Recurrent Aggressive Fibromatosis Research Articles

Long-term diagnostic value of MRI in detecting recurrent aggressive fibromatosis at two multidisciplinary sarcoma centers

PurposeTo assess the diagnostic accuracy of MRI in detecting recurrent aggressive fibromatosis (AF) during long-term follow-up at two multidisciplinary sarcoma centers. MethodsSeventy-nine patients from two sarcoma centers were included in this IRB-approved study and were examined postoperatively using 1.5-T MRI. MRI follow-up scans were reviewed for true-positive/-negative and false-positive/-negative results. Available pathological reports and MRI follow-ups were set as reference. ResultsThe median age of the patients was 38.1 ± 15.3 years. Of the patients 27.9 % showed recurrent AF lesions. The most common localizations of AF were the axilla/shoulder (n = 15) and the thigh (n = 11). From 498 postoperative MRI follow-ups, 24 true-positive, 16 false-positive, 6 false-negative, and 452 true-negative MRI follow-ups were identified. The overall sensitivity and specificity for detecting recurrent AF was 80 % and 97 %, respectively. There was no significant difference in the diagnostic accuracy at the two sarcoma centers. All false-negative results were found in small lesions. False-positive results mostly mimicked streaky (n = 10) and small ovoid/nodular (n = 5) lesions. The configuration of recurrent AF was significantly most often fascicular (50 %; p = 0.001−0.005). ConclusionMRI shows a high long-term diagnostic value in detecting AF recurrences. Nevertheless, radiologists should pay close attention when lesions are small, as they may remain undetected. Although the configuration of recurrent AF is most often fascicular, recurrences may also appear in different shapes.

Recurrent Aggressive Fibromatosis with Laryngeal Involvement: A Case Report and Literature Review

Aggressive Fibromatosis (AF) is a rare, benign neoplasm originating from musculoaponeurotic stromal structures characterized by aggressive growth and infiltration of local tissues. To date, there are only six previously reported cases of AF involving the larynx. Our case involves a 70-year-old female with a 5-year history of hoarseness and an enlarging neck mass consistent with aggressive recurrent fibromatosis. MRI showed a large, solid mass arising from the left anterior cervical space displacing the trachea and upper airway to the right. A year after the initial radical resection, the patient presented with recurrence. A second radical excision was performed not including a laryngectomy. Radiation therapy was consulted for possible local treatment to prevent subsequent recurrence of tumor. Literature has reported up to 40-70% recurrence rates of AF within 18 months. There is minimal literature to guide treatment. Surgery seems to be the treatment of choice for head and neck AF, but management of recurrence is controversial.

Configuration of Primary and Recurrent Aggressive Fibromatosis on Contrast-Enhanced MRI with an Evaluation of Potential Risk Factors for Recurrences in MRI Follow-Up

To analyze the appearance of primary and recurrent aggressive fibromatosis (AF) on MRI with a focus on configuration and to determine potential risk factors for recurrences detected on MRI follow-up scans. From 79 consecutive patients with histologically proven diagnosis of AF, 39 patients underwent a minimum of four 1.5 T MRI follow-up scans after resection of primary AF between 2008 and 2018. The primary and recurrent tumors were radiographically examined for configuration, limitation and extent on MRI. Epidemiological data and loco-regional subcutaneous edema, muscle edema and post-operative seroma were included. The mean age of the patients was 39 ± 2.6 years. Primary and recurrent AF most often occurred in the thigh. The main appearance of primary AF was significantly most often fascicular (p = 0.001-0.01) with heterogeneous and marked contrast enhancement. 21 % (n = 8) of the patients developed recurrences of AF. A fascicular configuration with homogeneous/heterogeneous contrast enhancement was the main appearance of recurrent AF, but recurrent AF appeared nodular, polycyclic, ovoid or streaky/flat as well. Recurrent AF significantly most often occurred within the first 9 months after primary tumor resection (p = 0.009), especially in patients up to 25 years of age (RR = 6.1; 95 % CI: 1.8-20.9; p = 0.004). The cases of recurrent AF were altogether significantly smaller than the primary tumors (p = 0.001). Post-treatment subcutaneous and muscle edema were present in 77 % and 56 %, respectively. Patients with muscle edema after primary tumor resection had a significantly higher risk for AF recurrences (relative risk ratio (RR) = 1.8; 95 % CI: 1.16-2.8; p = 0.0096). There was no significant difference detected in patients with complete or incomplete resection of the primary tumor. Primary and recurrent aggressive fibromatosis has a mostly fascicular configuration, but may appear ovoid, nodular, streaky/flat or polycyclic as well. High risks for tumor recurrences are detected for patients up to 25 years of age, patients within the first 9 post-operative months and patients with muscle edema after primary tumor resection. · Primary aggressive fibromatosis mostly has a fascicular configuration with heterogeneous contrast enhancement. · Recurrent aggressive fibromatosis usually has a fascicular configuration with heterogeneous/homogeneous contrast enhancement. · Patients within the first 9 post-operative months and up to 25 years of age have a significantly higher risk for recurrences. · Muscle edema after resection of primary aggressive fibromatosis is associated with a significantly higher risk for recurrences. · Sedaghat S, Surov A, Krohn S et al. Configuration of Primary and Recurrent Aggressive Fibromatosis on Contrast-Enhanced MRI with an Evaluation of Potential Risk Factors for Recurrences in MRI Follow-Up. Fortschr Röntgenstr 2020; 192: 448 - 457.

