Rare Middle Mediastinal Paraganglioma Mimicking Metastatic Neuroendocrine Tumor

Mehta, Christopher K.; Gillespie, Colin T.; Lin, Xiaoqi; Yeldandi, Anjana V.; DeCamp, Malcolm M.; Bharat, Ankit

doi:10.1016/j.athoracsur.2014.09.068

Cited by 11 publications

(29 citation statements)

References 7 publications

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“…Paragangliomas of the middle mediastinum are generally non-functional and occur in older patients (as in our case), while those of the posterior mediastinum are usually functional and affect younger people (6,12,16).…”

Section: Discussionmentioning

confidence: 57%

“…Paragangliomas are rare tumors (affecting 2 to 5 people per million per year) (10) with low grade of malignancy and non-epithelial origin, that are thought to arise from neural crest progenitor cells of the autonomic nervous system in extra-adrenal chromaffin tissues (1,2,6,12). Endothoracic paragangliomas can grow in the lung or in the mediastinum.…”

Section: Discussionmentioning

confidence: 99%

“…However, functional middle mediastinum paragangliomas have been described (12,17), as well as non-functional posterior mediastinum ones (18) and even asymptomatic but functional paragangliomas of the posterior mediastinum (14,19).…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary and mediastinal paragangliomas: rare endothoracic malignancies with challenging diagnosis and treatment

Palma¹,

Lorusso²,

Gennaro³

et al. 2018

J. Thorac. Dis

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Background: Pulmonary and mediastinal paragangliomas are rare tumors that may have neuroendocrine activity or be non-functional, incidental, in asymptomatic patients, or causing mass effect symptoms. Although being low-grade tumors, they can display an aggressive behaviour, developing local infiltration and distant metastases. We report our experience with three endothoracic paragangliomas and a Literature review, to point out diagnostic difficulties and problems related to surgical treatment. Methods: From 2009 to 2017, we treated 3 patients with histological diagnosis of paraganglioma: 2 pulmonary, 1 mediastinal. No one presented catecholamine-secreting syndromes; pulmonary cases were asymptomatic, while the mediastinal one had aspecific cough and dyspnea. Imaging diagnosis was based on chest computerized tomography (CT) and magnetic resonance imaging (MRI) scan. No patient had preoperative histological diagnosis. Intraoperative pathological examination was suggestive for malignancy: in pulmonary cases, wedge resection and lobectomy were performed; the middle mediastinal mass was completely removed after challenging dissection, isolation and section of numerous vascular pedicles. Results: Postoperative course was uneventful in all cases. No patient received adjuvant treatments. At a median follow-up of 47 months (range, 6-102 months), two patients are alive, without local or distant recurrence; one patient died 6 months after surgery, due to disease progression. Conclusions: Endothoracic paragangliomas, rare and often asymptomatic tumors, are of difficult diagnosis and should be considered malignant tumors, due to the potential aggressive behaviour of cases with high mitotic index and the frequent possibility of recurrence and metastases. Surgical resection is the treatment of choice and careful intraoperative manipulation is recommended, due to the high vascularity of these tumors, to prevent complications. After complete excision, long-term prognosis is generally good. However, even after surgical removal, a close, periodical and lifelong follow-up is mandatory.

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Section: Discussionmentioning

confidence: 57%

Section: Discussionmentioning

confidence: 99%

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Pulmonary and mediastinal paragangliomas: rare endothoracic malignancies with challenging diagnosis and treatment

Palma¹,

Lorusso²,

Gennaro³

et al. 2018

J. Thorac. Dis

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“…Its low-frequency forces to establish a differential diagnosis with other pathologies such as thymoma, thymic carcinoma, metastases or angiosarcoma. However, these pathologies do not express somatostatin receptors, so 111 In-Pentetreotide scintigraphy exclude them [5,6,7]. The first line diagnosis of paraganglioma is the study of biochemical parameters and structural imaging tests (CT, MRI).…”

