A complete resection was attempted involving resection of most of the extensor muscles except the. Fibrosarcoma of the bone is a rare malignant bone tumor which may occur as a primary lesion, or secondarily after radiation treatment, dedifferentiation from other tumors 3 or pathologies such as paget disease, bone infarction, or chronic osteomyelitis. We present a 4monthold girl that initially presented with a small red spot lesion over the right wrist since two weeks of life which had progressively increased in size. B mri t1weighted image shows a large, welldefined, lobulated. However, with better definition of tumors such as fibromatosis and malignant fibrous histiocytoma which were previously lumped together with fibrosarcoma, fibrosarcoma now accounts for only 5 to 10% of all sarcomas. The mass appeared isointense on t1 imaging, hyperintense on t2 imaging. Ifs grows rapidly, while tumor grade is a strong predictor of outcome 3. Congenital infantile fibrosarcoma cif is rare and represents less than 1% of all childhood cancers. Spontaneous regression in a patient with infantile fibrosarcoma. Although imaging findings are variable and not specific for infantile fibrosarcoma it should be considered in the differential diagnosis of a large mass developing in a limb at birth or during the neonatal period when mr imaging depicts a wellcircumscribed mass, isointense t1 and hyperintense t2weighted, sometimes heterogeneous and septate. For the majority of patients, in addition to imaging of the initial tumor by. Imaging suggested a diagnosis of lymphovenous malformation fig. Fibrosarcoma most commonly affects either a lower leg or arm.
Infantile fibrosarcoma is a rare soft tissue tumor in infants and children mostly. Fibrosarcoma epidemiology this tumor was formerly considered the most common soft tissue sarcoma. He was treated with chemotherapy and extremitysparing surgery. Canale s1, vanel d, couanet d, patte c, caramella c, dromain c. After a brief discussion of the rarity of soft tissue sarcomas in children and of the limited ability of magnetic resonance imaging to provide a tissue diagnosis, this article discusses the incidence, presentation, treatment, prognosis, and imaging characteristics of the more common and unusual pediatric soft tissue sarcomas. It is an aggressive and rapidly developing tumor that can grow to large sizes. Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a softtissue tumor presenting in. Nodular fasciitis is the most common benign softtissue tumor of fibrous origin. Infantile fibrosarcoma ifs, also known as congenital fibrosarcoma, is a rare soft tissue childhood tumor, accounting for approximately 12% of all childhood malignancies. Fibrosarcoma in children danafarberboston childrens. Fibrosarcoma fibroblastic sarcoma is a malignant mesenchymal tumour derived from fibrous connective tissue and characterized by the presence of immature proliferating fibroblasts or undifferentiated anaplastic spindle cells in a storiform pattern.
Fibrosarcoma of the bone radiology reference article. Infantile fibrosarcoma ifs is a malignant tumor of infancy that is typically seen in children below age 1. It presents as a rapidly growing mass at birth or shortly after. We report an infant with congenital infantile fibrosarcoma diagnosed as. Congenital infantile fibrosarcoma in the premature infant. Congenital infantile fibrosarcoma cifs is a rare pediatric soft tissue tumor. Fibrosarcoma differential diagnoses medscape reference. Imaging of musculoskeletal fibromatosis radiographics. Infantile fibrosarcoma represents less than 1% of all childhood cancers, but it is the most common softtissue sarcoma in those under 1 year of age. Ct and mri images from 10 adult patients with pathologically proven fibrosarcomas were retrospectively analyzed with regard to tumor location, size, number, shape, margins, attenuation, signal intensity, and enhancement patterns on mr images. Infantile fibrosarcoma successfully treated with chemotherapy, with occurrence of calcifying aponeurotic fibroma and pleomorphicspindled celled lipoma at the site 12.
Infantile fibrosarcoma ifs, a lowgrade nonrhabdomyosarcoma soft tissue sarcoma nrsts, is an uncommon tumor. Cellular mesoblastic nephroma infantile renal fibrosarcoma. Infantile ntrkassociated mesenchymal tumors jessica l. Childhood fibrosarcoma danafarber cancer institute.
