SDHC-Related Paraganglioma and Gastric Stromal Sarcoma (Gastric GIST)

SDHC-Related Paraganglioma and Gastric Stromal Sarcoma (Gastric GIST) are rare growths that arise from nerve-related cells called paraganglia. These cells sit along blood vessels and nerves and help control body functions. When a germline change (mutation) in the SDHC gene is present, the paraganglioma belongs to an inherited group called PGL3 syndrome. SDHC is part of the succinate dehydrogenase (SDH) enzyme in mitochondria. If SDHC is faulty, the SDH enzyme does not work properly. Cells then build up succinate, which sends false “low-oxygen” signals and can drive tumor growth. SDHC-related tumors most often occur in the head and neck (for example, at the carotid body or middle ear). They are usually benign and rarely spread, though careful follow-up is important. Laboratory tests may or may not show extra stress hormones (catecholamines). Imaging uses MRI, CT, and special PET scans that target somatostatin receptors. Genetic counseling is advised for families. MDPI+2www.elsevier.com+2

Paragangliomas are rare tumors that grow from nerve-related cells called paraganglia. Some make stress hormones (catecholamines), which can cause high blood pressure, headaches, sweats, and a fast heartbeat. “SDHC-related” means a harmful change (pathogenic variant) in the SDHC gene—one part of the mitochondrial enzyme succinate dehydrogenase (SDH)—has driven the tumor to form. When SDH does not work, the cell builds up succinate, which stabilizes a “low-oxygen” signal (HIF pathway) and pushes the cell toward tumor behavior (“pseudohypoxia”). SDHC tumors often arise in the head and neck and may be non-secreting, but can also appear in other sites. Modern reviews and guidelines emphasize genetic testing of all SDH-deficient tumors and life-long follow-up because new tumors can appear over time. PMC+2Wiley Online Library+2

Older reports called these tumors “gastric stromal sarcoma,” but today we use gastrointestinal stromal tumor (GIST). A special subgroup lacks the usual KIT or PDGFRA mutations and is SDH-deficient. Many arise in the stomach of adolescents or young adults, can be multifocal, and tend to grow slowly but may recur. SDH deficiency can happen from SDHx gene variants (including SDHC) or from epigenetic silencing (promoter methylation) of SDHC—classically seen in Carney triad (gastric GIST + paraganglioma + lung chondroma). These SDH-deficient GISTs behave differently and respond poorly to imatinib; care is specialized. Viamedica Journals+2Frontiers+2

“Gastric stromal sarcoma” is the older name for gastrointestinal stromal tumor (GIST) of the stomach. GISTs come from the interstitial cells of Cajal—the “pacemaker” cells of the gut. Most GISTs have KIT or PDGFRA gene mutations. A smaller subgroup is SDH-deficient GIST, where the SDH enzyme is lost; this includes tumors linked to SDHx genes (like SDHC) and to SDHC promoter hypermethylation (an epimutation) seen in Carney triad. SDH-deficient gastric GISTs tend to occur in children, teens, and young adults, often in the stomach, and show loss of SDHB protein by immunostaining with strong DOG1 and often KIT (CD117) positivity. These tumors behave differently from typical, KIT-mutant GISTs and require tailored testing and management. PubMed+4Meridian+4Grupo Geis+4

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Other names

• SDHC-related paraganglioma: “PGL3 syndrome,” “SDHC-mutated paraganglioma,” “SDHx-related PGL” (SDHx means any SDH subunit gene). MDPI

• Gastric stromal sarcoma: “Gastric GIST,” “SDH-deficient GIST” (if SDH lost), “KIT-mutant GIST” or “PDGFRA-mutant GIST” (if those driver mutations are present). Grupo Geis+1

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Types

SDHC-related paraganglioma

• By location: Head and neck paraganglioma (carotid body, jugular, vagal, tympanic), thoracic or abdominal sympathetic paraganglioma (less common in SDHC). Head/neck tumors predominate. www.elsevier.com

