A desmoid tumor, also known as aggressive fibromatosis, is a rare benign neoplasm that arises from fascial or musculoaponeurotic tissues. It can occur in any anatomical location, most commonly the abdominal wall, shoulder girdle and retroperitoneum. The typical clinical presentation is a painless mass with a slow and progressive invasion of contiguous structures. It is associated with a high local recurrence rate after resection. Many issues regarding the optimal treatment of desmoid tumors remain controversial. Aggressive surgical resection with a wide margin (2-3 cm) remains the gold standard treatment with regard to preserving quality of life. Radiotherapy alone has been shown to be effective for the control of unresectable or recurrent lesions. Desmoid tumors tend to be locally infiltrative, therefore, the fields must be generous to prevent marginal recurrence. The radiation dose appropriate for treating desmoid tumors remains controversial. We present a 25-year-old Caucasian man with local recurrence of a desmoid tumor after repeated surgical resection, treated with radiotherapy. The patient achieved complete tumor regression at 4 mo after radiotherapy, and he is clinically free of disease at 12 mo after the end of treatment, with an acceptable quality of life. The patient developed short bowel syndrome as a complication of second surgical resection. Consequently, radiotherapy might have worsened an already present malabsorption and so led to steatohepatitis.
Desmoid tumor; Aggressive fibromatosis; Fatty liver
Desmoid tumors are benign but locally aggressive tumors of mesenchymal origin which are poorly circumscribed, infiltrate the surrounding tissue, lack a true capsule and are composed of abundant collagen. History of trauma to the site of tumor origin is elicited in up to 1 in 4 cases and they most commonly develop in the anterior abdominal wall and shoulder girdle but they can arise in any skeletal muscle. The clinical behavior and natural history of desmoid tumors are unpredictable and management is difficult with many issues remaining controversial, mainly regarding early detection, the role, type and timing of surgery and the value of non-operative therapies.
We report a case of a 23 year old male referred from a district hospital to a national referral hospital in Kenya, after developing a huge abdominal wall desmoid tumor following laparotomy for a blunt abdominal injury fourteen months earlier. The tumor was successfully excised and the abdominal wall defect reconstructed using a vicryl/prolene mesh and a unilateral groin flap. The patient had a non-eventful recovery and was discharged through radiotherapy clinic.
Wide margin tumor excision alone is a reasonable option in the management of desmoid tumors.
Desmoid tumors are rare soft-tissue neoplasms with limited data on their management. We sought to determine the rates of recurrence following surgery for desmoid tumors and identify factors predictive of disease-free survival.
Between January 1983 and December 2011, 211 patients with desmoid tumors were identified from three major surgical centers. Clinicopathologic and treatment characteristics were analyzed to identify predictors of recurrence.
Median age was 36 years; patients were predominantly female (68 %). Desmoid tumors most commonly arose in extremities (32 %), abdominal cavity (23 %) or wall (21 %), and thorax (15 %); median size was 7.5 cm. Most patients had an R0 surgical margin (60 %). The 1- and 5-year recurrence-free survival was 81.3 and 52.8 %, respectively. Factors associated with worse recurrence-free survival were: younger age (for each 5-year increase in age, hazard ratio [HR] = 0.90, 95 % confidence interval [95 % CI] 0.82–0.98) and extra-abdominal tumor location (abdominal wall referent: extra-abdominal site, HR = 3.28, 95 % CI, 1.46–7.36) (both P <0.05).
Recurrence remains a problem following resection of desmoid tumors with as many as 50 % of patients experiencing a recurrence within 5 years. Factors associated with recurrence included age, tumor location, and margin status. While surgical resection remains central to the management of patients with desmoid tumors, the high rate of recurrence highlights the need for more effective adjuvant therapies.
