Calcifying aponeurotic fibroma: Radiologic-pathologic analysis of ten cases and review of the literature.

Calcifying aponeurotic fibroma (CAF) is a very rare benign entity that principally affects the volar fascia, tendons, and aponeuroses of the hands and feet with a peak incidence of between 5 and 15 years, although there have been cases found for a wide age range and at various anatomical sites. We present ten CAF cases; consisting of eight children and two adults. CAF occurred in the extremities in nine of the cases and in the chest wall in one case. CAF ultrasound and radiological findings are nonspecific but may help orientate diagnosis. Magnetic resonance imaging should be performed when there are doubtful cases, when occurring in nontypical sites, and when there are cases of nontypical clinical presentation. Histologically, all cases showed two components, a fibromatosis-like component and a nodular component. Chondroid areas were present in five cases. Calcifications were observed in nine cases. ERG immunostaining showed the same patterns in all the cases; diffuse positivity in pericalcified areas, and patchy positivity in areas away from calcifications. CAF has distinctive histopathological features which should aid in the differential diagnoses with other entities.

[Soft tissue tumours with FN1 (Fibronectin 1) fusion gene]. / Tumeurs des tissus mous avec fusion du gène FN1 (Fibronectine 1).

Translocations involving FN1 gene have been described in several tumours, which share the presence of a cartilaginous matrix with or without calcifications and a good prognosis. They encompass: soft tissue chondroma, synovial chondromatosis, calcifying aponeurotic fibroma, phosphaturic mesenchymal tumour and a new spectrum of tumours: "the calcified chondroid mesenchymal neoplasms". We review all the clinical, histopathological and molecular data of these tumours and discuss the differential diagnoses.

Calcifying aponeurotic fibroma around Achilles tendon: A case report.

Calcifying aponeurotic fibroma is an uncommon benign fibrous tumor, typically develops around the fascia and tendons and commonly observed in children and adolescents. It usually occurs in the distal portion of the extremities. Presented as slow growing painless mass, treated with complete surgical excision with high recurrence tendency. We report a case of calcifying aponeurotic fibroma in a 4-year-old girl located posterior to Achilles tendon. Clinical, radiographic, and magnetic resonance imaging findings are described. To the best of our knowledge, this is the first reported case of CAF located posterior to Achilles tendon.

Calcifying aponeurotic fibroma of the thigh: A differential diagnosis for infant knee contracture and leg length inequality (a case report).

INTRODUCTION: Calcifying aponeurotic fibroma is an uncommon type of tumor that primarily occurs in the distal extremities of young children. It usually appears as a slow-growing and asymptomatic soft-tissue mass and rarely causes complications, such as joint contractures. PRESENTATION OF CASE: We present a case of calcifying aponeurotic fibroma that caused knee contracture and leg length inequality, in which the affected leg was longer than the other. On the patient's first visit to our hospital at 8 years of age, a solid nodule was palpable at the medial distal area of her right thigh. Magnetic resonance imaging showed a T1WI-low, fs-T2WI-slightly high 60-mm lesion on the vastus intermedius muscle with enhancement caused by a gadolinium agent. Tumor resection surgery, which was preceded by an incisional biopsy that resulted in calcifying aponeurotic fibroma, improved the knee's range of motion (extension-flexion improved from 0-80° to 0-120°). For the remaining leg length inequality of 35 mm, epiphyseal arrest surgery is planned. DISCUSSION AND CONCLUSION: Very infrequently, calcifying aponeurotic fibroma can cause infant knee contracture and leg length inequality. These symptoms were seemingly caused, respectively, by fascia adhesion and by increased blood flow around the metaphysis. Timely diagnosis and appropriate surgical intervention may improve functional prognosis in affected patients.

Ultrasound and Magnetic Resonance Imaging Features of Calcifying Aponeurotic Fibromas.

