Extra-abdominal Desmoid Tumor Research Articles

Initial treatment for surgery-naïve desmoid tumors by high intensity focused ultrasound.

Desmoid tumor (DT) is a rare proliferative disease occurring in connective tissues, characterized by high infiltration and recurrence rates. While surgery remains the primary treatment, its recurrence risk is high, and some extra-abdominal desmoid tumors are inoperable due to their locations. Despite attempts with radiotherapy and systemic therapy, the efficacy remains limited. We used low-power cumulative high-intensity focused ultrasound (HIFU) therapy as an initial treatment for desmoid tumor patients either ineligible or unwilling for surgery. Low-power cumulative HIFU employs slower heat accumulation and diffusion, minimizing damage to surrounding tissues while enhancing efficacy. Fifty-seven non-FAP desmoid tumor patients, previously untreated surgically, underwent low-power cumulative HIFU therapy. Among them, 35 had abdominal wall DT, 20 had extra-abdominal DT, and 2 had intra- abdominal DT, with an 85% median ablation ratio. Abdominal wall DT patients showed significantly better response rates (91.4% vs. 86%) and disease control rates (100% vs. 32%) than that of non-abdominal wall DT patients. Median event- free survival time was not reached after a median follow-up duration of 34 months. With its high response rate, durable efficacy, and mild adverse effects, our findings suggest that low-power cumulative HIFU presents a promising novel treatment for desmoid tumors, particularly abdominal wall DT patients.

Abstract No. 175 Transarterial chemoembolization with Doxorubicin-eluting beads for extra-abdominal desmoid tumors: initial experience

To evaluate the efficacy and safety of Doxorubicin-eluting bead transarterial chemoembolization (DEB-TACE) in extra-abdominal desmoid tumor (DT)

Extra-abdominal desmoid fibromatosis: An evaluation of clinical factors affecting local recurrence rates.

The aim of this study was to determine the prognostic factors for recurrence in patients with extra-abdominal desmoid tumors (EDTs) treated surgically. This single-institution, retrospective study included patients with a histologically-proven extra-abdominal desmoid tumor between 2007 and 2018. The demographic characteristics (age, sex) of the patients, tumor characteristics (region, size, proximity to neurovascular structures, margins), treatment management (surgery and/or adjuvant radiotherapy), and clinical results were analyzed. The effects of these possible prognostic factors on overall and disease-free survival rates and the risk of local recurrence were evaluated. Evaluation was made of 22 patients (16 females, 6 males) with a mean age at diagnosis of 34.7 years (range = 22-76 years). The mean follow-up was 104 months (range = 4.8-168). Tumor localization was in the upper extremity in 4 patients (18.1%), the lower extremity in 11 (50.0%), and the trunk in 7 (31.8%). The mean tumor size (maximum diameter) was 5.2 cm (range = 0.6-13 cm; median = 5.8 cm), and the mean tumor volume was 181.3 ± 531.4 ml. All the 22 patients were treated surgically along with adjuvant radiotherapy (RT) administered to 8 in addition to surgery for the primary treatment of the tumor. Following primary surgery, resection margins were R0 in 11 patients, R1 in 9 and R2 in 2. Local recurrence (LR) developed in 6 patients (27.2%) during the follow-up period. Recurrence-free survival rate (RFS) was 90.9% at one year, 74.1% at 5 years, and 61.7% at 10 years. During the follow-up, no patient died, and distant metastasis was not detected. Tumor length, resection margins, and adjuvant RT were observed to influence the risk of local recurrence (P < 0.05). The results of this study have demonstrated that tumor size ≥ 5 cm and the presence of microscopic or macroscopic positive surgical margins can increase the risk of LR, and adjuvant RT can reduce the development of LR in the management of EDT.