Desmoid tumor is a locally invasive, myofibroblastic, nonmetastatic tumor. Its pathogenesis remains unclear and it may involve genetic abnormalities, sex hormones and traumatic injury, including surgery. Postoperative intra-abdominal desmoid tumor is rare, especially in the retroperitoneum. We report a case of postoperative retroperitoneal desmoid tumor that developed 29 mo after the first excision of a gastrointestinal stromal tumor. Sporadic trauma-related intra-abdominal desmoid tumors reported in the English literature are also reviewed. Despite an extremely low incidence, postoperative desmoid tumor should be considered in the differential diagnosis when a recurrent neoplasm is found at least one year after operation. However, it is a clinical challenge to distinguish recurrent malignant neoplasms from desmoid tumors, and surgical resection is the treatment option depending on the anatomic location.
Desmoid tumor; Gastrointestinal stromal tumor; Recurrence; Retroperitoneum; Surgery
Desmoid tumours represent aggressive fibroblastic proliferation of the musculoaponeurotic structures commonly from the anterior abdominal wall. These tumours infiltrate locally, recur frequently but do not metastasize. Antecedent trauma, pregnancy and estrogens play a role in the etiopathogenesis of these tumours. In familial adenomatous polyposis (FAP) genetic history associated with chromosomal abnormality and familial incidence as in Gardner's syndrome is reported and most of these tumours are intraperitoneal either in the mesentery or pelvis and may be multiple and they carry poor prognosis. Surgery is the most preferred treatment and requires wide excision with 1 cm margin followed by reconstruction of the defect in the anterior abdominal wall either with local musculoaponeurotic layers or with synthetic mesh. In intra-abdominal cases associated with FAP in addition to surgery, hormonal treatment, chemotherapy and Radiotherapy are also advised depending upon the particular condition but usually prognosis is not encouraging.
In this article we present our personal experience in the successful treatment of six cases of sporadic desmoids, five in females of child bearing age, and all in the anterior abdominal wall and one extra abdominal in a child aged 13 y in the gluteal region (Case 6). It is very interesting and unique to see two desmoid tumours developing in the same patient (Case2)one in each of the Rectus abdominal muscles (Right & Left).
Abdominal fibromatosis; Desmoids; Surgery
Desmoid-type fibromatosis is a rare mesenchymal neoplasm with local aggressiveness. The incidence of desmoid-type fibromatosis is 2–5/million/year with intra-abdominal fibromatosis, such as that which is reported in this clinical case, occurring only in 12–18% of cases. After having analyzed the pathogenetic hypotheses of desmoid-type fibromatosis, the authors point out that the diagnosis of this disease, especially in the intra-abdominal form, is often late, specifically when highly demolitive interventions are needed or when the limits of radical surgery have been exceeded. In the clinical case reported, the tumor was infiltrating both ileus and sigma. The authors consider the differential diagnosis of desmoid-type fibromatosis, especially with GISTs, with regard to both the radiological preoperative diagnostic and histological studies on the surgical specimen. Radical surgical excision is not always, for this disease, a sign of healing; in fact, even when the resection margins are negative, the incidence of recurrence is between 13 and 68%. The average time of recurrence is between 15 and 24 months; in this case report, the patient, who has not been subjected to complementary therapies, is tumor-free for over 30 months since surgery; his prognosis may be satisfactory if we consider the negativity of resection margins, which in any case remains the most important prognostic factor.
Desmoid tumors (also called desmoids fibromatosis) are rare slow growing benign and musculoaponeurotic tumors. Although these tumors have a propensity to invade surrounding tissues, they are not malignant. These tumors are associated with women of fertile age, especially during and after pregnancy. We report a young female patient with a giant desmoid tumor of the anterior abdominal wall who underwent primary resection. The patient had no history of an earlier abdominal surgery. Preoperative evaluation included abdominal ultrasound, computed tomography, and magnetic resonance imaging. The histology revealed a desmoid tumor. Primary surgical resection with immediate reconstruction of abdominal defect is the best management of this rarity. To the best of our knowledge and PubMed search, this is the first case ever reported in the medical literature of such a giant desmoid tumor arising from anterior abdominal wall weighing 6.5 kg treated surgically with successful outcome.
Spontaneous ureteric rupture is a rare entity that presents as an extravasation of urine from the ureter without previous surgery, ureteric manipulation and external trauma of the ureter. We report the case of a desmoid tumour presenting as spontaneous ureteric rupture which was managed in our institution.