However, with better definition of tumors such as fibromatosis and malignant fibrous histiocytoma which were previously lumped together with fibrosarcoma, fibrosarcoma now accounts for only 5 to 1 0% of all sarcomas. Congenital fibrosarcoma is an unusual pediatric spindlecell tumor of soft tissues that usually presents in the first few years of life. Infantile fibrosarcoma accounts for approximately % of pediatric fibroblastic and myofibroblastic tumors and 12% of softtissue malignancies in infants 56. Fibrosarcoma is an aggressive subtype of soft tissue sarcoma categorized in infantile congenitaltype and adulttype. Fibrosarcoma is a rare tumor in children with limited information on imaging features of these tumors in the literature.
It is a tumor that originates in the connective fibrous tissue found at the ends of long bones and then spreads to other surrounding soft tissues. Mr images show a large, heterogeneous softtissue mass with welldefined or infiltrative margins 5758. Although these tumors display histological features of malignancy and frequently recur, they have a. This type of tumor is the most common soft tissue sarcoma found in children under one year of age. Infantile fibrosarcoma usually presents as a rapidly.
Very young children, usually less a year old, are typically diagnosed with infantile fibrosarcoma. This particular case shows minimal collagen in the interstitium. The imaging findings of these tumors are often nonspecific. It is usually found in children less than 2 years of age and may be in form ofa solitary, nontender mass in the soft tissue layer of the extremities 1,2. Imaging of extraabdominal desmoid tumor and aggressive infantile fibromatosis imaging and treatment of extraabdominal desmoid tumor and of aggressive infantile fibromatosis are similar and therefore are discussed together. Consequently, in the majority of cases, histological and immunohistochemical studies are. Uptodate practical imaging evaluation of neonatal softtissue tumors. It can be mistaken as a benign vascular tumour as both share similar clinical presentation and imaging features. Infantile fibrosarcoma usually occurs in infants and young children but it may also be found before birth by ultrasound. The image shows a relatively uniform population of spindle cells with a hemangiopericytomalike vascular pattern. Congenital infantile fibrosarcoma cifs, perhaps the most well studied of these lesions is characterized by a recurrent etv6ntrk3 gene fusion.
Overall, infantile fibrosarcoma ifs has a good prognosis. Childhood soft tissue sarcoma is a disease in which malignant cancer cells form in soft tissues of the body. Fibrosarcoma in children danafarberboston childrens cancer. Uptodate practical imaging evaluation of neonatal softtissue. Infantile fibrosarcoma if, also known as congenital myofibroblastic sarcoma, is a rare lowgrade malignancy seen in children.
The biopsy demonstrated a congenital infantile fibrosarcoma with compact spindle cells with an interfascicular growth and cd99 positivity figure 3. Unlike adult fibrosarcoma, congenital ifs is characterized by a low rate of metastasis 8% and a high longterm survival rate 90% at 5 years. Most cases are detected in children younger than 2 years, and nearly half the cases are diagnosed at birth or antenatal imaging 2, 56. Definition of infantile fibrosarcoma nci dictionary of. Cases with abundant collagen have a greater resemblance to. This tumor was formerly considered the most common soft tissue sarcoma.
It is t2 hyperintense, demonstrates diffusion restriction and enhances. Having certain diseases and inherited disorders can increase the risk of childhood soft tissue sarcoma. Infantile fibrosarcoma ifs is an uncommon softtissue sarcoma. Poorly circumscribed, lobulated mass of small to large spindled cells in fascicles or herringbone pattern.
There are no identified risk factors for infantile fibrosarcoma, but the cause is associated with genetic aberrations. Fibrosarcoma affecting older children, adolescents and adults is sometimes called adulttype fibrosarcoma atfs. Fibrosarcoma, 144,145 a rare neoplasm, occurs as distinct clinicopathologic entities. Infantile fibrosarcoma if is a rare malignant soft tissue tumour in infancy. This form of fibrosarcoma is usually slowgrowing, and tends to be more benign than fibrosarcoma in older children, which behaves more like the. Magnetic resonance imaging mri may be the best modality overall for examining softtissue masses and for detecting the intraosseous and extraosseous extent of many bony sarcomas. Fibrosarcoma is a tumor of mesenchymal cell origin that is composed of malignant fibroblasts in a collagen background. Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a softtissue tumor presenting in infancy, located in an extremity and showing tumoral hemorrhage. They are a type of soft tissue sarcoma that can be grouped by the patient age, i. Aggressive lesions may be highly cellular at histologic analysis and may mimic an infantile fibrosarcoma, 1. Claudia elena teodorescu,md department of ultrasound, calea floreasca nr. Unusual localization and aggressive progression of large infantile.