OBJECTIVES: To report characteristic imaging findings of calcifying aponeurotic fibromas (CAFs) on ultrasound (US) and magnetic resonance imaging (MRI). METHODS: Eight patients with CAF based on our institutional pathology database from 2000 to 2019 were consecutively included. Images were assessed as follows: with plain radiographs for the presence of soft tissue calcifications; with US for the presence of microlithiasis (a nonshadowing hyperechoic focus <3 mm) and discrete calcifications, size, location, margin, echogenicity, and relationship with surrounding structures; and with MRI for the presence of MRI microlithiasis (scattered tiny signal-void dots), margin, signal intensity, contrast enhancement pattern, and relationship with surrounding structures. RESULTS: Calcifying aponeurotic fibromas occurred most commonly in the foot (n = 5), followed by the hand, ankle, and knee, respectively. Half of the lesions (n = 4) showed intralesional calcifications on plain radiographs. On US, all lesions showed microlithiasis. They involved the subcutaneous (n = 5), perifascial (n = 2), and intermuscular (n = 1) layers. Margins were circumscribed (n = 3) or irregular (n = 5), whereas the echogenicity of the underlying tumor matrix was hyperechoic (n = 4), isoechoic (n = 2), or hypoechoic (n = 2). All lesions were abutting (n = 7) or encasing (n = 1) an adjacent tendon. On MRI, all CAFs showed tiny signal-void dots and irregular margins. Signal intensity was mostly hyperintense (n = 5) on T2-weighted images and all hypointense on T1-weighted images. Three lesions showed heterogeneous contrast enhancement, and 3 showed uneven marginal enhancement. CONCLUSIONS: A CAF should be considered a differential diagnosis if a soft tissue mass abutting or encasing the tendon or fascia shows US or MRI microlithiasis.

What is new in pericytomatous, myoid, and myofibroblastic tumors?

Recent advances in molecular techniques in soft tissue pathology, including the widespread application of next-generation sequencing, have led to significant progress in our understanding of mesenchymal tumors. Recognition of the genetic signatures of these neoplasms not only clarifies the relationship of these entities but also provides a mechanism for more accurate diagnosis. More importantly, insight into the genetic underpinnings of these lesions may offer therapeutic targets for cases not amenable to surgical treatment. This review highlights the clinicopathologic features and novel molecular findings in pericytic, myoid, and myofibroblastic tumors.

Calcifying aponeurotic fibroma of the sole of the foot in an elderly patient.

Calcifying aponeurotic fibroma (CAF) is a rare, locally aggressive fibroblastic lesion that occurs predominantly in the distal extremities of children and adolescents. In the present study, a case of pathologically proven CAF arising in the sole of the right foot of a 84-year-old man is presented. Physical examination revealed a firm, immobile, non-tender mass. Plain radiographs showed a faintly calcified soft tissue mass without bone involvement. Magnetic resonance imaging (MRI) revealed a well circumscribed subcutaneous mass. The patient underwent an excisional biopsy. Histologically, the tumor showed a biphasic pattern, composed of a moderately cellular fibromatosis-like component and irregular calcified areas with polygonal epithelioid cells. There has been no evidence of local recurrence six months following surgery. To the best of our knowledge, this case describes the oldest patient with this condition.

Intraarticular calcifying aponeurotic fibroma of the wrist: mimicking gout or calcium pyrophosphate dihydrate deposition disease.

Calcifying aponeurotic fibroma is a rare, benign fibroblastic tumor that typically occurs in the palms of the hands and soles of the feet in children and adolescents. We report an unusual case of a calcifying aponeurotic fibroma with diffuse intra-articular involvement of the carpal joints in a 59-year-old female. Radiographs and computed tomography scans revealed a large lobulated soft tissue mass with multiple stippled calcifications around the carpal joints and numerous erosions of the second to fifth carpometacarpal and intercarpal joints. Magnetic resonance imaging showed diffuse multinodular synovial proliferation with inhomogeneous hypo- to isointense signal intensity on T1-weighted images, inhomogeneous hypointense to hyperintense signal intensity on T2-weighted images, and inhomogeneous intense enhancement on fat-suppressed contrast-enhanced T1-weighted images. Radiologic diagnosis included gout, calcium pyrophosphate dihydrate deposition disease, and tenosynovial giant cell tumor. Surgical excision was performed, and the mass was diagnosed on pathologic examination as a calcifying aponeurotic fibroma. There has been no reported case of a calcifying aponeurotic fibroma with diffuse intra-articular involvement of the carpal joints in the literature.