PRESENTATION OF CASE
A 28 years old healthy male presented with a four day history of generalised abdominal pain secondary to spontaneous right ureteric rupture. Patient was initially managed via insertion of nephrostomy tube and antibiotics. After unsuccessful attempts of retrograde and antegrade ureteric stent insertion, patient was subsequently managed via elective surgical intervention. The excised specimen revealed desmoid tumour as cause of the ureteric rupture.
Desmoid tumours are rare benign tumours arising from fascial or musculoaponeurotic structures that do not metastasise, but tend to invade locally. It is often initially managed medically prior to undertaking a definitive surgical intervention. To our knowledge this is the first reported case of ureteric perforation secondary to a desmoid tumour of the mesentery.
Spontaneous rupture of the ureter is often misdiagnosed as other conditions. History taking and examination can be unreliable, hence a high level of suspicion and further investigations should be utilised. Once the diagnosis is made, treatment can be individualised based on aetiology.
Desmoid tumour; Ureteric rupture; Spontaneous ureteric rupture
Desmoid fibromatoses are benign, slow growing fibroblastic neoplasms, arising from musculoaponeurotic stromal elements. Desmoids are characterized by local invasion, with a high rate of local recurrence and a tendency to destroy adjacent structures and organs. Desmoid fibromatoses are rare in children, and though they may occur in the head and neck region, are extremely rare in the paranasal sinuses. Here we report a case of extraabdominal desmoid fibromatosis in a seven-year-old boy involving the sphenoid sinus, one of only six published reports of desmoid fibromatosis of the paranasal sinuses. The expansile soft tissue mass eroded the walls of the sphenoid sinus as well as the posterior ethmoid air cells extending cephalad through the base of the skull. We discuss the clinicopathologic features of this lesion, including structural and ultrastructural characteristics, and we review the literature regarding treatment and outcome.
Desmoid tumors are rare benign tumors that originates in the fibrous sheath or musculo-aponeurotic structure. Histologically benign, they tends to invade locally and to be recurrent.
PRESENTATION OF CASE
We report a rare case of an intra-abdominal desmoid tumor in a patient affected by familial adenomatous polyposis, which degenerated into abscess. Male, 38 years, was hospitalized for abdominal pain, bowel obstruction and fever. The computed tomography showed a big dishomogeneous mass occupying the whole mesentery with internal massive liquefaction. The mass extended from the epigastrium for 13 cm up to L3. On the right mesogastric side a solid, thick mass of about 2 cm, with a length of 4.5 cm, was identified; it was not cleavable from the wall and from some of the loops. We decided to perform a computed tomography-guided percutaneous drainage. Two hundred ml of purulent necrotic material was aspirated, and washing with antibiotic solution was carried out. Cytological examination of fluid drainage showed histiocytes and neutrophils. At follow-up, the patient's clinical condition had improved. An abdominal ultrasound showed a substantial reduction in the diameter of the mass.
Diagnosis and treatment of desmoids tumor in patients with familial adenomatous polyposis may be difficult, especially when desmoids are located intra-abdominally and in the mesentery. Seldom will desmoid tumors be complicated by abscess formation.
The management of desmoids tumors is not easy and the choice of the best treatment may be difficult due to the different possible anatomical presentations.
DT, desmoid tumor; FAP, Familial Adenomatous Polyposis; APC, Adenomatous Polyposis Coli; CT, computed tomography; MRI, Magnetic resonance imaging; Desmoid tumor; familial adenomatous polyposis
Desmoid tumors are rare nonencapsulated benign lesions that invade the musculoaponeurotic tissues of the body. Such tumors infiltrate insidiously, and attempts to remove them frequently fail, usually leaving residual tumor. The use of adjuvant radiotherapy to decrease the recurrence rate in partially resected extra-abdominal desmoids has been reported by several authors. The role of irradiation in the management of desmoid tumors is illustrated in a case that combined surgery, intraoperative radiotherapy, and postoperative external beam radiotherapy in the management of this lesion.
Desmoid tumor is a monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures. This connective tissue hyperplasia infiltrates locally, recurs frequently after resection but does not metastasize. Abdominal desmoid occurs sporadically, in association with some familial syndromes and often represents a clinical dilemma for surgeons. The enigmatic biology and anatomical location of abdominal desmoids make treatment recommendations difficult. This distinct pathological entity is reviewed with a specific focus on aetiology and management.