Management of rapidly growing recurrent extra-abdominal pediatric desmoid tumor: case report

: Desmoid tumors are rare, benign soft tissue tumors that occur spontaneously, or less frequently, in patients with a genetic predisposition. With an unpredictable course, desmoid tumors are often locally aggressive and have a high rate of recurrence even after complete surgical excision. Desmoid tumors often become a chronic problem for patients, making them a major source of morbidity, and may even lead to mortality. Management of these tumors can be frustrating, with patients often requiring multiple modes of therapy in pursuit of a cure. We present a patient with a history of familial adenosis polyposis (FAP) who presented early on in life with an extra-abdominal desmoid tumor that proved to be a major source of morbidity during his childhood. We review a chronological approach to the management of our patients’ tumor with the resulting outcomes following each mode of therapy, which included observation, surgical resection, systemic therapy, and radiotherapy. Additionally, we review the currently literature with regards to the recommended management of desmoid tumors in different settings, including an in-depth discussion on the outcomes and failures of management following observation, systemic therapy with non-cytotoxic and cytotoxic therapies, surgical resection alone, radiation therapy (RT) alone, and a combination of surgical resection and RT.

Percutaneous cryoablation for advanced and refractory extra-abdominal desmoid tumors

To assess efficacy and safety of percutaneous cryoablation (CA) for advanced and refractory extra-abdominal desmoid tumors. This retrospective study reviewed 30 consecutive patients with symptomatic desmoid tumors evolving after "wait and watch" periods, and despite medical treatment, treated by CA between 2007 and 2019. Progression free survival (PFS), objective response rate, pain reduction (decreased of visual analogic scale pain (VAS)[Formula: see text] 3 or disappearance of pain), total volume lesion (TVL) and complications were documented. Kaplan Meier method was used to outline PFS. Paired sample t test was used to compare volume of tumors before treatment and at 1 and 3year. With a median follow-up of 18.5months (range 6-93months, interquartile range (IQR): 12-55), the PFS was 85.1% at 1year and 77.3% at 3years. Objective response was obtained for 80% of patients with a complete response for 43% patients. Pain reduction was obtained for 96.7% (95% confidence interval (95% CI): 90.3, 100) of patients. Median volume of desmoid tumor before treatment was 124.1cm3 (range 2-1727cm3, IQR: 54-338cm3). Median change of TLV after ablation was 66.6% (95% CI: 37.2, 72.3; p = 0.002) at 1year and 76.4% (95% CI: 59.1, 89.8; p = 0.002) at 3year. Adverse events rate was 36.6%, the most common was edema and temporary increase of pain in the days following CA. Four patients experienced a major complication (13.3%): 2 skin necrosis, 1 infection and 1 brachial plexopathy. CA is an effective treatment for advanced and refractory extra-abdominal desmoid tumor, that induces durable responses.

Does A Vertebra Fracture Cause A Tumor? A Dorsolumbar Paraspinal Desmoid Tumor: Case Report

AbstractExtraabdominal desmoid tumors are uncommon soft-tissue tumors. The etiology of the tumor is still unclear. Injury is one of the etiological factors of soft-tissue tumors. A 41-year-old female patient who had a traumatic vertebral body fracture on the thoracic spine was treated conservatively. Two and a half years later, she presented a painful, palpable swelling on the thoracolumbar region. In the present report, was discuss the patient, who underwent a surgery to remove the desmoid tumor (aggressive fibromatosis), within the context of the current literature. The literature on desmoid tumor caused by a trauma is rare. This is the first case that demonstrates an extraabdominal desmoid tumor following a spinal fracture. The swelling on the region of the trauma must be examined carefully and desmoid tumor must be kept in mind as a possible diagnosis.