• By hormone production: Non-secretory (most head/neck tumors) vs secretory (release of norepinephrine or dopamine is possible but less common in SDHC). OUP Academic

• By genetics: Germline SDHC pathogenic variant with a “second hit” in the tumor; very rarely epigenetic silencing. NCBI

• By behavior: Benign in the vast majority; metastatic disease is rare in SDHC compared with SDHB. Eshonline+1

Gastric GIST

• By driver mutation: KIT-mutant, PDGFRA-mutant, SDH-deficient (SDHx mutation or SDHC epimutation), NF1-associated, BRAF/RAS, or rare fusion-driven tumors. PMC

• By SDH status: SDH-deficient (loss of SDHB by IHC) vs SDH-competent. Grupo Geis

• By site/age: Stomach (most common), small intestine; pediatric/young adult cases are often SDH-deficient. Frontiers

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Causes

Note: “Cause” here means the underlying reasons or risk contexts that allow these tumors to form. Some apply to SDHC-PGL, some to gastric GIST, and some to the shared SDH-deficient pathway.

1. Germline SDHC mutation (PGL3): A harmful change in one SDHC copy is present from birth; the tumor forms after a second hit in the same gene in certain cells. MDPI

2. Loss of SDH enzyme activity: Faulty SDHC disrupts the SDH complex in mitochondria, causing energy pathway changes that favor tumor growth. MDPI

3. Succinate buildup (“oncometabolite”): Excess succinate blocks enzymes that normally clear “low oxygen” signals, pushing cells toward growth. MDPI

4. Pseudohypoxia signaling: The cell “thinks” oxygen is low, stabilizes HIF proteins, and turns on growth programs. MDPI

5. SDHC promoter hypermethylation (epimutation): In some gastric GISTs (and Carney triad), the SDHC gene is “silenced” by methyl tags rather than mutated. PubMed

6. Carney triad: A non-inherited condition linking gastric GIST, paraganglioma, and pulmonary chondroma; many cases show SDHC epimutation. Frontiers

7. Carney-Stratakis syndrome (dyad): Inherited pairing of GIST and paraganglioma due to germline SDHx mutations (including SDHC). PMC

8. SDHB, SDHD, SDHA variants (contrast group): Other SDH gene changes cause overlapping PGL syndromes; included here to show the SDH pathway’s central role. (SDHC is our focus.) MDPI

9. KIT mutation (GIST): Many gastric GISTs form because KIT is turned on by mutation, driving uncontrolled cell growth. PMC

10. PDGFRA mutation (GIST): Another common GIST driver; certain PDGFRA changes are linked to stomach location and unique behavior. PMC

11. NF1-associated GIST: People with neurofibromatosis type 1 may develop GIST through RAS pathway activation. PMC

12. BRAF/RAS pathway changes: Rare “wild-type” GISTs can be driven by BRAF or RAS mutations. PMC

13. Gene fusions (rare GIST): Uncommon fusions can drive GIST when KIT/PDGFRA are not mutated. PMC

14. Second-hit tumor genetics: After an inherited SDHC variant, tumor cells lose the remaining normal copy (loss of heterozygosity), allowing growth. NCBI

15. Age/sex patterns (SDH-deficient GIST): SDH-deficient gastric GISTs often occur in young patients and females, reflecting underlying biology rather than lifestyle. Frontiers

16. Somatic mosaicism for SDHC epimutation: In Carney triad, only certain tissues show SDHC methylation, hinting the change happened after conception. Ovid

17. Non-secretory neural crest cell origin (head/neck PGL): Many SDHC PGLs arise in parasympathetic paraganglia that usually do not make much adrenaline. This biology shapes symptoms and testing. www.elsevier.com

18. Family inheritance pattern (autosomal dominant): One altered SDHC copy can pass to children; each child has a 50% chance to inherit it. MDPI

19. Environmental modifiers (theoretical): Hypoxia and other stresses may influence tumor behavior in SDH-deficient states, but heredity is the main driver. (Framed cautiously; evidence strongest for genetics.) MDPI

20. Epigenetic re-programming in SDH loss: SDH defects alter DNA/histone methylation patterns globally, shifting cells toward tumor pathways. Wiley Online Library

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Symptoms

Symptoms vary. SDHC-related head/neck paragangliomas often cause local pressure symptoms. Gastric GISTs often cause bleeding, anemia, or fullness. Not everyone has all symptoms.

