Abdominal Wall Desmoid Research Articles

Desmoid of the Abdominal Wall: Mimicking an Ovarian Mass

Abdominal wall desmoid tumors (DTs) are a rare group of fibrous tumors of soft tissues and are locally aggressive. These tumors have a strong tendency for local invasion and recurrence without any metastatic potential. In large DTs with predominantly intra-abdominal component, the origin of mass becomes difficult to predict unless a keen observation for origin is done on advanced imaging such as magnetic resonance imaging (MRI). Surgery is the optimal treatment. We report a case of DT in a 32-year-old woman with a history of cesarean section 3 years back who presented with a chief complaint of progressive mass in the lower abdomen, noticed from the last 3 to 4 months. It was diagnosed as an ovarian mass on ultrasonography, but MRI confirmed a large abdominal wall mass. Resection of the tumor was done, and histopathology revealed a spindle cell tumor favoring fibromatosis. Large-sized DTs with predominantly intra-abdominal component may be mistaken for mass with intra-abdominal origin like uterine leiomyoma and ovarian mass as seen in our case. Therefore, careful attention is to be given to the origin if the mass is large and abutting the abdominal wall. MRI plays a crucial role in determining a diagnosis or narrowing the differential diagnosis, thus facilitating effective management.

Massive Abdominal Wall Desmoid Tumor Following Deep Dorsal Vein Arterialization: A Case Report

Massive Abdominal Wall Desmoid Tumor Following Deep Dorsal Vein Arterialization: A Case Report

Management of desmoid disease

Desmoid disease results when the wnt signaling pathway in fibroblasts is constantly active. It is characterized by enlarging tumors and sclerosing sheets of tissue. In patients with familial adenomatous polyposis (FAP) this can happen in the abdomen, the abdominal wall and in extra-abdominal sites. This review describes the clinical sequelae of desmoid disease in patients with FAP and their management. While abdominal wall desmoids cause symptoms due to their increasing size and prominence, they can be resected with a recurrence rate of about 30%. Intra-abdominal desmoid disease often involves the small bowel mesentery and cannot be resected without enterectomy. Various medical options are available for their management but none are predictably successful. A staging system has been developed that predicts mortality and allows rational treatment choices. Specific complications such as obstruction of the ureter and small bowel, small bowel perforation with abscess and fistula, and hemorrhage may need surgical management. Most FAP patients with desmoid disease can live in equilibrium with the desmoids. The keys are to mitigate symptoms while avoiding toxic or dangerous therapy.

Desmoid Tumor: Rare Abdominal Wall Lump in a Multiparous Woman

ABSTRACT Background Desmoids are rare tumors. Abdominal wall desmoids are more common in women of reproductive age, especially postpartum women. Here we present a case of desmoid tumor in a 37-year-old multiparous woman, who came with the complaint of a slowly growing painless abdominal lump. Case report A 37-year-old multiparous female presented with the complaint of a gradually increasing, painless abdominal lump. On physical examination, a well-defined, firm, mobile mass was palpated in the suprapubic region. Ultrasound revealed a homogeneous hypoechoic lesion in the right anterolateral abdominal wall. Computed tomography (CT) of the abdomen showed a well-defined homogeneous mass in relation to the inferior aspect of right rectus abdominis muscle. These clinical and imaging features were highly suggestive of abdominal wall desmoid, which was confirmed on fine needle aspiration cytology and postoperative tumor histopathology. Discussion Desmoids are uncommon benign, locally aggressive fibrous lesions that have an insidious course. Conventionally, these are classified as abdominal desmoids (occurring in abdominal wall, mesentery, or retroperitoneum) and extra-abdominal desmoids (affecting shoulder girdle, trunk, lower extremities, etc.). The definitive diagnosis of desmoid has to be established on histopathology. Wide local excision remains the definitive treatment of abdominal wall desmoids. Radiation therapy, chemotherapy, and endocrine therapy are the other treatment options. How to cite this article Garg M, Prabhakar N, Prakash M, Dahiya D, Khandelwal N. Desmoid Tumor: Rare Abdominal Wall Lump in a Multiparous Woman. J Postgrad Med Edu Res 2018;52(1):31-33.

