Synthetic educational example. Do not self-treat: a qualified treating physician must verify the sources, patient data, contraindications, and final plan.
Patient
VERIFIED-PHEOCHROMOCYTOMA-L1-PHEO_LOCALIZED_1L_ALPHA_BLOCKA · Algorithm: ALGO-PHEO-1L
Etiological driver
Etiological driver · etiologically_driven archetype
Pheochromocytoma
- Sporadic (~60–70%)
- VHL (von Hippel-Lindau) syndrome — bilateral adrenal pheochromocytoma; norepinephrine-secreting
- MEN2A / MEN2B (germline RET — M918T highest risk) — bilateral adrenal medullary disease, often preceded by medullary thyroid carcinoma
- Neurofibromatosis type 1 (germline NF1)
- Hereditary paraganglioma–pheochromocytoma syndromes — germline SDHB, SDHC, SDHD, SDHAF2 (SDHB highest malignancy risk; SDHD parent-of-origin paternal transmission)
- TMEM127 / MAX germline mutations (rare familial forms)
- FH (HLRCC) — rare paraganglioma association
Clinical significance of mutations (ESCAT)
Tumor-board context — the engine does not use these tiers to rank tracks
| Biomarker | Variant | ESCAT | Evidence | Clinical significance | Drugs | Sources |
|---|
| No clinically actionable variants matched in this profile. |
Primary current-line option
Local therapy plan
★ DEFAULT- Indication
- IND-PHEO-LOCALIZED-1L-ALPHA-BLOCKADE-ADRENALECTOMY
- Regimen
- Preoperative alpha-adrenergic blockade (phenoxybenzamine, before pheochromocytoma/paraganglioma surgery)
- Drugs + NSZU
- Phenoxybenzamine (DRUG-PHENOXYBENZAMINE) Starting 10 mg PO BID, titrated in 10-20 mg/day increments · Daily, titrated over the preoperative period to blood-pressure normalization and symptom control (typically roughly 1-2 weeks before surgery) · PO ✗ Not registered in UA
- Reason
- Provisional current-line default from ALGO-PHEO-1L: step 3 did not select a treatment branch. Default MIBG-non-avid metastatic systemic therapy -- sunitinib (IND-PHEO-METASTATIC-1L-SUNITINIB) per FIRSTMAPPP (only randomized, placebo-controlled trial of a systemic agent in metastatic PPGL; 12-mo PFS 36% vs 19% placebo). WIRING NOTE (independent post-hoc review, 2026-07-11...
Other current-line alternatives (3 tracks)
Same treatment line; review when biomarker, access, contraindication, or patient-context assumptions change.
- Indication
- IND-PHEO-METASTATIC-1L-MIBG
- Regimen
- I-131 MIBG radiopharmaceutical therapy (MIBG-avid metastatic pheochromocytoma/paraganglioma)
- Drugs + NSZU
- Iodine-131 MIBG (iobenguane I 131) (DRUG-IODINE-131-MIBG) Weight-based / patient-specific therapeutic activity per pre-therapy dosimetry and current product labeling -- see DRUG-IODINE-131-MIBG typical_dosing · Administered as one or more staged intravenous therapeutic doses in a specialized nuclear-medicine facility with radiation-safety isolation · IV ✗ Not registered in UA
- Reason
- Current-line alternative presented for HCP consideration
- Indication
- IND-PHEO-METASTATIC-1L-SUNITINIB
- Regimen
- Sunitinib monotherapy (metastatic non-MIBG-avid pheochromocytoma/paraganglioma; FIRSTMAPPP)
- Drugs + NSZU
- Sunitinib (DRUG-SUNITINIB) 37.5 mg PO once daily · Continuous daily dosing until progression or unacceptable toxicity (FIRSTMAPPP protocol -- same continuous-dosing schedule already used for pNET, not the intermittent 50 mg 4-weeks-on/2-weeks-off RCC schedule) · PO ⚠ Out-of-pocket
- Reason
- Current-line alternative presented for HCP consideration
- Indication
- IND-PHEO-METASTATIC-1L-CABOZANTINIB
- Regimen
- Cabozantinib monotherapy (metastatic non-MIBG-avid pheochromocytoma/paraganglioma, alternative to sunitinib)
- Drugs + NSZU
- Cabozantinib (DRUG-CABOZANTINIB) 60 mg PO daily · Continuous daily dosing; take without food (>=1h before or 2h after meal), mirroring the standard cabozantinib monotherapy dose already used elsewhere in this KB (REG-CABOZANTINIB-RCC, REG-CABOZANTINIB-MTC-1L) · PO ✗ Not registered in UA