1. Painless neck mass or fullness (HNPGL): A slow-growing lump near the jaw or along the neck vessels. www.elsevier.com

2. Pulsatile tinnitus: “Whooshing” sound in one ear from a vascular mass in the middle ear. www.elsevier.com

3. Hearing loss or ear fullness: Tumor behind the eardrum can affect hearing. www.elsevier.com

4. Hoarseness or voice change: Pressure on the vagus nerve or recurrent laryngeal nerve. www.elsevier.com

5. Trouble swallowing: A parapharyngeal tumor can narrow the throat. www.elsevier.com

6. Headache, palpitations, sweating, or high blood pressure: If the tumor releases catecholamines (less common in SDHC HNPGL but possible in other sites). OUP Academic

7. Lightheadedness: Blood pressure swings from catecholamines (if secretory). OUP Academic

8. Abdominal pain or discomfort (GIST): A stomach mass can ache or feel sore. Grupo Geis

9. Early fullness after small meals: A gastric mass reduces stomach capacity. Grupo Geis

10. Black stools (melena) or vomiting blood: GISTs can ulcerate and bleed into the stomach. Grupo Geis

11. Anemia symptoms: Tiredness, pale skin, shortness of breath due to chronic blood loss from GIST. Grupo Geis

12. Unexplained weight loss: From chronic illness or decreased intake. Grupo Geis

13. Abdominal mass: Large GISTs may be felt on exam. Grupo Geis

14. Reflux or indigestion: Non-specific stomach upset can occur with gastric tumors. Grupo Geis

15. Nerve-related deficits (HNPGL): Tongue weakness, shoulder droop, or palate asymmetry if cranial nerves are compressed. www.elsevier.com

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Diagnostic tests

A) Physical examination (what the clinician observes)

1. Vital signs and blood pressure pattern: Hypertension, variable readings, and tachycardia suggest catecholamine release (when present). Careful, calm measurement reduces false alarms. OUP Academic

2. Neck and cranial nerve exam: Inspection and palpation can reveal a firm, mobile mass and nerve deficits (voice, swallowing, shoulder, tongue). www.elsevier.com

3. Otoscopy (ear exam): A red/blue pulsating mass behind the eardrum suggests a tympanic paraganglioma. www.elsevier.com

4. Abdominal exam: Tenderness, fullness, or a palpable mass may indicate a large gastric GIST. Grupo Geis

5. Signs of anemia: Pale conjunctiva, rapid pulse, and fatigue point to GIST-related bleeding. Grupo Geis

B) Manual/bedside tests (simple clinic maneuvers)

6. Orthostatic blood pressure check: Standing BP may drop after surges of catecholamines subside or from anemia; it helps link symptoms to physiology. OUP Academic

7. Focused swallowing assessment: Water swallow tests can flag dysphagia and the need for imaging of a parapharyngeal mass. www.elsevier.com

8. Bedside hearing screening: Simple tests (whisper test, tuning forks) triage patients with suspected ear PGL for audiology and imaging. www.elsevier.com

9. Stool guaiac (fecal occult blood) at bedside: Detects hidden blood from a bleeding gastric GIST. Positive tests require endoscopic confirmation. Grupo Geis

10. Abdominal tenderness localization: Gentle palpation may point to tumor size/location and guide next imaging. Grupo Geis

C) Laboratory and pathological tests

11. Plasma free metanephrines: First-line biochemical test for PPGLs; very sensitive when done in a calm, supine setting. Borderline results may need repeat or clonidine suppression. OUP Academic+1

12. 24-hour urinary fractionated metanephrines: Alternative first-line biochemical screen, especially when plasma testing is not feasible. OUP Academic

13. Chromogranin A: A supportive marker that can rise in neuroendocrine tumors, used with caution because many non-tumor factors can elevate it. First Pediatrics UOA

14. Complete blood count (CBC): Looks for iron-deficiency anemia from chronic GIST bleeding; low hemoglobin supports GI blood loss. Grupo Geis

15. Tumor immunohistochemistry (biopsy/resection):

• SDHB IHC: Loss confirms SDH-deficient status.