Abdominal Wall Reconstruction Following Excision of Abdominal Wall Desmoid Tumours

Background: Although lacking metastatic potential, abdominal wall desmoid tumours can grow to large sizes and destruct the surrounding structures. Patients and methods: The current study is a prospective study including 17 patients with abdominal wall desmoid tumours conducted during the period from April 2011 till September 2014 in Ain Shams University and Dar Al-Hekma Hospitals. The patients were treated by radical resection of their tumour followed by immediate reconstruction of the abdominal wall by double face proceed mesh after confirmation of negative resection margins by frozen section technique. Results: All tumours were resected with a safety margin of at least 1 cm as proved by frozen section except for one tumour (5.88%). The mean operative time was 116.18 ±12.61 min (range 95-140 min). The mean amount of intraoperative blood loss was 511.33 ±166.56 mL (range 220-800 mL). There was no intraoperative vascular or organ injury and no postoperative abdominal hypertension. The mean visual analogue scale was 3.29 ±1.05 (range 2-5). The mean hospital stay was 3.35 ±1.11 days (range 2-5 days). Postoperative complications were mild and resolved conservatively including wound seroma in 2 patients (11.76%) and superficial wound infection in 1 patients (5.88%). There was no tumour recurrence or incisional hernia during the follow-up period. Hypertrophic scars occurred in 2 patients (11.76%) and improved with local 2 cortisone and fractional Co laser. Conclusion: Radical resection of abdominal wall desmoid tumours with frozen section confirmation of free margins followed by abdominal wall reconstruction by double face proceed mesh offers the best option for treating such tumours.

Intrathoracic Desmoid Tumor Presenting as Multiple Lung Nodules 13 Years after Previous Resection of Abdominal Wall Desmoid Tumor.

Desmoid tumors are rare soft tissue tumors considered to have locally infiltrative features without distant metastasis until now. Although they are most commonly intraabdominal, very few cases have extra-abdominal locations. The origin of intrathoracic desmoid tumors is predominantly the chest wall with occasional involvement of pleura. True intrathoracic primary desmoid tumors with no involvement of the chest wall or pleura are extremely rare. We recently experienced a case of true intrathoracic desmoid tumor presenting as multiple lung nodules at 13 years after resection of a previous intraabdominal desmoid tumor.

Sporadic Abdominal Wall Desmoid: Is it Time to Change Our First-Line Approach?

The team of the Royal Marsden of London reported a series of 50 patients who underwent the resection of a primary sporadic abdominal wall desmoid tumor (DT). This field is changing rapidly as new data accumulates, and a personalized approach that takes into account the initial tumor size and/or the evolution after initial surveillance is becoming the preferred method. This series confirms recent data indicating the strong predominance (96 %) of females, particularly those at reproductive age, with tumors in this location. During the study period, the authors systematically performed surgical resection following a biopsy. This approach resulted in an abdominal wall defect that required a prosthetic mesh in 94 % of patients. Not surprisingly, prolonged postoperative pain, hernia or infection affected approximately one patient out of five. Interestingly, no obstetrical impairment due to the mesh repair was observed among the ten pregnancies that reached full term. This study confirms the reports of others that the prognosis is good for abdominal wall DT compared with extra-abdominal DT. In other words, excellent local control was achieved with surgery alone, with low but significant morbidity. Combining all studies that examined the resection of abdominal wall DT, the estimated recurrence rate after surgery calculated by the authors is approximately 5 %. The current issue is when and for whom should we propose this efficient surgical resection? The authors acknowledge that their strategy has evolved recently to adopt active surveillance as a first-line therapy, as better knowledge of the disease has been achieved through the most recent analysis. Indeed, a recent study reported on 147 patients with abdominal wall desmoid, 102 of whom underwent an initial observation. Approximately one-third of the patients remained stable and one-third exhibited spontaneous regression. Only 16 % of the patients received operations at 3 years because of pain or progression. The vast majority of progressions occur during the first 3 years after diagnosis. Adopting a ‘wait and see’ approach enables the identification of patients who really require treatment, the best of which is surgery in this location, as shown in this paper. It is likely that abdominal wall desmoids were overtreated in the past, and surgical resection should now only be proposed for selected cases. The risk of a ‘wait and see’ approach is that the progression may create the need for a more extensive operation, and this risk should be weighed against the risk of performing an unnecessary surgery in the majority of patients. Therefore, the main issue is to detect the few patients who are at a higher risk of progression. In this paper, a tumor size greater than 7 cm was the only factor that affected disease-free survival. This threshold was also found in previous papers. Moreover, when we observe significant percentages of progression according to RECIST (Response Evaluation Criteria In Solid Tumors), the baseline tumor size is important to consider. Others factors, such as the location relative to the inguinal ligament and patient compliance to strict initial monitoring should also be taken into consideration. In the event of progression, the best salvage treatment in this location is clearly surgery. Whether medical treatment can be interposed as a secondline before deciding to resort to surgery is under investigation; patients who develop a DT just after pregnancy are likely the best candidates for this approach. The risk of progression for an in situ DT during pregnancy was evaluated recently in a small series (29 patients) and found to be approximately 50 %, but the condition was safely managed in the majority of cases. We need further Society of Surgical Oncology 2014