Calcifying aponeurotic fibroma: a core biopsy-based diagnosis.

Calcifying aponeurotic fibroma (CAF) is a very rare tumor of the extremities, which can be difficult to diagnose due to its wide cyto-architectural pattern. We herein report the clinicopathologic features of a case of CAF localized on the dorsal face of the foot in a 5-year-old male child, diagnosed by needle core biopsy. Differential diagnostic problems are discussed. The present case emphasizes that the diagnosis of CAF can be confidentially rendered on core needle biopsy if the main morphological components of this tumor are concurrently present; however, before making the diagnosis of CAF, the clinical and radiological context should be considered.

Multiple calcifying aponeurotic fibroma: case report and review of the literature.

We describe the first case of diagnosis of generalized calcifying aponeurotic fibroma in 52 year-old man receiving long-term therapy for seronegative rheumatoid arthritis with rheumatoid nodules. The prevalence of lesions (presence of multiple subcutaneous nodules in the aponeuroses and fascia of the head, neck, trunk, upper and lower extremities with massive deposition of calcium salts), and a combination with monoclonal secretion (IgGκ serum, BJκ in the urine), raised inflammation markers, suggest that this case of the disease is unique, so both in domestic and foreign literature contains no description of this unusual course of this type of mesenchymal tumor. We have shown that subcutaneous nodules biopsy followed by morphological and immunohistochemical study is required in the diagnosis of the disease. We have given the literature data on the clinical manifestations, methods of diagnosis and differential diagnosis of this disease with a variety of pathologies, accompanied by the development of multiple calcification.

Calcifying aponeurotic fibroma in a girl: MRI findings and their chronological changes.

Calcifying aponeurotic fibroma (CAF) is a rare benign tumor that typically develops in the fascia and tendons. On MRI, CAF usually shows isointensity on T1WI and heterogeneous high intensity on T2WI. We report a case of 17-month-old girl with CAF on her right ankle. MRI showed an usual signal intensity for CAF. At 3 years and 4 months of age, MRI revealed hyperintense nodules on T1WI and T2WI inside of the CAF. These fatty areas corresponded to coarse calcified nodules on CT. Radiologists should be aware of the fact that fat-like signals can appear in CAF on MRI.

Calcifying Aponeurotic Fibroma of the Knee: a Case Report with Radiographic and MRI Finding

Calcifying aponeurotic fibroma (CAF) is an uncommon benign tumor and a locally aggressive fibroblastic lesion. It commonly affects the palmar side of the hand and fingers, and the soles of the feet. The typical clinical manifestations are known as a poorly circumscribed, slow-growing, and asymptomatic firm mass. Most CAFs usually reveal low to intermediate or isointensity on T1-weighted images, and strong heterogeneous enhancement. However, various signal intensities on T2-weighted images have been reported depending on the degree of hypocellularity or the amount of calcification or collagen within the tumor. This report provides the radiographic and MRI finding of CAF arising in uncommon site, the knee region, of a 19-year-old male who presented with a painful and palpable mass.

FN1-EGF gene fusions are recurrent in calcifying aponeurotic fibroma.