Percutaneous Cryoablation of an Extra-Abdominal Desmoid Tumor Abutting the Skin Surface and Peritoneum

Percutaneous Cryoablation of an Extra-Abdominal Desmoid Tumor Abutting the Skin Surface and Peritoneum

Clinical prognostic factors for pediatric extra-abdominal desmoid tumor: analyses of 66 patients at a single institution

Background and purposePediatric desmoid tumor (PDT) is rare and has a high local recurrence rate. The purpose of the present study was to analyze clinical risk factors of local recurrence in PDT patients.Materials and methodsWe reviewed clinical data of 66 PDT patients from 2004 to 2015. All patients underwent macroscopically complete resection, and some recurrent tumors were prescribed radiotherapy. Factors such as sex, age at presentation, location, and proximity to nerves or vasculature were analyzed. The local recurrence rate and recurrence-free survival were analyzed with these factors.ResultsAll patients in the present study were children and had extra-abdominal tumors. The median follow-up time was 6.6 years. Thirty-six (55%) patients had local recurrence. Age, sex, tumor site, tumor size, and proximity to nerves/vasculature had a significant impact on prognosis in univariate analysis. Radiotherapy decreased the local recurrence rate. In multivariate analysis, younger age, tumor location in buttocks, larger tumor, and proximity to important nerves/vasculature were independent risk factors for poor prognosis.ConclusionsFavorable therapeutic strategies could be selected according to the preoperative prognostic risk factors. Radiotherapy should be considered for local recurrence of PDT.

Extra abdominal desmoid tumor (fibromatosis) of the breast

Desmoid tumors (or fibromatosis) of the breast are very rare findings. Their clinical and imaging features can easily mislead diagnosis, the lesions being diagnosed as breast cancer. The case of a 46-year old female patient, who presented a desmoid tumor in the area of a previously excised accessory breast, is reported. A review of the international literature revealed no other similar case. The differential diagnosis and current opinion on treatment of these tumors is discussed.

Plantar Fibromatosis: Pathophysiology, Surgical and Nonsurgical Therapies: An Evidence-Based Review.

Level V: Expert opinion.

EXTRA-ABDOMINAL DESMOID TUMOR: ANALYSIS OF 23 CONSECUTIVE CASES IN A SINGLE INSTITUTION.

ABSTRACTObjective:Extra-abdominal desmoid tumor (EDT) is a rare condition, caused by proliferation of fibroblasts. Despite being a benign tumor, it is locally aggressive and has unpredictable clinical behavior. The objective of this study is to present the clinical outcomes of patients with EDT treated surgically between 1995 and 2016.Methods:This is a retrospective series of 23 patients with histopathological diagnosis of EDT that underwent surgery at the orthopedic oncology service of our hospital. The information was obtained from the institute's clinical and pathology reports.Results:A total of 223 medical records with histopathological reports were evaluated. Only 23 cases of EDT were included in the present study. The mean age was 22.5 years. Twelve (52.2%) patients had the tumor on the lower limbs, seven (30.4%) on the upper limbs and four (17.4%) cases were reported on the back. Five (21.7%) patients had tumors measuring less than 5 cm, while eighteen (78.3%) patients had tumors measuring more than 5 cm. All patients underwent surgery as the definitive treatment in our institute. Twelve (52.2%) cases presented negative margins (NM) and eleven (47.8%) cases had positive margins (PM). Local recurrence (LR) occurred in eleven (47.8%) patients.Conclusion:Impairment of the surgical margin was the only prognostic factor found for LR of EDT. Level of Evidence IV, Case Series.

Limb Salvage Treatment of Large Multicentric Extra-Abdominal Desmoid Tumor of Lower Extremity

Limb Salvage Treatment of Large Multicentric Extra-Abdominal Desmoid Tumor of Lower Extremity

The Impact of Radiotherapy in the Treatment of Recurrent Desmoid Tumor of Neck: A Case Report with Literature Review

Background: Extra-abdominal desmoid tumor of the head and neck in particular commonly infiltrate vital neurovascular structures. we report a case of a large and recurrent extra-abdominal desmoid tumor causing significant morbidity and who was treated successfully by radiotherapy. Case presentation: A 34-year-old male patient presented with a history of right neck mass for 15 months, which was gradually increasing in size. Cervical CT scan revealed a large mass of the posterior soft tissues of the neck extended to the soft tissues of the posterior thoracic wall. The CT density of the mass was that of fat tissue. It was measuring 12.4 × 85.9 mm and enhanced after injection of the contrast product. A biopsy was taken. The histopathological findings were suggestive of desmoid tumor. Discussion: This case was discussed in multidisciplinary oncological team and radiotherapy was planned. The surgery was proposed but not performed due to the recurring character of the tumor and the risk of a high postoperative morbidity. After 4 weeks of treatment, the clinical evaluation showed a good response. Conclusion: Alternative treatment modalities such as primary radiotherapy may be preferable to mutilating surgery.

