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Post - Paget’s Sarcoma

Summary

Paget's sarcoma is a secondary sarcoma that occurs in patients with long-standing Paget's disease. The incidence is declining due to improved treatment of Paget's disease.

Most patients are older than 60.
This tumor occurs in patients with known Paget's disease who have experienced a fracture or a new painful swelling in an involved bone.
On x-ray, an aggressive destructive lesion in a bone that is already involved with Paget's disease is seen. A pathological fracture may be the first sign of this process.
The prognosis is generally poor. This is a very aggressive sarcoma it is difficult to treat. Wide surgical removal and chemotherapy are used as much as possible.
Complete Information on this Tumor
Introduction and Definition: 

Paget's disease of bone is characterized by an excessive and abnormal remodeling of bone. Paget's sarcoma is a rare form of secondary osteosarcoma (or secondary sarcoma) that occurs in a small number of patients with long-standing Paget's disease. Recent data show that the incidence has been declining, possibly due to improved treatment strategies for Paget's disease. It is now expected that less than 1% of patients with Paget's disease will develop Paget's sarcoma.

As treatment of Paget's disease evolves, the presentation of Paget's sarcoma has changed, patient presenting at an older age, with monostotic Paget's disease, and lower serum alkaline phosphatase levels. Despite these changes, prognosis remains poor.

Incidence and Demographics: 
Most patients are 55-80 years old. The most common sites of malignant transformation are the femur, pelvis, humerus and craniofacial bones in that order. Paget's sarcoma has a very poor prognosis, with 8-10% survival at 5 years and a mean survival of one year.
Symptoms and Presentation: 

Paget's sarcoma usually presents as a new, progressive pain in a patient with long standing Paget's disease. Other symptoms can include soft tissue swelling or pathological fractures. The serum alkaline phosphatase which is elevated with Paget's disease may rise further with onset of sarcoma.
Patients often complain of pain before the tumor can be easily seen on a plain radiography and the diagnosis can often be delayed. Eventually, cortical destruction and an ill defined tumor mass extending into the soft tissue with a background of Paget's disease become apparent. If one Paget's
sarcoma is found, the entire body should be surveyed as the tumor often arises in more than one site.

X-Ray Appearance and Advanced Imaging Findings: 
The x-ray appearance shows characteristic findings of Paget's disease, with a new area of aggressive lytic destruction of the bone, which may also demonstrate a soft tissue mass. There is cortical destruction, an ill-defined, permeative lesion that extends into the soft tissue and destroys the surrounding pagetoid bone. Pathological fracture through pagetoid bone may signal the onset of Paget's sarcoma.
Differential Diagnosis: 
pathological fracture, metastatic adenocarcinoma
Histopathology findings: 
The exact pathology of lesions arising from Paget's disease need to be confirmed by biopsy. The tumor cells of Paget's sarcoma are derived from osteoblasts, have large, pleomorphic nuclei, are poorly differentiated and produce osteoid. The tumor can have different appearances depending on the matrix produced: bone (osteoblastic), fibrous (fibroblastic), cartilage (chondroblastic) or blood vessels (telangiectatic). Periosteal and juxtacortical sarcomas do not arise from Paget's disease.
Treatment Options for this Tumor: 
Treatment for Paget's sarcoma depends on the tumor stage and the general status of the patient, and can vary from wide resection and chemotherapy to palliative radiation for pain control.
Preferred Margin for this Tumor: 
Wide
Outcomes of Treatment and Prognosis: 
Paget's sarcoma has a very poor prognosis, with 8-10% survival at 5 years and a mean survival of one year'. Factors contributing to mortality include the aggressive and high grade anaplastic tumors, the poor health of the age group in which it occurs, surgically inaccessible tumor sites, and the increased vascularity of the bone that occurs with Paget's disease which predisposes to hematological metastatses (especially to the lungs). Other tumors that occur in Paget's disease include malignant fibrous hystiocytomas andchondrosarcomas.
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