmoid tumors in patients after ileal pouch-anal anastomosis for familial adenomatous polyposis

PURPOSE: The aim of this study was to review the management of desmoid tumors in patients who have undergone ileal pouch-anal anastomosis for familial adenomatous polyposis and their outcome. METHODS: A retrospective review of case notes was undertaken in a series of 196 patients with familial adenomatous polyposis who had had an ileal pouch-anal anastomosis. Eleven patients were identified in whom desmoid tumor had developed (4 male; median age, 30 (range, 16–43) years). RESULTS: Desmoid tumors developed in the abdominal wall alone in 4 of 11 patients. Three enlarging tumors were resected with no subsequent recurrence. No patient received adjuvant treatment and pouch function remained stable at a median follow-up of eight years. Mesenteric desmoid tumors developed in seven patients; the pouch has been excised in two because of ischemia; two other patients have had defunctioning stomas created because of slow progression of their desmoid tumors. One patient had a single-lumen pouch excised and a new S-pouch inserted, with no subsequent recurrence of the desmoid tumor, and in two patients the mesenteric desmoid tumor has remained unchanged. All of these patients received long-term Clinoril® (sulindac, Merck & Co., Inc., West Point, PA). Although there has been no significant deterioration in pouch function in patients in whom the pouch remainsin situ, the pouch function was significantly worse compared with a control group of patients after ileal pouch-anal anastomosis for familial adenomatous polyposis in whom desmoid tumors did not develop. CONCLUSION: Desmoid tumors developed in 5.6 percent of 196 patients after ileal pouch-anal anastomosis for familial adenomatous polyposis. No patient with abdominal-wall desmoid tumor had a recurrence after local excision. By contrast, mesenteric desmoid tumors eventually resulted in pouch failure in four of seven affected patients.Read at the meeting of The American Society of Colon & Rectal Surgeons, Philadelphia, Pennsylvania, June 22 to 26, 1997.     

Chemotherapy for desmoid tumors in association with familial adenomatous polyposis

PURPOSE: This study was designed to assess the effect of chemotherapy on complex desmoid tumors associated with familial adenomatous polyposis. METHODS: Five patients (3 males, 2 females; age range, 29–45 years) had symptomatic, unresectable intra-abdominal desmoid tumors in association with familial adenomatous polyposis that were unresponsive to conventional medical therapy. Each patient was treated with a cytotoxic chemotherapeutic regimen consisting of doxorubicin and dacarbazine followed by carboplatin and dacarbazine. Response to treatment was assessed by measurement of tumor size using computerized tomography. Follow-up has been for a mean of 22 (range, 10–30) months. RESULTS: One patient has had a complete response, and three patients have had a partial response, with a reduction in tumor volume of at least 50 percent. One patient had a minimum response to treatment and developed a rapid increase in tumor size on cessation of therapy. Complications of treatment included febrile neutropenia, severe epistaxis, and subclavian vein thrombosis. CONCLUSIONS: The cytotoxic chemotherapeutic regimen described is effective in the treatment of selected unresectable desmoid tumors associated with familial adenomatous polyposis and should be considered in symptomatic patients who do not respond to conventional medical therapy.Read at the meeting of The American Society of Colon and Rectal Surgeons, Seattle, Washington, June 9 to 14, 1996. No reprints are available.     

Development of invasive adenocarcinoma following colectomy with ileoanal anastomosis for familial polyposis coli

PURPOSE: Proctocolectomy with ileoanal anastomosis has gained increasing acceptance for the prophylactic treatment of patients with familial polyposis coli. Longterm surveillance of the ileal pouch and the pouch-anal anastomosis has not been emphasized despite concern regarding retained rectal mucosa following the procedure. METHODS: A 34-year-old patient with a strong family history of familial polyposis coli was treated at 14 years of age by single-stage proctocolectomy with straight ileoanal anastomosis. Follow-up proctoscopic examinations revealed development of adenomatous changes at the ileoanal anastomosis. RESULTS: This report presents a patient with familial polyposis coli who developed invasive adenocarcinoma at the ileoanal anastomosis 20 years after proctocolectomy with ileoanal anastomosis. CONCLUSIONS: We stress the need for lifelong proctoscopic surveillance in patients with familial polyposis coli treated by proctocolectomy with ileoanal anastomosis.     

