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.     

韧带样型纤维瘤病的CT表现与病理对照

目的：探讨韧带样型纤维瘤病的CT特征。方法：回顾性分析15例经手术病理证实的韧带样型纤维瘤病CT表现,并与病理结果对照。结果：15例韧带样型纤维瘤病表现为腹壁或腹内软组织肿块,CT平扫病灶呈稍低密度或等密度,动态增强扫描动脉期病灶大部分呈不均匀轻度强化,静脉期病灶持续强化,延迟期病灶强化明显,周边部分残留有斑片状、条状无强化低密度区。结论：韧带样型纤维瘤病的CT表现有一定特征,CT对该病有较高的诊断价值。     

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.     

Metachronous multicentric aggressive fibromatosis with mediastinal involvement

We describe the CT appearance of a metachronous multicentric aggressive fibromatosis that presented with an extensive middle mediastinal mass associated with esophageal involvement. Correspondence to: T. Franquet     

Desmoid-type fibromatosis: Tumour response assessment using magnetic resonance imaging signal and size criteria

IntroductionThis study aimed to establish a specified magnetic resonance imaging (MRI) signal and size criterion for assessing the response of desmoid-type fibromatosis (DF).MethodsThis retrospective study included 129 patients with DF who received non-surgical therapy. All patients underwent pretreatment and 6-month-interval follow-up MRI for >3 years (6 follow-up visits). The correlation between signal grade and size was determined. Signal grade and size among three response groups (partial response [PR], stable disease [SD], progression disease [PD]) were compared. The specified signal and size criterion was established, used to assess tumour response at each follow-up, and compared with the reference. The Response Evaluation Criteria in Solid Tumours (RECIST)1.1 criterion at the end of the 3rd year was considered the reference.ResultsMRI signals were moderately correlated with size changes (r = −0.56 and −0.41 for T2 grade and contrast-enhanced T1 grade, respectively). Changes in T2 grade and size in the three response groups were significantly different (all p < 0.01). The signal and size criterion accurately predicted 95% of PR patients at 2nd follow-up and 81.2% of PD patients at the 3rd follow-up, while only 13.1% of PR and 56.3% of PD patients were predicted by RECIST1.1. However, the accuracy of the signal & size criterion for predicting SD was lower than that of RECIST1.1.ConclusionsMRI signal is useful in assessing the response of DF. Signal & size criterion can identify patients with PR and PD earlier than RECIST1.1.     

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.     

99mTc(V)-DMSA and67Ga-citrate images in a patient with recurrent extraabdominal fibromatosis

⁶⁷Ga-citrate and^99mTc(V)-DMSA images were obtained in a patient with recurrent extraabdominal fibromatosis of the left calf. The⁶⁷Ga-citrate image showed abnormal uptake in the left calf lesion and right calf, which was considered due to probable myositis caused by overexertion. On the other hand, the^99mTc(V)-DMSA image showed abnormal uptake only in the left calf lesion. Surgical and radiation therapy was performed. Two years after the treatment, two lesions of the recurrent extraabdominal fibromatosis followed, and a^99mTc(V)-DMSA image could demarcate the two lesions.^99mTc(V)-DMSA images were considered to be useful in following up of recurrent extraabdominal 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.     

Percutaneous Microwave Ablation of Desmoid Fibromatosis

ObjectiveTo demonstrate the feasibility of percutaneous microwave ablation in desmoid fibromatosis with respect to tumor volume control and improvement in the quality of life.Materials and MethodsTwelve microwave ablations were performed in 9 patients with a histological diagnosis of desmoid fibromatosis between January 2010 and January 2019. The study population included 6 female and 3 male, with an age range of 21–76 years (mean = 46.6 years; standard deviation [SD] = 19.3 years). The mean major axis of the tumors was 10.9 cm (SD = 5.2 cm) and mean lesion volume was 212.7 cm³ (SD = 213 cm³). Their anatomical distribution was as follows: 3 lesions in the thigh, 2 in the gluteus, 2 in the leg and 2 in the periscapular region. We evaluated the reduction in tumor volume and improvement in the quality of life based on the Eastern Cooperative Oncology Group (ECOG) scale.ResultsAn average tumor volume reduction of 70.4% (SD = 24.9) was achieved, while the quality of life (ECOG scale) improved in 88.9% of patients.ConclusionPercutaneous microwave ablation may potentially be a safe, effective, and promising technique for controlling tumor volume and improving the quality of life in patients with desmoid fibromatosis.     

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。     

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.     

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.     

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.     

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.     

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.     

韧带样纤维瘤病MRI特征表现及其病理基础

目的：探讨韧带样纤维瘤病（DF）的MRI特点,重点分析MRI特征影像及其病理基础,评价MRI在DF诊断中的价值。方法回顾性分析经手术病理证实14例韧带样纤维瘤病的MRI表现（14个病灶均行M RI增强及延迟扫描）,与术后病理结果进行比较,并探讨M RI特征影像的病理基础。结果14例均为单发病灶（腹外型7例、腹壁型4例、腹内型3例）,其中有2例为术后复发,肿瘤沿着肌肉长轴生长。T1 WI呈等或稍低于肌肉的信号,T2 WI呈高于肌肉信号,增强扫描病灶均呈不均匀强化,以延迟强化为明显,病灶内可见条状或带状 T1 WI及 T2 WI低信号,增强后未见强化。14例病灶内均未见坏死、囊变及瘤周水肿。病理组织学上肿瘤由不同比例的成纤维细胞、纤维组织和胶原纤维束组成,纤维细胞及成纤维细胞密集区域T2 WI信号增高,而肿瘤内胶原纤维区域在 T1 WI、T2 WI均为低信号。肿瘤内见较多血管壁完整的毛细血管,这是病灶延迟强化的病理基础。结论病灶内可见T1 WI、T2 WI均呈带状低信号且增强扫描不强化以及病灶延迟强化是DF的特征性MRI表现。MRI可以很好评估肿瘤侵犯范围及其与周围结构的关系,有助于DF的诊断和鉴别诊断。     

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.     

Positron emission tomography in patients with aggressive fibromatosis/desmoid tumours undergoing therapy with imatinib

Purpose  

We used ¹⁸F-FDG PET to evaluate the FDG uptake in patients with aggressive fibromatosis (AF, also known as desmoid tumours) undergoing therapy with imatinib (imatinib mesylate, Glivec).