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 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 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.
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 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 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
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.
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 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 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-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
We report here a review of the current medical literature on pregnancy associated desmoids, including 10 cases of our own. The pertinent findings are that a large percentage of desmoids in females arise in and around pregnancy. Most occur in the abdominal muscles, particularly the right rectus abdominus, perhaps related to trauma from abdominal stretching and fetal movement. While these tumors may regress spontaneously after delivery most can be surgically resected with low recurrence rates even with R1 resections and this is clearly the treatment of choice. Subsequent pregnancies do not appear to result in recurrence in either FAP or non FAP patients. It is not clear from currently available data whether pregnancy associated desmoids are molecularly distinct from other desmoids.
desmoid; pregnancy; post-partum; female
Aggressive fibromatosis is a rare soft tissue tumor. Although it lacks metastatic potential, it can grow aggressively in a locally infiltrating pattern. The tumors frequently recur after surgical excision, which remains the treatment of choice. Optional combinations of radiotherapy and/or chemotherapy have been used postoperatively for recurrent disease and/or inoperable cases. A palpable mass was detected in the popliteal fossa of the right lower extremity in a 48-year-old man. Magnetic resonance imaging showed a contrast-enhancing noncalcified lesion initially felt to represent a vascular tumor. An invasive mass adherent to the surrounding tissue was visualized intraoperatively and extensively debulked. The patient's postoperative course was uneventful. Histologic examination of the surgical specimen was consistent with an extra-abdominal desmoid tumor. After appropriate recognition, wide local excision may be the most appropriate treatment for fibromatosis of the extremity. However, the rarity of this tumor and the difficulty inherent in distinguishing it from similar-appearing tumors are necessitating histologic confirmation of the diagnosis.
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 tumours are rare, benign tumours arising from fibrous tissue in muscle fascia or aponeurosis. They are most common in women of child-bearing age and most often appear during or after pregnancy in this age group. The recommended treatment is wide surgical excision, if possible, but unresectable tumours may be treated with radiotherapy, anticancer drugs, nonsteroidal anti-inflammatory agents or antiestrogenic compounds. The recurrence rate is high and seems to be related to the achievement of resection margins free of tumour. The literature is not specific about how to counsel woment who have had a desmoid tumour and subsequently wish to have a child. Patients should be advised that these tumours may be estrogen sensitive but subsequent pregnancy is not necessarily a risk factor for recurrence or development of new disease.
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 characterized by infiltrative growth and local recurrence often occurs after surgery. To reduce the local recurrence rate, adjuvant therapy, such as radiotherapy and pharmacotherapy with cytotoxic agents, anti-estrogen agents and non-steroidal anti-inflammatory drugs, is often applied. In addition, these non-surgical treatments are also performed in patients with unresectable desmoid tumors. We successfully treated a patient with a desmoid tumor with tranilast; an anti-allergic agent.
A 48-year-old Japanese man with a slow-growing desmoid tumor on his chest wall was treated with an oral administration of tranilast (300 mg per day, three times a day). Two years and two months after the commencement of his therapy, the tumor became impalpable. At this time, the oral administration of tranilast was discontinued. Two years after discontinuation of the treatment, a physical examination showed no recurrence of the tumor and he continued in a state of remission. We were successfully able to reduce the size of the tumor and thereafter maintain the reduced size.
Tranilast was clinically effective in our case, and is probably comparable to cytotoxic agents or anti-estrogen agents. Because tranilast has substantially fewer adverse effects than cytotoxic agents, it could be a very useful therapeutic agent for desmoid tumor.
Desmoid tumors are soft-tissue neoplasms arising from fascial or musculo-aponeurotic structures. Most reported thoracic desmoid tumors originate from the chest wall. However, intrathoracic desmoid tumors are rare. We present a case of a 35-year-old male patient complaining of mild shortness of breath. The patient was diagnosed to have a huge intrathoracic desmoid tumor, which was successfully resected.
