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Endocrinology and metabolism
Published in Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan, Essential Notes for Medical and Surgical Finals, 2021
Kaji Sritharan, Jonathan Rohrer, Alexandra C Rankin, Sachi Sivananthan
A phaeochromocytoma is an adrenal medulla tumour. A paraganglioma is a tumour arising from the sympathetic and parasympathetic ganglia. » 25% occur due to mutations in the VHL, NF1, c-Ret, SDH-B, -C, -D genes and are associated with familial phaeo/paraganglioma syndromes. Clinical features: classical symptoms are headache, sweating and palpitations, which may be paroxysmal, occurring in attacks lasting minutes to hours; and hypertension which may be persistent rather than episodic.
Imaging of the Neck
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Paragangliomas are benign vascular tumours derived from primitive neural crest and occur anywhere along the carotid sheath as far as the skull base, the most common being the carotid body tumour (CBT).181 CBT occurs at the bifurcation of the common carotid artery with resultant characteristic splaying of the internal and external carotid arteries (Figure 37.42). As the tumour increases in size, it may encase the carotid vessels but does not narrow their calibre.182 Because of its site a CBT can be mistaken for an abnormal lymph node in the upper cervical chain. Other common paragangliomas are the glomus jugulare and glomus vagale arising from the jugular vein and vagus nerve respectively. The glomus vagale characteristically displaces the internal carotid artery anteriorly or medially and the jugular vein posteriorly. Paragangliomas may be familial and in such cases may be multiple and may very rarely show malignant transformation. On US paragangliomas do not appear particularly vascular despite showing avid enhancement on contrast-enhanced CT and MRI. They may show a characteristic ‘salt and pepper’ appearance on MRI (Figure 37.43) with the ‘pepper’ or low-signal appearance representing flow voids of feeding vessels.
Jugular Foramen Lesions and their Management
Published in John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed, Paediatrics, The Ear, Skull Base, 2018
The biological behaviour of paragangliomas is usually indolent: it can take many years or even decades for slight growth to become apparent. However, even without any substantial increase in the volume of the lesion, symptoms of progressive cranial nerve deficits may develop, presumably as the result of local erosion and compression by the tumour. In Figures 107.4 and 107.5, two illustrative cases are shown.
Metastatic brain carotid body paraganglioma with endocrine activity: a case report and literature review
Published in British Journal of Neurosurgery, 2019
Xiang Wang, Xianglan Zhu, Jinxiu Chen, Yanhui Liu, Qing Mao
The patient underwent a craniotomy, and a total resection of the tumor was performed (Figure 1(f)). The tumor was hard and solid, located on the left parietal lobe, and originated from the cerebral parenchyma with infiltration of the dura matter. The tumor was surrounded by multiple thickened blood vessels with some necrosis inside (Figure 1(g)). Histopathological examination revealed that the tumor tissue consisted of clusters of chief cells surrounded by sustentacular cells arranged in nest-like, trabecular, and adenoidal gland pattern, known as so-called “Zellballen” (Figure 1(h)). The chief cells had abundant pale cytoplasm and vesicular nuclei with conspicuous nucleoli, and some cells showed prominent atypia. Nuclear mitosis was easily detected, and the blood supply of the tumor was abundant (Figure 1(i)). The sustentacular cells tested locally weak-positive to S-100. The chief cells were positive for synaptophysin and chromogranin A, but negative for CK and GFAP (Figure 1(j,k)). Further, the positive percentage of MIB-1was around 15% (Figure 1(l)). The pathological diagnosis was paraganglioma.
The spectrum of SDHD mutations in Russian patients with head and neck paraganglioma
Published in International Journal of Neuroscience, 2018
Marina V. Shulskaya, Maria I. Shadrina, Natalia A. Bakilina, Svetlana V. Zolotova, Petr A. Slominsky
Head and neck paragangliomas (HNPGLs) are rare neoplasms representing about 0.03% of all human tumors [1]. These are hypervascular tumors that arise from neuroectodermal cells and are located mainly in the carotid bifurcation, as well as in the vagus nerve, the jugular foramen, or the middle ear [2]. This type of tumor is characterized by slow progression and rather mild clinical symptoms that occur predominantly in adulthood [3]. HNPGLs are the most common benign tumors of the middle ear [4] and account for 80–90% of jugular foramen lesions [5]. The rate of functioning HNPGLs is low: less than 3% of them are present with elevated catecholamines and subsequent tachycardia, tremor and hypertension [1,4,6]. Autosomal dominant and sporadic forms of paraganglioma are distinguished based on the nature of their family inheritance. To date, there is no clear picture of the frequency of the occurrence of the HNPGL familial form; according to various estimates, it ranges between 30% and 40% [7–9].
Para-aortic paraganglioma mimicking lymph node metastasis in an ovarian carcinoma: a case report
Published in Journal of Obstetrics and Gynaecology, 2018
Gozde Sahin, Ceyhan Ugurluoglu, Serra Akar, Ayhan Gul, Aysegul Kebapcilar, Cetin Celik
Paragangliomas are rare neuroendocrine tumours that arise from specialised paraganglion cells, which are spread over the entire body from the skull to the pelvic floor. Paragangliomas are known to arise in specific locations, such as the carotid body, jugular foramen, middle ear, aortico-pulmonary region, posterior mediastinum and abdominal para-aortic region, including Zuckerkandl’s body, or the aortic body (Feng et al. 2009). The adrenal medulla has the largest collection of receptor cells, which accounts for the high rate of tumour occurrence at this site. In contrast, extra-adrenal retroperitoneal paragangliomas arise from the symmetrically distributed paraganglia situated close to the aorta and sympathetic chain (Wen et al. 2010).