Significance of Incidental Desmoids Identified During Surgery for Familial Adenomatous Polyposis

PURPOSE The behavior of intra-abdominal desmoids in familial adenomatous polyposis is incompletely understood. Findings range from typical mass lesions to flat sheets, termed the desmoid reaction or desmoid precursor lesion. The latter often are incidental findings of uncertain significance. The study was designed to describe the natural history of incidental intra-abdominal desmoid tumors with particular reference to the desmoid reaction.METHODS Patients who underwent laparotomy for familial adenomatous polyposis at the Cleveland Clinic Foundation were identified. The incidence of incidental intra-abdominal desmoid tumors was determined by review of operative records. Intra-abdominal desmoid tumors were classified as mass lesions if three-dimensional or desmoid reaction if two-dimensional. The incidence of clinically apparent intra-abdominal desmoid tumors (typical mass lesions on physical examination or cross-sectional imaging in symptomatic patients) was determined by chart review. The incidence of clinical intra-abdominal desmoid tumors between groups was compared by Fishers exact test.RESULTS A total of 266 patients (153 females; median age, 26 (range, 9–63) years) underwent abdominal surgery for familial adenomatous polyposis. Incidental intra-abdominal desmoid tumors were identified in 34 patients: 8 at the index surgery and 26 at relaparotomy. These lesions influenced the planned procedure in eight cases (26 percent), including preventing ileoanal pouch in 3 of 19 patients in whom this was intended. The median follow-up from the time of identification of intra-abdominal desmoids was 42 (range, 2–178) months at which point four patients (11 percent) had developed clinical intra-abdominal desmoid tumors. There was no significant difference in incidence of clinical intra-abdominal desmoid tumors between mass and desmoid reaction groups (P = 0.27).CONCLUSIONS Incidental intra-abdominal desmoid tumors are a common finding at relaparotomy in patients with familial adenomatous polyposis. These lesions influence planned surgery in a minority of cases. Desmoid reaction may have little bearing on the subsequent development of clinically significant intra-abdominal desmoid tumors.Presented at the meeting of The American Society of Colon and Rectal Surgeons, Chicago, Illinois, June 4 to 8, 2002.Reprints are not available.     

Ileorectal anastomosis--familial adenomatous polyposis.

Familial adenomatous polyposis is a generalized growth disorder. The predominant cause of death is, however, colorectal cancer in the untreated patient. Prophylactic colectomy, whether it be proctocolectomy and ileostomy, colectomy with ileorectal anastomosis, or colectomy with an ileoanal pouch procedure, will dramatically reduce the mortality from large bowel cancer. It is important, however, to realize that because of the generalized nature of this condition and the possibility of developing extracolonic malignancy, the surgical approach chosen should not be considered one that will necessarily cure the disease. Colectomy with ileorectal anastomosis still leaves the potential for rectal cancer. However, death from rectal cancer is distinctly uncommon. The risk of death following ileorectal anastomosis from rectal cancer is actually less than the risk of dying from cancer of the duodenum or from desmoid tumors. Both of these lesions are unpredictable in patients with familial adenomatous polyposis, and usually occur following the patient's prophylactic colectomy. With this low incidence of death from rectal cancer following ileorectal anastomosis it would seem to be debatable whether the complexities of the ileoanal pouch procedure together with its longer recovery and higher complication rate are justified in the routine management of young people with an early diagnosis of familial adenomatous polyposis.     

Anal transitional zone cancer after restorative proctocolectomy and ileoanal anastomosis in familial adenomatous polyposis: report of two cases

