Aggressive intraabdominal fibromatosis

BACKGROUND: Intraabdominal or mesenteric fibromatosis is a rare benign nonmetastatic neoplasm that appears as a sporadic lesion or in patients with familiar polyposis. Its evolution is unpredictable. About 10% of the cases shaw a very aggressive growth. CASE REPORT: We presented a 22-year-old woman in whom an aggressive intraabdominal fibromatosis had appeared during the first pregnancy as a well circumscribed ovoid tumor, involving the terminal ileum, the caecum, the ascending colon, the right kidney, the ureter, and the right common iliac artery. The tumor was excised with right colectomy, nephroureterectomy and resection of the involved artery using arterial reconstruction with graft interposition. Two years after the surgery the patient developed an inoperable tumor recurrency with a fatal outcome. CONCLUSION: In spite of a successful surgical excision during the original surgery intraabdominal or mesenteric fibromatosis might have an aggressive evolution leading to an inoperable tumor recurrency and a fatal outcome.     

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.     

Aggressive fibromatosis: MRI features with pathologic correlation

OBJECTIVE: We present the MRI features with pathologic correlation of aggressive fibromatosis, incorporating 203 cases over a 5-year period from the Royal Marsden Hospital Sarcoma Unit database. MATERIALS AND METHODS: Sixty patients had imaging available for retrospective review of which 29 had preoperative MRI and final histopathologic diagnosis of aggressive fibromatosis. RESULTS: The average age at diagnosis was 41.3 years with a female-to-male sex ratio of 1.2:1. Twenty lesions were extraabdominal; six, intraabdominal; and three, in the abdominal wall (classic desmoid). The average tumor size was 6.4 cm (range, 2.2-13.7 cm). Intraabdominal aggressive fibromatosis produced the largest tumors, averaging 9.5 cm. Most lesions were ovoid (52%) or infiltrative (34.5%) in outline with an irregular or lobulated contour (76%). The lesions crossed major fascial boundaries in 31% of cases overall and in 66% of patients referred for recurrent disease. On MRI, homogeneous isointensity or mild hyperintensity on T1-weighted images and heterogenous high signal on T2-weighted or STIR images were seen. All lesions enhanced after IV gadolinium, usually avidly. In contrast to previous reports, 38% of cases failed to show low signal on all pulse sequences and no abnormalities were seen in local bone structures. Histology showed sheets of bland spindle cells in dense collagen and did not vary with the MRI signal characteristics of the lesion. Patients referred for recurrent disease were most likely to have a recurrence after surgery. MRI and pathology findings did not predict recurrence. CONCLUSION: Accurate diagnosis and staging of aggressive fibromatosis by MRI have important treatment and prognostic implications.     

Aggressive fibromatosis of the tongue: MR demonstration

A case of aggressive fibromatosis of the tongue is described in a 9-year-old boy. Magnetic resonance was used in the initial evaluation and revealed a well defined mass in the anterior third of the tongue which was mildly hypointense on a T1-weighted image and minimally hyperintense on a T2-weighted image. Magnetic resonance was useful in indicating the benign nature of the tumor, in defining its extent, and for follow-up after local surgical resection.     

Aggressive fibromatosis of the neck: MR findings.

We present a case of aggressive fibromatosis of the scalene and longus colli muscles with surgically proved secondary involvement of the brachial plexus and carotid sheath in a 29-year-old woman in whom MR imaging failed to show involvement of the carotid sheath. The well-defined lesion was isointense on T1-weighted images and hyperintense on T2-weighted images relative to adjacent normal muscle and enhanced brightly.     

左颈动脉鞘侵袭性纤维瘤病1例

患者 女,55岁.因左颈前区疼痛性肿物伴声音嘶哑、饮水呛咳3个月入院.入院检查:患者左颈前区可触及一约10cm× 3cm长条形肿物,质硬,有压痛,不随吞咽活动,活动度差,左侧Horner征阳性.超声检查:左颈动脉血管鞘前后壁可见低回声,回声较均匀,下端起自颈动脉分支下约2cm处,上行至颌下角深部,内血管壁结构显示清晰,内壁粘膜光滑,血流清晰,频谱正常,内径较对侧略窄,提示左颈动脉血管鞘增厚,纤维病变不除外.     

