侵袭性纤维瘤病CT表现及鉴别诊断

目的 分析侵袭性纤维瘤病的CT表现,以提高对本病的认识及诊断水平.资料与方法 回顾性分析21例经病理证实的侵袭性纤维瘤病的CT表现,21例均行CT平扫,18例行增强扫描.结果 21例中,腹内7例(肠系膜5例,腹膜后及腹腔各1例),腹壁7例(腹直肌2例,腹内斜肌5例),腹外7例(胸壁3例,颌骨、骶骨、颈部及大腿软组织各1例).骨外侵袭性纤维瘤病19例,12例为类圆形或梭形,7例为不规则形.CT平扫示17例肿瘤密度为等或略低于肌肉密度,2例肿瘤为囊实性;增强扫描17例中,15例肿瘤为轻中度强化,2例肿瘤明显强化,4例肿瘤内见增强的血管影穿行.骨侵袭性纤维瘤病2例,CT平扫表现为轻度膨胀性骨质破坏,1例增强扫描肿瘤轻度强化.结论 侵袭性纤维瘤病可分为腹壁、腹内及腹外三型,各型CT表现具有一定特征性,根据发病部位不同需与其他肿瘤鉴别.     

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.     

Scintigraphic evaluation of aggressive fibromatosis

Despite its benign microscopic appearance, aggressive fibromatosis has potential to recur and infiltrate neighboring tissues. Therefore, it is necessary to determine the exact extent before therapy. In the present study, 11 cases of aggressive fibromatosis were examined scintigraphically using [99mTc(V)]dimercaptosuccinic acid (11 cases) and 67Ga-citrate (7 cases). Technetium-99m-(V)-dimercaptosuccinic acid demonstrated all lesions, while 67Ga-citrate detected 57% of the cases.     

Infantile fibromatosis of the mandible: a case report

We report an aggressive tumour in a 5-year-old girl causing facial disfigurement. Imaging confirmed a solid, diffusely enhancing mass at the right internal pterygoid muscle, infiltrating the adjacent bone. Surgical excision and reconstruction of the mandible were performed. Histology revealed aggressive infantile fibromatosis. No recurrence was noted 7 months later. Infantile fibromatosis may mimic malignancies and should be considered in aggressive mandibular soft tissue masses, in order to carefully plan biopsy and reconstructive surgery.     

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.     

多层螺旋CT对侵袭性纤维瘤病的诊断价值

目的:探讨侵袭性纤维瘤病的多层螺旋CT表现及诊断价值。方法:回顾性分析9例经病理证实的侵袭性纤维瘤病的多层螺旋CT表现。结果:9例病灶中7例发生于腹内,2例发生于腹外。发生于腹内者4例病灶呈类圆形、密度均匀、边缘光整;3例病灶略呈椭圆形或分叶状、混杂密度、侵犯周围组织,其中3例侵犯临近小肠,1例侵犯临近腹主动脉、右侧髂动脉及右侧腰大肌。发生于腹外的2例病灶表现为沿肌纤维浸润性生长的分叶状软组织肿块,累及多块肌肉。9例病灶中1例可见囊变,1例有点状钙化,9例均未见瘤周水肿。CT增强扫描,病灶以中等强化为主,其中2例可见小片状明显强化区。结论:侵袭性纤维瘤病的CT表现有一定的特征性,CT可以较好的显示肿瘤与周围组织结构关系,对诊断该病有重要价值。     

Aggressive fibromatosis of the mandible in childhood

Aggressive fibromatosis, or infantile fibrosarcoma, is an uncommon form of juvenile fibromatosis which rarely involves the head and neck. Skeletal involvement is infrequently demonstrated by radiography in this condition. Two unusual cases with similar radiographic changes in the mandible are presented, a situation not previously described. Clinical, pathologic, and radiographic features of aggressive fibromatosis are discussed.     

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.     

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。     

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.     

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.     

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.     

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。     

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     

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.     

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...     

Anomalous atlantoaxial portions of vertebral and posterior inferior cerebellar arteries

Summary In a review of the vertebral angiograms of 300 patients free from disease at the craniovertebral junction, we found atlantoaxial arterial anomalies in 2,3%. These were: 2 cases in which the vertebral artery ran in the spinal canal below C1, 3 cases of duplication of the vertebral artery above and below C1, and 2 cases of origin of the posterior inferior cerebellar artery at C2. Although these arteries ran in the spinal canal between C1 and C2, they never encroached upon the posterior third of the canal. From the survey of another 21 patients having bony abnormalities at the craniovertebral junction, the first type of arterial anomaly described above was seen in 4 patients and associated with failure of segmentation of the embryonic sclerotome such as occipitalization of the atlas or Klippel-Feil syndrome. It is possible to relate the development of these anomalous vessels to malarrangement of the embryonic segmental arteries. Our results indicate that one must be cautious with lateral C1/2 puncture or surgical exposure of the region.     

Thoracic cage extent of melorheostosis depicted by multislice CT

The ribs and vertebrae are rarely affected by melorheostosis. We present a case of melorheostosis of the ribs and thoracic vertebrae, in which multislice computed tomography (CT) was useful in depicting its extent and defining its sclerotome distribution.     

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.     

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.