Meta-analysis of the influence of surgical margin and adjuvant radiotherapy on local recurrence after resection of sporadic desmoid-type fibromatosis.

BACKGROUND: Extra-abdominal desmoid-type fibromatosis (DF) is a rare, locally aggressive neoplasm that is usually managed conservatively. When treatment is indicated, it typically involves surgical resection, possibly with adjuvant radiotherapy. The indications for postoperative radiotherapy and its effectiveness are unclear. The objective of this study was to estimate the effect of surgical resection margins and adjuvant radiotherapy on rates of recurrence of DF. METHODS: Literature published between 1999 and 2015 was extracted from MEDLINE, Embase, Cochrane Central Registry of Trials, Web of Science and Google Scholar. Recurrence rate was analysed by meta-analysis and compared between subgroups. RESULTS: Sixteen reports were included, consisting of a total of 1295 patients with DF. In patients treated by surgical resection alone, the risk of local recurrence was almost twofold higher for those with microscopically positive resection margins (risk ratio (RR) 1·78, 95 per cent c.i. 1·40 to 2·26). Adjuvant radiotherapy after surgery with negative margins had no detectable benefit on recurrence. In contrast, after incomplete surgical resection, adjuvant radiotherapy improved recurrence rates both in patients with primary tumours (RR 1·54, 1·05 to 2·27) and in those with recurrent DF (RR 1·60, 1·12 to 2·28). CONCLUSION: DF resected with microscopically positive margins has a higher risk of recurrence. Adjuvant radiotherapy appears to reduce the risk of recurrence after incomplete surgical resection, particularly in patients with recurrent tumours.

Carbon ion radiotherapy for desmoid tumor of the abdominal wall: a case report.

BACKGROUND: Desmoid tumors, which are associated with familial adenomatous polyposis (FAP), tend to occur frequently in the abdominal wall and mesentery. Currently, there are no recognized treatments other than surgery, and frequent surgeries result in gastrointestinal obstructions and functional gastrointestinal disorders. CASE PRESENTATION: After surgery that was performed on a 39-year-old patient with FAP, we performed a second tumor excision which was the procedure used for frequently occurring mesenteric desmoid tumors. It was determined that the enlarged tumor would be difficult to operate on through an abdominal incision. Subsequently, the carbon ion radiotherapy of 50 Gy was then performed on the patient. Three years later, the tumor still remains reduced in size. In addition, we have not observed any negative effect on the digestive tract. CONCLUSIONS: This is the first instance that the carbon ion radiotherapy has been effective for the unresected desmoid tumor, and it is believed that this will become the one effective option for the treatment of desmoid tumors.

Proton therapy versus photon radiation therapy for the management of a recurrent desmoid tumor of the right flank: a case report.

Desmoid tumors are benign mesenchymal tumors with a strong tendency for local recurrence after surgery. Radiotherapy improves local control following incomplete resection, but nearby organs at risk may limit the dose to the target volume. The patient in this report presented with a recurrent desmoid tumor of the right flank and underwent surgery with microscopically positive margins. Particular problems presented in this case included that the tumor bed was situated in close proximity to the liver and the right kidney and that the right kidney was responsible for 65% of the patient's renal function. Intensity-modulated radiation therapy plans delivering 54 Gy necessarily exposed the right kidney to a V18 of 98% and the liver to a V30 of 55%. Proton therapy plans significantly reduced the right kidney V18 to 32% and the liver V30 to 28%. In light of this, the proton plan was utilized for treatment of this patient. Proton therapy was tolerated without gastrointestinal discomfort or other complaints. Twenty-four months after initiation of proton therapy, the patient is without clinical or radiographic evidence of disease recurrence. In this setting, the improved dose distribution associated with proton therapy allowed for curative treatment of a patient who arguably could not have been safely treated with intensity-modulated radiation therapy or other methods of conventional radiotherapy.

Outcome of aggressive fibromatosis treated with radiation therapy.

INTRODUCTION: The purpose of this study is to report the clinical course and outcome in 7 patients with aggressive fibromatosis. MATERIAL AND METHODS: Between the years 2000 and 2003, 7 patients who were treated with combined modalities were evaluated retrospectively. Patients' demographic information, including age and gender, tumour characteristics, surgical resection, and the use of radiotherapy were recorded and evaluated. RESULTS: The mean patient age was 34 years. The median time to follow-up was 15.5 months. Resection was performed with positive surgical margins in three cases. Three patients were evaluated as inoperable and one patient was treated with debulking surgery. All patients received radiation therapy with a median dose of 51 Gy. At follow-up, three patients had no evidence of disease, three patients were alive with disease, and one patient died 15 days after radiotherapy. CONCLUSION: Local control is the primary problem in aggressive fibromatosis. There is no appropriate treatment for aggressive fibromatosis and the type of treatment depends on tumour characteristics and location as well as patient characteristics.

Desmoid-type fibromatosis involving the brachial plexus.

Desmoid-type fibromatosis involving the brachial plexus is a rare and challenging disease. Due to involvement of crucial neurovascular structures, wide local excision of the associated fibromas is rarely feasible and recurrence is common. The authors describe their experience in four surgically treated patients with desmoid-type fibromatosis involving the brachial plexus and review the relevant neurosurgical literature. All tumors were assessed for c-KIT oncogene mutations in hopes of establishing a biological basis for using the tyrosine kinase inhibitor imatimib mesylate as an adjuvant therapy. Three patients experienced tumor recurrence requiring reoperation. Fractionated radiotherapy achieved local control in three patients, and the disease in one patient progressed beyond the treatment field. Single base pair changes at exon 10 of the c-KIT oncogene were identified in three tumors. One tumor with this mutation did not respond to treatment with imatimib mesylate. A review of the literature revealed 17 additional patients in two different case series. Analysis of these cases emphasizes the need for careful resection in patients with desmoid-type fibromatosis and supports the conclusion that without adjuvant radiotherapy a high local recurrence rate can be anticipated. For optimal local disease control, the authors recommend postsurgical radiation therapy regardless of the extent of resection achieved. The mutational status of the c-KIT oncogene remains an intriguing biological marker that in the future may predict which lesions will be responsive to imatimib mesylate; larger series will be necessary to test this hypothesis.