Intra-lesional NSAIDs Injection: Unreported Treatment in (Extra-abdominal) Desmoid Tumors.

Desmoid tumors are a rare benign tumor that can be locally aggressive and causemorbidity to a patient. The best way of treatment, whether surgical or non-surgical, hasyet to be found. We report an unreported method of treatment in (extra-abdominal)desmoid tumor which showed a positive result. Our patient presented with (extraabdominal)desmoid tumor on her left forearm that caused her pain and paresthesia inthe left forearm. The mass was located near an important neurovascular structure makingthe authors opt for conservative management. Desmoid tumor cells show expressionof COX-2 and beta-catenin. NSAIDs the COX enzyme inhibitors are used orally toconservatively treat (extra-abdominal) desmoid tumor. To our knowledge, NSAIDshave never been used locally. Here we report our treatment of (extra-abdominal)desmoid tumor by injecting NSAIDs directly into the tumor and follow up on theclinical and radiographic study of the patient. This diversion of treatment, which hadnever been done before, showed a positive outcome in decreasing symptoms and sizeof the tumor in our patient. In this reported case, local NSAIDs injection into (extraabdominal)desmoid tumor showed a positive result. We hope this case report can leadto a further study of this way of treatment and we hope that this report could be a pathto a further study for the cure of desmoid tumors.

Bilateral desmoid tumor of the breast: case seriesand literature review.

BackgroundDesmoid tumor of the breast is very rare and locally aggressive but has no distant metastasis. Bilateral lesions are extremely rare, found in only 4% of patients. Two cases of bilateral desmoid tumor of the breast are reported. The clinical presentation, diagnosis, imaging, treatment, and follow-up outcomes of recurrence as well as a brief literature review are provided.Case reportsCase 1 is a 31-year-old woman who presented with nipple retraction. An ultrasound revealed BIRAD V in both breasts. She underwent a bilateral excisional biopsy under ultrasound mark with the pathology result of extra-abdominal desmoid tumor in both breasts. The patient had a bilateral mastectomy with silicone implantation due to the involved margins by excision. She remained tumor free after 7-year follow-up. Case 2 is a 28-year-old woman who presented with a lump on her right breast that she had discovered ~2 months earlier. An ultrasound showed a spiculated mass in the right breast and some circumscribed hypoechoic masses in both breasts. A bilateral breast excision was done. The pathology result was an extra-abdominal desmoid tumor. She had recurrence on both sides and underwent a mastectomy and silicone implantation. The tumor has not recurred after 1-year follow-up.ConclusionImaging cannot distinguish between benign breast lesions and malignancy. Pathology results are helpful in making a definitive diagnosis. Given that the desmoid tumor is locally aggressive, a local excision with clear margins is recommended. Chemotherapy and hormonal treatment are controversial.

Extra-abdominal desmoid tumor: A case report

Desmoid tumor represents a rare monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Although histologically benign, desmoids are locally invasive and associated with a high local recurrence rate, but lack of metastatic potential. Many issues regarding the optimal treatment of patients with desmoids remain controversial. Surgical resection and radiotherapy are standard treatment options for these patients. Due to heterogeneity of the biological behavior of desmoids, including long periods of stable disease or even spontaneous regression, treatment needs to be individualized to optimize local tumor control and preserve patient's quality of life. Therapeutic approaches to the treatment of recurrent or unresectable desmoid tumors comprise anti-hormonal therapy, non-steroidal anti-inflammatory drugs, classic chemotherapy regimens and tyrosine kinase inhibitor, with highly variable results. It has not yet been possible to establish an optimal therapy protocol for this disease. In this case report we present our experience with the treatment of recurrent extra-abdominal desmoid tumor.