Radiation Therapy for Aggressive Fibromatosis: The Association Between Local Control and Age

Radiation therapy (RT) is often used in the treatment of unresectable or recurrent aggressive fibromatosis (also known as desmoid tumor) typically with excellent local control. Prior reports have suggested that local control in pediatric patients with aggressive fibromatosis is poor. We aimed to report a long-term single-institution experience with the radiotherapeutic treatment of these tumors with a focus on age-dependent outcomes. A total of 101 patients treated with RT for aggressive fibromatosis between 1975 and 2015 at a single institution were identified. A variety of demographic and treatment-related variables were abstracted from patients' medical records. Kaplan-Meier analyses were performed to investigate the relationship between these variables and local control. Overall survival was excellent (98% and 95% at 5 and 10years, respectively); local control was likewise excellent (82% and 78% at 5 and 10years, respectively). Patients aged <20years at diagnosis had significantly worse 5-year local control than those aged >40years at diagnosis (72% vs 97%; hazard ratio, 9.0; P= .009). Patients treated with once-daily fractionation had significantly improved 5-year local control compared with those treated with twice-daily fractionation (90% vs 73%; hazard ratio, 0.3; P = .008). Neither the presence of gross versus microscopic residual disease, initial versus recurrent presentation, number of prior surgical procedures, nor tumor size had any effect on 5-year local control. In a total of 36.6% of patients, Common Terminology Criteria for Adverse Events grade 3 or 4 toxicity developed following treatment; the frequency of toxicities was reduced in patients treated during or after 1995 (24.5%) relative to those treated prior to 1995 (51.9%, P = .02). RT for aggressive fibromatosis offers excellent local control and should remain the standard of care for patients with unresectable or recurrent disease. Younger patients have diminished local control relative to older patients, suggesting possible biological differences contributing to radioresistance in the pediatric and young adult population.

Safety and efficacy of intralesional steroid injection for aggressive fibromatosis

BackgroundTreatment of recurrent aggressive fibromatosis (AF) following surgical resection is a clinical challenge. Non-steroidal anti-inflammatory drugs (NSAIDs) have been reported to be an effective option for controlling the disease. However, long-term NSAID use can result in unfavorable complications. This study was a trial of the use of intralesional steroid injection (ILSI) including investigation of safety margins and clinical outcomes of high-dose steroids for local use treatment of AF.MethodsA prospective cohort study was conducted to evaluate the safety and efficacy of particulate corticosteroids for AF. Intralesional steroid injections of Kanolone® guided by ultrasound were given monthly for three consecutive months with 1 mg/kg/episode (a total of 3 mg/kg). Patients were followed up monthly for 3 months at the time of each monthly injection and then for an additional 3 months after the last injection. Complications from the procedure and clinical outcomes were monitored.ResultsEight recurrent AF patients completed the full 6-month evaluation process. No procedure-related complications were reported either during the injection period or the follow-up period. None of the patients developed Cushingoid features. The highest number of complication events, all of which were mild or detectable only by laboratory analysis, occurred during the month following the second injection. Triamcinolone levels were significantly increased 24 h after injection, and four of the eight cases developed hypothalamic-pituitary-axis suppression. Tumors were stabilized in 83.3% of the cases during the study period, and pain and functional ability scores improved significantly.ConclusionsIntralesional steroid injection appears to be a safe and effective alternative treatment for recurrent AF.Trial registrationTCTR20150409001; Registered date: 9 April 2015; The safety and result of intratumoral steroid injection for aggressive fibromatosis.

