Atypical neurofibromatous neoplasm of uncertain biologic potential (ANNUBP) progression t...

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

ID	`RF-MPNST-TRANSFORMATION-PROGRESSION`
Type	Red flag
Status	reviewed 2026-04-27 \| pending_clinical_signoff
Diseases	`DIS-MPNST`
Sources	`SRC-NCCN-SARCOMA` `SRC-ONCOKB`

Red Flag Origin

Definition	Atypical neurofibromatous neoplasm of uncertain biologic potential (ANNUBP) progression to frank MPNST (CDKN2A/B biallelic loss, increased cellularity, mitotic activity), rapid local progression on AI neoadjuvant, new metastatic disease (lung, pleura, bone), or unresectable recurrence — triggers re-staging, re-biopsy, salvage AI intensification or pazopanib + clinical trial enrollment.
Clinical direction	hold
Category	transformation-progression

Trigger Logic

{
  "any_of": [
    {
      "finding": "annubp_to_mpnst_transition",
      "value": true
    },
    {
      "finding": "rapid_progression_on_chemo",
      "value": true
    },
    {
      "finding": "new_metastatic_disease",
      "value": true
    },
    {
      "finding": "unresectable_local_recurrence",
      "value": true
    }
  ],
  "type": "composite_clinical"
}

Notes

Continuum from plexiform NF → ANNUBP → low-grade MPNST → high-grade MPNST is well-described in NF1 patients; CDKN2A/B loss is the inflection point. Surveillance MRI + functional imaging (FDG-PET SUVmax >3) recommended in NF1 for high-burden plexiform NF. Rapid progression on AI: re-biopsy at progression, consider switch to pazopanib (PALETTE; limited MPNST data but FDA-approved for non-GIST STS) or trial. New lung metastases: pulmonary metastasectomy if oligometastatic + adequate lung function. Salvage AI re-induction has diminishing returns; trial enrollment (selumetinib + AI; cabozantinib in NF1-MPNST) preferred.

Used By

No reverse references found in the YAML corpus.