OpenOnco · Treatment Plan

Treatment plan — Pheochromocytoma

PLAN-VERIFIED-PHEOCHROMOCYTOMA-L1-PHEO_LOCALIZED_1L_ALPHA_BLOCKA-V1 · v1 · 2026-07-15

Patient

VERIFIED-PHEOCHROMOCYTOMA-L1-PHEO_LOCALIZED_1L_ALPHA_BLOCKA · Algorithm: ALGO-PHEO-1L

DiagnosisPheochromocytoma

MOH / ICD-10D35.0

ICD-O-38700/0; C74.1

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.

Standard plan

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

Standard plan

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

Standard plan

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

Pheochromocytoma / paraganglioma surgery (adrenalectomy) is planned or imminent WITHOUT documented adequate preoperative alpha-adrenergic blockade (phenoxybenzamine or doxazosin). Adequate alpha-blockade is mandatory before surgery to prevent an intraoperative hypertensive crisis from catecholamine release during tumor manipulation (Endocrine Society 2014 PPGL guideline). This is a hard sequencing / safety gate, NOT a treatment-selection preference.
Models the narrative's hard requirement that "adequate preoperative alpha-blockade ... is MANDATORY before surgery to prevent intraoperative hypertensive crisis" as an explicit engine-visible safety gate (clinical_direction: hold) rather…
RF-PHEO-INADEQUATE-ALPHA-BLOCKADESRC-ENDOCRINE-SOCIETY-PPGL-2014SRC-NCCN-NET-2025

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)

Social worker / case manager recommended
Plan includes drugs without NSZU reimbursement — patient access pathway must be assessed.

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	Domain
Cellular therapy specialist (CAR-T)	`cellular_therapy_specialist`	v0.1.0	2026-04-25	cellular_therapy
Clinical pharmacist	`clinical_pharmacist`	v0.1.0	2026-04-25	clinical_pharmacy
Hematologist / oncohematologist	`hematologist`	v0.1.0	2026-04-25	hematology_oncology
Hematopathologist (lymphoma / leukemia / myeloma)	`hematopathologist`	v0.1.0	2026-04-25	hematopathology
Infectious disease / hepatology	`infectious_disease_hepatology`	v0.1.0	2026-04-25	infectious_diseases
Medical oncologist (solid-tumor chemotherapist)	`medical_oncologist`	v0.1.0	2026-04-25	solid_oncology
Molecular geneticist / molecular oncologist	`molecular_geneticist`	v0.1.0	2026-04-25	molecular_oncology
Palliative care	`palliative_care`	v0.1.0	2026-04-25	palliative_care
Pathologist (general)	`pathologist`	v0.1.0	2026-04-25	pathology
Primary care / family physician	`primary_care`	v0.1.0	2026-04-25	primary_care
Psycho-oncologist	`psychologist`	v0.1.0	2026-04-25	psychosocial
Radiation oncologist	`radiation_oncologist`	v0.1.0	2026-04-25	radiation_oncology
Radiologist	`radiologist`	v0.1.0	2026-04-25	diagnostic_imaging
Social worker / case manager	`social_worker_case_manager`	v0.1.0	2026-04-25	psychosocial
Surgical oncologist	`surgical_oncologist`	v0.1.0	2026-04-25	surgical_oncology
Transplant specialist (BMT)	`transplant_specialist`	v0.1.0	2026-04-25	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)

Third plan track — open-enrollment trials from ClinicalTrials.gov. Render-time metadata; engine selection is not affected by this block (CHARTER §8.3). Last synced: 2026-07-15.