Results of Surgical Treatment of Anterior Abdominal Wall Desmoid Tumours: 13 Cases Reviewed with Literature

Background : We retrospectively evaluated the results of surgical treatment for anterior abdominal wall desmoid tumours.Methods : Records for 13 patients operated on for desmoid tumours from 1997–2013 were searched for age, gender, abdominal/pelvic surgical history, pregnancy, Gardner’s syndrome, pre-operative radiological examinations, tumour size, multifocality, surgical procedure, tumour presence at surgical margins, recurrence, morbidity, and mortality. Local recurrence-free survival probabilities were estimated by the Kaplan-Meier method and stratified by various clinicopathologi-cal variables.Results : There were 11 female (84,6%) and 2 male (15,4%) patients with a median age of 36 years. Seven (53,8%) patients had previous abdominal/pelvic surgery, five (38,5%) had a history of pregnancy, and one (7,6%) had Gardner’s Syndrome. Two (15,3%) patients had multifocality on their pre-operative radiological examinations. Mean tumour diameter was 4,6 cm (SD 3,2 cm; range 2–12 cm). After the excision of the masses in five (38,5%) patients, synthetic materials were used to close the abdominal wall defects. Two (15,3%) patients with positive surgical margins after surgery were re-operated. Three (23%) patients required a second surgical intervention after the mass excisions were performed. Mean follow-up time was 56,7 months. Recurrence was observed in three patients during follow-up. Increased tumour size, history of previous abdominal/pelvic surgery, and the presence of multifocality had a negative effect on local recurrence-free survival. There was no mortality during follow-up.Conclusions : Desmoid tumours are characterized by high recurrence, even after proper surgical excisions. Preoperative differential diagnoses of these tumours should be done and a post-operative follow-up protocol should be followed.

Spontaneous Regression of Primary Abdominal Wall Desmoid Tumors: More Common than Previously Thought

The relevance of the initial observational approach for desmoid tumors (DTs) remains unclear. We investigated a new conservative management treatment for primary abdominal wall DTs. Data were collected from 147 patients between 1993 and 2012. The initial therapeutic approaches were categorized as front-line surgery [surgery group (SG), n = 41, 28 %] and initial observation or medical treatment [nonsurgery group (NSG), n = 106, 72 %]. The cumulative incidence of the last strategy modification was estimated using competing risk methods with variable censoring times. Of the 147 patients, 143 were female (97 %). In the SG, 27 patients (66 %) required full-thickness abdominal wall mesh repair. In the NSG, 102 patients (96 %) underwent initial observation and four received medical treatment. In the NSG, the 1- and 3-year incidences of changing to medical treatment (no further changes during the follow-up) were 19 % [95 % confidence interval (CI) 11-28] and 25 % (95 % CI 17-35), respectively, and the 1- and 3-year incidences of a final switch to surgery were 14 % (95 % CI 8-22) and 16 % (95 % CI 9-24), respectively. An initial tumor size of >7 cm was associated with a higher strategy modification risk (p = 0.004). Of the 102 patients initially observed, 29 experienced spontaneous regression over a median follow-up period of 32 months. All second-intent resections were macroscopically completed, with R0 resections achieved in 82 % of patients. This study supports an initial nonsurgical approach to abdominal wall DTs ≤7 cm, followed by surgery based on tumor growth in select cases.

Abdominal Wall Desmoid during Pregnancy: Diagnostic Challenges

Background. Desmoids are benign tumors, with local invasive features and no metastatic potential, which have rarely been described to be pregnancy associated. Case. We described the rapid growth of an anterior abdominal wall mass in a 40-year-old pregnant woman. Due to its close proximity to the enlarged uterus, it was misdiagnosed to be a uterine leiomyoma by ultrasound examination. Final tissue diagnosis and radical resection were done at the time of abdominal delivery. Conclusion. Due to the diagnostic limitations of imaging techniques, desmoids should always be considered when the following manifestations are observed in combination: progressive growth of a solitary abdominal wall mass during pregnancy and well-delineated smooth tumor margins demonstrated by imaging techniques. This case emphasizes the importance of entertaining uncommon medical conditions in the differential diagnosis of seemingly common clinical manifestations.

