What is the most common type of dwarfism, and what are its manifestations? The most common type of dwarfism is achondroplasia. This is a rhizomelic (shortening of the proximal bones) autosomal dominant disorder. Typical characteristics include a large head with frontal bossing, a trident configuration of the hands, genu varum (bowed legs), and an exaggerated …
Bony changes of sickle cell anemia Sickle cell anemia is secondary to a disorder of “sickling” of red blood cells resulting from abnormal hemoglobin molecules. Clumped sickled cells form venous (and sometimes arterial) thromboses, affecting multiple organs. Osseous manifestations include patchy sclerotic changes in bone from infarctions. A more specific sign includes a “Lincoln log” …
Causes of palpable soft tissue mass in Children Most soft tissue masses in children are benign and include desmoid tumors, infantile myofibromatosis, peripheral nerve sheath tumors, hemangiomas, lipomatous lesions (lipoma, which is more common in adults, and lipoblastoma, more common in children). Malignant tumors include rhabdomyosarcoma, primitive peripheral neuroectodermal tumor (PNET), extraosseous Ewing sarcoma, and …
How should a suspected osteoid osteoma be evaluated? Osteoid osteoma is a benign neoplasm with a nidus of osteoid-rich tissue that typically causes an intense sclerotic reaction in surrounding bone. An osteoid osteoma may occur in the cortical, cancellous, or periosteal regions of any bone (or rarely in adjacent soft tissues). Most patients are between …
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Benign pediatric primary bone tumors Benign pediatric primary bone tumors can be categorized into groups as follows: Cartilaginous: Osteochondroma, enchondroma, chondroblastoma Cysts: Unicameral/simple bone cyst, aneurysmal bone cyst Fibrous: Fibrous cortical defect/nonossifying fibroma, fibrous dysplasia Osseous: Osteoid osteoma, osteoblastoma Other: Langerhans cell histiocytosis/eosinophilic granuloma
What are the most common pediatric primary tumors that metastasize to the osseous structures? Neuroblastoma, lymphoma, clear cell sarcoma/Wilms tumor, rhabdomyosarcoma, retinoblastoma, Ewing sarcoma (more commonly metastasizing to lung), Osteosarcoma (more commonly metastasizing to lung).
When and where do pediatric primary tumors of bone occur? Ewing sarcoma and osteosarcoma are the most common primary pediatric bone tumors and typically occur between ages 10 to 25 years. The most common sites are the pelvis, thigh, lower leg, upper arm, and ribs, but soft tissue may also be the primary site of …
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