Contralateral recurrence of aggressive fibromatosis in a young woman: A case report and review of the literature.

Aggressive fibromatosis (AF) is a benign non-encapsulated tumor of mesenchymal origin, with a tendency for local spread along fascial planes. Local invasion can lead to extensive morbidity and even mortality due to destruction of the bones, organs and soft tissues. This rare lesion is observed 1,000 times more frequently in patients with familial adenomatous polyposis or Gardner's syndrome due to the inheritance of the adenomatous polyposis coli (APC) gene. While AF does not metastasize, local recurrence is common. Distant recurrence is extremely rare, but is observed in those with a germ line APC mutation. The present study details the case of a 20-year-old woman with a melanoma of the right shoulder, treated definitively with surgery. The patient then developed a painful mass at the surgical site; a surgical biopsy demonstrated that the mass was AF. The patient was treated with surgical resection, radiation therapy, and a course of tamoxifen. Five years later, the patient presented with left forearm pain and diminished range of motion due to an infiltrating mass. This was excised and a clinical diagnosis of recurrent AF was made, in this patient lacking familial predisposition to the disease.

AGGRESSIVE MESENTRIC FIBROMATOSIS: A RARE CASE REPORT AND REVIEW OF LITERATURE

AbstractMesentric fibromatosis is a proliferative fibroblastic neoplasm of the small intestinal mesentery with varied clinical presentation. Giant mesentric fibromatosis is uncommon and its rarity poses a diagnostic and therapeutic challenge. This paper presents a recurrent aggressive fibromatosis in a 38 year old male patient, who had initially undergone a laparotomy outside for mass abdomen but only pus was evacuated and definitive diagnosis was not made.

Multicentric synchronous recurrent aggressive fibromatosis

Extra-abdominal desmoid tumors are known as aggressive fibromatosis (AFM). Synchronous and metachronous multicentric aggressive fibromatosis are rare lesions and pose dilemma in diagnosis and management. A rare and interesting case of recurrent multicentric, synchronous AFM is presented which to the best of our knowledge has not been reported earlier. A young male presented with well defined, hard, fixed swelling on the thigh. Resected tumor mass on histopathology was diagnosed as an extra abdominal fibromatosis. He presented again after two months with swelling at the same site; and two more swellings on the foot. Fine needle aspiration cytology (FNAC) from all three sites was performed; and was suggestive of benign spindle cell lesion of fibrogenic origin with the possibility of multicentric synchronous recurrent aggressive fibromatosis.

Imatinib for progressive and recurrent aggressive fibromatosis (desmoid tumors): an FNCLCC/French Sarcoma Group phase II trial with a long-term follow-up

BackgroundImatinib evaluated as a new treatment option in patients with recurrent or established progressive aggressive fibromatosis/desmoid tumor (AF/DT). Patients and methodsForty patients with unresectable and progressive symptomatic AF/DT were treated with imatinib (400 mg/day for 1 year) in a Simon's optimal two-stage phase II study (P0=10%, P1=30%, α=5%, β=10%). The primary end point was non-progressive at 3 months (RECIST). ResultsThe study population consisted of 28 women and 12 men, with a mean age of 41 (range 20–72 years). Most of the primary sites were extra-abdominal (24, 54.5%). Familial adenomatous polyposis was observed in six (15%) cases. The median follow-up was 34 months. Imatinib toxicity was similar to that previously reported in literature. Tumor assessment was validated by a central independent radiology committee for 35 patients At 3 months, one (3%) complete and three (9%) partial confirmed responses were observed. The non-progression rates at 3, 6 and 12 months were, respectively, 91%, 80% and 67%. The 2-year progression-free and overall survival rates were 55% and 95%, respectively. Two patients with mesenteric AF/DT died from progressive disease. ConclusionImatinib is active in the treatment of recurrent and progressive AF/DT, providing objective response and long-term stable disease in a large proportion of patients.

