Excision of A Rare Bilateral Malignant Carotid Paraganglioma: What We Have Done and What’s to Do
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2019, Vol 14, Issue 1
Abstract
Paragangliomas are non-epithelial neuroendocrine neoplasms. They are most commonly identified in the head and neck, most frequent in the carotid body, but only about 5% of carotid paragangliomas are bilateral. Surgery is an effective treatment although it may be difficult in patients with the advanced types. Here we present a rare case of bilateral malignant carotid paraganglioma in a female patient treated with two-step surgery and life-long surveillance.Paragangliomas (PGL) are non-epithelial neuroendocrine neoplasms (NEN) [1] that derive from paraganglia, which are seen in close association with components of the sympathetic and parasympathetic nervous systems [2,3]. They can arise in any location where paraganglia normally reside [4]. Because of widespread distribution of paraganglia, paragangliomas can occur at virtually all locations in the body except within the brain and in bone although they have been reported in the extremities [5]. They are most commonly identified in the head and neck, being most frequent in the carotid body, followed by jugulo tympanic paraganglia, vagal nerve and ganglium nodosum, as well as laryngeal paraganglia. Abdominal sites include the well-known urinary bladder neoplasms that originate in the Zuckerkandl organ. However, other unusual sites of origin include peri-adrenal, paraaortic, inter-aortocaval, and paracaval retroperitoneal sites, as well as neoplasms in organs where they may not be expected such as thyroid, parathyroid, pituitary, gut, pancreas, liver, mesentery, lung, heart and mediastinum. Carotid body neoplasms represent approximately 65% of all head and neck paragangliomas and only about 5% of carotid body neoplasm are bilateral. In the literature, most of the cases presented concern benign and unilateral neoplasms. We present here a rare case of bilateral malignant carotid paraganglioma in a female patient.A 40-years-old woman was admitted to the 2nd division of General Surgery Department of “A. Cardarelli” Hospital of Naples with a 15-years history of bilateral neck swelling and diagnosis of bilateral lymphadenopathy. She denied any history of similar swelling elsewhere in body. There was no history of similar swelling in any family members. There was no history of fever, weight loss or loss of appetite and no other constitutional symptoms were described such as episodes of palpitations or hypertension. Physical examination was negative except for bilateral neck masses with pulsation. The masses were firm with free mobility. Results of cranial nerve examinations were normal and the tests for pheochromocytoma were negative. Several ultrasound examinations had been repeated over the years always highlighting the presence of two irregular solid nodules of soft tissue density, measuring 3.7 cm and 1.7 cm located in the right and left carotid artery bifurcation, respectively. Additionally, RM examination, performed 2 years before the hospitalization, revealed heterogeneous reinforcement in the bilateral carotid sheath area, however the boundary between the masses and right and left carotid arteries was clear.
Authors and Affiliations
Grasso Marica, Costigliola Luciana, Ciorra Francesca Romana, De Sena Gabriele, Esposito MG, Puzziello Alessandro
Keywords
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- EP ID EP587604
- DOI 10.26717/BJSTR.2019.14.002483
- Views 152
- Downloads 0
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