Desmoid; Abdomen; Fibromatosis; Familial adenomatous polyposis; Gardner’s syndrome
Desmoid-type fibromatosis (DTF) is an uncommon nonmetastatic fibrous neoplasm. Sporadic intraperitoneal DTF is rarely described in current literature. We herein report a case of DTF of unknown cause involving the pancreatic head. A 41-year-old man presented with recurrent epigastric pain and weight loss. An abdominal computed tomography scan showed a well-delineated solid cystic mass inside the pancreatic head. Pylorus-preserving pancreaticoduodenectomy was performed due to the patient’s debilitating symptoms and suspected malignancy. The pathological examination revealed massive fibroblastic proliferation arising from the musculoaponeurotic tissues, consistent with a diagnosis of DTF. Immunohistochemical phenotyping determined positive immunoreactivity to vimentin and β-catenin, but negative immunoreactivity to smooth muscle actin, CD117, CD34, or S-100, confirming the diagnosis of DTF. No local recurrence or distant metastasis was found during a 24-month follow-up. Radical resection is recommended as first-line treatment for pancreatic DTF. Long-term follow-up studies are required to establish the prognosis of pancreatic DTF.
Desmoid-type fibromatosis; Desmoid tumor; Aggressive fibromatosis; Pancreatic head; Musculoaponeurotic tissues; Immunohistochemistry
Desmoid tumors are histologically benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. There is similar biological behaviour between benign fibrous proliferations and fibrosarcoma. These neoplasms have the tendency to locally invade and erode adjacent muscular tissue and bone. Within the head and neck area they also tend to encase vital structures. We present a case of a 28-year-old white female with a five-month history of a painful mass in her left posterior cervical triangle who was treated by excisional biopsy of the lesion with preservation of the accessory nerve and post-operative radiation. The histopathologic characteristics of desmoid tumors and treatment of these lesions are also reviewed.
Fibromatosis; aggressive; extraabdominal; neck
Aggressive fibromatosis (or desmoid tumor) refers to a histologically benign but locally aggressive lesion arising from musculoaponeurotic structures in various anatomic sites. Extra-abdominal desmoids represent about one third of all desmoid tumors; of these only about 11 to 15% arise in the head and neck. Desmoid tumors arising in the infratemporal fossa are exceedingly rare; to our knowledge only one such tumor has been reported in the literature. We present a desmoid tumor arising in the infratemporal fossa with intracranial extension in a twenty-seven year old male and review the literature on this rare condition.
Desmoid tumor is a rare entity, and most desmoid tumors are located in abdominal wall or extra-abdominal tissues. Occurrence of desmoid tumor in mesentry is extremely rare.
we report a mesenteric desmoid tumor in a 73-years-old woman who had undergone total gastrectomy reconstructed with jejunal pouch interposition for gastric carcinoma. After 1 year, a tumor was originating from mesentery of the interposed jejunal pouch was identified, and the patient underwent resection of the large mass which was found to invade pancreas. Histological examination revealed desmoid tumor.
Desmoid tumor is rare, and it was difficult for the differential diagnosis of desmoid tumor or recurrent tumor.
Desmoid tumors are very rare, benign fibrous neoplasms arise from the musculoaponeurotic structures throughout the body.
The patient was a seven-year old boy with a large mandibular mass growing over a period of six months. His CT-scan showed a large mass, 13 cm in diameter in the cheek area extending to the neck and trachea. Biopsy was compatible with desmoid fibromatosis. He was given neoadjovant treatment with vinblastin and methotrexate. The patient underwent a tracheostomy. Then a complete hemimandibulectomy and submandibular gland excision was performed. Finally reconstruction with latisimus dorsi free flap was performed.
Despite rarity desmoid tumors should be kept in mind of an otorhinolaryngologist as a differential diagnosis in children with head and neck mass.
