Secondary chondrosarcoma in cartilage bone tumors: report of 32 patients

What is the Proportion of Patients With Multiple Hereditary Exostoses Who Undergo Malignant Degeneration?

“…In our respondents, malignant degeneration of lesions occurred most often in the pelvis (eight of 21) and scapula (four of 21), a distribution similar to those reported previous studies that noted location of chondrosarcoma lesions [11,13]. This may be attributable to a delayed diagnosis of lesions in these areas [1]. Nevertheless, the risk of chondrosarcoma in patients with multiple hereditary exostoses is low, albeit greater than the risk to the general population.…”

Insights into Enchondroma, Enchondromatosis and the risk of secondary Chondrosarcoma. Review of the literature with an emphasis on the clinical behaviour, radiology, malignant transformation and the follow up

“…Most subtypes are non-hereditary, while some are autosomal dominant or recessive [5]. Clinically, the bone deformities as well as malignant progression of enchondromas may require (multiple) surgical interventions [9][10][11][12][13].…”

“…In our literature review the age of patients with secondary chondrosarcoma (sCS) arising from tion bias, the risk of developing a secondary chondrosarcoma in solitary enchondroma described to be up to 4% [10]. It is estimated that primary Chondrosarcoma is approximately two times more common than CS arising from a solitary enchondroma [14].…”

“…The malignant progression of an enchondroma was in most cases evident if one of the following criteria was present (in MRI): cortical destruction, moth-eaten or permeative osteolysis, spontaneous pathologic fracture, periosteal reac- -multilocular appearance on contrast-enhanced T1-weighted images (in discussion) -Soft tissue mass -Endosteal scalloping > 2/3 of the cortex (with limitation in the metaphyseal region where the cortex is thin, especially in the proximal fibula) -Extent of endosteal scalloping superior to two-thirds of the lesion length -Cortical thickening and enlargement of the medullary cavity -Increased uptake in scintigraphy (more than that of the anterior iliac crest) -Lesion size > 5-6 cm (risk factor) Pre-existing LesionRisk of malignant transformation [10,15,24] -Enchondroma: risk of malignant transformation up to 4%, on average about 2% -Enchondromatosis / Ollier disease: risk of malignant transformation up to 46% -Maffucci syndrome: risk of malignant transformation up to 55% Genetics [8, 15, 34-37, 40, 41] -Mutation in parathyroid hormone receptor 1 (PTHR1)? -Rearrangements of chromosome 6 and chromosome 12 -PTPN11 mutations -Alterations at some level in the pRb pathway -Other highly variable genetic alterations tion, edema surrounding the tumor on MR images, and soft tissue mass [2,29,[42][43][44][45][46][47]49].…”

Biopsia endoscópica de osteocondroma de fosa infratemporal

“…El intervalo de tiempo entre diagnóstico y malignización varía ampliamente. En un trabajo de Altay y cols 13 se describe un promedio de tiempo de 9,8 años. Así, la presentación del condrosarcoma secundario a OC se produce generalmente durante la tercera década de vida 9,12 .…”