| multipele osteochondromen van pijpbeen (aandoening) | | multipele osteochondromen van pijpbeen | | Multiple osteochondroma | | Bessel Hagen disease
Multiple osteochondroma of long bone
| | The development of two or more cartilage capped bony outgrowths of the long bones.Develop and increase in size in the first decade of life, ceasing to grow when the growth plates close at puberty. The number may vary significantly within and between families. The majority are asymptomatic and located in bones that develop from cartilage, especially the long bones of the extremities, predominantly around the knee. The most important complication is malignant transformation towards secondary peripheral chondrosarcoma. Germline mutations in EXT1 or EXT2, are found in almost 90% of patients. An autosomal dominant disorder. |
| | Id | 716742001 | | Status | Primitive |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | D16.9 | | Term | Benigne neoplasma van bot en gewrichtskraakbeen, niet gespecificeerd |
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| SNOMED CT to Orphanet simple map | 321 |
| SNOMED CT to ICD-10 extended map | | Target | D16.9 | | Rule | TRUE | | Advice | ALWAYS D16.9 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE | | Correlation | SNOMED CT source code to target map code correlation not specified |
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