Imaging assessment of desmoid tumours in familial adenomatous polyposis: is state-of-the-art 1.5 T MRI better than 64-MDCT?

Desmoid tumour is a common extraintestinal manifestation of patients with familial adenomatous polyposis (FAP) who have undergone prophylactic colectomy. We aimed to determine whether MRI provides equivalent or better assessment of desmoid tumours than CT, the current first-line investigation. Following ethics approval and informed consent, FAP patients with known desmoid tumour underwent contrast-enhanced 64-slice multidetector CT (MDCT) and 1.5 T MRI (incorporating T(1) weighted, T(2) weighted, short tau inversion-recovery and T(1) weighted with contrast, axial, sagittal and coronal sequences). The number, site, size, local extent, tumour signal intensity and desmoid-to-aorta enhancement ratio were analysed. MRI identified 23 desmoid tumours in 9 patients: 9 intra-abdominal desmoid (IAD) tumours, 10 abdominal wall desmoid (AWD) tumours and 4 extra-abdominal desmoid (EAD) tumours. CT identified only 21 desmoids; 1 EAD and 1 AWD were not identified. The two modalities were equivalent in terms of defining local extent of desmoid. Five IAD tumours involved the bowel, six caused ureteric compression and none compromised the proximal superior mesenteric artery. There was no difference in median desmoid size: 56.7 cm(2) (range 2-215 cm(2)) on MDCT and 56.3 cm(2) (3-215 cm(2)) on MRI (p=0.985). The mean MRI enhancement ratio, at 1.12 (standard deviation 0.43), was greater than the CT enhancement ratio, which was 0.48 (0.16) (p<0.0001). High signal intensity on T(2) MRI was associated with increased MRI enhancement ratio (p=0.006). MRI is at least equivalent (and may be superior) to MDCT for the detection of desmoid tumours in FAP. Coupled with the advantage of avoiding radiation, it should be considered as the primary imaging modality for young FAP patients.

Imaging assessment of desmoid tumours in familial adenomatous polyposis: is state of the art 1.5T MRI better than 64-MDCT?

Objective: Desmoid tumour is a common extraintestinal manifestation of patients with familial adenomatous polyposis (FAP) who have undergone prophylactic colectomy. We aimed to determine whether MRI provides equivalent or better assessment of desmoid tumours than CT, the current first-line investigation. Methods: Following ethics approval and informed consent, FAP patients with known desmoid tumour underwent contrast-enhanced 64-slice multidetector CT (MDCT) and 1.5 T MRI (incorporating T1 weighted, T2 weighted, short tau inversion–recovery and T1 weighted with contrast, axial, sagittal and coronal sequences). The number, site, size, local extent, tumour signal intensity and desmoid-to-aorta enhancement ratio were analysed. Results: MRI identified 23 desmoid tumours in 9 patients: 9 intra-abdominal desmoid (IAD) tumours, 10 abdominal wall desmoid (AWD) tumours and 4 extra-abdominal desmoid (EAD) tumours. CT identified only 21 desmoids; 1 EAD and 1 AWD were not identified. The two modalities were equivalent in terms of defining local extent of desmoid. Five IAD tumours involved the bowel, six caused ureteric compression and none compromised the proximal superior mesenteric artery. There was no difference in median desmoid size: 56.7 cm (range 2–215 cm) on MDCT and 56.3 cm (3–215 cm) on MRI (p50.985). The mean MRI enhancement ratio, at 1.12 (standard deviation 0.43), was greater than the CT enhancement ratio, which was 0.48 (0.16) (p,0.0001). High signal intensity on T2 MRI was associated with increased MRI enhancement ratio (p50.006). Conclusions: MRI is at least equivalent (and may be superior) to MDCT for the detection of desmoid tumours in FAP. Coupled with the advantage of avoiding radiation, it should be considered as the primary imaging modality for young FAP patients. Received 11 December 2010 Revised 22 January 2011 Accepted 16 March 2011

Prognostic Factors Influencing Progression-Free Survival Determined From a Series of Sporadic Desmoid Tumors: A Wait-and-See Policy According to Tumor Presentation

