Table 5.
Treatment Manual for PPGL
| Scenario | Intervention | Ref | # | |
|---|---|---|---|---|
| Staging and blockade | Elevated plasma or urine normetanephrine and/or metanephrine | a. alpha-adrenoceptor blocker, eg, doxazosin 1–2 mg, increase 2–4 mg weekly to maximum tolerated dosage for ≤30 mg/d. | (1) | 1.1 |
| b. Localization studies CT, MRI, or PET/CT. | (168) | 1.2 | ||
| Localized stage | Thoracic or abdominal/pelvic | Curative resection, if safe. | (1) | 2.1 |
| HN | Surgery, external beam radiation, locoregional therapy, or watchful waiting. If not possible, follow algorithm for malignant disease 5.3.1. | (41) | 2.2 | |
| Metastatic stage | Elevated plasma or urine normetanephrine and/or metanephrine | a. Palliative doxazosin 1–2 mg, increase 2–4 mg weekly. Balance maximum tolerated dosage to quality of life. | (191) | 3.1.1 |
| b. Before start of any treatment, doxazosin according to 1.1. | 3.1.2 | |||
| Confined disease | Surgery, external radiation, or locoregional therapy if safe and with acceptable morbidity. If not, proceed to 3.3.1. | 3.2 | ||
| Disseminated disease | Medical treatment to alleviate hormone or mass effect alternatively at disease progression. Perform 123I-MIBG scintigraphy and 68Ga-DOTATATE PET/CT. | 3.3 | ||
| First-line 131I-MIBG or 68Ga-DOTATATE positivea | 123I-MIBG = 68Ga-DOTATATE, choose 131I-MIBG. | 3.4.1 | ||
| 123I-MIBG > 68Ga-DOTATATE, choose 131I-MIBG. | 3.4.2 | |||
| 123I-MIGB < 68Ga-DOTATATE, choose 177Lu-DOTATATE. | 3.4.3 | |||
| Second-line or first-line 123I-MIBG/68Ga-DOTATATE negative | Priority I. Rechallenge 123I-MIBG or 68Ga-DOTATATE. | 3.5.1 | ||
| Priority II. CVD,a if WHO performance status >1 or wish for nonhospitalization, proceed to 3.5.3. | 3.5.2 | |||
| Priority III. Temozolomide, Tyrosine kinase inhibitor or experimental therapy. | (213, 214) | 3.5.3 |
a
CVD chemotherapy may be considered as first-line therapy in patients where the investigator considers the disease as rapidly progressing; recommendations are based on own experience with PPGL.