Calcifying aponeurotic fibroma (CAF) is a soft tissue neoplasm with a predilection for the hands and feet in children and adolescents. Its molecular basis is unknown. We used chromosome banding analysis, fluorescence in situ hybridization (FISH), mRNA sequencing (RNA-seq), RT-PCR, and immunohistochemistry to characterize a series of CAFs. An insertion ins(2;4)(q35;q25q?) was identified in the index case. Fusion of the FN1 and EGF genes, mapping to the breakpoint regions on chromosomes 2 and 4, respectively, was detected by RNA-seq and confirmed by RT-PCR in the index case and two additional cases. FISH on five additional tumours identified FN1-EGF fusions in all cases. CAFs analysed by RT-PCR showed that FN1 exon 23, 27 or 42 was fused to EGF exon 17 or 19. High-level expression of the entire FN1 gene in CAF suggests that strong FN1 promoter activity drives inappropriate expression of the biologically active portion of EGF, which was detected immunohistochemically in 8/9 cases. The FN1-EGF fusion, which has not been observed in any other neoplasm, appears to be the main driver mutation in CAF. Although further functional studies are required to understand the exact pathogenesis of CAF, the composition of the chimera suggests an autocrine/paracrine mechanism of transformation. Copyright © 2015 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Clinicopathological features of six cases of calcifying aponeurotic fibroma / 临床与实验病理学杂志

Purpose To investigate the clinicopathologic features of calcifying aponeurotic fibroma (CAF). Methods The clinical and pathologic profiles of 6 cases of CAF were retrospectively analyzed. Results CAF appeared a firm, painless and slowly growing mass with a predilection for the distal portions of the extremities in young children and adolescents. Microscopically, the tumor consis-ted mainly of benign appearing spindle cells, chondroid cells and calcific debris. Immunohistochemically, the tumor cells were positive for vimentin, HHF-35, S-100, Calponin and CD68. Conclusion CAF is a rare, locally aggressive fibroblastic lesion. It can make an accurate diagnosis by integrating the clinical location with histopathological features. Repeated minor trauma to the extremities and some embryologic cell nests maybe its pathogenesis.

Calcifying aponeurotic fibroma of the finger in an elderly patient: CT and MRI findings with pathologic correlation.

Calcifying aponeurotic fibroma (CAF) is a rare, locally aggressive fibroblastic lesion that occurs predominantly in the distal extremities of children and adolescents. In the present study, a case of pathologically proven CAF arising in the right little finger of a 69-year-old woman is presented. Physical examination revealed a firm, immobile, non-tender mass. Plain radiographs showed a faintly calcified soft tissue mass without bone involvement and computed tomography scans clearly demonstrated the presence of the lesion. Magnetic resonance imaging revealed that the lesion exhibited low to intermediate signal intensity on T1-weighted images and heterogeneous high signal intensity with small foci of low signal intensity on T2-weighted spectral presaturation with inversion recovery images. Contrast-enhanced fat-suppressed T1-weighted images demonstrated intense heterogeneous enhancement throughout the mass. The patient underwent an excisional biopsy. Histologically, the tumor showed a biphasic pattern, composed of a moderately cellular fibromatosis-like component and irregular calcified areas with polygonal epithelioid cells. There has been no evidence of local recurrence four months following surgery. To the best of our knowledge, this case report describes the oldest patient with this condition.

Calcifying aponeurotic fibroma: case report with radiographic and MR features.

Calcifying aponeurotic fibroma is a rare, benign fibroblastic tumor. The lesion has a propensity for local invasion and a high recurrent rate. Therefore, accurate preoperative diagnosis and complete excision are important to prevent the recurrence of the tumor after surgical removal. However, radiographic and magnetic resonance imaging findings of calcifying aponeurotic fibroma have been extremely rarely described in the radiology literature. Thus, we report a rare case of calcifying aponeurotic fibroma affecting the dorsal wrist in a 67-year-old man, describe radiographic and MR findings, and discuss the differential diagnosis of the tumor.