Desmoid tumors are mesenchymal fibroblastic/myofibroblastic proliferations with locoregional aggressiveness and high ability to recur after initial treatment. We present the results of the largest series of sporadic desmoid tumors ever published to determine the prognostic factors of these rare tumors. Four hundred twenty-six patients with a desmoid tumor at diagnosis were included, and the following parameters were studied: age, sex, delay between first symptoms and diagnosis, tumor size, tumor site, previous history of surgery or trauma in the area of the primary tumor, surgical margins, and context of abdominal wall desmoids in women of child-bearing age during or shortly after pregnancy. We performed univariate and multivariate analysis for progression-free survival (PFS). In univariate analysis, age, tumor size, tumor site, and surgical margins (R2 v R0/R1) had a significant impact on PFS. PFS curves were not significantly different for microscopic assessment of surgical resection quality (R0 v R1). In multivariate analysis, age, tumor size, and tumor site had independent values. Three prognostic groups for PFS were defined on the basis of the number of independent unfavorable prognostic factors (0 or 1, 2, and 3). This study clearly demonstrates that there are different prognostic subgroups of desmoid tumors that could benefit from different therapeutic strategies, including a wait-and-see policy.

Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients

Background:The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients.Methods:Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management, and outcome were analysed retrospectively. Progression-free survival (PFS) rates and final outcome were compared for surgical vs non-surgical treatment, for intra-abdominal and extra-abdominal desmoids separately. Also, pharmacological treatment was evaluated for all desmoids.Results:Median follow-up was 8 years. For intra-abdominal desmoids (n=62), PFS rates at 10 years of follow-up were comparable after surgical and non-surgical treatment (33% and 49%, respectively, P=0.163). None of these desmoids could be removed entirely. Eventually, one fifth died from desmoid disease. Most extra-abdominal and abdominal wall desmoids were treated surgically with a PFS rate of 63% and no deaths from desmoid disease. Comparison between NSAID and anti-estrogen treatment showed comparable outcomes. Four of the 10 patients who received chemotherapy had stabilisation of tumour growth, all after doxorubicin combination therapy.Conclusion:For intra-abdominal desmoids, a conservative approach and surgery showed comparable outcomes. For extra-abdominal and abdominal wall desmoids, surgery seemed appropriate. Different pharmacological therapies showed comparable outcomes. If chemotherapy was given for progressively growing intra-abdominal desmoids, most favourable outcomes occurred after combinations including doxorubicin.

Prognostic factors for extra‐abdominal and abdominal wall desmoids: A 20‐year experience at a single institution

Previous reports even large studies discussing the prognosis of desmoids have included tumors from intra- and extra-abdominal sites as well as incomplete resection. The purpose of this study was to explore prognostic factors associated with the recurrence free survival (RFS) rate in surgically treated extra-abdominal and abdominal wall desmoids. A total of 198 consecutive desmoid patients were treated with surgery over a 20-year period at a single institution. Of these, 151 patients with extra-abdominal and abdominal wall tumors were retrospectively reviewed. One hundred thirteen patients were referred for the primary tumor and the other 38 for recurrent disease initially treated elsewhere. All patients underwent a macroscopically complete resection. The median follow-up interval was 102 months. Thirty-one patients (20.5%) had a local recurrence (LR). No patients died of the disease. The 5- and 10-year RFS was 79.7% and 78.5%, respectively. Admission status, gender, tumor size, margin status, location, and number, were predictors of LR in univariate analysis. Tumor size and margin status were independent prognostic factors in multivariate analysis. Positive margins were predictive of recurrence of primary disease, and also showed a trend for recurrent disease, which was not statistically significant. The selective use of adjuvant radiation did not show significant benefit over local control. Regardless of primary or recurrent disease, microscopically negative margins should always be the goal for extra-abdominal desmoids surgery, if no cosmetic defects or function demolition is encountered. Extra-abdominal desmoids deserve more attention and should be treated more aggressively, especially when leaving positive margins.

A comparison of methods for the analysis of low abundance proteins in desmoid tumor cells

The desmoids are a group of rare clinically diverse, deep-seated fibrous neoplasms. The exact etiology is unknown, but several factors are considered to be positively correlated with their development and growth, i.e., genetic and hormonal factors and trauma. These tumors may be sporadic or associated with a genetic disease such as familial adenomatous polyposis (FAP). Devoid of metastatic potential, they tend to form large, infiltrative masses which, if not completely excised, recur repeatedly. Although surgery is widely accepted as the first-line treatment for extra-abdominal and abdominal wall desmoids, a proportion of cases are successfully palliated with either estrogen antagonists (tamoxifen, toremifene, and raloxifene) or nonsteroidal anti-inflammatory drugs. We describe and compare four methods for evaluating the expression of estrogen receptors α/β and COX-1 and COX-2 in desmoid tumor-derived cells and tissues: immunocytochemistry, immunohistochemistry, RT-PCR, and two-color Western blot detection with the Odyssey infrared imaging system. Through this comparative analysis, Western blot with Odyssey was recognized as the best method to analyze the expression particularly of low expressed proteins in desmoid-derived cells. The use of a specific and reliable assessment method becomes fundamental in the evaluation of the presence and modulation of proteins which are important but weakly expressed in these rare tumors.