PURPOSE: Restorative proctocolectomy with ileal pouch-anal anastomosis is accepted as the surgical treatment of choice for many patients with familial adenomatous polyposis. The risk of cancer developing in the ileal pouch after this surgery is unknown. Cancer may arise from the ileal pouch after restorative proctocolectomy, but that arising from the anal transitional zone has not been documented in familial adenomatous polyposis. We report two cases of this cancer from the anal transitional zone in patients with familial adenomatous polyposis, with a review of the literature. METHODS: All patients with familial adenomatous polyposis treated with restorative proctocolectomy and ileal pouch-anal anastomosis in The Cleveland Clinic were included in the study. Patients whose surveillance biopsy of the anal transitional zone revealed invasive adenocarcinoma were studied. RESULTS: Among a total of 146 patients with familial adenomatous polyposis who underwent restorative proctocolectomy and ileal pouch-anal anastomosis from 1983 to 2001 in our institution, none developed cancer of the anal transitional zone at up to 18 years of follow-up. However, there were two patients, both of whom underwent surgery elsewhere but who were followed up here, who developed invasive adenocarcinoma of the anal transitional zone. In one of them, cancer was diagnosed three years after a double-stapled ileal pouch-anal anastomosis, whereas in the other, cancer occurred eight years after a straight ileoanal anastomosis with mucosectomy.CONCLUSIONS: Cancer may develop in the anal transitional zone after restorative proctocolectomy with ileal pouch-anal anastomosis for familial adenomatous polyposis. Long-term surveillance of the anal transitional zone needs to be emphasized.     

Anal Transitional Zone Cancer After Restorative Proctocolectomy and Ileoanal Anastomosis in Familial Adenomatous Polyposis

PURPOSE: Restorative proctocolectomy with ileal pouch-anal anastomosis is accepted as the surgical treatment of choice for many patients with familial adenomatous polyposis. The risk of cancer developing in the ileal pouch after this surgery is unknown. Cancer may arise from the ileal pouch after restorative proctocolectomy, but that arising from the anal transitional zone has not been documented in familial adenomatous polyposis. We report two cases of this cancer from the anal transitional zone in patients with familial adenomatous polyposis, with a review of the literature. METHODS: All patients with familial adenomatous polyposis treated with restorative proctocolectomy and ileal pouch-anal anastomosis in The Cleveland Clinic were included in the study. Patients whose surveillance biopsy of the anal transitional zone revealed invasive adenocarcinoma were studied. RESULTS: Among a total of 146 patients with familial adenomatous polyposis who underwent restorative proctocolectomy and ileal pouch-anal anastomosis from 1983 to 2001 in our institution, none developed cancer of the anal transitional zone at up to 18 years of follow-up. However, there were two patients, both of whom underwent surgery elsewhere but who were followed up here, who developed invasive adenocarcinoma of the anal transitional zone. In one of them, cancer was diagnosed three years after a double-stapled ileal pouch-anal anastomosis, whereas in the other, cancer occurred eight years after a straight ileoanal anastomosis with mucosectomy. CONCLUSIONS: Cancer may develop in the anal transitional zone after restorative proctocolectomy with ileal pouch-anal anastomosis for familial adenomatous polyposis. Long-term surveillance of the anal transitional zone needs to be emphasized.     

Systemic cytotoxic chemotherapy and radiation therapy for desmoid in familial adenomatous polyposis

Forty-two of 416 familial adenomatous polyposis (FAP) patients in the FAP registry at the Cleveland Clinic had desmoid tumors. The role of cytotoxic chemotherapy and radiation therapy in the management of these patients was investigated. Eight intra-abdominal desmoid tumors were treated by systemic cytotoxic chemotherapy. Two had complete remission, and one had partial remission. Five patients died as a result of the desmoid tumor or late complications of chemotherapy. Three intra-abdominal desmoids were treated by radiation therapy with no response. Neither cytotoxic chemotherapy nor radiation therapy is recommended as a first-choice treatment for intra-abdominal desmoid tumors in patients with FAP.     

Should ileoanal pouch surgery be staged for patients with mucosal ulcerative colitis on immunosuppressives?

