Aggressive fibromatosis

Four patients with aggressive fibromatosis are presented as illustrative examples of this rare non-metastasizing fibroblastic soft tissue tumor. The skeleton was involved in all four of these cases. The radiographic and histological appearances are reviewed and a summary of the literature is presented.     

Desmoid tumor of bone with enchondromatous nodules,mistaken for chondrosarcoma

Desmoid tumor of bone, also termed desmoplastic fibroma or aggressive fibromatosis, is a rare, locally aggressive fibroblastic tumor. We present a 16-year-old male with a huge desmoid tumor involving the iliac wing. It was associated with enchondromatous nodules mimicking malignancy. The tumor in this patient was mistaken for chondrosarcoma and hemipelvectomy was performed. To our knowledge, such a case has not previously been documented fully in the English literature. The radiographic and pathologic findings and a possible mechanism of enchondromatous nodule formation in fibrous bone tumors are discussed.     

Mesenteric desmoid tumor: De novo occurrence or recurrence following appendectomy?

Desmoid type fibromatosis (DF) is a rare, locally aggressive but benign proliferation of fibrous tissue which produces a fibroblastic mass that can cause a wide range of symptoms secondary to mass effect. When resected, these masses most commonly recur in the first 2 years. We present a case of a 33-year-old male with a history of an appendectomy 2 years prior, though his pathology report did not identify inflammation in the appendix, who presented with gradual onset of abdominal pain, and radiographs that demonstrated a large mass in the right lower abdomen. Given his symptoms the mass was resected and pathologic evaluation revealed a desmoid tumor. This case presents a unique possibility of a recurrent desmoid tumor in which the patient''s surgical history and radiographic findings can contribute to the overall management strategy of the patient given the evolving options for treatment of desmoid fibromatosis.     

Breast fibromatosis associated with breast implants

Fibromatosis refers to an extra-abdominal desmoid tumor or aggressive fibromatosis. Breast fibromatosis can develop in association with the capsule around a breast implant, although reports of cases of fibromatosis associated with breast implants are rare. As the demand for breast augmentation has increased, it is important to understand the diseases associated with breast implants. In the present report, we describe a case of breast fibromatosis that developed adjacent to a breast implant and demonstrated a relatively well-defined border even though it invaded the surrounding structures. We also explore the specific imaging features for diagnosing breast fibromatosis in association with implants by reviewing previous literature.     

Synchronous multicentric desmoid tumors (aggressive fibromatosis) of the extremities

Synchronous multicentric aggressive fibromatosis does not appear to have been previously reported. Two such cases are described. The tumors were identified by magnetic resonance (MR) imaging. The incidence of synchronous multicentric aggressive fibromatosis is not known. It is anticipated that increased use of coronal MR imaging will reveal more tumors of this type, both synchronous and metachronous. In a patient with known or suspected aggressive fibromatosis, every other soft tissue nodule or mass in the same limb has to be regarded as an additional tumor of the same histology.     

Infantile desmoid-type fibromatosis with involvement of temporal bone: case report

Infantile desmoid-type fibromatosis is a rare, benign, infiltratively growing tumor of the soft tissue. The histological classification is often confusing due to various patterns. Although surgical excision is the treatment of choice, local recurrence is high. Ultrasound, CT, and MRI cannot differentiate the different types of fibrous lesions, but are very essential, showing the localization and infiltrated tissues. We report an unusual case of desmoid-type fibromatosis destroying the temporal bone in a now-5-year-old boy. The literature contains only few reports of radiolological findings in patients with infantile fibromatosis. Correspondence to: T. Hagen     

Sonography of plantar fibromatosis

OBJECTIVE: Plantar fibromatosis is a rare benign fibroproliferative disorder of the plantar fascia that can be evaluated on sonography. Our study details the sonographic appearances of plantar fibromatosis. MATERIALS AND METHODS: We conducted a retrospective review of the clinical presentation, sonographic appearances, and clinical progress in 14 patients (range, 35-85 years; mean age, 53.1 years;) with plantar fibromatosis. Sonography was performed using either a 13-5-MHz multidimensional or 12.5-MHz linear array transducer. The location, sonographic appearances, and size of the plantar fibromatosis nodules were noted and correlated with symptom duration and clinical outcome. RESULTS: A total of 25 fibromatosis nodules in 19 feet were examined. On sonography, plantar fibromatosis was seen as a discrete fusiform nodular thickening of the plantar fascia, separate from the calcaneal insertion. Approximately one third (36%) of lesions were bilateral, and one quarter (26%) were multiple. All lesions were located either medially (60%) or centrally (40%) in the fascia. Most were hypoechoic (76%), were well defined (64%), and showed no acoustic enhancement (80%) or intrinsic vascularity (92%). No correlation was found between the echogenicity and size of plantar fibromatosis nodules or duration of symptoms (p < 0.01). One quarter of the affected feet had coexistent thickening of the plantar fascia at the calcaneal insertion with no related symptoms. CONCLUSION: Although the sonographic appearances of plantar fibromatosis vary, the appearances are characteristic enough to allow a specific diagnosis to be made. No clear relationship was found among the sonographic appearances, duration of symptoms, or clinical outcome.     

