Multiple paragangliomas of head and neck associated with hepatic paraganglioma: a case reportReport as inadecuate




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BMC Medical Imaging

, 15:38

First Online: 25 September 2015Received: 27 March 2015Accepted: 18 September 2015

Abstract

BackgroundParagangliomas PGs are neuroendocrine tumors derived embryonically from the neural crest cells of the autonomic nervous system. Approximately 3 % of all paragangliomas occur in the head and neck area. Head and neck paragangliomas HNPGs are rare and highly vascularized tumors, the majority of which are benign. Multiple HNPGs with hepatic paraganglioma are exceedingly rare.

Case presentationWe report a 59-year-old male patient with a 40-year history of an enlarged mass at the right side of the neck and two months of epigastric discomfort. Neck physical examination revealed a 6 × 6 cm, ovoid, firm mass on the right side of the neck. A pre-contrast computed tomography CT scan of the head and neck revealed bilateral heterogeneous soft tissue masses at the bifurcation of the carotid artery with indistinct border, the size of which was 2.4 cm × 2.6 cm on the left and 5.4 cm × 4.3 cm on the right. The lesions were intensely and heterogeneously enhanced with the internal and external carotid arteries surrounded and pushed anteriorly after contrast administration. Magnetic resonance imaging MRI showed a hyperintense signal on T2 weighted images compared to the surrounding muscle tissue and an intense contrast enhancement on T1 weighted images. Digital subtraction angiography DSA exhibited a highly vascularized masses that occupied and deformed both sides of the carotid bifurcation. As for the hepatic mass, non-contrasted CT imaging of the upper abdomen showed a 6.1 cm × 5.5 cm × 5.8 cm low density mass in the liver with indistinct border. On late arterial phase, the mass showed slight enhancement with an enlarged hepatic artery pushed around the lesion. MR imaging of the lesion in the liver demonstrated low signal intensity on T1 weighted images but heterogeneous high signal intensity on T2 weighted images. On diffusion weighted images, the mass showed high signal intensity whereas low signal intensity was seen on the image of apparent diffusion coefficient ADC. Moreover, the contrast-enhanced MRI showed that the lesion was intensely but heterogeneously enhanced.

ConclusionMultiple HNPGs with hepatic paraganglioma are exceedingly rare. Advanced medical imaging modalities such as ultrasound US, CT, MR, DSA and I-metaiodobenzylguanidine I-MIBG are helpful in the evaluation of the patients with PGs. Increased awareness of their concomitant occurrence and familiarity with their characteristic features are critical for clinicians and radiologists to avoid diagnostic and therapeutic pitfalls and to facilitate the early diagnosis.

KeywordsParaganglioma Head and neck Liver Immunohistochemistry CT MR AbbreviationsPGsParaganliomas

HNPGsHead and neck paragangliomas

CBPGCarotid body paraganglioma

CTComputed tomography

MRMagnetic resonance imaging

USUltrasound

DSADigital subtraction angiography

I-MIBGI-metaiodobenzylguanidine

HCCHepatocellular carcinomas

CTAComputed tomographic angiography

MPRMultiple-planner reconstruction

ADCApparent diffusion coefficient

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Author: Zebin Xiao - Dejun She - Dairong Cao

Source: https://link.springer.com/



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