Desmoid tumors of the chest wall.

Desmoid tumors are rare soft tissue neoplasms derived from fascial or musculoaponeurotic structures. These tumors are histologically benign but may behave aggressively at the local level with multiple recurrences after complete resection being common. Chest wall desmoids account for approximately 20% of all patients with desmoid tumors. Patients with these lesions are often asymptomatic and thus commonly present with lesions greater than 10 cm in size. The treatment for these neoplasms remains margin negative surgical excision, and, given the often large size at presentation, may require extensive chest wall resection. When this is required, chest wall reconstruction with either prosthetic material and/or autologous tissue may be performed. There may be recurrence in as many as 75% of patients. Neither adjuvant radiotherapy or chemotherapy have been shown to reduce the rate of recurrence. Recurrence should also be treated with surgical resection because patients who undergo complete surgical resection of recurrence are as likely to remain disease free after resection as patients who present with primary disease.     

Desmoid tumors of the chest wall.

OBJECTIVE: Desmoid tumors of the chest wall are uncommon fibromatous tumors characterized by their local invasion and frequent recurrences. They are considered low-grade malignant sarcomas. We analyze our results and evaluate our experience in their surgical treatment. PATIENTS AND METHODS: From 1980 and 1999, eight patients were operated in our service for desmoid tumors of the chest wall. There were five males and three females with a mean age of 32 years old (17-52 years). The diagnosis was established on the basis of clinical signs and imaging studies (Chest X-ray and computed tomography). Surgery was done by direct approach of the tumors. RESULTS: None of the patients had Gardner's syndrome. Complete resection was done in seven of the eight cases. Histology confirmed all cases. The follow-up ranged from 24 and 180 months, for a mean of 82 months. There was one death from cardiac and renal failure 3 months after a fifth resection associated with adjuvant radiation therapy. No other post-operative complications were observed. Recurrences occurred in 50% of cases, all treated by surgery, associated to radiotherapy in one case. CONCLUSION: Surgical treatment of primary desmoid tumors of the chest wall requires wide local resection. In cases of recurrences, multimodality therapies should be considered.     

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.     

Desmoid of the anterior chest wall

The tumor is of interest for several reasons. It occurs in males but in a minority of cases. Likewise it usually is found in connection with the musculo-aponeurotic structures of the anterior abdominal wall and only occasionally in other sites. A definite history of trauma was obtained from the patient which gives further support to the theory of traumatic origin in connection with individual predisposition as a cause for the appearance of these tumors.     

Desmoid tumor of the chest wall foiling surgery

AIM OF THE STUDY: The Authors report on a patient personally observed owing to a tumefaction, mimicking a pomelo, located on the posterior chest wall, in the same site of another past surgical operation dating back to four years ago. This tumefaction was clear at the inspection, not aching and hard-elastic at the palpation, mobile synchronously with respiratory movements, but unable to alter respiratory mechanics. CASE REPORT: The patient was subjected to laboratory tests, which showed nothing pathological, and to instrumental tests (RX and TAC of the chest, bony scintigraphy) which showed a roundish solid tumefaction, with no "secondary" interest of bony tissue. In this case, it was executed a posterior-lateral thoracothomy, at the VI intercostal space, in the area circumscribing the past surgical scar. The careful removal of the adhesions between the mass and the costal plane, not without the sacrifice of the periosteum, permitted us the total exeresis. The anatomo-pathological test showed a desmoid fibromatosis (desmoid tumor) extra-abdominal (12.5 x 9 x 5 cm). About this kind of neoplastic masses, the risk of post-surgical relapse is very high; so many Authors consider opportune a radio-chemical adjuvant therapy. In this case, the radical excision allowed the Authors to avoid the post surgery pharmacological treatment and to get no relapses after two years from the operation.     

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.     

