Surgical management of large desmoid tumour of the anterior abdominal wall

Desmoid tumours are uncommon. They are locally invasive and incomplete excision leads to recurrence, which can pose a significant management challenge. Patients therefore require effective treatment, which essentially entails tumour excision with a clear surgical margin. The resulting wide defect may lead to difficulty in closure of the anterior abdominal wall. We report our experience in treating large desmoid tumours of the anterior abdominal wall. Between January 2000 and December 2001, three patients with large desmoid tumour of the anterior abdominal wall were treated with wide excision, which included a 3-cm margin of uninvolved tissues. This led to a considerable abdominal wall defect. The peritoneal defect was closed as a separate layer, though under considerable tension, while the abdominal wall musculature defect was closed with a polypropylene mesh. All three patients recovered well with no immediate or late postoperative morbidity. Follow-up until December 2006 has not revealed any tumour recurrence or hernia development. Wide excision of an anterior abdominal wall desmoid tumour with a clear margin of 3 cm including the peritoneum should be considered when managing such tumours. Closure under tension of the peritoneum did not seem to produce any morbidity.     

Desmoid tumors of the abdominal wall.

Four cases of desmiod tumors of the anterior abdominal wall recently treated at the University of Chicago Hospitals and Clinics are presented. From our experience we conclude: 1. Inadequate excision of desmoid tumors results in local recurrence. 2. Adequate excision of desmoid tumors ot the abdominal wall sometimes necessitates creation of an abdominal wall defect too large for primary closure. 3. Marlex mesh has been used successfully in reconstruction of the abdominal wall following adequate excision of desmoid tumors. 4. One patient (Case 4) with an abdominal wall desmoid tumor associated with familial polypisis and mesenteric fibromatosis has benefited greatly following wide excision of abdominal wall and reconstriction using Marlex mesh, despite the fact that the mesenteric fibrous tumor was unresectable.     

Abdominal wall reconstruction with intraperitoneal prosthesis in desmoid tumors surgery

Desmoid tumor (DT), also known as aggressive fibromatosis, is a rare soft tissue neoplasm. For those tumors localized in the anterior abdominal wall, radical resection and reconstruction with mesh are indicated. Due to the rarity of this disease, there have been no randomized trials, but in reported retrospective series, although it is considered a benign lesion, it is clear that local recurrence is not uncommon. Records from seven consecutive patients (1 man, 6 women; mean age 35 years, range 25-60 years) presenting with desmoid tumors of the anterior abdominal wall were analyzed. In all cases the surgical strategy was the same: wide surgical excision and immediate plastic reconstruction with ePTFE mesh after intraoperative confirmation by frozen section of disease-free margins >1 cm. No immediate postoperative complications were recorded, and no patients developed recurrence after a median follow-up period of 60 months. The long-term mean of global health status recorded was 100%. Radical resection aided by intraoperative margin evaluation by frozen sections, followed by immediate mesh reconstruction, is a safe procedure and can provide a definitive cure without functional limitations for patients with desmoid tumors of the anterior abdominal wall.     

Problems in resection of chest wall sarcomas.

To illustrate the problems of reconstruction in major chest wall resection, five patients with a variety of soft tissue tumors of the chest wall, located at different sites, are presented. Patients, who underwent a lateral or posterolateral chest wall resection required removal of two to five ribs sequentially as well as the adjacent soft tissue. Those who underwent an anterior chest wall resection required resection of the manubrium or the body of sternum as well as of adjacent costal cartilages. To prevent instability of the chest, herniation, and to minimize flailing, the chest defect was bridged with the use of Marlex mesh. Whenever possible, the omentum was brought into the chest cavity to increase the vascularity of the reconstruction. Since, in most instances, the tumors involved the skin because of previous damage from radiation therapy, extensive skin coverage was planned well in advance of resection. Pedicle skin flaps or rotation flaps were used to cover the skin defect. Ventilatory support by volume respirator, was required for three to four days. In all patients, the chest wall was completeley stable after three to six weeks.     

