- NCIt
- C176944
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Data completeness0 / 7 dimensions
Computed from entity_counters.completeness · refreshed after each connector run
Definition
A rare inherited form of myofibromatosis caused by autosomal dominant mutation(s) in the NOTCH3 gene, encoding neurogenic locus notch homolog protein 3. The condition is characterized by the onset of solitary or multicentric benign tumors in the skin, striated muscles, bones, and viscera. Soft tissue lesions may regress spontaneously whereas visceral lesions are associated with high morbidity and mortality.
Data updated 22 days agoSource updated 26.08e
Taxonomy
Several hierarchies coexist; edges are listed per hierarchy type with their source.
Parents
Children (0)
Leaf node — no children.
Anatomy
Data updated 18 hours agoSource updated unknowncounters aggregate over descendants
Names
Only the preferred name is recorded so far.
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Key figures
Each figure carries its unit, period and source; ranks link to "Why this rank?".
No registry-level ancestor: burden figures are published for the mutually exclusive top-level site groups only, and this entity does not descend from one. Global figures (IARC / GLOBOCAN) stay under license review and SEER awaits credentials.
Data updated 18 hours agoSource updated unknownregistry figures: none · counters aggregate over descendants