Imaging of musculoskeletal fibromatosis.

The musculoskeletal fibromatoses comprise a wide range of lesions with a common histopathologic appearance. They can be divided into two major groups: superficial and deep. The superficial fibromatoses are typically small, slow-growing lesions and include palmar fibromatosis, plantar fibromatosis, juvenile aponeurotic fibroma, and infantile digital fibroma. The deep fibromatoses are commonly large, may grow rapidly, and are more aggressive. They include infantile myofibromatosis, fibromatosis colli, extraabdominal desmoid tumor, and aggressive infantile fibromatosis. Radiographs typically reveal a nonspecific soft-tissue mass, and calcification is common only in juvenile aponeurotic fibroma. Advanced imaging (ultrasonography, computed tomography, and magnetic resonance [MR] imaging) demonstrates lesion extent. Involvement of adjacent structures is common, reflecting the infiltrative growth pattern often seen in these lesions. MR imaging may show characteristic features of prominent low to intermediate signal intensity and bands of low signal intensity representing highly collagenized tissue. However, fibromatoses with less collagen and more cellularity may have nonspecific high signal intensity on T2-weighted images. Local recurrence is frequent after surgical resection due to the aggressive lesion growth. It is important for radiologists to recognize the imaging characteristics of musculoskeletal fibromatoses to help guide the often difficult and protracted therapy and management of these lesions.     

侵袭性纤维瘤病的CT和MRI表现

目的:探讨CT和MRI在侵袭性纤维瘤病中的诊断价值.方法:回顾性分析20例经手术或病理证实的侵袭性纤维瘤病的CT和MRI表现.结果:瘤体发生于四肢8例,躯干12例,直径2.5～17.0 cm,CT平扫与肌肉对比呈等密度者14例,稍高密度者6例,多数呈渐进性强化,强化不均.瘤体在MRI T1WI上呈等低信号,在T2WI上...     

侵袭性纤维瘤病的CT诊断

目的：对侵袭性纤维瘤病的腹壁外组及腹壁组作比较，重点讨论腹壁外侵袭性纤维瘤病的ＣＴ表现。材料和方法：对４０例４３个经手术及病理证实的侵袭性纤维瘤病术前行ＣＴ检查，肿瘤位于腹壁外２９个，腹壁１４个。结果：腹壁外肿瘤比较特征性的ＣＴ征象为肿块较大，肿瘤呈爪样浸润正常肌肉组织，平扫病灶密度均匀。增强后强化，表现为密度大部分均匀呈等或高密度，偏中心数个低密度改变，或密度不均匀，小梁状、条索状改变；或肿瘤均匀等密度。腹壁肿瘤则较小，平扫与增强密度都均匀。ＣＴ还能帮助评价肿瘤的侵袭范围以及与周围结构的关系。结论：提高对腹壁及腹壁外侵袭性纤维瘤病的ＣＴ表现的认识，有助于术前定性诊断。     

Aggressive infantile fibromatosis. Pulmonary metastases documented by plain film and computed tomography

Aggressive infantile fibromatosis, one of the juvenile fibromatoses, is generally considered a benign lesion, but it does tend to recur locally because of its invasive nature. Distant metastases are considered rare. This case report documents pulmonary metastases arising 2 years after resection of the primary lesion from the thigh of an infant. This is the seventh reported case of distant metastasis from aggressive infantile fibromatosis. Computed tomography of the original lesion as well as the metastases are presented.     

Sclerotome distribution of melorheostosis and multicentric fibromatosis

A case of melorheostosis affecting the C7 sclerotome is described, in association with synchronous multicentric fibromatosis. The foci of fibromatosis also affected the C7 sclerotome, suggesting a similar underlying pathogenesis for both diseases.     

Fibromatosis of the breast in a male patient

Fibromatosis of the breast is a rare soft tissue lesion that arises from the mammary tissue or the pectoral fascia. We present a case of fibromatosis in a 39-year-old male patient who developed a right lateral breast mass in several weeks without prior trauma or surgery. Ultrasound-guided core needle biopsy findings included differential diagnoses of nodular fasciitis and fibromatosis. The patient was referred to a breast surgeon and underwent excisional biopsy. Final pathology report confirmed fibromatosis. The patient tolerated the surgery well and will continue to follow up post-operatively for recurrence.     