• SDHA IHC: If both SDHA and SDHB are lost, SDHA is likely mutated; if SDHB lost but SDHA present, another SDHx gene (like SDHC) is implicated.

• KIT (CD117) and DOG1: Strong markers for GIST; DOG1 helps when KIT is negative. Meridian+1

16. Molecular testing (NGS panel): Detects SDHC germline variants for PGL and KIT/PDGFRA/SDHx status for GIST; guides counseling and follow-up. Nature

17. SDHC promoter methylation assay: Identifies epimutations typical of Carney triad and some SDH-deficient GISTs; useful when sequencing is negative. PubMed+1

18. Iron studies (ferritin, transferrin saturation): Support the diagnosis of iron-deficiency anemia from GI bleeding due to GIST. Grupo Geis

D) Electrodiagnostic / physiologic monitoring

19. 12-lead ECG: Checks for arrhythmias or ischemic changes triggered by catecholamine surges (if a secretory PGL is present). NCBI

20. Ambulatory ECG or blood pressure monitoring: Captures intermittent palpitations or BP spikes that correlate with symptoms. These help triage to biochemical testing and imaging. NCBI

E) Imaging tests (how we find and stage tumors)

• MRI of head/neck: Preferred for suspected head/neck paraganglioma; high sensitivity without radiation. Nature

• CT of neck/chest/abdomen: Maps tumor size, relation to vessels and organs, and looks for spread. OUP Academic

• 68Ga-DOTATATE PET/CT: The most sensitive functional imaging for many SDHx-related paragangliomas and head/neck lesions; often outperforms MIBG and FDG for lesion detection. PMC+2Thieme+2

• 18F-FDG PET/CT: Helpful in more aggressive tumors or when somatostatin receptor expression is variable; complementary to DOTATATE. BioMed Central

• 123I-MIBG scintigraphy: Historically used, but much less sensitive in SDHx-related disease, especially SDHB; now used selectively. PMC+1

• Upper endoscopy (EGD): Directly visualizes gastric GISTs, checks for ulceration, and allows biopsy when safe. Grupo Geis

• Endoscopic ultrasound (EUS): Defines layer of origin (submucosa/muscularis propria) and permits fine-needle sampling for GIST. Grupo Geis

• CT enterography / abdominal MRI: Stages gastric GIST and looks for liver or peritoneal spread. Grupo Geis

Non-pharmacological treatments

1. Multidisciplinary care and genetic counseling
   Purpose: Coordinate surgery, oncology, endocrinology, genetics, and imaging; inform relatives.
   Mechanism: Team planning reduces delays and tailors surveillance for SDHx families; cascade testing finds carriers early. ScienceDirect

2. Pre-operative alpha-blockade and BP control for functional tumors
   Purpose: Prevent dangerous blood pressure surges during anesthesia and surgery.
   Mechanism: Alpha-blockers blunt catecholamine effects; guidelines recommend 7–14 days of blockade and liberal salt/fluid intake. Nature

3. Surgical resection when feasible (PGL or gastric GIST)
   Purpose: Remove the tumor for cure or symptom relief.
   Mechanism: Surgery eliminates hormone source or bleeding mass; approach varies by site (open resection often for paraganglioma; organ-sparing when appropriate). PubMed

4. Endoscopic surveillance for small gastric SDH-deficient GIST
   Purpose: Watch indolent lesions and time intervention.
   Mechanism: Serial endoscopy/EUS and imaging track size/bleeding risk; many SDH-deficient gastric GISTs are slow-growing. Viamedica Journals

