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148

P. Imbach

 

 

12.3.8 Prognosis

Grades 1 and 2: 10-year survival rate in 70% of patients

Grades 2 and 4: 10-year survival rate in 30–40% of patients

Age:

Below 5 years of age: 80% survival rate, metastatic spread in 4–8% of patients

In children older than 10 years, 60% survival rate, metastatic spread in 50% of patients

12.4Synovial Sarcoma

12.4.1 Incidence

>10% of soft tissue sarcoma in childhood

Mainly in adolescents and young adults

Ratio of males to females is 1.2:1

12.4.2 Location

In the extremities, 80–90%, mostly in the lower extremities

Frequency (ranked): thigh, foot, knee, forearm, lower leg, and hand; rarely head and neck, chest, spine, and skull

12.4.3 Pathology and Cytogenetics

Tumor rarely connected with the joints

Histology: two cellular types: spindle cells, structured in whirls or sheets, surrounded by epithelial cells with periodic acid-Schiff (PAS)-positive polysaccharide and glandular-like structures

Immunohistochemistry: keratin antibodies detectable

Cytogenetics: translocation t(x;19) (q11;Xp11) or t(x;18p11;q11.2)

12.4.4

Clinical Manifestations

Painless swelling in about 60%, pain-sensitive swelling in 22%, and painful

 

tumor in 18% of patients

Metastatic spread: lung, lymph node, bone, rarely brain

12.4.5

Radiological Diagnosis

MRI and/or CT: frequently calcifications are observed within the tumor

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