Introduction and objective Much debate has revolved around whether patients with mucosal ulcerative colitis (MUC) receiving immunosuppression should be weaned off immunosuppressives before undergoing ileal pouch surgery. Therefore, the aim of this study was to assess the affect of immunosuppressive drugs on postoperative complications after ileoanal pouch surgery. Materials and methods A retrospective medical record review of patients with MUC who underwent ileal pouch surgery while taking immunosuppressive drugs such as azathioprine, 6-mercaptopurine (6-MP), methotrexate, and cyclosporin A was performed. Postoperative complications in the study group were compared to three matched groups: patients with MUC who had ileoanal pouch surgery while taking systemic steroids, patients with MUC not receiving any immunosuppressive drugs, and patients with familial adenomatous polyposis. Results Twenty-two patients with MUC who underwent ileoanal pouch surgery while taking immunosuppressive drugs were identified from a prospectively entered database of patients who had this surgery between 1988 and 2005. All but two patients underwent temporary fecal diversion. Fifteen patients were taking 6-MP or azathioprine; six were on cyclosporine A, and one both on azathioprine and cyclosporine A. Fifteen patients were also taking steroids at the time of ileoanal pouch surgery. Early (within 30 days of surgery) and late complications occurred in 36 and 50% of the study group patients, respectively, but did not significantly differ from a matched group of patients with MUC who did not take immunosuppressive drugs. Patients with familial adenomatous polyposis had a significantly lower long-term complication rate. Conclusion This retrospective case-matched study suggests that the use of immunosuppressive drugs and cyclosporine A may not be associated with an increased rate of complications after ileoanal pouch surgery. This paper was presented at the annual meeting of the American Society of Colon and Rectal Surgeons, June 2–7, 2001, San Diego, CA and at the biennial meeting of the International Society of University Colon and Rectal Surgeons, April 10–14, 2002, Osaka, Japan.     

Secondary reconstruction of an ileal reservoir in patients with failed straight ileoanal pull-through: report of two cases

The ileoanal reservoir is a widely accepted option for the treatment of mucosal ulcerative colitis and familial adenomatous polyposis. Function of an ileoanal anastomosis without the reservoir may be unacceptable. The aim of this study was to assess the technical feasibility of conversion of a straight ileoanal anastomosis to an ileoanal reservoir anastomosis. Two patients underwent straight ileoanal anastomosis and then underwent subsequent conversion to an ileoanal reservoir anastomosis. A 16-year-old girl with mucosal ulcerative colitis and a 38-year-old woman with familial adenomatous polyposis presented with 20–25 bowel movements per day and severe diarrheal-related symptoms within 11 months of ileoanal anastomosis. In each case, the anastomosis was reversed and an ileal Jpouch was fashioned and anastomosed to the dentate line; there was no postoperative morbidity. The 38-year-old patient reported 4–5 bowel movements per day without the need for any medication at 1–8 months after ileostomy closure. The 16-year-old patient is waiting ileostomy closure. In conclusion, it is technically possible to convert a straight ileoanal anastomosis to an ileoanal reservoir anastomosis with expectations of improvement in function. Received: 30 January 2002 / Accepted: 25 September 2002     

Ileoanal Pouch Neoplasia in Familial Adenomatous Polyposis: An Underestimated Threat

PURPOSE Ileal pouch-anal anastomosis is one of the two main options available for the surgical treatment of patients with familial adenomatous polyposis. Its main advantage is the minimal risk of rectal cancer but a possible Achilles heel is the recurrence of epithelial neoplasia at the ileal pouch-anal anastomosis and within the ileal pouch. The significance of ileoanal anastomotic and ileal pouch adenomas is not yet fully appreciated, and there is a false sense of security about this operation. The consequences of worsening pouch polyposis are serious in that endoscopic treatment is unlikely to be an effective way of controlling it. This study has been done to alert those caring for patients with familial adenomatous polyposis to the looming danger of pouch polyposis and to suggest ways to deal with it.METHODS Studies reporting ileoanal pouch adenomas, ileal pouch-anal anastomotic cancers, and ileal pouch cancers in patients with familial adenomatous polyposis were reviewed. Reports of adenomas in Kock pouches and in Brooke ileostomies in the setting of familial adenomatous polyposis were included. The primary end points of the study were the time between pouch construction and the diagnosis of neoplasia, the age of the patients at the diagnosis of neoplasia, and the severity of the neoplasia.RESULTS There were 18 studies reporting pouch neoplasia, 15 with adenomas, and 3 with cancer. Ten were case reports, five were retrospective studies, and three were prospective studies. All three prospective studies showed that the incidence of pouch adenomas increases with time of follow-up and that the severity of the polyposis varies. The median time from pouch construction to diagnosis of pouch adenomas was 4.7 years and the range was 0.5 to 12 years. There were six studies reporting eight patients with cancer at the ileal pouch-anal anastomosis, diagnosed a median of 8 years after pouch construction (range, 3–20 years). One-half of the cancers were locally advanced (T4) and one-half were not (T1 or T2). One-half followed stapled anastomosis and one-half were after mucosectomy. There were eight case reports of cancer described in an ileostomy in patients with familial adenomatous polyposis. The median time from ileostomy construction to the ileostomy cancers was 25 (range, 9–40) years.DISCUSSION The combination of fecal stasis, adenomatous epithelium, and a germline APC mutation is a potent recipe for epithelial neoplasia. There is increasing evidence that this happens in an ileostomy but that the process is much faster in an ileal pouch. Endoscopic treatment of ileal adenomas is likely to be difficult, reducing the options for their control to excising the entire pouch or chemoprevention.Reprints are not available.     