Results of chest wall resection for recurrent or locally advanced breast malignancies

Between 1998 and 2003 we observed 15 women who underwent full thickness chest wall resection (FTCWR) followed by plastic reconstruction for locally recurrent or primary breast cancer. Preoperative symptoms were: pain (5 patients), malodorous ulceration (3 patients), presence of tumour mass (4 patients) and thoracic deformity (2 patients). One patient was asymptomatic. Surgery was partial sternectomy with rib resection in 9 patients, rib resection alone in 5, and total sternectomy in one. No perioperative mortality or major morbidity occurred; minor complications occurred in 3 patients (20%). Five of the six surviving patients reported a positive overall outcome in a telephonic interview. Median overall and disease-free survival were 23.4 and 17.5 months, respectively. In conclusion, FTCWR is a safe procedure with low morbidity and mortality that can provide good symptoms palliation in patients with locally advanced breast malignancies, so it should be considered more often by interdisciplinary care providers in those patients who fail to respond to classic multimodality treatment.     

Desmoid tumor of the chest wall in an elderly woman

We report a case of desmoid tumor of the chest wall in a 79-year-old woman. The patient was referred to our hospital for management of a chest wall mass. Four months previously, the patient noted a small lump in the right lateral chest wall that had rapidly increased in size. Magnetic resonance imaging of the chest revealed a soft tissue tumor in the right lateral chest wall with unclear margins that extended into the intercostal muscles. Positron emission tomography with ¹⁸F-fluorodeoxyglucose (FDG) showed slight FDG accumulation at the lesion. Because open biopsy suggested a desmoid tumor, full-thickness chest wall resection with reconstruction was performed. The final diagnosis was desmoid tumor of the chest wall. Wide surgical resection during the initial operation is an essential element in the treatment of this tumor.     

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 tumors of the anterior abdominal wall. Apropos of a case

The authors describe a case of a desmoid tumor of the anterior abdominal wall. From a review of the world literature, the main characteristics of these tumors are detailed, and the various pathophysiological hypotheses, demonstrating the necessity of exclusively surgical treatment, are presented.     

Malignant bone tumors of the chest wall.

Malignant bone tumors constitute only 0.2% of all tumors. Bone sarcomas occur at a rate approximately one tenth that of sarcomas of the soft tissue. Malignant bone tumors of the chest wall and sternum are even more rare because most bone tumors occur in the long bones or joints. Because of the relative paucity of experience treating these malignancies, progress in successful therapies has been limited. Chondrosarcomas remain the most common bony malignant chest wall lesions and are discussed elsewhere in this issue. Other lesions in descending order of incidence include Ewing's sarcoma, osteosarcoma, malignant fibrous histiocytoma, solitary plasmacytoma, and Askin tumors. This article reviews these remaining five malignant bony chest wall tumors, along with their symptoms, presentations, and current approaches to therapy.     

Primary chest wall tumors

The differential diagnosis of chest wall tumors is diverse, including both benign and malignant lesions (primary and malignant), local extension of adjacent disease, and local manifestations of infectious and inflammatory processes. Primary chest wall tumors are best classified by their primary component: soft tissue or bone. Work-up consists of a thorough history, physical examination and imaging to best assess location, size, composition, association with surrounding structures, and evidence of any soft tissue component. Biopsies are often required, especially for soft tissue masses. Treatment depends on histological subtype and location, but may include chemotherapy and radiotherapy in addition to surgical resection.     

Primary chest wall tumors

A retrospective study of 53 primary chest wall tumors, 26 benign and 27 malignant, was carried out to review their clinical radiological and pathological features. Forty-nine of the 53 lesions presented in the ribs and the remaining 4, in the sternum. The overall 5-year survival for patients with primary malignant neoplasms of the chest wall was 33.3%, and the 10-year survival was 18.5%. All of the deaths were disease related. All of the patients with benign tumors were treated by excision without recurrence or death. Distinction between benign and malignant chest wall tumors was not possible using radiographic criteria unless cortical destruction and involvement of soft tissues were visualized. On the basis of our analysis, we believe that all tumors of the chest wall should be considered malignant until proven otherwise and that wide excision should be carried out. This is necessary not only to obtain an adequate diagnosis but also to provide the best chance for cure in both benign and malignant lesions.