Pheochromocytoma and paraganglioma: imaging characteristics

Abstract

The accurate diagnosis of adult pheochromocytoma and paraganglioma necessitates a multidisciplinary approach that includes clinical history, biochemical testing, and multimodality imaging such as computed tomography, magnetic resonance imaging, and nuclear medicine studies. This review illustrates the different imaging characteristics of primary adult pheochromocytomas as well as both sympathetic and parasympathetic paragangliomas. The review also describes known genetic associations and shows common metastatic patterns. Knowledge of the diverse appearance of pheochromocytomas and paragangliomas can result in early initial diagnosis or detection of disease recurrence thereby affecting patient management and prognosis.

Figure 1

A 41-year-old woman with a history of Von Hippel-Lindau and breast cancer presented with worsening abdominal and back pain. (a) An enhancing right adrenal mass is noted (black arrow) on the contrast-enhanced CT. Numerous cystic lesions arising from the tail of the pancreas are noted in addition to a microcystic serous cystadenoma (white arrow). (b) A heterogeneously enhancing left renal mass was proven on biopsy to represent a renal cell carcinoma (arrow). Numerous cysts are seen in the right kidney and pancreas consistent with known history of Von Hippel-Lindau (arrowhead). (c) [¹²³I]MIBG scan demonstrates intense radiotracer uptake in the right adrenal gland confirming the diagnosis of pheochromocytoma (arrow).

Figure 2

A 45-year-old man undergoing placement of an adjustable gastric band experienced retroperitoneal bleeding during the procedure secondary to an incidentally discovered mass. Postoperative tests showed increased levels of plasma metanephrine, 24-h urine catecholamines, vanillylmandelic acid, and metanephrines consistent with a diagnosis of pheochromocytoma. (a) Contrast-enhanced axial CT image of the abdomen demonstrates a complex left adrenal mass with thick enhancing walls. There is a central, high density component with fluid levels consistent with internal hemorrhage. (b) Coronal T2-weighted MR image demonstrates a heterogeneous adrenal mass with multiple internal foci of T2 prolongation with septation (arrow).

Figure 3

A 57-year-old woman initially presented with severe left hip and flank pain without a history of trauma. CT demonstrated a left adrenal mass (not available). (a) T2-weighted coronal MRI of the abdomen shows a heterogeneous mass with cystic components arising from the left adrenal gland (thick arrow). A liver cyst is incidentally noted (thin arrow). (b) Axial [¹⁸F]FDG-PET image demonstrates FDG uptake in the adrenal mass (arrow). (c) [¹⁸F]FDG-PET coronal maximal intensity projection (MIP) again demonstrates the FDG-avid primary left adrenal mass consistent with pheochromocytoma (arrow).

Figure 4

A 77-year-old man had an adrenal mass incidentally discovered on a restaging scan following resection of a gastrointestinal stromal tumor (GIST). (a) Coronal CT image with oral and intravenous contrast demonstrates a homogeneous, hypodense mass (solid arrow) adjacent to the surgical bed representing local recurrence of GIST. An enhancing retroperitoneal mass adjacent to the aorta corresponded to the location of the sympathetic ganglia and raised the possibility of paraganglioma (dotted arrow). (b) [¹²³I]MIBG scan demonstrates intense radiotracer uptake to the left of midline in the midabdomen corresponding to the previously noted paraganglioma (arrow). The upper abdominal GIST does not demonstrate any radiotracer uptake. Physiologic uptake of [¹²³I]MIBG is noted in the salivary glands, liver, bladder, and bowel. (c) Coronal [¹⁸F]FDG-PET MIP shows intense FDG uptake in both the GIST (dotted arrow) and pheochromocytoma (solid arrow) as both tumors are metabolically active. The combination of GIST and paraganglioma is diagnostic of Carney-Stratakis syndrome. Numerous other foci of FDG uptake within the liver and lymph nodes are consistent with metastatic disease.