- Reason
- Current-line alternative presented for HCP consideration
Pre-treatment investigations
Investigations before treatment start · critical / standard / desired · merged across tracks
| ID | Name | Priority | Category | Where to order | Needed for |
|---|
| TEST-CT-CHEST-ABDOMEN-PELVIS | CT chest + abdomen + pelvis with IV contrast | Critical | imaging | — | all tracks |
| TEST-GERMLINE-MULTI-GENE-PANEL | Germline multi-gene hereditary cancer panel (NGS) | Standard | genomic | CSD Lab: M089 | desired (local_therapy, standard) |
| TEST-METANEPHRINES-PLASMA-PARAGANGLIOMA | Plasma free metanephrines | Standard | lab | CSD Lab ✓ (code TBC) | all tracks |
Red flags — PRO / CONTRA aggressive
PRO-AGGRESSIVE
Triggers that push toward the aggressive track
CONTRA-AGGRESSIVE
Hard contraindications to escalation
What NOT to do
Explicit prohibitive rules, each grounded in a regimen / supportive care / contraindication entity
Local therapy plan (IND-PHEO-LOCALIZED-1L-ALPHA-BLOCKADE-ADRENALECTOMY)
- Do not proceed to adrenalectomy or paraganglioma resection without documented adequate preoperative alpha-blockade -- risk of intraoperative hypertensive crisis.
- Do not start a beta-blocker before alpha-blockade is underway -- unopposed alpha-stimulation can precipitate hypertensive crisis.
- Do not default to total bilateral adrenalectomy without considering cortical-sparing technique in bilateral/hereditary disease -- risk of lifelong adrenal insufficiency.
Standard plan (IND-PHEO-METASTATIC-1L-MIBG)
- Do not initiate I-131 MIBG therapy without confirmed MIBG avidity on diagnostic scintigraphy.
- Do not administer therapy without prior thyroid blockade (potassium iodide/iodate).
Standard plan (IND-PHEO-METASTATIC-1L-SUNITINIB)
- Do not use sunitinib as first-choice therapy in MIBG-avid disease without considering I-131 MIBG first.
- Do not disregard hypertension on sunitinib in a patient with a catecholamine-secreting tumor -- effects may be additive.
Standard plan (IND-PHEO-METASTATIC-1L-CABOZANTINIB)
- Do not use cabozantinib ahead of sunitinib as first choice without a clinical rationale (intolerance, prior progression, patient preference).
Timeline
Treatment timeline — derived from regimen + monitoring schedule
Standard plan
Induction · Sunitinib monotherapy (metastatic non-MIBG-avid pheochromocytoma/paraganglioma; FIRSTMAPPP)
28-day cycles × Continuous until progression or unacceptable toxicity
Standard plan
Induction · Cabozantinib monotherapy (metastatic non-MIBG-avid pheochromocytoma/paraganglioma, alternative to sunitinib)
28-day cycles × Until progression or unacceptable toxicity
MDT brief
MDT talk tree (1 steps)
| # | Owner | Topic | Action |
|---|
| 1 | social_worker_case_manager | Specialist review | Plan includes drugs without NSZU reimbursement — patient access pathway must be assessed. |
Skills (recommended) — for consideration (1)
Data quality
Usable with caveats. No critical default-track gap was found, but the MDT should review the listed caveats before final sign-off.
- Biomarker coverage: 0/0 known (100%), 0 missing, 0 default-track gaps
- Unevaluated RedFlags: RF-CASCADE-VHL-FDR-POSITIVE, RF-MEN2-CONFIRMED-CARRIER, RF-PHEO-INADEQUATE-ALPHA-BLOCKADE, RF-SDH-CONFIRMED-CARRIER, RF-SDH-FAMILY-HISTORY-SUSPICION, RF-SDHA-SPECIFIC-CARRIER, RF-SDHB-SPECIFIC-CARRIER, RF-SDHC-SPECIFIC-CARRIER, RF-VHL-CONFIRMED-CARRIER
Technical MDT skill metadata (1/16 activated in this plan)
All registered virtual specialists. ✓ — activated for this case; ○ — not activated (available for other clinical scenarios).