Aggressive fibromatosis in childhood computed tomographic findings in three patients

Three children with aggressive fibromatosis are presented and the value of computed tomography in defining the extent of the lesions and for follow-up and treatment planning is discussed. Aggressive fibromatosis is a nonmetastizing fibrous lesion with a propensity for local invasion and recurrence after surgery. The lesions usually have a poorly defined margin on computed tomography and the attenuation coefficient is less than that of muscle with poor enhancement following intravenous administration of contrast material.     

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.     

Aggressive fibromatosis of the prevertebral and retropharyngeal spaces: MR and CT characteristics.

A 47-year-old man had aggressive fibromatosis, and CT and MR showed a large, multilobulated soft-tissue mass within the prevertebral and retropharyngeal spaces. On CT, the lesion was slightly higher in attenuation than adjacent muscle; on MR, it was intermediate between muscle and fat on unenhanced T1-weighted images, isointense with fat on intermediate-weighted images, hyperintense relative to fat on T2-weighted images, and markedly enhanced after administration of gadopentetate dimeglumine. Multiple small focal and linear areas of decreased signal intensity that did not enhance with gadopentetate dimeglumine were observed on all pulse sequences.     

Scintigraphy of aggressive fibromatosis

Thirteen patients with aggressive fibromatosis underwent skeletal scintigraphy and computed tomography as part of their preoperative staging. Bone involvement was visible on plain radiographs of three patients. For the other 10 patients, the presence or absence of bone involvement was shown best by computed tomography (CT) in five instances, and best by scintigraphy only once. The two studies were equally useful four times. Although skeletal scintigraphy has previously been found to be very accurate for the evaluation of bone involvement by soft tissue sarcomas, it was less useful in these patients with aggressive fibromatosis.     

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.     

Magnetic resonance appearance of fibromatosis

We reviewed retrospectively the magnetic resonance (MR) images of 14 soft-tissue lesions of fibromatosis (desmoid tumors) encountered in 11 patients. The lesions were typically inhomogeneous in texture and round to oval in configuration. Margins were well-defined in 78% of the lesions at presentation and were infiltrating in all recurrences. On T₁-weighted spin echo MR images, the predominant signal intensity was either isointense or minimally hyperintense when compared with skeletal muscle. On T₂-weighted MR images the predominant signal intensity was typically intermediate between skeletal muscle and subcutaneous fat or isointense to fat. Linear and curvilinear areas of decreased signal intensity were distributed throughout the lesions on both pulse sequences in 86% of cases. This pattern strongly suggested fibromatosis. Speculation concerning possible etiologies of this appearance are discussed, and the relevant literature on previously reported cases is reviewed.The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army, the Department of Defense, or the Uniformed Services University of the Health Sciences     

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.     

侵袭性纤维瘤病的MRI诊断

目的 分析侵袭性纤维瘤病(AF)的MRI特点,提高对该病的认识和诊断水平.方法 回顾性分析66例经手术病理证实的AF( 19例原发,47例复发)的临床资料和MRI表现,并与术后病理进行对照.结果 66例患者中位年龄31岁,男女比例1∶3.4.共检出肿瘤80个.浅表纤维瘤病5个;深部纤维瘤病75个,其中发生于腹内2个,腹壁6个,腹壁外67个.肿瘤长径平均大小为(8.7±5.4)cm,浅表肿瘤长径为(5.7±2.8)cm,深部肿瘤长径为(8.9±5.5)cm.98.8％( 79/80)表现为肌肉内占位病变;58.8％ (47/80)肿瘤呈卵圆形或分叶状,27.5％(22/80)呈不规则浸润状;60％(48/80)病灶边界清晰,其中4个可见假包膜.大部分肿瘤与肌肉相比在T1WI呈等、稍高或稍低信号,T2WI呈不均匀高信号,增强后可见明显不均匀强化.所有患者均未见囊变坏死和瘤周水肿.2个肿瘤见邻近骨质破坏.结论 侵袭性纤维瘤病在MRI上具有一定特征性,MRI对该病的诊断、肿瘤侵犯范围及其与周围结构关系的评估有重要价值.