Adrenal phaeochromocytoma: correlation of MRI appearances with histology and function

Jacques, Audrey; Sahdev, Anju; Sandrasagara, M.; Goldstein, Rick; Berney, Daniel M.; Rockall, Andrea; Chew, Shern L.; Reznek, Rodney H.

doi:10.1007/s00330-008-1073-z

Cited by 99 publications

(82 citation statements)

References 33 publications

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“…Patients with multiple endocrine neoplasia 2 and neurofibromatosis 1 mutations have been found to have an 'adrenergic' phenotype, whereas Von Hippel-Lindau (VHL), SDH-B, and SDH-D rarely show increased EPI/MN. If they do, the levels are proportionally smaller than the elevations in NE [37,42,43]. Furthermore, mutations especially of SDH-B have been found to exhibit a 'noradrenergic' and 'dopaminergic' phenotype [36,37].…”

Section: Biochemistrymentioning

confidence: 97%

Pheochromocytomas and paragangliomas: assessment of malignant potential

Korevaar

Grossman

2011

Endocrine

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Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting tumors which arise from the adrenal glands or sympathetic neuronal tissue. Malignant transformation of these tumors occurs in a significant proportion and may therefore lower overall survival rates. In patients with PPGLs it is impossible to identify malignant disease without the presence of metastatic disease, something which can occur as long as 20 years after initial surgery. Early identification of malignant disease would necessitate a more aggressive treatment approach, something which may result in better disease outcome. We have therefore reviewed possible predictors of malignancy and current developments in order to help clinicians to swiftly assess malignant potential in patients with PPGLs. Currently, there is no absolute marker which can objectively reflect malignant potential. Tumor size is the most reliable predictor and should therefore be used as the baseline characteristic. The combination of various clinical markers (extra-adrenal disease and post-operative hypertension), biochemical markers (high dopamine, high norepinephrine and epinephrine to total catecholamine ratio) and/or histological markers (SNAIL, microRNAs and/or microarray results) can raise or lower the suspicion of malignancy. Furthermore, we discuss how clinical markers may affect biochemical results linked to malignancy, how biochemical results may distinguish hereditary syndromes, the role of imaging in determining malignant potential and tumor detection, and recent results of proposed histological markers.

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Section: Biochemistrymentioning

confidence: 97%

Pheochromocytomas and paragangliomas: assessment of malignant potential

Korevaar

Grossman

2011

Endocrine

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“…The high attenuation (40 HU) and well-circumscribed homogenous appearance seen on our patient's CT are similar to what would be expected for a classic pheochromocytoma, and therefore the level of suspicion for a CP or any malignant component was low [20][21][22]. It should be noted, though, that radiologic features are neither specific nor diagnostic for CP [23].…”

Section: Discussionmentioning

confidence: 63%

An Unusual Case of a Composite Pheochromocytoma With Neuroblastoma

Steen

2014

J Endocrinol Metab

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Composite pheochromocytoma (CP) is a rare tumor of the adrenal medulla, consisting of neuroendocrine and neural components. Despite similar neural crest origins, pheochromocytomas and neurogenic tumors are distinct entities. Symptoms may arise from hypersecretion of hormones by either component; however, not all patients present with classic symptoms. Moreover, various medical conditions and substances can confound screening tests and complicate the diagnosis. The patient, a 46-year-old male, was seen in the endocrine clinic regarding a 2-year history of paroxysmal headaches, palpitations and diaphoresis. His medical history was significant for hypothyroidism and substance misuse (nicotine, marijuana and cocaine). Family history was negative for pheochromocytoma, hyperparathyroidism or thyroid malignancies. He was found to be hypertensive in clinic, and two cafe-au-lait spots and bilateral axillary freckles were noted on dermatological examination. Pheochromocytoma was suspected and investigations revealed 24-h urinary catecholamines, metanephrines and vanillylmandelic acid levels that were substantially elevated. He was asked to repeat the urine collection while abstaining from cocaine, given the potential confounding with cocaine-induced elevations of catecholamines and their metabolites; however, the patient did not comply with this. His plasma free metanephrines and chromogranin A were later found to be elevated. Abdominal computed tomography revealed a 5.8 × 5 cm mass in the left adrenal gland, with an attenuation of 40 Hounsfield units. He underwent an uncomplicated laparoscopic left adrenalectomy, after which his blood pressure normalized. Histological features of the tumor revealed findings consistent with a typical pheochromocytoma with neuroblastomatous infiltrates comprising < 5% of the tumor. The mitosis karyorrhexis index was low and there was no ganglioneuromatous infiltrate evident. Medical oncology was consulted in view of the histopathological findings, and no adjuvant therapy was recommended. Unfortunately, he did not fully comply with repeat imaging or biochemical evaluation. Six months postoperatively, the patient remains completely asymptomatic and normotensive. Cocaine-induced catecholamine effects can clinically mimic pheochromocytoma, thereby complicating the diagnosis. In addition, the biologic behavior of CP with neuroblastoma is uncertain and warrants oncologic assessment and surveillance.

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“…12.29a-e ). In a recent study of T2-weighted appearances of 44 adrenal pheochromocytomas, the "classical" homogeneous high signal intensity ("light-bulb," isointense to cerebrospinal fl uid, CSF) was only seen in 11 % [ 110 ]. Thirty-four percent appeared homogeneous, isointense, or minimally hyperintense to liver and spleen (hypointense to CSF), 16 % had a heterogeneous and marbled (swirl-like areas of mixed high and lower signal intensity) appearance, and 39 % appeared heterogeneous, with multiple high signal intensity pockets or cystic appearance.…”

Section: Imaging Featuresmentioning

confidence: 97%