Desmoid tumors: a novel approach for local control.

BACKGROUND AND OBJECTIVES: After resection, desmoid tumors are associated with a recurrence rate that is typically 25-50%. Although this is an unusual problem, we instituted a prospective cohort study with neoadjuvant chemotherapy and radiation, followed by surgical resection, in an effort to improve local control. METHODS: Between 1985 and 1999, 13 patients with potentially resectable disease were managed with a treatment protocol of preoperative doxorubicin (30 mg continuous infusion daily for 3 days) and radiotherapy (10 x 300 cGy). Resection was performed 4-6 weeks later. All lesions were resected with an intended margin of 1 cm, but clear adventitial margins were accepted in order to preserve critical structures. RESULTS: The median follow-up was 71 months (range, 22-109). Six patients (46%) presented after failure of a previous surgery. Clear microscopic margins were obtained in 11 patients, and 2 patients had positive margins. There were two local recurrences (15% local recurrence). Both recurrences followed resection of large thigh lesions, which appeared at 30 and 49 months of follow-up. In one patient with a chest wall tumor, two new primary desmoid tumors developed outside the treatment area, in the ipsilateral arm and forearm. Eleven patients have been disease free for a median of 71 months (range, 22-109). CONCLUSIONS: For potentially resectable lesions, this protocol provides excellent local control, even in those with recurrent disease. Neoadjuvant treatment with doxorubicin and radiotherapy appears to be a better option than surgery alone, or surgery and adjuvant radiotherapy. These results need to be confirmed in larger, prospective randomized trials.

The efficacy of radiotherapy as postoperative treatment for desmoid tumors.

PURPOSE: The purpose of this study was to determine if radiotherapy is a beneficial adjuvant treatment after desmoid tumor resection. METHODS AND MATERIALS: A retrospective analysis was performed on 54 patients who underwent surgery without prior radiation at our institution between 1982 and 1998 to remove a desmoid tumor. Thirty-five patients had adjuvant radiation therapy after surgery, and 19 patients had surgery alone without immediate postoperative radiation. Sixteen of the 35 patients who underwent immediate postoperative radiation treatment had at least one prior resection before reoperation at our institution. Recurrence was defined as radiographic increase in tumor size after treatment. Follow-up interval (mean 39 months) and duration of local control were measured from the date of surgery at our institution. Potential prognostic factors for time to tumor progression were analyzed. RESULTS: Adjuvant treatment with radiation was the only significant prognostic factor for local control. The five-year actuarial local control rate was 81% for the 35 patients who underwent radiation in addition to surgery, compared to 53% for the 19 patients who underwent surgery alone (p = 0.018). For the patients who did not receive adjuvant radiation, only younger age at the time of surgery was associated with increased risk of failure (p = 0.035). Gross or microscopic margin status and number of prior operations were not detected as prognostic for local failure. For patients who did receive postoperative radiation, only abdominal location was associated with increased risk of failure (p = 0.0097). CONCLUSION: Radiation treatment as an adjuvant to surgery improved local control over surgery alone. Multiple operations before adjuvant radiation did not decrease the probability of subsequent tumor control. Radiation should be considered as adjuvant therapy to surgery if repeated surgery for a recurrent tumor would be complicated by a significant risk of morbidity.

Regression of an aggressive abdominal desmoid tumor in a patient with familial adenomatous polyposis by hyperthermoradiotherapy.

A case of huge desmoid tumor successfully treated by hyperthermoradiotherapy is described. A 23-year-old man with familial adenomatous polyposis was operated upon for a desmoid tumor in the mesenterium involving the right kidney and small intestine in 1988. In 1990, the tumor recurred and could not be resected because of the involvement of the vena cava. The tumor grew larger and larger, and occupied two-thirds of the right lower quadrant. Several therapies using sulindac, tamoxifen, prednisolone, indomethacin, luteinizing hormone-releasing hormone analogue, and ascorbate were all ineffective. Finally, the combination of radiation and hyperthermia was used over a 6-month period. At the end of the hyperthermoradiotherapy, the tumor in the abdominal wall was markedly reduced in size, and the protruded abdominal wall became flat. To our best knowledge, this is the first report of the successful treatment of a huge desmoid tumor by hyperthermoradiotherapy.

Triple-phase bone scan findings in aggressive fibromatosis. Before and after radiation therapy.

The authors present comparative triple-phase bone scan findings in three cases of histologically proven aggressive fibromatosis both before (initial evaluation) and after radiation therapy. The purpose of the study was to compare triple-phase bone scan findings in aggressive fibromatosis both before and after radiation therapy and to determine whether any additional physiological information could be obtained. Before radiation therapy, the triple-phase bone scintigraphy demonstrated increased flow and radiotracer pooling in the areas of tumors on dynamic flow and immediate blood pool images, respectively. However, the delayed static images demonstrated variable radiotracer uptake. When compared to preradiation therapy triple-phase bone scan, decreased vascularity was well demonstrated in all three patients after radiation therapy. In addition, it also provided information regarding the changes in the size and extent of tumor, noninvaded underlying bone, and remainder of the skeleton. This additional information can be particularly useful in patients with equivocal or questionable histologic diagnosis especially from small, unrepresentative biopsies.