Figure 5

A 35-year-old woman with a paternal family history of pheochromocytoma was initially diagnosed with paraganglioma at the base of the bladder causing left hydronephrosis and hypertension for which she underwent partial cystectomy and resection of the mass. (a) Axial T2-weighted MRI demonstrates heterogeneous, hyperintense signal at the base of the bladder to the left of midline (arrow) consistent with recurrence. (b) Coronal T2-weighted MRI demonstrates the same mass at the base of the bladder (thin arrow). At the level of the aortic bifurcation, a left of midline mass demonstrates intermediate signal (thick arrow) corresponding to an organ of Zuckerandl paraganglioma. An enlarged pelvic lymph node is seen on the left (dotted arrow).

Figure 6

A 51-year-old woman presented with a cough. Axial contrast-enhanced CT through the level of the aortic arch demonstrates an intensely enhancing mediastinal mass in the anterioposterior window (arrow). Surgical pathology confirmed the diagnosis of paraganglioma.

Figure 7

A 40-year-old woman recently postpartum presented with right-sided neck pain, minimally relieved with standing. (a) Coronal contrast-enhanced T1-weighted MRI demonstrates enhancing masses adjacent to the bilateral carotid arteries inferior to the jugular foramen on the right (dashed arrow) and at the level of the carotid bifurcation on the left (solid arrow). (b) [¹¹¹In]Octreotide scan shows radiotracer uptake in the bilateral neck masses confirming the diagnosis of paraganglioma.

Figure 8

A 61-year-old man with a history of treated right glomus jugulare and right glomus vagale tumors presented with progressive right facial droop, dysphagia, and right eyelid ptosis. (a) Axial contrast-enhanced T1-weighted MRI demonstrates an enhancing right jugular fossa mass involving the right petrous apex (white arrow) and clivus. The mass extends into the cavernous sinus. (b) Coronal [¹⁸F]FDG-PET MIP demonstrates FDG uptake within a right lower lobe mass (arrow). (c) [¹¹¹In]Octreotide scan demonstrates radiotracer uptake in the right lower lobe (white arrow) compatible with metastases. Uptake was also noted in the mediastinum and bilateral hila consistent with lymphadenopathy (black arrows).

Figure 9

A 70-year-old man with a history of diabetes and hypertension initially presented with upper abdominal pain. Coronal contrast enhanced CT 1 year after the initial presentation (baseline studies not available) of a paraganglioma demonstrates widely metastatic enhancing mediastinal (white arrow) and retroperitoneal (black arrow) lymph nodes. Some of the lymph nodes are centrally hypodense.

Figure 10

A 52-year-old woman initially presented with a sensation of mid-epigastric fullness. After initial imaging (not available), the patient underwent surgical excision of a 9-cm abdominal mass with the diagnosis of paraganglioma. (a) Postexcision coronal [¹⁸F]FDG-PET MIP demonstrates focal FDG uptake within the frontal bone, liver (solid arrow), femur (dashed arrow), and throughout mediastinal and retroperitoneal lymph nodes consistent with metastatic paraganglioma. (b) Fused axial [¹⁸F]FDG-PET/CT illustrates intensely FDG-avid retroperitoneal lymph nodes and a lumbar vertebral body lesion. (c) Fused coronal [¹⁸F]FDG-PET/CT demonstrates an intensely FDG-avid right femoral metastasis (arrow).

Figure 11

A 57-year-old man initially presented with episodic sweating and flushing secondary to a right adrenal pheochromocytoma that was resected. One year later, he noticed enlarged cervical lymph nodes that were found to be metastatic pheochromocytoma on biopsy. (a) Axial noncontrast CT image of the upper thorax demonstrates a left subpectoral mass that is slightly hypodense to muscle (arrow). (b) Axial noncontrast CT images show a right middle lobe lung nodule representing metastatic pheochromocytoma (arrow) in addition to bilateral pleural effusions.

Figure 12

A 47-year-old man initially presented with abdominal pain. (a) Axial CT scan with oral and intravenous contrast demonstrates an enhancing retroperitoneal mass with central necrosis (arrow). At pathologic examination, this was a paraganglioma. (b) [^99mTc]methylene diphosphonate bone scan performed 2 years after the initial CT demonstrates increased radiotracer uptake in the thoracolumbar spine (arrows). (c) Sagittal T1-weighted MRI with contrast obtained 2 years after the bone scan demonstrates diffuse bone marrow heterogeneity within the vertebral bodies consistent with metastases. An enhancing soft tissue component seen at the level of the T5 vertebral body extends into the right neural foramen and compresses the thecal sac (arrow).