Fibromatosis; Fibrous Tissue; Mandibular Diseases; Neoplasms
Intra-abdominal desmoid tumors are rare and most often occur in patients with a history of familial adenomatous polyposis, surgery, or pregnancy. We report a case of an intra-abdominal desmoid tumor mimicking the recurrence of gastric cancer. A 57-year-old male had undergone distal gastrectomy for advanced gastric cancer. Serum levels of carcinoembryonic antigen were found to be elevated 27 months after surgery. Computed tomography revealed a 15-mm mass in the mesentery of the transverse colon. In addition, radiotracer fluorodeoxyglucose uptake of the tumor was detected by positron emission tomography. The patient was diagnosed with gastric cancer recurrence, and chemotherapy consisting of cisplatin and S-1 was commenced. After five courses of chemotherapy, although no significant clinical response was seen, no new lesions were seen either. Thus, a curative resection of the recurrent tumor seemed possible, which was successfully performed. Histological examination of the resected specimen revealed spindle-shaped tumor cells with collagen fiber progression; no cancer cells were detected. The tumor was diagnosed as an intra-abdominal desmoid tumor. We report a rare case of an intra-abdominal desmoid tumor that mimicked a recurrent tumor arising from gastric cancer. In patients with history of surgery for intra-abdominal malignancies, it may be difficult to distinguish the recurrence of malignancy from desmoid tumors but the possibility of desmoid tumors must be considered in the differential diagnosis.
Desmoid tumor; Gastric cancer; Recurrence; Surgery
Background: To define the efficacy of postoperative irradiation in patients with recurrent extra-abdominal desmoid tumors in
whom surgical intervention has resulted in microscopically or grossly positive surgical margins.
Methods: A retrospective analysis was performed on all patients referred to the department of radiation oncology at the
Detroit Medical Center with a diagnosis of recurrent extra-abdominal desmoid tumor. This analysis includes all patients
seen from 1 January 1990 through 31 December 1999. A total of 11 patients were treated to 13 sites. Ten had
microscopically positive margins and three had gross residual disease. Three patients were noted to have multifocal disease at
the time of initial representation. Local control, survival, follow-up, and subsequent development of new tumors are
measured from the last day of treatment with irradiation.
Results: Thirteen sites were treated. Seven patients had received chemotherapy/hormonal therapy prior to surgery and/or
irradiation. The most commonly used drug was tamoxifen (n=6). The type of radiation delivered included external beam
irradiation alone (n=3), combined external beam irradiation and brachytherapy (n=4), brachytherapy alone (n=3) and
252-Cf neutron brachytherapy alone (n=3). Follow-up has ranged from 29 to 115 months (median=76 months). Three
patients have failed locally at 17, 24 and 29 months. One of these was treated for gross residual disease. No patient has died
of tumor-related causes. Salvage at the failed sites was possible in twom of three with re-irradiation using external neutrons
and/or aggressive surgical intervention and systemic therapy. Complications were most often noted to include decrease range
in motion, especially in joint areas, and skin reactions which were normal in presentation. In one site there was development
soft tissue necrosis.
Conclusion: Based on our experience we recommend postoperative irradiation for all recurrent extra-abdominal desmoid
lesions with microscopically or grossly positive surgical margins. Furthermore, patients with recurrent desmoid tumors
involving the bony structures of the hand or feet are poor candidates for brachytherapy alone. For patients with extremity
lesions, brachytherapy may be a reasonable treatment option provided adequate margins around the tumor bed are covered.
The continued recommended use of irradiation in this group of patients is warranted.
Desmoid tumours are benign monoclonal myofibroblastic neoplasms arising from musculoaponeurotic stromal tissue. They infiltrate local tissue but have no known metastatic potential. The management of desmoid tumours is complicated by their unpredictable nature and rarity, which makes study into their behaviour, and therefore treatment, a challenge. We present a case of intra-abdominal desmoid tumour and discuss the recommended approach to management.
Desmoid tumours are rare, poorly circumscribed tumours that have a firm consistency and, although benign, have a remarkable tendency to infiltrate into surrounding structures. Extra-abdominal desmoid tumours involve mainly the extremities or the chest wall and are usually managed by wide radical resection. Moreover, desmoid tumours involving the chest wall are locally aggressive tumours with a high recurrence rate. We report a case of a pathologically proven desmoid tumour of the chest wall in a patient with a history of bilateral breast cancer and oesophageal cancer. We discuss the imaging appearances of this tumour on positron emission tomography combined with computed tomography (PET/CT) and magnetic resonance imaging.
Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner’s syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner’s syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner’s syndrome.
Desmoid tumours; Gardner’s syndrome; Radiofrequency ablation
Desmoid tumours or fibromatoses are rare entities characterized by the benign proliferation of fibroblasts, which can be life-threatening due to their locally aggressive properties. Surgery is widely accepted as the first line of treatment for extra-abdominal desmoids; however, it is not recommended for intra-abdominal desmoids because of the high-risk of recurrence and difficulties with the operation. Here, we report on a patient with sporadic intra-abdominal desmoid tumours, who showed partial response following the intake of non-steroidal anti-inflammatory drugs.
A 73-year-old man presented with swelling and pain of the right leg. Computed tomography showed an abnormal multilocular soft-tissue mass (95 × 70 mm) in the right pelvis, which was revealed by biopsy to be a desmoid tumour. Immunohistochemical analysis showed that the tumour cells expressed vimentin, but not smooth-muscle actin, CD34, or desmin. Very few Ki-67-positive cells were found. Non-cytotoxic treatment with etodolac (200 mg/day) was chosen because of the patient's age, lack of bowel obstruction, and the likelihood of prostate cancer. Two years after the commencement of non-steroidal anti-inflammatory drug administration, computed tomography showed a decrease in tumour size (63 × 49 mm), and the disappearance of intratumoural septa.
Our case report suggests that non-steroidal anti-inflammatory drug treatment should be taken into consideration for use as first-line treatment in patients with sporadic intra-abdominal desmoid tumours.
Desmoid tumors (DTs) are benign tumors that exhibit fibroblastic proliferation, which arises from fascial or musculoaponeurotic structures. The aim of this study was to investigate the characteristics and outcomes of patients with resectable DTs. A total of 21 patients were included and their clinicopathological characteristics were retrospectively analyzed. The 21 patients (16 females and 5 males) were identified through reviewing the patient charts at our institute. The tumor was located in the lower extremities in 7 cases, in the upper extremities in 4 cases, in the abdominal region in 9 cases and in the neck region in 1 case. Patients who had been initially treated by surgical excision were included in the study. Of these 21 patients, a positive surgical margin (SM) was reported in 11 patients, 7 of whom received postoperative radiotherapy (RT). Ten patients had a negative SM and 6 received RT. A total of 5 patients (46%) in the positive SM group and 4 (40%) in the negative SM group had documented disease relapse (P>0.05). The median relapse-free survival (RFS) was 20.5 months for the patients treated by surgery alone and 50 months for those treated with surgery followed by adjuvant RT (P>0.05). Age, gender, SM and adjuvant RT were not identified as predictors of recurrence. No predictive factors appeared to indicate local DT recurrence following surgery.
desmoid disease; radiotherapy; surgery
To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors.
The standard management of desmoid tumors is resection, but many recur locally. Other options include observation or novel chemotherapeutics, but little guidance exists on selecting treatment.
Patients undergoing resection during 1982-2011 for primary or locally recurrent desmoids were identified from a single-institution prospective database. Cox regression analysis was used to assess risk factors and to create a recurrence nomogram, which was validated using an international, multi-institutional dataset.
Desmoids were treated surgically in 495 patients (median follow-up 60 months). Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence–free survival (LRFS) was 69%. Eight patients died of disease, all after R2 resection. Adjuvant radiation was not associated with improved LRFS. In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. Abdominal wall tumors had the best outcome (5-year LRFS 91%). Age, site, and size were used to construct a nomogram with concordance index 0.703 in internal validation and 0.659 in external validation. Integration of additional variables (R1 margin, gender, depth, and primary vs. recurrent presentation) did not importantly improve concordance (internal concordance index 0.707).
A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. Systemic therapies may be appropriate for young patients with large, extremity desmoids, but surgery alone is curative for most abdominal wall lesions.