Familial adenomatous polyposis complicated by an intrahepatic desmoid tumor

Desmoids are uncommon proliferations of fibroblasts that occur with disproportionate frequency in patients with familial adenomatous polyposis. They do not metastasize and are histologically benign. Despite this, the unpredictable and often aggressive nature of familial adenomatous polyposis-associated desmoids and their tendency to occur in intra-abdominal sites means that they present a difficult management problem, and they are a leading cause of death in patients with familial adenomatous polyposis who have undergone colectomy. We report a case of a patient with familial adenomatous polyposis who had extensive and aggressive desmoid disease and whose management was further complicated by a large intrahepatic desmoid. There are no previous reports of desmoids occurring in the liver.     

Extended follow-up of patients treated with cytotoxic chemotherapy for intra-abdominal desmoid tumors

BACKGROUND: Cytotoxic chemotherapy can achieve a good initial response in inoperable desmoid tumors that have caused progressive obstruction of the gastrointestinal and urinary tracts and have caused unrelenting pain. METHODS: We have reviewed 8 patients (3 male) with desmoid tumors and familial adenomatous polyposis who underwent cytotoxic chemotherapy for inoperable gastrointestinal obstruction and/or uncontrolled pain. They were treated with doxorubicin and dacarbazine followed by carboplatin and dacarbazine. RESULTS: Follow-up after cytotoxic chemotherapy in the 7 patients for whom it was available was a mean of 42 (range 24-54) months. Two patients achieved complete remission after therapy. Four patients achieved a partial remission after completing all or some of the chemotherapy regimen; of these, three remained in stable remission, whereas the other was lost to follow-up. There were two recurrences that required further therapy; one of these patients was treated with further chemotherapy, which induced a second remission, and the other was treated with pelvic exenteration and has subsequently died. CONCLUSIONS: Most patients had a substantial response to cytotoxic chemotherapy; however, two patients required additional therapy 24 and 30 months after cytotoxic chemotherapy, respectively. Cytotoxic chemotherapy is effective in producing short-term and long-term remission in these difficult patients.     

Surgery for large intra-abdominal desmoid tumors: report of four cases

PURPOSE: Desmoids are rare, locally aggressive but nonmetastasizing fibrous masses that occur sporadically and in association with familial adenomatous polyposis. Therapeutic options are limited, and there is reluctance to operate on mesenteric desmoids because of the risk of complications or recurrence. Consequently, there is an increasing reliance on alternative therapeutic modalities. Nonsurgical options are of variable efficacy, however, and surgery is still required for the complications of desmoids. Each of the last four patients with desmoids presenting to this unit has required life-saving surgery as a result of failure of nonsurgical treatments or the development of complications. METHODS: We present case reports of four consecutive patients with large mesenteric desmoid tumors requiring surgical management. RESULTS: All four patients had massive mesenteric desmoids. Three cases associated with familial adenomatous polyposis had developed their desmoids after colectomy while the sporadic desmoid had continued to grow rapidly after diagnosis at laparotomy. Sulindac and toremifene were unsuccessful in all cases and one patient with familial adenomatous polyposis suffered dramatic erosion of her desmoid through the abdominal wall during antisarcoma chemotherapy. Two others required emergency laparotomy for complications, and the sporadic case underwent elective resection for symptomatic relief. Three had complete excision of their desmoid, and all remained well with no recurrence at a median follow-up of 12 (range, 7-14) months. CONCLUSION: Despite the risks, there remains a role for surgery in the management of large mesenteric desmoids.     