Calcifying Aponeurotic Fibroma: Case Report with Radiographic and MR Features

Calcifying aponeurotic fibroma is a rare, benign fibroblastic tumor. The lesion has a propensity for local invasion and a high recurrent rate. Therefore, accurate preoperative diagnosis and complete excision are important to prevent the recurrence of the tumor after surgical removal. However, radiographic and magnetic resonance imaging findings of calcifying aponeurotic fibroma have been extremely rarely described in the radiology literature. Thus, we report a rare case of calcifying aponeurotic fibroma affecting the dorsal wrist in a 67-year-old man, describe radiographic and MR findings, and discuss the differential diagnosis of the tumor.

Calcifying Aponeurotic Fibroma with Gait Disturbance Caused by Trauma / 대한피부과학회지

A calcifying aponeurotic fibroma is a rare benign fibrous tumor with well-characterized histopathological features. Calcifying aponeurotic fibromas are generally slowly-growing lesions related to tendons and aponeuroses in the distal extremities of children and adolescents. While the neoplasm is usually asymptomatic, cases of progressive local discomfort have been reported. The exact etiology of this neoplasm is unknown, and there is frequently no history of trauma or local irritating factors. A 10-year-old female presented with a 2.5x2.0 cm, subcutaneous mass on the right sole 4 years after she bumped against a table. The histopathologic examination showed a poorly circumscribed fibrotic mass with foci of chondroid differentiation and calcification. These findings were consistent with the diagnosis of a calcifying aponeurotic fibroma. After total excision of the mass, there has been no recurrence of the lesion in 8 months of follow-up.

Calcifying Aponeurotic Fibroma: An Atypical Case

Calcifying aponeurotic fibroma is a very rare benign soft tissue tumor with predilection for the fingers and palms of children and adolescents. We describe an unusual case of a calcifying aponeurotic fibroma in old age that circumferentially involved the dorsal and palmar side of the third metacarpal head.We performed a wide excision using two incisions, resulting in satisfactory clinical result. A calcifying aponeurotic fibroma must be considered in the differential diagnosis of a calcifying soft tissue lesion occuring in the hand of old age.

Calcifying Aponeurotic Fibroma Occurring on the Chin: A Case Report

PURPOSE: Fewer than 100 cases of calcifying aponeurotic fibroma have been reported in the literature since this entity was initially described by Keasbey in 1953 who called it calcifying juvenile aponeurotic fibroma. The tumor is a slowly growing, painless mass. In most cases the mass is poorly circumscribed and causes neither discomfort nor limitation of movement. Most lesions occur in children, with a peak incidence ages of 8-14 years. There is no evidence of any increased familial prevalence. Predilection sites are palm, finger, toe, but it also occurs in the wrist, forearm, elbow, upper arm, neck, abdominal wall, lumbar paravertebral area, leg and ankle. We herein describe a rare case of calcifying aponeurotic fibroma occurring on the chin with review of the literature. METHODS: A 14-year-old male had painless, slowly growing mass(phi2.5cm) on a chin for a year. The tumor was excised elliptically under local anesthesia and the excisional site was repaired directly. Due pathological examination was processed. RESULTS: Histological examination revealed an ill- defined fibrous growth that extends with multiple processes into the surrounding tissue with centrally located foci of calcification. The tumor is composed of short spindled plump fibroblasts with round or ovoid nuclei separated by collagenous stroma, showing vaguely palisading pattern. Diagnosis of calcifying aponeurotic fibroma was conferred. Postoperatively, the patient did well, and the lesion had not recurred. CONCLUSION: Fewer than 100 cases of calcifying aponeurotic fibroma have been reported in the literature. The most common occurring sites are palm, finger & toe, but it has been reported in the wrist, forearm, elbow, upper arm, neck, abdominal wall, lumbar paravertebral area, leg and ankle. Two cases of calcifying aponeurotic fibroma occurring on the neck have been reported in the literature. To the authors knowledge, our case of calcifying aponeurotic fibroma occurring on the chin is the first to be reported.