Wide resection of the upper right hemithorax combined with amputation of the right arm for a recurrent desmoid tumor

A 66-year-old woman had a recurrent desmoid tumor in the right thoracic apex. The tumor infiltrated the brachial plexus and eventually rendered the upper right extremity functionless. The tumor was removed by aggressive wide resection of the right upper hemithorax with simultaneous amputation of the functionless right arm. Reconstruction of the chest wall was accomplished, utilizing the soft tissues of the remnant arm as a pedicled flap to cover the full-thickness defect. Aggressive wide resection of the chest wall with limb amputation is technically challenging but unavoidable in some cases of recurrent desmoid tumor.     

Desmoids tumors

Desmoid tumor can be defined as a pseudoencapsulated infiltrative growth of well-differentiated collagenous fibroblasts and fibrocytes arising either in fascia or musculoaponeurotic structures. The etiology of desmoid tumors is poorly defined. The most commonly implicated etiologic factors are trauma, hormonal disturbances, and genetic or hereditary factors. Desmoid tumors of the anterior abdominal wall are much less common than extra-abdominal desmoids; they may occur at any age but are most common in the third and fourth decades. Although both sexes may be affected, abdominal desmoids predominate in females, particularly in females of childbearing age. Extra-abdominal desmoids, which most commonly occur on the back, chest wall, head and neck, or lower extremity, have a male predominance. Most patients complain of a painless mass of several months or years' duration. The primary consideration in surgical treatment of desmoid tumors should be the prevention of local recurrence. In most instances, this can be achieved by wide local excision or muscle group resection. There is no clear evidence that irradiation or chemotherapy are effective in controlling desmoid tumors.     

Necrotizing fasciitis of the chest wall with a retropharyngeal abscess: case report and literature review

We herein report a case of a diabetic 49-year-old woman with anterior chest wall necrotizing fasciitis that caused a retropharyngeal abscess and discuss the literature surrounding necrotizing soft tissue infections of the chest wall. The patient was treated with serial wound debridements, antibiotic therapy, and percutaneous drainage and serial irrigation of the retropharyngeal abscess with a silicone catheter. The resulting defect was grafted with a split thickness skin graft.     

Abdominal wall and foot reconstruction after extensive desmoid tumor resection with free tissue transfer

BACKGROUND: Desmoid tumors are rare connective tissue tumors, also referred to as deep, aggressive fibromatosis. Although histologically benign, they show an invasive growth behavior and have a high local recurrence rate. METHODS: The treatment of choice is surgical resection with wide negative margins, while the use of radiotherapy remains controversial. Wide resection of greater desmoid tumors may result in considerable defects and functional impairment. Few papers discuss different options for defect coverage after desmoid tumor resection. Two cases of extensive desmoid tumors, one at the trunk, one at the foot, both with compromised wound margins due to multiple previous surgeries, are presented. To achieve a stable and functional soft tissue cover, the defects were treated with microvascular soft tissue transfer (one free latissimus dorsi, one free radial forearm flap). RESULTS: Both flaps healed uneventfully and patients regained full function of the abdominal wall and foot, respectively. CONCLUSIONS: The presented cases demonstrate the efficacy of free flap coverage as an ultimate therapeutic option in selected cases of critical defects after extra-abdominal desmoid tumor resection. Furthermore, free flaps provide a well vascularized ground for adjuvant radiotherapy.     

Proceed补片对腹壁硬纤维瘤切除后遗留巨大腹壁缺损修补的疗效分析

目的探讨Proceed网片对腹壁缺损修补的疗效。方法回顾性分析了我院2年来对15例硬纤维瘤患者以Proceed补片进行腹壁缺损修补的手术方法及随访资料。结果术后3例发生皮下积液,全部患者随访1个月~2年,无感染、切口疝发生,无局部不适。结论Proceed补片具有抗粘连、复发率低等优点,可在腹壁硬纤维瘤切除术后遗留巨大腹壁缺损修补中加以应用。     

Intrathoracic localisation of desmoid tumours: a familial case of isolated desmoid tumours and current published data

We present the case of large desmoid tumor of the anterior chest wall with pleural involvement, which persistently progressed despite hormonotherapy, chemotherapy and surgery. After many years of therapeutic failures, given the tumor size and its hemodynamic repercussions, the patient was presumed to be incurable and only supportive measures were given. One year later, the desmoid tumor had completely disappeared. Even though wide surgical excision is an essential element in the treatment of desmoid tumors, spontaneous regression may occur in very advanced disease.     