Rare Coincidence of Familial Adenomatous Polyposis and a Retroperitoneal Fibromyxoid Sarcoma: Report of a Case

Purpose Familial adenomatous polyposis is an autosomal-dominant inherited disease with development of as many as thousands of adenomas within colon and rectum. All untreated patients will develop colorectal adenocarcinoma. A variety of extracolonic manifestations can occur, although malignant tumors are rare. An association of familial adenomatous polyposis and sarcomas was reported in a few cases only. Methods We present the exceptional case of a 24-year-old male with genetically verified familial adenomatous polyposis (deletion of 10 base pairs at position 228–237 of exon 15A). The patient underwent prophylactic subtotal proctocolectomy and ileal-pouch rectal anastomosis in 2003. Two years later, an obstruction of the left ureter caused by a retroperitoneal mass was diagnosed. Results Histopathologic findings after complete tumor resection showed a low-grade fibromyxoid sarcoma. CT scan and clinical follow-up through 15 months postoperatively revealed no recurrent tumor growth. Conclusions To our knowledge, this is the first reported case of familial adenomatous polyposis with metachronous retroperitoneal fibromyxoid sarcoma. Proctocolectomy or total colectomy and complete tumor resection is the treatment of choice in this case. In addition to more common semimalignant retroperitoneal desmoid tumors in familial adenomatous polyposis patients, a malignant soft-tissue tumor also has to be considered for differential diagnosis. Presented at the Cancer Congress of Saxony-Anhalt, Magdeburg, Germany, March, 23 to 24, 2007. Reprints are not available.     

Prior pregnancy ameliorates the course of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis

PURPOSE: Intra-abdominal desmoid tumors occur in 12 percent of patients with familial adenomatous polyposis. A minority grow quickly and are lethal, most are relatively inert, and some cause problems by obstructing adjacent organs. Desmoid tumors may be estrogen-dependent, and estrogen-blocking drugs are part of the usual treatment of these tumors. This study was performed to examine the effect of pregnancy on the course of patients with familial adenomatous polyposis and intra-abdominal desmoids. METHODS: All females with familial adenomatous polyposis and an intra-abdominal desmoid treated or followed up at this institution were eligible. Stable, asymptomatic desmoids were followed up yearly with examination and CT scan. Growing or symptomatic desmoids were followed up at least every six months. Maximum tumor size was grouped as follows: <10 cm, 10 to 20 cm, and >20 cm. A change in tumor size was defined as a change of ±50 percent or more of maximum diameter. Stable tumors showed no change in diameter during the study period; variable growth was defined as a significant change in either direction that was followed by a return to previous dimensions or a stabilization of growth. Rapid growth was a doubling of diameter within three months. Pregnant females were compared with nonpregnant females. Subgroups of females were matched for age at diagnosis of desmoid. RESULTS: Twenty-two females had never been pregnant, whereas 25 had been pregnant at least once. Eleven pairs were matched for age. There were no differences between groups in the incidence of extracolonic manifestations of familial adenomatous polyposis, family history of desmoids, number or type of surgeries done for familial adenomatous polyposis, length of follow-up, or time from surgery to desmoid diagnosis. Desmoids in pregnant females had a significantly more benign course: 18 were stable (vs. 6 nonpregnant females), 2 had variable growth (vs. 10), 1 had rapid growth (vs. 5), and 4 disappeared (vs. 1). There were also trends to smaller, less symptomatic tumors requiring treatment less often in pregnant females. CONCLUSIONS: Pregnancy seems to ameliorate the course of abdominal desmoid tumors significantly in females with familial adenomatous polyposis. This finding raises questions about the most appropriate hormonal treatment for these tumors. Perhaps progesterone or prolactin therapy should be tried, alone or in combination with estrogen. If further studies confirm these findings, females with a family history of desmoid tumors should not be advised against pregnancy.Read at The American Society of Colon and Rectal Surgeons' 100th Anniversary and Tripartite Meeting, Washington, D.C., May 1 to 6, 1999.     

Multiple adenomas in terminal ileum 25 years after restorative proctocolectomy for familial adenomatous polyposis

A patient with familial adenomatous polyposis was treated with colectomy, mucosal proctectomy, and a straight ileoanal anastomosis in 1962. Thirteen to 21 years later recurrent adenomas developed at the ileoanal anastomosis, and 25 years after the operation multiple adenomas were found in the terminal ileum up to 12 cm from the ileoanal anastomosis. It is concluded that colectomy and mucosal procectomy, with or without an ileoanal reservoir, does not eliminate the future risk of adenoma formation. Thus, this method should be used only in selected polyposis patients