NCT	Title	Phase	Status	Sponsor	UA	Signals
NCT05636540	In Vivo PARP-1 Expression With 18F-FluorThanatrace PET/CT in Patients With Pheochromocytoma and Paraganglioma	EARLY_PHASE1	RECRUITING	Heather Wachtel	—	Small N (<50) Single country
NCT01660984	Natural History Study of Children and Adults With Medullary Thyroid Cancer	N/A	RECRUITING	National Cancer Institute (NCI)	—	Single country
NCT00001238	Von Hippel-Lindau (VHL): Clinical Manifestations, Diagnosis, Management and Molecular Bases of Inherited Renal and Other Urologic Malignant Disorders	N/A	RECRUITING	National Cancer Institute (NCI)	—	Surrogate endpoint only Single country
NCT00107289	Iodine I 131 Metaiodobenzylguanidine in Treating Patients With Recurrent, Progressive, or Refractory Neuroblastoma or Malignant Pheochromocytoma or Paraganglioma	PHASE2	RECRUITING	Memorial Sloan Kettering Cancer Center	—	Single country
NCT03050268	Familial Investigations of Childhood Cancer Predisposition	N/A	RECRUITING	St. Jude Children's Research Hospital	—	Single country
NCT06427798	Somatostatin-Receptors (SSTR)-Agonist [212Pb]VMT-alpha-NET in Metastatic or Inoperable SSTR+ Gastrointestinal Neuroendocrine Tumor and Pheochromocytoma/Paraganglioma Previously Treated With Systemic Targeted Radioligand Therapy	PHASE1 / PHASE2	RECRUITING	National Cancer Institute (NCI)	—	Surrogate endpoint only Single country
NCT05702944	The Effect and Safety of Omitting Preoperative Alpha-adrenergic Blockade for Normotensive Pheochromocytoma	PHASE4	RECRUITING	Seoul National University Hospital	—	Small N (<50) Single country
NCT07282587	Study of ONC206 (JZP3507) in Advanced Pheochromocytoma and Paraganglioma	PHASE2	RECRUITING	Jazz Pharmaceuticals	—	Surrogate endpoint only Single country
NCT03344016	Multicenter Pheochromocytoma and Paraganglioma Evaluation	NA	RECRUITING	Felix Beuschlein	—	Single country
NCT06444607	Hereditary Pheochromocytoma Assessment of Tumour Immunologies	N/A	RECRUITING	Radboud University Medical Center	—	Single country

Verify recruitment status directly with the trial site. ctgov data can lag behind current UA-site status.

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
Trial · NCT05636540 In Vivo PARP-1 Expression With 18F-FluorThanatrace PET/CT in Patients With Pheochromocytoma and Paraganglioma No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT01660984 Natural History Study of Children and Adults With Medullary Thyroid Cancer No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT00001238 Von Hippel-Lindau (VHL): Clinical Manifestations, Diagnosis, Management and Molecular Bases of Inherited Renal and Other Urologic Malignant Disorders No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT00107289 Iodine I 131 Metaiodobenzylguanidine in Treating Patients With Recurrent, Progressive, or Refractory Neuroblastoma or Malignant Pheochromocytoma or Paraganglioma No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT03050268 Familial Investigations of Childhood Cancer Predisposition No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT06427798 Somatostatin-Receptors (SSTR)-Agonist [212Pb]VMT-alpha-NET in Metastatic or Inoperable SSTR+ Gastrointestinal Neuroendocrine Tumor and Pheochromocytoma/Paraganglioma Previously Treated With Systemic Targeted Radioligand Therapy No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT05702944 The Effect and Safety of Omitting Preoperative Alpha-adrenergic Blockade for Normotensive Pheochromocytoma No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT07282587 Study of ONC206 (JZP3507) in Advanced Pheochromocytoma and Paraganglioma No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT03344016 Multicenter Pheochromocytoma and Paraganglioma Evaluation No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT06444607 Hereditary Pheochromocytoma Assessment of Tumour Immunologies No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor

Cost information is orientation. Verify with a specific pharmacy / foundation / trial site. Status updated: 2026-07-15.

plan_id: PLAN-VERIFIED-PHEOCHROMOCYTOMA-L1-PHEO_LOCALIZED_1L_ALPHA_BLOCKA-V1 | version: 1 | generated: 2026-07-15T13:33:23.158188+00:00

Per FDA non-device CDS positioning (CHARTER §15): Outpatient oncology treatment planning to support tumor-board discussion. Not a medical device; not for autonomous clinical decision-making.

Per FDA non-device CDS positioning (CHARTER §15): Both treatment options below are presented for review. The engine's selection of a 'recommended' default does not constitute a clinical decision. Final treatment selection requires HCP judgment incorporating information not available to the system.

This document is an informational resource supporting tumor-board discussion (per CHARTER §11). It is not a system that makes clinical decisions. Every recommendation must be verified by the treating physician.