Desmoid Tumors of the Chest Wall (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.
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.
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.
Lung India, 2012
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.
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.
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.
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.
Desmoid tumors: need for an individualized approach
Expert Review of Anticancer Therapy, 2009
Desmoid tumor, also known as aggressive fibromatosis or desmoid-type fibromatosis, is a rare monoclonal, fibroblastic proliferation arising in musculoaponeurotic structures. Although histologically benign, desmoids are often locally invasive and associated with a high local recurrence rate after resection. Since it is a heterogeneous disease, in particular regarding clinical presentation, anatomic location and biological behavior, treatment should be individualized to reduce local tumor control failure with concurrently acceptable morbidity and preservation of quality of life. Many issues regarding optimal treatment of desmoids remain controversial. However, wide surgical excision remains the treatment of choice, except when surgery is mutilating and is associated with considerable function loss or major morbidity. Involvement of surgical margins is probably associated with an increased risk of local recurrence. Postoperative radiotherapy results in a significant reduction of the local recurrence rate, but only in the case of involved surgical margins. Radiotherapy for gross disease is considerably effective, but is associated with a relatively high rate of complications, which are usually mild or moderate and radiation dose dependent. Radiotherapy should only be applied where anatomic constraints preclude complete resection and radiotherapy is not too toxic. Risk factors for local tumor control failure include young age, large size, presentation as recurrent disease, limb/girdle or intra-abdominal location, involved surgical margins, omission of radiotherapy, radiation dose less than 50 Gy and insufficient radiation field size. Increased comprehension of the pathogenesis and biological behavior of desmoids resulted in the emerging applicability of systemic therapies and a wait-and-see policy. Systemic treatment may be indicated in patients that have anatomic barriers to effective surgery or radiotherapy. Considering the significant morbidity of surgery and/or radiotherapy for certain locations, especially mutilation and loss of function, and the tumor's natural history, which is often characterized by prolonged periods of stability or even regression, a period of watchful waiting may compose the most appropriate management in selected asymptomatic patients. Attempts to complete eradication of the disease may be worse than the disease itself.