5. Lifelong follow-up (imaging + biochemistry)
   Purpose: Detect new primaries or recurrence early.
   Mechanism: Periodic metanephrines and targeted imaging are recommended because SDHx tumors can recur years later. OUP Academic

6. Anemia management in bleeding gastric GIST
   Purpose: Restore iron and hemoglobin to improve symptoms.
   Mechanism: Iron therapy/transfusion as needed; treat bleeding source definitively (resection or endoscopic therapy). Viamedica Journals

7. Dietary salt and volume optimization around surgery
   Purpose: Reduce post-op low blood pressure after tumor removal.
   Mechanism: Alpha-blockade can cause vasodilation; extra salt/fluids maintain volume per guideline suggestions. PubMed

8. Radiation-safety education for radiopharmaceutical therapies
   Purpose: Protect patient/family after I-131 MIBG or Lu-177 DOTATATE.
   Mechanism: Standard precautions limit exposure; amino-acid infusions protect kidneys during Lu-177 therapy. FDA Access Data+1

9. Fertility/pregnancy counseling
   Purpose: Plan therapy timing; avoid teratogenic exposures.
   Mechanism: Many cancer drugs and radio-therapies harm a fetus; planning reduces risk. (See drug labels below for pregnancy warnings.) FDA Access Data

10. Lifestyle risk reduction
    Purpose: General cardiometabolic benefit during long surveillance.
    Mechanism: Stop smoking, maintain healthy weight, regular activity—supportive for surgical recovery and BP control. (General guidance consistent with endocrine/oncology survivorship reviews.) Endocrine Society

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Drug treatments

Dosing here summarizes FDA-labeled adult regimens where applicable; individual dosing must be personalized by the treating team.

1. Iobenguane I-131 (Azedra®) — Radiotherapeutic for PPGL
   Class: Radiopharmaceutical (targets norepinephrine transporter).
   Dose/Time: Weight/organ-dose–based; dosimetry precedes therapy; typically two therapeutic doses weeks apart per label.
   Purpose/Mechanism: Delivers targeted radiation to PPGL cells that take up MIBG, shrinking tumors and lowering catecholamines. Side effects: Myelosuppression, hypothyroidism, nausea; radiation precautions required. FDA Access Data+2FDA Access Data+2

2. Lutetium Lu-177 dotatate (Lutathera®) — For SSTR-positive NETs; used in SSTR-expressing lesions
   Class: Peptide receptor radionuclide therapy (PRRT) to somatostatin receptors.
   Dose/Time: 7.4 GBq (200 mCi) IV every 8 weeks × 4; amino-acid infusion for renal protection.
   Purpose/Mechanism: Binds SSTR-positive cells and delivers beta radiation. Side effects: Nausea (from amino acids), myelosuppression; radiation safety needed. FDA Access Data+1

3. Imatinib (Gleevec®) — TKI for GIST (not effective in SDH-deficient GIST, but standard in KIT/PDGFRA-mutant)
   Class: Tyrosine kinase inhibitor (KIT/PDGFRA).
   Dose/Time: 400 mg daily typical (GIST); adjust for organ function.
   Purpose/Mechanism: Blocks KIT/PDGFRA signaling. Side effects: Edema, cytopenias, GI upset, hepatotoxicity. Note: SDH-deficient GISTs are usually imatinib-resistant; listed here for completeness and for mixed clinics. FDA Access Data+2FDA Access Data+2

4. Sunitinib (Sutent®) — TKI active in imatinib-refractory GIST; used in some PPGL series
   Class: Multitarget TKI (VEGFR, PDGFR, KIT).
   Dose/Time: 50 mg daily (4 weeks on/2 off) or 37.5 mg continuous—per label.
   Purpose/Mechanism: Anti-angiogenic and anti-KIT activity; standard second-line GIST. Side effects: Hypertension, fatigue, HFSR, cytopenias, thyroid dysfunction. FDA Access Data+1