Infantile fibrosarcoma demonstrated by f18fdg petct. Ijms free fulltext ptx3 modulates neovascularization. Postoperative magnetic resonance imaging revealed a residual mass on the heel. Ab clinical as well as radiologic diagnosis of infantile fibrosarcoma ifs is often a challenging problem due to similarities with tumors of vascular origin. Ct and mri findings of soft tissue adult fibrosarcoma in extremities. Childhood soft tissue sarcoma is a disease in which malignant cancer cells form in soft tissues of the body soft tissue sarcoma occurs in children and adults. Congenital infantile fibrosarcoma has nonspecific imaging characteristics but should be high on the differential diagnosis in a softtissue tumor presenting in infancy, located in an extremity and.
Infantile fibrosarcoma is a rare soft tissue malignant tumor, when it occurs, it is usu. Imaging has a key role in the evaluation of lesion origin, extent, and. Fibrosarcoma cells and its surrounding immune inflammatory infiltrates overexpress or induce the expression of fibroblast growth factor2 fgf2 that have a crucial role in tumor progression and angiogenesis. Infantile fibrosarcoma as the great mimicker of infantile. Ct and mri findings of soft tissue adult fibrosarcoma in. It is useful in providing information about the local extent, lesion size, and involvement of the neurovascular structures. Home soft tissue fibrous tissue fibrosarcoma congenital infantile fibrosarcoma slide 17 of 19. Magnetic resonance imaging mri findings showed 45cm.
Congenital infantile fibrosarcoma is a rare mesenchymal tumor seen in. Its occurrence in the jaw especially in childhood is very rare. In newborns and infants up to about three years old, it is called infantile fibrosarcoma or congenital infantile fibrosarcoma cfs. Infantile fibrosarcoma shares this translocation and a virtually identical histologic appearance with mesoblastic nephroma. To characterize and evaluate ct and mri features of extremity soft tissue adult fibrosarcoma. Fibrosarcoma is a tumor of fibroblasts, the cells that create fibrous tissues. We report an infant with congenital infantile fibrosarcoma diagnosed as hemangiopericytoma. A malignant tumor consisting of fibroblasts connective tissue cells that produce the collagen found in scar tissue fibrosarcoma may occur as a mass in the soft tissues or may be found in bone. Pathology outlines fibrosarcoma of soft tissue infantile.
Soft tissues of the body connect, support, and surround other body parts and organs. Magnetic resonance imaging mri revealed a solid heterogeneous mass in the. Early surgery is feasible for a very large congenital infantile. In these cases the tumor is typically found immediately after birth. Infantile fibrosarcoma usually occurs in the first year of life and in many cases is present at birth. Ct and mr imaging have respective advantages in diagnosing adult fibrosarcoma. Congenital infantile fibrosarcoma cif is a rare malignant. About of cases are congenital and most occur in preschool aged children. An infantile and an adult form are described showing identical histopathological features but differing in survival prognosis. Fibrosarcomas are malignant tumours of fibroblast origin.
Infantile fibrosarcoma misdiagnosed as vascular tumors. Congenital infantile fibrosarcoma journal of perinatology. An infantile form of fibrosarcoma which occurs in children less than one year of age has an excellent prognosis when appropriately treated. However, a notable subset of locally aggressive congenital infantile soft tissue lesions with similar morphologic features to cifs, have not todate, shown evidence of any canonical molecular aberration. Prenatal management of congenital infantile fibrosarcoma wiley. Mr imaging is the preferred imaging modality for the evaluation of infantile fibrosarcoma. Scientists and doctors distinguish two common forms of the condition. Statesseer program 19751995, national cancer institute, seer program. It is the most common soft tissue sarcoma occurring in children younger than 1 year of age. Infantile fibrosarcoma usually occurs in children younger than 1 year. It occasionally occurs in children up to age 4 years.
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