Vinay Raja

Soft Tissue Sarcomas CASE 1. Granulocytic Sarcoma: Presentation With Nodal and Skin Involvement

Malignancies arising in connective tissue comprise a clinically important and histologically diverse category of pediatric cancers. These have traditionally been classified by presumed cell of origin or type of differentiation, with the caveat that some diagnoses originate from undefined cell types. Newer genetic data indicates that molecular perturbations, particularly translocations and their derivative chimeric fusions, correlate with histological types and better predict clinical behavior and outcome. Many diagnostic imaging techniques can be profitably applied to these tumors for the purposes of diagnosis, staging, and disease monitoring. These range from ultrasonography to newer positron emission tomography (PET) scans. Rhabdomyosarcomas constitute a large proportion of sarcomas in children, but in older children and adolescents, non-rhabdomyosarcomatous soft tissue sarcomas (NRSTS) predominate as a group. This review will cover classification, grading, morphological and genetic diagnosis, and diagnostic imaging features of these diverse lesions, Rarely adult types of sarcomas occur in children, but they will be included in this relatively brief chapter.

Soft Tissue Sarcomas

Malignancies arising in connective tissue comprise a clinically important and histologically diverse category of pediatric cancers. These have traditionally been classified by presumed cell of origin or type of differentiation, with the caveat that some diagnoses originate from undefined cell types. Newer genetic data indicates that molecular perturbations, particularly translocations and their derivative chimeric fusions, correlate with histological types and better predict clinical behavior and outcome. Many diagnostic imaging techniques can be profitably applied to these tumors for the purposes of diagnosis, staging, and disease monitoring. These range from ultrasonography to newer positron emission tomography (PET) scans. Rhabdomyosarcomas constitute a large proportion of sarcomas in children, but in older children and adolescents, non-rhabdomyosarcomatous soft tissue sarcomas (NRSTS) predominate as a group. This review will cover classification, grading, morphological and genetic diagnosis, and diagnostic imaging features of these diverse lesions, Rarely adult types of sarcomas occur in children, but they will be included in this relatively brief chapter.

Case 1. Granulocytic sarcoma: Presentation with nodal and skin involvement

Malignancies arising in connective tissue comprise a clinically important and histologically diverse category of pediatric cancers. These have traditionally been classified by presumed cell of origin or type of differentiation, with the caveat that some diagnoses originate from undefined cell types. Newer genetic data indicates that molecular perturbations, particularly translocations and their derivative chimeric fusions, correlate with histological types and better predict clinical behavior and outcome. Many diagnostic imaging techniques can be profitably applied to these tumors for the purposes of diagnosis, staging, and disease monitoring. These range from ultrasonography to newer positron emission tomography (PET) scans. Rhabdomyosarcomas constitute a large proportion of sarcomas in children, but in older children and adolescents, non-rhabdomyosarcomatous soft tissue sarcomas (NRSTS) predominate as a group. This review will cover classification, grading, morphological and genetic diagnosis, and diagnostic imaging features of these diverse lesions, Rarely adult types of sarcomas occur in children, but they will be included in this relatively brief chapter.