5. Regorafenib (Stivarga®) — Third-line GIST
   Class: Multikinase inhibitor (VEGFR, RAF, PDGFR, KIT).
   Dose/Time: 160 mg daily 3 weeks on/1 week off.
   Purpose/Mechanism: Improves progression-free survival after imatinib/sunitinib. Side effects: Hand–foot reaction, hypertension, fatigue; wound-healing caution. FDA Access Data+1

6. Ripretinib (Qinlock®) — Fourth-line GIST
   Class: Broad KIT/PDGFRA switch-control inhibitor.
   Dose/Time: 150 mg once daily until progression/intolerance.
   Purpose/Mechanism: Inhibits a spectrum of resistant KIT mutations; improves PFS in heavily pretreated GIST. Side effects: Alopecia, myalgia, fatigue; monitor for skin toxicity and hypertension. FDA Access Data+1

7. Avapritinib (Ayvakit®) — For PDGFRA exon 18 (D842V) GIST
   Class: PDGFRA-selective TKI.
   Dose/Time: 300 mg daily (GIST indication).
   Purpose/Mechanism: Highly active in PDGFRA D842V GIST (not an SDH-deficient biology, but important for differential). Side effects: Cognitive effects, edema, GI symptoms—dose modify as needed. FDA Access Data+1

8. Belzutifan (Welireg®) — HIF-2α inhibitor; now FDA-approved for PPGL (2025 supplement)
   Class: Hypoxia-inducible factor-2α inhibitor.
   Dose/Time: 120 mg daily (labels vary by indication).
   Purpose/Mechanism: Blocks HIF-2 signaling central to SDH-deficient pseudohypoxia biology; FDA accepted supplemental approval for PPGL in 2025. Side effects: Anemia, hypoxia; embryo-fetal toxicity warnings. FDA Access Data+2FDA Access Data+2

9. Octreotide LAR (Sandostatin LAR®) — Somatostatin analog for SSTR-positive symptoms ± antiproliferative effect
   Class: Somatostatin analog.
   Dose/Time: IM every 4 weeks; dose titrated by response.
   Purpose/Mechanism: Binds SSTR to reduce hormone-related symptoms and may slow growth in NETs; often paired with imaging/PRRT strategies. Side effects: Gallstones, glucose changes; B12 monitoring. FDA Access Data+1

10. Lanreotide depot (Somatuline® Depot) — Somatostatin analog (NETs)
    Class: Somatostatin analog.
    Dose/Time: Deep SC every 4 weeks; adjust per response.
    Purpose/Mechanism: Similar to octreotide; improves progression-free survival in GEP-NETs; useful for symptom control in SSTR-positive disease. Side effects: GI upset, gallstones; storage/refrigeration specifics in label. FDA Access Data+1

11. Temozolomide (Temodar®) — Alkylating agent (used in some metastatic PPGL cases)
    Class: Alkylating chemotherapy.
    Dose/Time: Common schedules include 150–200 mg/m²/day × 5 days q28d (label onc indications differ; PPGL use is off-label).
    Purpose/Mechanism: DNA alkylation/cytotoxic; clinical activity reported particularly in SDHB-mutant PPGL cohorts. Side effects: Myelosuppression, nausea, fatigue. FDA Access Data+1

12. Metyrosine (Demser® and generics) — Pre-op and palliative control of catecholamine excess
    Class: Tyrosine hydroxylase inhibitor (blocks catecholamine synthesis).
    Dose/Time: Oral; dose titrated to symptom control in PPGL (per label family).
    Purpose/Mechanism: Lowers norepinephrine/epinephrine production to stabilize BP and limit crises; often combined with alpha-blockers. Side effects: Sedation, depression, extrapyramidal effects—monitor closely. FDA Access Data+2FDA Access Data+2

(Other TKIs such as pazopanib or cabozantinib have labels for other cancers and are sometimes used in refractory settings; selection depends on individual factors and trial data.) FDA Access Data+3FDA Access Data+3FDA Access Data+3

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Dietary molecular supplements

No supplement cures these tumors. Discuss all products with your care team, especially around surgery or radiation. Evidence is supportive/adjunctive only.