| Specialist | skill_id | Version | Last reviewed | Sign-offs | Domain |
|---|
| Cellular therapy specialist (CAR-T) | cellular_therapy_specialist | v0.1.0 | 2026-04-25 | 0 | cellular_therapy |
| Clinical pharmacist | clinical_pharmacist | v0.1.0 | 2026-04-25 | 0 | clinical_pharmacy |
| Hematologist / oncohematologist | hematologist | v0.1.0 | 2026-04-25 | 0 | hematology_oncology |
| Hematopathologist (lymphoma / leukemia / myeloma) | hematopathologist | v0.1.0 | 2026-04-25 | 0 | hematopathology |
| Infectious disease / hepatology | infectious_disease_hepatology | v0.1.0 | 2026-04-25 | 0 | infectious_diseases |
| Medical oncologist (solid-tumor chemotherapist) | medical_oncologist | v0.1.0 | 2026-04-25 | 0 | solid_oncology |
| Molecular geneticist / molecular oncologist | molecular_geneticist | v0.1.0 | 2026-04-25 | 0 | molecular_oncology |
| Palliative care | palliative_care | v0.1.0 | 2026-04-25 | 0 | palliative_care |
| Pathologist (general) | pathologist | v0.1.0 | 2026-04-25 | 0 | pathology |
| Primary care / family physician | primary_care | v0.1.0 | 2026-04-25 | 0 | primary_care |
| Psycho-oncologist | psychologist | v0.1.0 | 2026-04-25 | 0 | psychosocial |
| Radiation oncologist | radiation_oncologist | v0.1.0 | 2026-04-25 | 0 | radiation_oncology |
| Radiologist | radiologist | v0.1.0 | 2026-04-25 | 0 | diagnostic_imaging |
| Social worker / case manager | social_worker_case_manager | v0.1.0 | 2026-04-25 | 0 | psychosocial |
| Surgical oncologist | surgical_oncologist | v0.1.0 | 2026-04-25 | 0 | surgical_oncology |
| Transplant specialist (BMT) | transplant_specialist | v0.1.0 | 2026-04-25 | 0 | cellular_therapy |
Sources cited
- SRC-ENDOCRINE-SOCIETY-PPGL-2014: Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline
(2014)
- SRC-FIRSTMAPPP-BAUDIN-2024: Sunitinib for metastatic progressive phaeochromocytomas and paragangliomas: results from FIRSTMAPPP, an academic, multicentre, international, randomised, placebo-controlled, double-blind, phase 2 trial (2024)
- SRC-NCCN-NET-2025: NCCN Neuroendocrine and Adrenal Tumors (v.2.2025)
Experimental options (clinical trials)
Last synced: 2026-09-09 · ctgov.
No active trials matched this scenario in ctgov.
Option availability in Ukraine
Per-track UA registration · NSZU · cost · access pathway. Render-time metadata; engine selection does not depend on these fields (CHARTER §8.3).
| Option | UA registration | NSZU | Cost orientation | Access pathway |
|---|
| Local therapy plan Preoperative alpha-adrenergic blockade (phenoxybenzamine, before pheochromocytoma/paraganglioma surgery) (REG-ALPHA-BLOCKADE-PREOP-PHEO) 1/1 component drug(s) not registered in Ukraine +1 | ✗ not registered | ✗ out-of-pocket | ₴-? — verify pathway | not recorded |
| Standard plan I-131 MIBG radiopharmaceutical therapy (MIBG-avid metastatic pheochromocytoma/paraganglioma) (REG-IODINE-131-MIBG-PHEO) 1/1 component drug(s) not registered in Ukraine +1 | ✗ not registered | ✗ out-of-pocket | ₴-? — verify pathway | not recorded |
| Standard plan Sunitinib monotherapy (metastatic non-MIBG-avid pheochromocytoma/paraganglioma; FIRSTMAPPP) (REG-SUNITINIB-PHEO) 1/1 component drug(s) not on NSZU formulary | ✓ registered | ✗ out-of-pocket | ₴-? — verify pathway | not recorded |
| Standard plan Cabozantinib monotherapy (metastatic non-MIBG-avid pheochromocytoma/paraganglioma, alternative to sunitinib) (REG-CABOZANTINIB-PHEO) 1/1 component drug(s) not registered in Ukraine +1 | ✗ not registered | ✗ out-of-pocket | ₴-? — verify pathway | not recorded |
Cost information is orientation. Verify with a specific pharmacy / foundation / trial site. Status updated: 2026-09-09.