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A case of cervico-mediastinal paraganglioma mimicking an ectopic goiter

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Abstract Introduction and importance Mediastinal paragangliomas are rare neuroendocrine tumors that originate from extra-adrenal paraganglia, occasionally secreting catecholamines. Nonfunctional mediastinal paragangliomas present nonspecific clinical and radiological features and represent a diagnostic challenge. Case presentation A 53-year old woman presented with cough and dyspnea increasing over time. CT-scan and ultrasonography showed a large vascularized cervico-mediastinal mass, consistent with an intrathoracic ectopic goiter. Preoperative angiography showed a blood supply from neck vessels. The lesion was completely removed through a cervical approach. The diagnosis of paraganglioma was a histological surprise. The patient is alive without recurrence 30 months after surgery. Clinical discussion When preoperatively diagnosed, the treatment of choice of a mediastinal paraganglioma is surgical excision. However, a preoperative diagnosis of mediastinal paraganglioma is difficult to obtain, especially in cases of nonfunctional lesions. Distinction between an intrathoracic goiter and a nonfunctional paraganglioma can be extremely difficult and, given the rarity of the latter, an ectopic goiter is suspected in first instance. CT-scan and ultrasonography are of little use in the differential diagnosis. However, scintigraphy with 123 I-metaiodobenzylguanidine can be an useful diagnostic tool when a paraganglioma is suspected. In case of vascularized cervico-mediastinal mass, such as paragangliomas or intrathoracic goiter, preoperative angiography should be performed to study the blood supply and orient the surgical approach. Conclusion Although uncommon, paragangliomas should be considered in the differential diagnosis of mediastinal masses, especially when an ectopic goiter is suspected. Highlights
Title: A case of cervico-mediastinal paraganglioma mimicking an ectopic goiter
Description:
Abstract Introduction and importance Mediastinal paragangliomas are rare neuroendocrine tumors that originate from extra-adrenal paraganglia, occasionally secreting catecholamines.
Nonfunctional mediastinal paragangliomas present nonspecific clinical and radiological features and represent a diagnostic challenge.
Case presentation A 53-year old woman presented with cough and dyspnea increasing over time.
CT-scan and ultrasonography showed a large vascularized cervico-mediastinal mass, consistent with an intrathoracic ectopic goiter.
Preoperative angiography showed a blood supply from neck vessels.
The lesion was completely removed through a cervical approach.
The diagnosis of paraganglioma was a histological surprise.
The patient is alive without recurrence 30 months after surgery.
Clinical discussion When preoperatively diagnosed, the treatment of choice of a mediastinal paraganglioma is surgical excision.
However, a preoperative diagnosis of mediastinal paraganglioma is difficult to obtain, especially in cases of nonfunctional lesions.
Distinction between an intrathoracic goiter and a nonfunctional paraganglioma can be extremely difficult and, given the rarity of the latter, an ectopic goiter is suspected in first instance.
CT-scan and ultrasonography are of little use in the differential diagnosis.
However, scintigraphy with 123 I-metaiodobenzylguanidine can be an useful diagnostic tool when a paraganglioma is suspected.
In case of vascularized cervico-mediastinal mass, such as paragangliomas or intrathoracic goiter, preoperative angiography should be performed to study the blood supply and orient the surgical approach.
Conclusion Although uncommon, paragangliomas should be considered in the differential diagnosis of mediastinal masses, especially when an ectopic goiter is suspected.
Highlights.

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