1. Oral iron (ferrous sulfate or equivalents) — Dose: usual elemental iron 40–65 mg once/twice daily as tolerated. Function/mechanism: Replenishes iron lost from slow gastric bleeding; improves fatigue and exercise tolerance. Viamedica Journals

2. Vitamin B12 — Dose: replacement if low. Mechanism: Corrects B12 deficiency that can occur with somatostatin analog therapy. FDA Access Data

3. Amino-acid infusions (arginine/lysine mixtures) given with Lu-177 therapy — Dose: per PRRT protocol. Mechanism: Compete for renal tubular uptake, lowering kidney radiation dose. FDA Access Data

4. Vitamin D — Dose: individualized to serum 25-OH-D. Mechanism: Bone health during long surveillance and possible TKI-related effects on bone turnover (supportive). (General oncology supportive care practice.)

5. Folate — Dose: diet or supplement if deficient. Mechanism: Supports RBC production during chronic iron-loss anemia. (General hematology practice.)

6. Electrolyte-balanced oral fluids — Dose: as advised around alpha-blockade and surgery. Mechanism: Supports volume expansion to reduce post-op hypotension. PubMed

7. Protein-adequate diet — Dose: dietitian-guided. Mechanism: Helps wound healing and muscle mass during long treatments. (General peri-operative nutrition guidance.)

8. Omega-3 fatty acids — Dose: typical 1 g/day EPA/DHA if no bleeding risk. Mechanism: May help triglycerides and inflammation; check with surgeon before procedures. (General cardiometabolic guidance.)

9. Proton-pump inhibitor or H2 blocker (not a “supplement” but often needed) — Mechanism: Protects stomach mucosa in bleeding-prone gastric tumors; physician-directed. (Standard GI supportive care.)

10. Multivitamin without iron (when not iron-deficient) — Mechanism: Covers mild diet gaps during treatment; avoid excess iron unless deficient. (General survivorship guidance.)

(Clinical nutrition for cancer care is individualized; these are supportive principles rather than disease-modifying therapies.)

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Surgeries (what they are and why)

1. Open resection of paraganglioma (site-appropriate approach)
   Why done: Definitive removal, symptom control, and to prevent catecholamine crises. Notes: Head–neck lesions often need vascular control; abdominal PGLs need strict pre-op alpha-blockade and high-volume experience. PubMed

2. Laparoscopic/robotic adrenalectomy (if adrenal pheochromocytoma present)
   Why done: Minimally invasive removal with faster recovery when criteria are met. Notes: Partial adrenalectomy may be considered in select hereditary cases. PubMed

3. Wedge/partial gastrectomy for gastric GIST
   Why done: Remove bleeding or growing gastric SDH-deficient GIST with margin negative resection; lymph nodes are rarely involved. Viamedica Journals

4. Endoscopic therapy for bleeding lesions
   Why done: Stabilize bleeding pre-definitive surgery (clips, coagulation) when appropriate. Viamedica Journals

5. Resection of limited metastases
   Why done: Symptom relief and, occasionally, disease control in carefully selected cases within a multimodal plan. Endocrine Society

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Preventions

1. Genetic testing and family cascade testing to find carriers early. ScienceDirect

2. Regular surveillance plan (biochemistry + imaging). OUP Academic

3. Pre-op alpha-blockade for any functional tumor before surgery. Nature

4. Avoid unmonitored decongestants/stimulants that can trigger BP spikes (e.g., pseudoephedrine). (Endocrine practice.)

5. Peri-procedural planning with anesthesia, flagging PPGL history. PubMed

6. Vaccination up to date (general oncology care).

7. Healthy BP, weight, and exercise program—cardiovascular resilience. (Survivorship care.)

8. Prompt iron/B12 correction to prevent complications of anemia. FDA Access Data

9. Radiation safety counseling when receiving I-131 MIBG or Lu-177 therapy. FDA Access Data+1

10. Keep a symptom diary (headaches, palpitations, flushing, black stools) and report new patterns early. OUP Academic

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When to see a doctor

• New or worsening headaches, sweating, palpitations, or blood pressure spikes. OUP Academic