Plantar fibromatosis: ultrasonography results]

INTRODUCTION: Plantar fibromatosis, or Ledderhose's disease, is a fibrous proliferation arising within the plantar fascia end exhibiting typical clinical nodular features. The lesion appears as a firm single thickening or a nodule, occasionally painful, localized to the medial portion of the sole of the foot. This unusual condition has not been extensively studied with US and very little has been written about it. MATERIAL AND METHODS: In 1998-99, six patients with plantar fibromatosis were submitted to US with 13 MHz linear array and 20 MHz mechanical annular array probes. All patients were examined in prone recumbency with the probe positioned on the sole of the foot. Only some of them were subsequently submitted to surgery. RESULTS: Plantar fibromatosis exhibited an almost pathognomonic pattern and US proved to be a quick, noninvasive and cost-effective technique to confirm clinical diagnosis. The nodule is typically single and isoechoic, with maximum diameter of about 1 cm, inhomogeneous internal structure and few thin hyperechoic septa. The nodular fibrous proliferation adheres with the major axis along the plantar fascia; it exhibits clear-cut margins and US beam transmission is good. No calcifications or fluid collections are seen within the nodule. Color and power Doppler show no flow inside. CONCLUSIONS: US is an adequate tool for the study of plantar fibromatosis.     

Desmoid-type fibromatosis in the head and neck: CT and MR imaging characteristics

Introduction

In the head and neck region, desmoid-type fibromatosis is an uncommon tumor, and the imaging features have not been well described. The purpose of this study was to describe imaging features with their pathologic correlation of desmoid-type fibromatosis in this region.

Methods

Computed tomographic (CT) and magnetic resonance (MR) images of nine consecutive patients (five women and four men; age range, 2–72 years; mean age, 28 years) with desmoid-type fibromatosis in the head and neck were retrospectively evaluated, focusing on lesion location, size, shape, presence of a rim of surrounding fat, CT attenuation, signal intensity, and enhancement characteristics on MR with pathologic correlation.

Results

Desmoid-type fibromatosis involved perivertebral space (n?=?5) and carotid space (n?=?1) in six adult patients. In three pediatric patients, the fibromatosis primarily involved submandibular space (n?=?2) and masticator space (n?=?1) with frequent invasion to the adjacent spaces (3/3). A mean greatest dimension of 5.8 cm, elongated shape (7/9), and rim of surrounding fat (8/9) were the common features of the desmoid-type fibromatosis. Tumors often showed iso (3/7) or high attenuation (3/7) on postcontrast CT, high signal intensity (6/9) on T2-weighted image, iso signal intensity (8/9) on T1-weighted image, and strong MR enhancement (8/9). Characteristic nonenhancing low signal intensity bands (8/9) on all MR sequences were well correlated with dense collagenous stroma.

Conclusions

Desmoid-type fibromatosis in the head and neck of adults frequently involves perivertebral space. Along with various common imaging features, desmoid-type fibromatosis shows characteristic nonenhancing low signal intensity bands on MR images.     

Desmoid-type fibromatosis of the lower extremity: A unique case of complete lesion resolution following core needle biopsy

Desmoid-type fibromatosis (DF) is a rare neoplasm characterized by fibroblastic and myofibroblastic proliferation. While characterized as a benign lesion that does not metastasize, desmoid-type fibromatosis exhibits a wide range of behavior from aggressive local tissue invasion and post-surgical recurrence to spontaneous regression. Tumor regression can occur following systemic medical therapy or rarely may occur in the absence of therapy. We present a case of a 50-year-old female with a left thigh vastus medialis intramuscular mass which underwent imaging work-up and subsequent core needle ultrasound-guided biopsy showing results of desmoid-type fibromatosis. Following biopsy, the tumor showed prompt, complete regression with complete MRI resolution 2 months following biopsy. The patient showed no evidence of disease recurrence out to one year on MRI surveillance. This case report will discuss desmoid-type fibromatosis imaging features, treatment strategies, spectrum of disease behavior, and atypical behavior such as the spontaneous tumor regression as seen in this case report. To our knowledge there have been no reported cases of DF spontaneous regression 2 months following a core needle biopsy. Understanding the variable behavior of desmoid-type fibromatosis can assist the radiologist in guiding management of these lesions with the goal of optimizing clinical outcomes and preventing unnecessary aggressive treatments for stable or regressing disease.     

