Case Of Aggressive Fibromatosis Research Articles

Aggressive Fibromatosis

Fibromatosis develops in the soft tissue and is the proliferation of well-differentiated fibroblasts and myofibroblasts. It is present most commonly in the abdominal region. Aggressive fibromatosis (AF) of the oral or para oral structures has been reported occasionally but, it is quite uncommon in the maxilla. We report a case of AF in a 68-year- old male in the maxilla, who came with a chief complaint of swelling in his upper left back tooth region which extended to the retromolar region and was clinico-histopathologically diagnosed as AF. All proliferative indurated growths are not always clear-cut malignancies.

Aggressive fibromatosis in the infratemporal fossa presenting as trismus: a case report

BackgroundHere we report a very rare entity of an infratemporal region aggressive fibromatosis in a 23-year-old Tharu man who had presented with the symptoms of painless but progressive trismus.Case presentationWe describe a case of aggressive fibromatosis in a 23-year-old Tharu man. Radiological imaging as well as an immunohistochemistry panel from a biopsy indicated a diagnosis of an aggressive fibromatosis. Since there was no aggravation in his trismus following surgery and because of his poor socioeconomic status, he was advised to attend regular follow-up visits without any adjuvant therapy.ConclusionsThis case report adds to the notion of keeping the differential diagnosis of an aggressive fibromatosis in all patients presenting with progressive but painless trismus. The characteristic imaging findings as well an immunohistochemistry panel will help us clinch the correct diagnosis.

1 Case Clinical Report of Cranium Aggressive Fibromatosis and Literature Review

Objective: The purpose of this study is to explore the clinical characteristics and therapeutic methods of aggressive fibromatosis (AF) in skull. Methods: The clinical data and operative therapy of one case of aggressive fibromatosis in skull in our department were examined and reviewed. The associated literatures were reviewed and discussed. Results: It was indicated that the main manifestation of AF in skull was headache and skull tumor. There was prominent osteolytic destruction found in X-ray plain film for skull AF. And CT scanning showed that skull sclerotin was disorganized and inhomogeneous, with widen diploe. The skull fibromatosis constituted by fibroblasts and myofibroblas, which were mainly spindle-shaped without heteromorphism. Immunohistochemistry showed positive expression of β-catenin and Vim in these cells. The enlarged incision was adopted for the strategy of operation in this patient of skull AF. After follow-up, there was no recurrence of AF discovered. Conclusions: Skull AF is very rare in neurosurgical clinic. The clinical manifestation and iconography of AF were lack of specificity. Therefore, skull AF is hard to diagnose preoperatively. The effective diagnose is mainly dependant on histopathologic examination. As for treatment, operation is the most optimal method so far, which has a good therapeutic effect.

Response of imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors

Aggressive fibromatosis (AF) is usually a slowly growing locally invasive tumor, but may exhibit a much more aggressive phenotype. The role of chemotherapy in AF is not well defined, but can be useful in some cases. We examined the response of a case to both imatinib and sunitinib. We report a case of an aggressive multicentric extra-abdominal AF that was responsive to sunitinib, but resistant to imatinib. A 23-year-old woman developed painful multifocal AF of both legs and gluteal muscles that progressed after surgery and treatment with methotrexate/vinblastine and pegylated-liposomal doxorubicin. She received six cycles of ifosfamide/etoposide (IMV), and obtained a good response with elimination of pain. After 5 months, she developed progression and again received six cycles of IMV, with cessation of symptoms. After 13 months, tumors recurred. Although the AF was symptomatic and progressing, she was hesitant to receive chemotherapy and began treatment with sunitinib 50 mg/day for 28 days of a 42-day cycle. At 4 months, she could walk on her heels without pain. After 13 months of sunitinib, therapy was changed to imatinib 400 mg/day; after 7 days she noticed increasing pain in the AF lesions and decreased knee flexibility. Imatinib was continued, but after 2 months of imatinib, she could only walk a few steps due to pain. Sunitinib was reinstituted at 37.5 mg/day and symptoms improved within 1.5 weeks, with a marked reduction of symptoms at 1 month. She was doing well with a normal activity level, 32 months after initially beginning sunitinib. We conclude that sunitinib may be useful in some cases of AF.