Desmoid tumor of the chest wall following chest surgery: Report of a case

Desmoid tumors of the chest wall following chest surgery are a rare occurrence. A case of this disease is reported herein together with a review of the literature. A 74-year-old man, who had previously undergone a right lower lobectomy for squamous cell carcinoma of the lung, was referred to our hospital with an abnormal shadow on his chest X-ray. The tumor, located in the right lateral chest wall, was successfully resected by an aggressive, wide extirpation, and a final diagnosis of a desmoid tumor originating in the chest wall was made. When following up patients after surgery for lung cancer, the possibility of desmoid tumors developing in the incised chest wall should therefore be kept in mind.     

Desmoid tumor: a case of mistaken identity

Desmoid tumors are rare tumors accounting for only 0.03% of all neoplasms. Mainly occurring in the fourth and fifth decades of life, these tumors originate in musculoaponeurotic tissues of the limbs, neck, trunk, abdominal wall, and mesentery. We present a rare case of a chest wall desmoid tumor that was mistaken for breast cancer on both physical examination and mammography, which highlights the unique risk these tumors present for confusion with other malignant processes. Although past literature contains numerous reports of other misdiagnoses, this case is unique in reporting the potential for misdiagnosis between chest wall desmoid tumors and breast cancer. In cases where suspicious breast findings do not correlate to usual diagnostic measures, such as fine-needle aspiration or core needle biopsy, the possibility of another pathology such as a chest wall desmoid tumor mimicking breast cancer should be considered in the differential diagnosis.     

Reconstruction of extensive abdominal wall defect using an eccentric perforator‐based pedicled anterolateral thigh flap: A case report

Reconstruction of extensive abdominal wall defects is a challenge for reconstructive surgeons. In this report, a case of reconstruction of a large abdominal wall defect using an eccentric perforator‐based pedicled anterolateral thigh (ALT) flap is presented. A 30‐year‐old man presented with recurrent desmoid‐type fibromatosis in the abdominal wall. The recurrent tumor was radically excised, and the en bloc excision resulted in a full‐thickness, large abdominal wall defect (25 cm × 20 cm). An eccentric perforator‐based pedicled ALT flap, including wide fascial extension, was transferred to the abdominal defect; fascial portions were sutured to the remnant abdominal fascia. Plication of the fascia along the sutured portion was performed to relieve the skin tension between the flap and the marginal skin of the abdominal defect. Eight months after surgery, the reconstructed abdomen had an acceptable esthetic appearance without tumor recurrence or hernia. The use of an eccentric perforator‐based pedicled ALT flap may be an alternative method for the reconstruction of extensive abdominal wall defects. © 2013 Wiley Periodicals, Inc. Microsurgery 33:482–486, 2013.     

腹壁韧带样瘤16例临床分析

目的 探讨腹壁韧带样瘤的临床特点和治疗方法.方法 回顾性分析1998年1月至2010年10月收治的16例腹壁韧带样瘤患者的临床资料,全组患者均经手术治疗.结果 本组16例患者均行广泛切除术,复发2例,复发率12.5%(2/16).结论 腹壁韧带样瘤呈侵袭性生长的特性,治疗应按低度恶性软组织肿瘤处理.治疗首选外科手术.     

Enucleation of a Giant Hepatic Hemangioma in a Jehovah’s Witness

We present two cases of desmoid tumour of the anterior abdominal wall in young women in whom the defect after radical excision could not be closed without using prosthesis. The first case warranted the use of a composite mesh, the second a polypropylene prosthesis. In both cases primary closure of the skin was possible. Both women are doing fine with no sign of relapse or incisional hernia.     