Chest wall; desmoid; fibromatosis; intrathoracic; tumor
Desmoid tumours are rare mesenchymal tumours, often locally invasive and characteristically associated with a high local recurrence rate after resection. A potential aetiological role for female hormones is indicated. Pregnancy-associated desmoid tumours are almost exclusively located in the abdominal wall. An essential issue is how to counsel women who have had a pregnancy-associated desmoid tumour and subsequently wish to bear a child. A considerably rare case of a patient with a resection of a giant pregnancy-associated, 33 cm in diameter, intra-abdominal desmoid tumour is presented. After a subsequent pregnancy, the patient delivered healthy twins 26 months later. Fifty-four months after treatment, there are no signs of recurrent or second desmoid tumour. Although rarely located in the abdomen, pregnancy-associated desmoid tumours should be included in the differential diagnosis of intra-abdominal tumours detected during or shortly after pregnancy. Based on this case and a few others reported in the literature, subsequent pregnancy does not necessarily seem to be a risk factor for recurrent or new disease.
Aggressive fibromatosis; Desmoid tumour; Intra-abdominal tumour; Pregnancy
Objective: Desmoid tumors are rare benign neoplasms of fibroblastic origin, frequently presenting in the abdomen of adult patients. Little is reported about clinical features and outcome of desmoid tumors appearing in infants and children, especially when they are located in the face. Methods: We report a girl with a desmoid tumor of her cheek, which was present at birth. Results: The tumor was treated by surgical excision, with no additional treatment, other than follow-up. No recurrence of the tumor was witnessed 15 months following surgical excision. Summary: Radical surgical treatment with tumor excision, sometimes accompanied by radiotherapy, is the current standard therapy for infantile desmoid tumors. Pharmacological treatment may be added in selected cases. These modalities may be challenging when desmoid tumors appear in the face because of risks of facial distortion and associated growth problems.
Extra-abdominal desmoid tumors preferentially affect the shoulders, arms, backs, buttocks, and thighs of young adults. Multicentric occurrence is rather rare but seems to be another distinctive feature of extra-abdominal desmoid tumors. In this article we report a rare case of multicentric extra-abdominal desmoid tumors arising in bilateral lower limbs.
desmoid; bilateral limbs; radiation
Desmoid tumors are benign neoplasms that most often arise from muscle aponeurosis and have been associated with both trauma and pregnancy. The etiology of desmoids has not been determined. We report the case of anterior abdominal wall desmoid tumor in a female patient with previous history of cesarean section. Preoperative ultrasound and computed tomography demonstrated a large mass mimicking a large hematoma or an intraabdominal mass. The tumor was removed by wide excision with safe margins. The abdominal wall defect was reconstructed with polypropylene mesh. Subsequent histology revealed a desmoid tumor. Desmoid tumors in females are often associated with pregnancy or occur post-partum. The reasons behind this association are unclear. The most common sites are in the abdominal muscles.
Desmoid tumors; post partum; surgery
Desmoid tumors are cytologically bland fibrous neoplasms originating from musculoaponeurotic structures throughout the body. The cause of desmoid tumors is uncertain, but may be related to trauma or hormonal factors, or may have a genetic association. These tumors can be found in some young women during pregnancy or just after giving birth. We report herein a case of desmoid tumor on the inner aspect of the abdominal wall that mimicked a large subserosal uterine leiomyoma. Initial clinical examination of the patient suggested a large abdominal wall tumor, while the imaging techniques including transabdominal ultrasound and magnetic resonance imaging suggested a large subserosal uterine leiomyoma as the initial diagnosis. This case emphasizes the importance of clinical examination during the diagnostic process.
diagnosis; lesion; ultrasound
Fibromatosis or desmoid tumor covers a broad spectrum of benign fibrous tissue proliferations. It is characterized by infiltrative growth and a tendency towards recurrence; however, unlike sarcoma, it never metastasizes.
We report on a case of extraabdominal fibromatosis originating from the retroperitoneal space in a 43-year-old woman. Seven years earlier she had undergone ureterolysis and ureteroureterostomy for ureteral obstruction. Computed tomography revealed a tumor between the iliocostalis and the psoas muscle. Histopathological evaluation revealed uniform proliferation of spindle cells, with a moderate amount of collagen fibers, suggesting extraabdominal fibromatosis (desmoid tumor). The tumor was surgically resected, and since then, the patient has remained asymptomatic without any restrictions of daily living activities and without any signs of tumor recurrence during the two-year follow-up.
Complete resection is the treatment of choice. Adjuvant therapy using non steroidal anti-inflammatory agents, tamoxifen, interferon, anti-neoplastic agents, and radiotherapy, either alone or in combination finds application for unresectable or recurrent cases.