Atlas One · Host Biology · Scale 07 — System

Schwannomatosis

Schwannomatosis is caused by germline SMARCB1 (~40%) or LZTR1 (~30%) mutations; multiple peripheral schwannomas WITHOUT bilateral vestibular schwannomas; chronic pain is the hallmark; distinct from NF2; treatment: surgical resection for symptomatic tumors.

Also known as: schwannomatosis, multiple schwannomatosis, SMARCB1 schwannomatosis, LZTR1 schwannomatosis, schwannomatosis type 1, schwannomatosis type 2, sporadic schwannomatosis, schwannomatosis NF2-negative, hereditary schwannomatosis

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
schwannomatosis

Schwannomatosis

Schwannomatosis is caused by germline SMARCB1 (~40%) or LZTR1 (~30%) mutations; multiple peripheral schwannomas WITHOUT bilateral vestibular schwannomas; chronic pain is the hallmark; distinct from NF2; treatment: surgical resection for symptomatic tumors.

Entry Metadata

FieldValue
IDschwannomatosis
NameSchwannomatosis
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toLztr1 connects-toSmarcb1 connects-toNeurofibromatosis Type 2 connects-toAtypical Teratoid Rhabdoid Tumor connects-toNf2 connects-toNoonan Syndrome connects-toPeripheral Nerve connects-toMpnst connects-toNeurofibromatosis Type 1 connects-toNervous System

Sources