The good, the bad, and the ugly: uncommon CT appearances of pheochromocytoma

Berry, Renu; Busireddy, Kiran K.; Chu, Linda C.; Johnson, Pamela T.; Fishman, Elliot K.

doi:10.1007/s00261-022-03447-y

Cited by 2 publications

(1 citation statement)

References 32 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…PCC can be cystic and giant cystic as well. 83 While these findings can also be found in adrenal adenomas, a characteristic finding for PCC is unenhanced attenuation of >10 HU. 84 , 85 Due to the polymorphic and unspecific nature of adrenal incidentalomas, contrast enhancement does not seem to be distinguishing but can be localizing in 88–100% of cases.…”

Section: Imagingmentioning

confidence: 97%

Pheochromocytoma: a changing perspective and current concepts

Kiriakopoulos,

Giannakis,

Menenakos

2023

Therapeutic Advances in Endocrinology

View full text Add to dashboard Cite

This article aims to review current concepts in diagnosing and managing pheochromocytoma and paraganglioma (PPGL). Personalized genetic testing is vital, as 40–60% of tumors are linked to a known mutation. Tumor DNA should be sampled first. Next-generation sequencing is the best and most cost-effective choice and also helps with the expansion of current knowledge. Recent advancements have also led to the increased incorporation of regulatory RNA, metabolome markers, and the NETest in PPGL workup. PPGL presentation is highly volatile and nonspecific due to its multifactorial etiology. Symptoms mainly derive from catecholamine (CMN) excess or mass effect, primarily affecting the cardiovascular system. However, paroxysmal nature, hypertension, and the classic triad are no longer perceived as telltale signs. Identifying high-risk subjects and diagnosing patients at the correct time by using appropriate personalized methods are essential. Free plasma/urine catecholamine metabolites must be first-line examinations using liquid chromatography with tandem mass spectrometry as the gold standard analytical method. Reference intervals should be personalized according to demographics and comorbidity. The same applies to result interpretation. Threefold increase from the upper limit is highly suggestive of PPGL. Computed tomography (CT) is preferred for pheochromocytoma due to better cost-effectiveness and spatial resolution. Unenhanced attenuation of >10HU in non-contrast CT is indicative. The choice of extra-adrenal tumor imaging is based on location. Functional imaging with positron emission tomography/computed tomography and radionuclide administration improves diagnostic accuracy, especially in extra-adrenal/malignant or familial cases. Surgery is the mainstay treatment when feasible. Preoperative α-adrenergic blockade reduces surgical morbidity. Aggressive metastatic PPGL benefits from systemic chemotherapy, while milder cases can be managed with radionuclides. Short-term postoperative follow-up evaluates the adequacy of resection. Long-term follow-up assesses the risk of recurrence or metastasis. Asymptomatic carriers and their families can benefit from surveillance, with intervals depending on the specific gene mutation. Trials primarily focusing on targeted therapy and radionuclides are currently active. A multidisciplinary approach, correct timing, and personalization are key for successful PPGL management.

show abstract

Section: Imagingmentioning

confidence: 97%