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METASTATIC PARAGANGLIOMA
Authored By: Bennett Greenspan and Archana Kantawala.
Patient: 29 year old female
History:

29 year old woman with a scalp mass.

She initially presented to her primary care physician with a small painless scalp mass 3 months prior to the imaging study.

She was given a course of antibiotics, for what was at that time thought to be a sebaceous cyst.

She was subsequently referred to a surgeon due to increasing size of the mass.

An outside hospital biopsy of the lesion was read as sarcoma and she was transferred to our institute for further care.

 She also had a history of hypertension.

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Multimedia: 242115_1_submitted.pptx
1.FDG PET- CT baseline and follow up after surgery. 2. Head CT. 3. Cerebral angiography. 4. Brain MRI.
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Findings:

PET-CT -There is a large extra cranial mass superior to the vertex that erodes the parietal bones and extends intracranially that displays increased FDG uptake with centrally decreased activity indicating central necrosis. There is another FDG-avid retroperitoneal mass measuring 4.0 x 3.6 cm with associated erosion of the S1 vertebral body. There is an area of mildly increased FDG uptake in the left infrapatellar fat.

IMPRESSION: 1. Increased uptake in known intracranial mass with a second distant lesion in the retroperitoneum eroding the S1 vertebral body.

2. Mildly increased FDG uptake in the left infrapatellar fat which may be due to recent trauma. Recommend correlation with trauma history.

Head CT- Large, permeative, destructive mass, centered in the skull apex, with intracranial, extradural extension that demonstrates post biopsy changes. No other mass is seen.

Cerebral angiography - 1. Large hypervascular extra-axial mass at the skull vertex which receives blood supply primarily from the right and left external carotid arteries. There is no significant blood supply to the mass via the internal carotid arteries.

2. Partial occlusion of the superior sagittal sinus with development of multiple venous collaterals along the anterior aspect of the superior sagittal sinus.

Brain/Brainstem MRI- 1. Large extra-axial paraganglioma centered at the skull apex, which demonstrates postbiopsy changes.

2. No additional mass lesions or areas of abnormal enhancement.

Follow up PET-CT - No residual disease.

DDx:

Malignant paraganglioma.

Lymphoma.

metastatic disease form a wide variety of primary tumors, for example- breast cancer.

Diagnosis:

Metastatic paraganglioma.

The patient underwent sucessive resections of her scalp lesion followed 2 months later by excision of the presacral mass.

At surgery, the presacral mass was determined to be paraganglioma arising in the organ of Zuckerkandl.

Surgical pathology of both lesions demonstrated paraganglioma.

 

General Discussion:

Paragangliomas are pheochromocytomas that arise from tissue of the extra-adrenal sympathetic nervous system.

They constitute about 10% of pheochromocytomas.

The signs and symptoms are related to overproduction of catecholamines.

About 10% of pheochromocytomas/paragangliomas are incidental findings on imaging done for atypical symptoms.

The skeletal sytem is the most common site for distant metastatic disease from a malignant paraganglioma.

References:

1. Ki Yeol Lee,Yu Whan Oh, Hyung Jun Noh, et al. Extra adrenal Paraganglioma of the Body:ImagingFeatures. AJR 2006;187:492-504.

2. Conor Heaney, Brian Mullan et al. F18 FDG PET/CT imaging of primary extra adrenal paragangliomas. J Nucl Med.2006;47(Supplement 1):442P.

3. David Taieb,Frederic Sebag et al. F18 FDG Avidity of Pheochromocytomas and Paragangliomas: A New Molecular Imaging Signature? J Nucl Med 2009;50:711 -717.

4. Barry L.DS Hulkin,Ioannis Ilias et al. Current trends in Functional Imaging of Pheochromocytomas and Paragangliomas.Ann. N.Y Acad.Sci 1073:374-382.

5. Linda D. Nosserman and Paul Fitzgerald. Malignant Paraganglioma.Commun Oncol 2004;1:47-52.  

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Additional Details:

Case Number: 242115Owner(s): Bennett Greenspan and Archana KantawalaLast Updated: 12-07-2011
Anatomy: Other   Pathology: Neoplasm
Modality: CT, MR, PETAccess Level: Readable by all users, writable by NucMed Certifiers
Keywords: paraganglionoma, ptnm

Case has been viewed 25 times.
Certified by Bennett Greenspan on 09-24-2011

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