EXTRA-ABDOMINAL DESMOID TUMOR: LOCAL RECURRENCE AND TREATMENT OPTIONS.

ABSTRACTObjective: To evaluate the rate of local recurrence of extra-abdominal desmoid tumor and compare the outcomes of surgical treatment and conservative treatment.Methods: Twenty one patients (14 women and seven men), mean age 33.0±8.7 years old, with a diagnosis of desmoid tumor were evaluated. The mean follow-up period was 58.5±29.0 months. Fourteen cases involved the lower limbs, four cases involved the upper limbs, and three cases involved the trunk. The average tumor size was 12.7±7.5 cm. Of the 21 patients, 14 did not undergo previous treatment and seven patients relapsed before the initial evaluation. Surgical treatment was performed in 16 patients and conservative treatment was performed in five patients.Results: Recurrence occurred in seven patients (33%) and six of them relapsed within the first 18 months. No significant difference was observed between conservative and surgical treatment. However, a significant difference was observed among patients undergoing wide resection and who experienced improved local control.Conclusion: The recurrence rate of desmoid tumor was 33.3%. There was no difference in recurrence between conservative and surgical treatment. In surgical treatment, wide margins showed better results for recurrence control. Level of Evidence III. Retrospective Observational Study.

Percutaneous Cryoablation of Extraabdominal Desmoid Tumors: A 10-Year Experience.

Extraabdominal desmoid (EAD) tumors pose a therapeutic challenge because they often recur locally and behave aggressively. Accepted management options include surgery, radiation, chemotherapy, and observation. The objective of this study was to assess the safety and efficacy of percutaneous cryoablation for the treatment of EAD tumors. A retrospective search of our cryoablation database was performed to identify patients with EAD tumors treated with percutaneous cryoablation between June 15, 2004, and June 15, 2014. During this 10-year time period, we treated 18 patients with 26 discrete tumors during 31 treatment sessions. After cryoablation, contrast-enhanced MRI or CT was performed. Any enhancing soft tissue was considered viable EAD tumor and was measured in three planes. Of the 26 EAD tumors treated, follow-up imaging with IV contrast material was available for 23 tumors. The mean imaging follow-up was 16.2 ± 20.0 (SD) months. All 31 sessions were technically successful procedures. No residual viable EAD tumor was observed in nine of 23 tumors (39.1%). Some degree of volume reduction was evident in 22 of 23 tumors (95.7%). Progressive disease was observed in one of the 23 tumors (4.3%). Of the cases with residual or progressive disease, the recurrence occurred at the margin of the treated tumor in all cases. No major complications were observed, and none of the complications was more severe than Clavien-Dindo grade I. Percutaneous cryoablation is a safe, effective, and repeatable treatment for achieving local control of EAD tumors.

Extra-Abdominal Desmoid Tumor in the Donor Site of an Extended Latissimus Dorsi Flap.

Extra-Abdominal Desmoid Tumor in the Donor Site of an Extended Latissimus Dorsi Flap.

Unusual Ultrasonography Findings of Recurred Mammary Fibromatosis Mimicking Subareolar Mastitis: A Case Report

본원 내원 당시 이학적 검사에서 유륜 주위에 수술 상처가 있 Fibromatosis, also known as an extra-abdominal desmoid tumor, rarely occurs in the breast and is often mistaken for carcinoma, clinically and radiologically. Desmoid tumor is a monoclonal myofibroblastic neoplasm which is locally aggressive, but rarely metastasizes. We herein report a case of a 64-year-old woman who experienced two episodes of recurrence of mammary fibromatosis. The mass was initially detected by screening mammography. It appeared as an irregularly shaped mass which was confined within the mammary zone. Recurrences were excised from the right breast 10 and 17 months later. The second recurrence occurred in the subareolar area accompanied by skin thickening and showed an anechoic component on ultrasonography, which mimicked subareolar mastitis with an abscess.