Section: Discussionmentioning

confidence: 99%

Paraganglioma mediastínico detectado con gammagrafía SPECT-TC con 111In-pentetreótida. Descripción de un caso

et al. 2017

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SUMMARYIntroduction: Mediastinal paragangliomas are low incidence tumors that arise from neural crest. Its diferential diagnosis include several malignant diseases, so its correct characterization is mandatory for an adecuate therapeutic management. 111In-Pentetreotide scintigraphy/SPECT-CT can detect paraganglioma, mainly in those cases of atypical or unsuspected location, and may have a significant role in follow-up of those patients diagnosed with multifocal and familiar paraganglioma. Case: Here we report a case of an extremely rare middle mediastinal paraganglioma, detected by scintigraphy and SPECT-CT with 111 In-Pentetreotide in a patient diagnosed of multifocal and family history of paraganglioma. Surgery was carried out by median sternotomy and extracorporeal circulation. The pathological examination of the surgical specimen showed a para-aortic low-grade tumor, positive for chromogranin and synaptophysin, cytokeratin AE1-AE3 negative and Ki67 lower than 5%, compatible with paraganglioma. Currently, the patient is tumor free, under clinical monitoring. Conclusions: 111In-Pentetreotide scan proved to be a helpful diagnostic method because of its potential to explore full body, so It allows us to locate unsuspected and atypical location paragangliomas. This finding suggests that patients diagnosed with multifocal head and neck paraganglioma should undergo periodical follow-up with 111In-Pentetreotide scan to detect unsuspected paraganglioma. KEYWORDS mediastinal paraganglioma; 111In-Pentetreotide scintigraphy; chemodectomas RESUMEN Introducción: Los paragangliomas mediastínicos son tumores de baja incidencia que surgen de la cresta neural. Su diagnóstico diferencial incluye varias enfermedades malignas, por lo que su correcta caracterización es obligatoria para un adecuado tratamiento. La gammagrafía

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“…Given the normal distribution of paraganglia associated with components of the autonomous nervous system, it should be no surprise that paragangliomas can occur in the gallbladder [ 11 , 12 ], and the liver [ 8 , 13 , 14 ], where they are likely to arise from small abdominal vagus nerve branches. Paraganglia are present and can give rise to paragangliomas in various sites including the orbit, mandible, paranasal sinuses and sellar region [ 8 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 ], adjacent to or within the thyroid gland [ 8 , 24 , 25 , 26 , 27 , 28 , 29 ], in the parathyroid [ 30 ], in the mediastinum [ 8 , 31 , 32 , 33 ], within the lungs and heart [ 8 , 34 , 35 , 36 , 37 , 38 ], gut [ 8 ], and pancreas [ 8 , 39 , 40 ] and in the mesentery [ 8 , 41 , 42 ].…”

Section: Distribution and Localization Of Paragangliomasmentioning

confidence: 99%

The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

Sylvia

Ezzat

Mete

2018

JCM

126

108

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Paragangliomas are neuroendocrine neoplasms, derived from paraganglia of the sympathetic and parasympathetic nervous systems. They are most commonly identified in the head and neck, being most frequent in the carotid body, followed by jugulotympanic paraganglia, vagal nerve and ganglion nodosum, as well as laryngeal paraganglia. Abdominal sites include the well-known urinary bladder tumors that originate in the Organ of Zuckerkandl. However, other unusual sites of origin include peri-adrenal, para-aortic, inter-aortocaval, and paracaval retroperitoneal sites, as well as tumors in organs where they may not be expected in the differential diagnosis of neuroendocrine neoplasms, such as thyroid, parathyroid, pituitary, gut, pancreas, liver, mesentery, lung, heart and mediastinum. The distinction of these lesions from epithelial neuroendocrine neoplasms is critical for several reasons. Firstly, the determination of clinical and biochemical features is different from that used for epithelial neuroendocrine tumors. Secondly, the genetic implications are different, since paragangliomas/pheochromocytomas have the highest rate of germline susceptibility at almost 40%. Finally, the characterization of metastatic disease is unique in these highly syndromic lesions. In this review, we summarize updated concepts by outlining the spectrum of anatomic locations of paragangliomas, the importance of morphology in establishing the correct diagnosis, the clinical implications for management, and the impact of genetics on the distinction between multifocal primary tumors compared with malignant disease.

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Rare Middle Mediastinal Paraganglioma Mimicking Metastatic Neuroendocrine Tumor

Cited by 11 publications

References 7 publications

Pulmonary and mediastinal paragangliomas: rare endothoracic malignancies with challenging diagnosis and treatment

Pulmonary and mediastinal paragangliomas: rare endothoracic malignancies with challenging diagnosis and treatment

Paraganglioma mediastínico detectado con gammagrafía SPECT-TC con 111In-pentetreótida. Descripción de un caso

The Diagnosis and Clinical Significance of Paragangliomas in Unusual Locations

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