Uptake characteristics of fluorodeoxyglucose (FDG) in deep fibromatosis and abdominal desmoids: potential clinical role of FDG-PET in the management

In this preliminary report, we explore the uptake pattern of fluorodeoxyglucose (FDG) in fibromatosis and hypothesize the potential clinical role of FDG-positron emission tomography (PET) in the management of this benign but locally aggressive heterogeneous group of soft-tissue tumours. Five patients were studied (two men and three women, age range 23-35 years), among whom were three cases of deep musculoskeletal fibromatosis, one of abdominal fibromatosis (abdominal desmoid) associated with familial adenomatous polyposis (Gardner's syndrome) and one case of both deep musculoskeletal fibromatosis and abdominal desmoid. The FDG uptake in the lesions was heterogeneous in four cases and relatively homogeneous in one case. The uptake ranged from low to moderate grade with areas or foci of relatively avid FDG uptake. The maximum standardized uptake value (SUV(max)) observed was up to 4.7; the avidity probably related to the biological aggressiveness and tendency for recurrence, characteristic of fibromatosis. A dual-point FDG-PET carried out over four active foci in two cases registered an increase in SUV ranging from 6.93% to 25.85% (mean 19.28%). Treatment monitoring with chemotherapy was carried out in two cases: the reduction in FDG uptake was consistent with the histological evidence of fibrosis and reduction in mitosis. Hence, a baseline FDG-PET can serve a valuable role in monitoring the effect of systemic pharmacotherapy in patients with recurrent progressive disease after unsuccessful local-regional treatment. The findings in this report can be extrapolated and have implications for studying the utility of FDG-PET in defining aggressiveness, guiding biopsy and defining excision site in a large tumour and in monitoring therapy in fibromatosis.     

A desmoid-type fibromatosis in the retroperitoneum of the gastro-pancreatic region that was resected with a distal pancreatomy: a case report

An 80-year-old man was referred to our hospital because of epigastric pain. Abdominal computed tomography revealed a well-defined circular intra-abdominal mass in the gastro-pancreatic region measuring 15 mm in diameter. After 6 months, the mass lesion was growing with mild enhancement, and weaker enhancement was found in the lower half of the mass on contrast-enhanced computed tomography. The mass lesion touched the stomach, whereas adipose tissue appeared to intervene between the mass and pancreas. On magnetic resonance imaging, the well-defined mass lesion had isointensity to muscle on T1-weighted imaging, slight hyperintensity to muscle on T2-weighted imaging, which indicated a rich fibrous tumor. Under general anesthesia, the patient underwent open surgery. Intraoperatively, the tumor was separated from the stomach and firmly attached to the pancreas. Therefore, we performed a distal pancreatomy with splenic resection. Pathological diagnosis was desmoid-type fibromatosis in the retroperitoneum, and the tumor margin was attached to the pancreas, splenic artery, and splenic vein. Since there are few reports of desmoid-type fibromatosis occurring in the retroperitoneum of the gastropancreatic region, it is difficult to distinguish from other soft tissue tumors and to identify the tumor origin. Close observation by radiological re-valuation was a useful option. Magnetic resonance imaging signals and an enhanced pattern may help distinguish a desmoid-type fibromatosis from other soft tissue tumors. A desmoid-type fibromatosis that is well-defined in radiological findings may infiltrate the surrounding organs with gross or pathological analyses.     

Ollier's disease in association with adjacent fibromatosis

Ollier's disease (enchondromatosis) is a nonhereditary disorder of mesodermal dysplasia. It is characterized by the presence of multiple enchondromas that typically affect the metaphyseal ends of bones. The association of Ollier's disease with adjacent fibromatosis has, to our knowledge, not been previously described. We report a case of Ollier's disease in association with soft tissue fibromatosis adjacent to the involved upper arm.     

侵袭性纤维瘤病的MRI特征

目的探讨侵袭性纤维瘤病的MR特征。方法对11例经手术病理证实的侵袭性纤维瘤病MRI征象进行回顾性分析。结果 11例侵性纤维瘤病表现为腹内或腹壁软组织肿块,肿块呈圆形、类圆形或不规则形、爪形。MRI检查,T2WI病灶均呈稍高信号;T1WI呈等信号6例,呈稍低信号3例,呈稍高信号2例;脂肪抑制T2像,病灶呈明显高信号,并能清晰显示肿瘤的边界及范围。其中4例病灶在各序列均见有条带状致密胶原纤维形成的低信号影。动态增强扫描动脉期及静脉期病灶轻中度不均匀强化,延迟期病灶明显强化并趋于均匀。其中4例病灶周边及内部残留有斑片状、条状无强化稍低密度区。结论侵袭性纤维瘤病的MRI表现具有一定特征性,MRI检查对该病有较高的诊断价值。