The Role of 68Ga-DOTA-Octreotate PET/CT in Follow-Up of SDH-Associated Pheochromocytoma and Paraganglioma

J Clin Endocrinol Metab. 2019 Nov 1;104(11):5091-5099. doi: 10.1210/jc.2019-00018.

Abstract

Purpose: Germline succinate dehydrogenase (SDHx) mutation carriers, especially SDHB, are at increased risk for malignancy and require life-long surveillance. Current guidelines recommend periodic whole-body MRI imaging. We assessed the incremental value of 68Ga-DOTA-octreotate (GaTate) positron emission tomography (PET)/CT compared with conventional imaging in such patients.

Methods: SDHx mutation carriers who had GaTate PET/CT were retrospectively reviewed. Detection of lesions were compared with MRI or CT on a per-patient and per-lesion basis. Proof of lesions were based on histopathology or clinical/imaging follow-up.

Results: Twenty consecutive patients (median age, 46 years; 10 males) were reviewed. Fourteen patients had SDHB, four, SDHD, one SDHC, and one SDHA mutation. Fifteen had prior surgery and/or radiotherapy. Indications for PET/CT were as follows: 7 patients for surveillance for previously treated disease, 9 residual disease, 2 asymptomatic mutation carriers, and 2 for elevated catecholamines. Median time between modalities was 1.5 months.GaTate PET/CT had higher sensitivity and specificity than conventional imaging. On a per-patient basis: PET/CT sensitivity 100%, specificity 100%; MRI/CT 85% and 50%. Per-lesion basis: PET/CT sensitivity 100%, specificity 75%; MRI/CT 80% and 25%. PET/CT correctly identified additional small nodal and osseous lesions. MRI/CT had more false-positive findings. Change of management resulted in 40% (8/20 patients): 3 received localized treatment instead of observation, 1 changed to observation given extra disease detected, 4 with metastases had radionuclide therapy.

Conclusions: GaTate PET/CT provided incremental diagnostic information with consequent management impact in SDHx-pheochromocytoma and paraganglioma. Incorporating this modality as part of a surveillance program seems prudent. Further research is needed to define the optimal surveillance strategy including use of MRI.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adrenal Gland Neoplasms / diagnostic imaging*
  • Adult
  • Aged
  • Catecholamines / metabolism
  • False Positive Reactions
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Multimodal Imaging
  • Mutation
  • Neoplasm Metastasis / diagnostic imaging
  • Organometallic Compounds*
  • Paraganglioma / diagnostic imaging*
  • Paraganglioma / radiotherapy
  • Paraganglioma / surgery
  • Pheochromocytoma / diagnostic imaging*
  • Pheochromocytoma / radiotherapy
  • Pheochromocytoma / surgery
  • Positron Emission Tomography Computed Tomography / methods*
  • Radiopharmaceuticals*
  • Retrospective Studies
  • Sensitivity and Specificity
  • Treatment Outcome
  • Young Adult

Substances

  • Catecholamines
  • Organometallic Compounds
  • Radiopharmaceuticals
  • gallium Ga 68 dotatate