Updated outcome with long-term follow-up of imatinib for the treatment of progressive or recurrent aggressive fibromatosis (desmoid tumor): A FNCLCC/ French Sarcoma Group phase II trial

10518 Background: We present updated results from a previously reported phase II trial assessing clinical efficacy of imatinib in progressive or recurrent aggressive fibromatosis (Fayette et al. ASCO 2007) Methods: Patients with aggressive fibromatosis not amenable to radiotherapy or non-mutilating surgery were eligible and received imatinib 400mg daily (increased to 800mg daily in case of progression) up to 1 year then stopped. The primary end point was non-progressive disease rate at 3 months. Independent radiological committee reviewed responses. The number of patients was calculated according to a optimal 2-stages design. Results: Forty patients were included between September 2004 and October 2005 in 15 centers. The median follow up is 33 months [95% CI: 32–35]. Toxicity of imatinib is similar to that previously reported. At 3 months, the nonprogression rate was 91% [95% CI: 77–96] with 1 (2%) complete and 3 (8%) partial confirmed responses observed. The non progression rates at 6, 9, and 12 months were 80%, 69% and 66% respectively. The median time to progression was 9.5 months. The 2-year progression-free survival (PFS) was 55%. No plateau was observed. The 2-year-overall survival was 95%. Two patients with mesenteric aggressive fibromatosis died from progressive disease. In multivariate analysis including clinical factors, only previous radiotherapy was associated with reduced progression free survival. None of the biological factors tested using IHC on TMA (PDGFR α, PDGFR β, β catenin, c-kit, E cadherine, MCSFR, cycline D1 and Erk) were found correlated to response, progression free or overall survival. Conclusions: With a 2.8 years median follow up, this is the largest study which confirms the high efficacy of imatinib (400 mg daily) in patients presenting with aggressive fibromatosis failing local treatment and with documented evidence of progressive disease before imatinib treatment. [Table: see text]

Aggressive fibromatosis in the maxilla

We present a rare case of recurrent aggressive fibromatosis of the maxilla in a 61-year-old woman, who was treated by resection of the left maxilla. Adjuvant treatments, particularly radiotherapy, are valuable if the tumour recurs.

Pediatric aggressive fibromatosis

Aggressive fibromatosis (AF) is a soft tissue tumor and is rare in childhood, with high potential for local invasiveness and recurrence. General recommendations for the clinical management of pediatric patients with AF remain undetermined. The authors retrospectively analyzed 13 children with AF who were diagnosed from 1987 until 2004 in the Erasmus MC-Sophia Children's Hospital, and a review of the pediatric literature was conducted. Two patients received preoperative chemotherapy with combined vincristine, actinomycin-D, and cyclophosphamide (VAC). All 13 patients underwent surgery. Three of six patients who underwent incomplete resection received adjuvant treatment, two patients received radiotherapy, and one patient received chemotherapy (VAC). The median follow-up was 3.9 years (range, 0.6-14.0 years). Three patients developed recurrent AF, including two recurrences after patients underwent incomplete resection without adjuvant treatment. Secondary resection was performed, which was incomplete in one patient who subsequently received chemotherapy (VAC). At the time of the current report, all 13 patients were in complete remission. Ten pediatric AF studies, including the current study, with a total of 187 patients were reviewed. Incomplete resection was the most important determinant for disease recurrence; in the authors' opinion, the role of adjuvant therapy needs to be studied further. Primary surgery with negative surgical margins was found to be the most successful primary treatment modality for children with AF. Positive margins after surgery indicated a high risk for disease recurrence. Multicenter, prospective (randomized) trials will be necessary to clarify the role of adjuvant treatment for patients with pediatric AF.

Interferon-induced remission of rapidly growing aggressive fibromatosis in the temporal fossa

Aggressive fibromatosis is the name for uncommon soft-tissue neoplasms arising within musculoaponeurotic tissue. They show benign histologic features but have an aggressive local behaviour and frequently recur after surgery or radiation. A 48-year-old black woman presented with recurrent aggressive fibromatosis after primary radiotherapy in the left temporal fossa involving the base of the skull. The patient received interferon α2a subcutaneously for 6 months. A slow but steady reduction of the tumour was observed, and pre-existing symptoms disappeared.