Intra-thoracic desmoid tumor (original) (raw)
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An unusual case of chest wall desmoid tumor
Indian Journal of Surgery, 2010
Desmoid tumor of the chest wall are uncommon fibromatous tumors characterized by their local invasion and frequent recurrences. Extra-abdominal sites are mainly the shoulder girdle, the pelvic girdle and distal ends of the lower limbs. The chest wall represents 8–10% of cases and the tumor is exceptionally intrathoracic. We present here a rare case of a large desmoid tumor of left antero-lateral chest wall. In the presented case, wide excision of the tumor and chest wall reconstruction was done. There was no recurrence in 3 years of follow up.
Desmoid Tumors of the Chest Wall
Asian Cardiovascular and Thoracic Annals, 2006
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.
Chest wall and intrathoracic desmoid tumors: surgical experience and review of the literature
European journal of medical research, 2009
Desmoid tumors are fibroblastic/myofibroblastic neoplasms, which originate from musculo-aponeurotic structures and are classified as deep fibromatoses. Despite their benign histologic appearance and lack of metastatic potential, desmoid tumors may cause aggres?sive local infiltrations and compression of surrounding structures. They are often associated with female gender, familial adenomatous polyposis (FAP) and sporadically may occur at sites of previous trauma, scars or irradiation. Molecular studies have demonstrated that these patients are associated with a bi-allelic APC mutation in the affected tissue. Radical tumor resection with free margins remains the first therapy of choice. In cases with anatomical or technical limitations for a wide excision, radiation therapy represents a proven and effective alternative or supplementary treatment.
Tuberculosis and Respiratory Diseases, 2015
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 desmoid tumors of the chest: long-term follow-up of 28 multimodally treated patients
European Journal of Cardio-Thoracic Surgery, 2011
Introduction: Desmoids of the chest are extremely rare borderline tumors. Radical surgical resection is considered to be the primary treatment. Achieving negative margins is often a challenge. Cases with positive surgical margins are associated with high risk of local recurrence. Methods: A retrospective multicenter review was undertaken of 28 patients who underwent surgery for sporadically appearing desmoids of the chest between 1988 and 2008. Clinico-pathological data were investigated in detail. Authors have statistically analyzed the relationships between gender, age, tumor size, radicality of the first surgery, impact of the pharmacologic treatment, estrogen receptor positivity, and the development of local recurrences after a median follow-up period of 104 months. Results: Primary surgery was radical in 14 patients (50%). Mean pathologic diameter was 72.14 mm. Wide surgical excision was performed in 27 primary cases, out of which 10 cases (37%) were full-and 17 cases (63%) partial-thickness chest wall resections. Synthetic mesh stabilization was used in nine cases and soft tissue coverage in six patients. Morbidity rate was 25%. Recurrences were found in 63% of the cases, with a mean time of 30.5 months to first recurrences. Our investigation confirmed that microscopically free surgical margin of the first tumor resection significantly affected local tumor control. Conclusions: Because the radicality of the first surgical resection is of essential importance for long-time local control of chest desmoids, accurate preoperative diagnostics and well-planned aggressive surgical resection of the primary tumor is recommended. Due to the low incidence of desmoids, multicentric randomized investigations would be mandatory to establish evidence-based protocol for desmoid tumors.
Desmoid-type chest wall fibromatosis. A six cases series
Orthopaedics & Traumatology: Surgery & Research, 2011
Purpose of the study: Desmoid-type fibromatosis is a fibroblastic proliferation that develops in the deep soft tissues and is characterized by infiltrative growth and a tendency towards local recurrence but does not metastasize. Fibromatosis of the chest wall is rare, representing only 10 to 20% of all deep fibromatoses. Case report: We report a six cases series of fibromatosis of the chest wall treated at our institution between 1996 and 2009. There were five men and one woman with a mean age of 42.2. Swelling of the chest wall was the most frequent symptom. Imaging showed an expansive homogeneous mass, invading the rib (four cases) or the sternum (two cases). Surgical resection was performed in all cases. The outcome was favorable (five cases) or marked by local recurrence (one case). Discussion: Although it is considered to be a benign lesion, this tumor can be locally very aggressive, and has a high local recurrence rate depending upon the efficacy of surgical resection.
Unique Treatment Considerations for Chest Wall Desmoid Tumor Invading the Breast
Archives of Surgery and Clinical Case Reports, 2018
Desmoid Tumors (DT) are rare tumors, characterized by infiltrative growth and a tendency toward local recurrence but with an inability to metastasize. Although surgery is the primary treatment modality, there remains significant controversy amongst surgeons regarding the management of disease involving adjacent breast given the possibility for significant functional and aesthetic compromise [1]. This is a rare and interesting case of a young woman with a massive chest wall DT displacing the breast necessitating extensive chest wall resection and right partial mastectomy.
Multidisciplinary treatment of intra-thoracic desmoid tumors: Case series and narrative review
Medical Science Monitor, 2012
Background: Primary intra-thoracic desmoids are exceedingly rare borderline tumors, with 34 reported cases in the English-language literature. The characteristic localized infiltrative growth and the high rate of recurrence can result in life-threatening conditions. Radical surgical resection is considered to be the primary treatment. Achieving negative surgical margins is a challenge. Cases with positive surgical margins are associated with a high rate of local recurrence; therefore, other multimodal approaches play a large role in their therapy.