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Tumors of Fibrous Tissue


Adamantinoma of the long bones, or extragnathic adamantinoma, is an extremely rare, slow growing, low-grade malignant tumor of epithelial origin, that occurs almost exclusively in the tibia and the fibula.
The tumor usually occurs in the second to fifth decade of life.
The patient usually has swelling that may be painful. The duration of symptoms can vary from a few weeks to years.Adamantinoma appears as an eccentric, well-circumscribed, and lytic lesion on plain x-ray.
Adamantinoma is treated by wide surgical excision

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Adamantinoma es

Adamantinoma de huesos largos es un tumor maligno sumamente raro de origen epitelial, bajo grado y crecimiento lento que se localiza casi exclusivamente en la tiba y fibula. Este tumor surge usualmente entre la segunda y quinta década de vida y el paciente suele sufrir hinchazones dolorosas. La duración de los síntomas puede variar de semanas a años. En radiografías simples, el adamantinoma aparece como una lesión lítica, excéntrica y propiamente circunscrita. El tratamiento conlleva excisión quirúrgica de tipo amplio.

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Aggressive Fibromatosis (Desmoid Tumor)

Aggressive fibromatosis (AF)(desmoid tumor) is a rare tumor characterized by a monoclonal proliferation of fibroblasts in muscles, tendons, and ligaments. It is histologically benign, but may be locally aggressive, invasive, and destructive. The tumor does not metastasize but recurs frequently following treatment. The clinical presentation, anatomic location, and biologic behavior of this tumor are variable and an individualized treatment approach is required.

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Desmoplastic Fibroma

Desmoplastic fibroma is an extremely rare benign tumor that progresses slowly with well-differentiated cells that produce collagen. The most common site is the mandible, followed by the femur and pelvis.
It occurs most often in the first 3 decades and is found equally in men and women.
Clinical findings include pain late in the clinical course and swelling. The diagnosis of desmoplastic fibroma is difficult to make radiologically. Plain xray shows an osteolytic, expansile, medullary lesion with well defined sclerotic margins.

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