Desmoid tumours complicating familial adenomatous polyposis.

Desmoid tumours are one of the most important and intriguing extracolonic manifestations of familial adenomatous polyposis (FAP). They have been studied only in small numbers of patients. Patients with FAP who also had desmoid tumour were identified from a polyposis registry database and their hospital notes were reviewed. There were 166 desmoids in 88 patients (median age 32 (interquartile range 22-38) years; 51 (58 per cent) female); 83 tumours (50 per cent) were within the abdomen and 80 (48 per cent) were in the abdominal wall. All but 16 individuals (18 per cent) had already undergone abdominal surgery, which was significantly more recent in women (P = 0.01, Mann-Whitney U test). Intra-abdominal desmoids caused small bowel and ureteric obstruction and resulted in ten deaths; survival was significantly poorer than in patients with abdominal wall desmoid alone (chi2 = 3. 93, 1 d.f., P = 0.047, log rank test), and eight of 22 patients who underwent resection of intra-abdominal desmoid died in the perioperative period. Abdominal wall desmoids caused no deaths or significant morbidity; although recurrence was common after excision, the treatment was safe. Intra-abdominal desmoids can cause serious complications and treatment is often unsuccessful; in particular, surgery for desmoids at this site is hazardous.

Occurrence of desmoid tumours in familial adenomatous polyposis and results of treatment.

Desmoids are of major concern in patients with familial adenomatous polyposis (FAP) besides the cancer risk. We estimated the risk for desmoid tumours and the results of their treatment in the Finnish Polyposis Registry. The analysis included 202 FAP patients, of whom 169 had undergone colectomy. Desmoids were observed in 29 cases: 15 in the mesentery, 10 in the abdominal wall and 4 in other sites. There were 12 male and 17 female patients with a mean age of 28.2 years (range 7 months to 59 years). The cumulative life-time risk was 21%; 1.5, 3.0, 8.9, 16 and 18% at ages of 10, 20, 30, 40 and 50 years, respectively. The risk of postcolectomy desmoids was 17% after ileorectal anastomosis and 12% after proctocolectomy. There were no deaths due to desmoids. One abdominal scar desmoid disappeared spontaneously and all the other abdominal wall desmoids could be excised without complications, but recurrence occurred in five (45%) cases. Excision was possible in only nine mesenteric desmoids (56%); in these cases recurrence was less common (two cases, 22%) but two others had life-threatening complications (bleeding, short bowel). We conclude that the desmoid problem concerns more than 20% of all FAP patients in long-term. Despite high recurrence rates and surgical hazards surgery remains a useful option for most desmoid tumours in FAP patients considering that other treatments are often ineffective or hazardous as well.

Treatment of Intra-abdominal and Abdominal Wall Desmoid Tumors With Drugs That Affect the Metabolism of Cyclic 3', 5'-Adenosine Monophosphate

A female patient with Gardner's syndrome was treated with delta1-testololactone (200 mg daily) because of growth of a large desmoid tumor in the pelvis and lower abdomen and a tumor in a scar from a previous laparotomy. There was also pain and swelling of the left leg. An immediate effect of the drug therapy was complete relief of pain followed shortly thereafter by disappearance of the edema of the leg. After two months, the numerous sebaceous cysts were less prominent. The gross measurements of the diameter of the pelvic and lower abdominal tumor clearly demonstrated tumor shrinkage following therapy. Small polyps scattered over the rectal mucosa and numerous osteomata were not demonstrably affected. After one year of treatment with delta1-testololactone, a laparotomy for partial small bowel obstruction was necessary. Obstruction was caused by the involvement of small bowel mesentery and the bowel itself in a contracted residuum of dense fibrous tissue. Substitution of theophylline and chlorothiazide for the testololactone in Januray 1974 was followed by further diminution of the measurable abdominal and pelvic desmoids. All of these compounds synergize the action of 3',5'-adenosine monophosphate and at least the latter two may function by inhibiting the action of 3',5'-adenosine monophosphate diesterase.