Desmoid tumor of the chest wall with pleural involvement

Although there have been reports of desmoid tumors of the chest wall, pleural extension, as well as overall size greater than 20 cm, is rare. We present the case of a large desmoid tumor involving the left anterior chest wall, upper abdomen, and diaphragm, which impinged on the left lung and displaced the liver. Wide surgical excision, reconstruction, and differential diagnosis from fibrosarcoma are essential elements in the treatment of these rare tumors.     

Desmoid Tumors of the Anterior Abdominal Wall: Results from a Monocentric Surgical Experience and Review of the Literature

Background  Desmoid tumor, also known as aggressive fibromatosis, is a rare soft tissue tumor. For those cases localized in the anterior abdominal wall, radical resection and reconstruction with a mesh is indicated. Because the rarity of the disease, randomized trials are lacking, but in reported retrospective series, it is clear that although it is considered a benign lesion, local recurrence is not uncommon. Methods  We analyzed the records of 14 consecutive patients (3 men, 11 women, mean age 36 years, range 25–51 years) with desmoid tumor of the anterior abdominal wall treated at the European Institute of Oncology. The surgical strategy was the same in all cases: wide surgical excision and immediate plastic reconstruction with mesh after intraoperative confirmation by frozen sections of disease-free margins of >1 cm. We considered long-term outcomes by using the European Organization for the Research and Treatment of Cancer QLQ-C30 as an instrument to evaluate the overall quality of the treatment delivered to these patients. Results  No immediate postoperative complication was registered, and no patient developed recurrence after a median follow-up period of 55 months. Two women experienced mesh bulging within 1 year after the operation. The long-term mean global health status registered was 97 out of 100. Conclusions  Radical resection aided by intraoperative margin evaluation via frozen sections followed by immediate mesh reconstruction is a safe procedure and can provide definitive cure without functional limitations for patients with desmoid tumors of the anterior abdominal wall.     

Desmoid tumors of the chest wall. A locally recurrent problem.

Desmoid tumors, in general, are known for their propensity to recur. To evaluate treatment and results, we reviewed the records of 32 patients admitted to our institution with a chest wall desmoid tumor from 1948 to 1988. There were 13 men and 19 women whose ages ranged from 12 to 67 years (median 36 years). Four patients had Gardner's syndrome. Treatment was wide resection. Median follow-up was 110 months. The overall 5-year survival was 93%; 5-year disease-free survival was 71%. The 5-year local recurrence rate was 29%. Since death from disease is uncommon after resection, but local recurrence is common, we recommend aggressive wide resection in patients who have desmoid tumors of the chest wall when seen initially.     

Pedicled superior epigastric perforator flap for reconstruction of a soft tissue defect at the ventral thoracic wall

The purpose of this paper is to present a new method of ventral chest wall reconstruction utilizing skin and fat from the abdominal wall pedicled on the superior epigastric artery and its comitant veins without sacrificing the rectus abdominis muscle. In a patient with a 13 × 15 cm left parasternal soft tissue defect of the thoracic wall, a paraumbilical perforator flap of skin and subcutaneous tissue was elevated with meticulous preparation of the perforators to the right superior epigastric vessels without sacrificing any muscle. The skin and fat island with its vascular pedicle was drawn through an epigastric subcutaneous tunnel to the ventral thorax and placed into the defect. Postoperative flap perfusion measured by laser Doppler flowmetry was excellent, abdominal wall reconstruction was easy, and 3 months after operation, the ability to perform sit-ups was as good as before the operation. In two further patients, we found the vascular anatomy to be unsuitable for this flap. The pedicled superior epigastric perforator (SEP) flap is suitable in certain cases but unreliable for reconstruction of soft tissue defects at the chest wall. Further investigations and operating experience may provide additional knowledge to allow wider and more secure use of the SEP flap.     

Reconstructive surgeries after extensive resections of anterior abdominal and chest walls for tumors

Experience of reconstructive surgeries after extensive resections of anterior abdominal and chest walls performed on account of tumors is described. Recovery of integrity of anterior abdominal wall was performed by serial grafting with dura mater and polypropylene net implant. The thorax was repaired with special grafts of the ribs and sternum. The integrity of the skin was reestablished with either suturing of the separated flaps or transposition of myodermal flap on vascular pedicle.