• Black stools, vomiting blood, or iron-deficiency anemia symptoms (fatigue, pica). Viamedica Journals

• A new neck mass, ear fullness, or voice change. Wiley Online Library

• Fainting, chest pain, severe anxiety spells (rule out catecholamine surge). OUP Academic

• Before any elective surgery, pregnancy, or fertility treatment if you have PPGL history. PubMed

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What to eat and what to avoid

1. If iron-deficient, favor iron-rich foods (lean meats, beans, greens) plus vitamin C for absorption; avoid tea/coffee with iron doses. Viamedica Journals

2. Balanced salt and fluids only as instructed during pre-op alpha-blockade; otherwise follow standard BP-friendly diet. PubMed

3. Small, regular meals if early satiety from gastric mass. Viamedica Journals

4. Limit alcohol (BP and bleeding risk).

5. Avoid high-caffeine/stimulant drinks if they trigger palpitations.

6. Adequate protein for healing and strength.

7. High-fiber foods unless bowel symptoms suggest otherwise; adjust individually.

8. Omega-3–containing foods (fish, flax) for heart health (confirm with surgeon pre-op).

9. Avoid herbal stimulants (yohimbine, synephrine) that can raise BP.

10. Discuss all supplements and fasting diets with the team before PRRT or MIBG. FDA Access Data

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FAQs

1) Is “gastric stromal sarcoma” the same as GIST?
Yes—older term. In SDH-deficient cases (often gastric), the tumor biology and drug response differ from “typical” KIT-mutant GIST. Viamedica Journals

2) What makes an SDHC-related tumor special?
Loss of SDH function drives a “pseudohypoxic” state (HIF activation). That biology shapes imaging choices and emerging drugs like belzutifan. PMC+1

3) Are SDHC tumors always hereditary?
Many are hereditary, but SDHC can also be epigenetically silenced (Carney triad). Testing clarifies this. Frontiers

4) Which first tests confirm a functional paraganglioma?
Plasma free or urinary fractionated metanephrines. OUP Academic

5) Which scans are best for SDHx disease?
MRI/CT for anatomy; FDG-PET and SSTR-PET are highly useful; MIBG is mainly for therapy planning. Urology Wiki

6) Do SDH-deficient GISTs respond to imatinib?
Generally poorly—other strategies and surgery/surveillance are used; later-line TKIs vary by case. Viamedica Journals

7) What new systemic options exist for PPGL?
FDA has approved Azedra (I-131 MIBG) and, in 2025, belzutifan for PPGL; PRRT is an option for SSTR-positive disease. FDA Access Data+2FDA Access Data+2

8) Are somatostatin analogs useful?
Yes, for SSTR-positive symptoms and disease control, and as partners with PRRT. FDA Access Data+1

9) Why is pre-op alpha-blockade critical?
It prevents life-threatening BP spikes during anesthesia and tumor handling. Nature

10) How long is follow-up?
Life-long, due to risk of new primaries or recurrence. OUP Academic

11) Can family members be at risk?
Yes—offer genetic counseling and cascade testing. ScienceDirect

12) Is there a role for temozolomide?
Yes, for selected metastatic PPGL (off-label), with documented activity in SDHx cohorts. FDA Access Data

13) What about pazopanib/cabozantinib?
Both are TKIs with anti-angiogenic activity used in refractory cases based on broader oncology labels and studies. FDA Access Data+1

14) Do I need special diet rules forever?
No disease-specific diet cures these tumors; nutrition supports strength, anemia correction, and safe surgery. Viamedica Journals

15) Who should manage my care?
A center experienced in PPGL/GIST with genetics, endocrine, nuclear medicine, surgical oncology, and GI surgery. ScienceDirect

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The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: November 11, 2025.

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