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F. Di Gennaro
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P2.17 - Thymic Malignancies/Esophageal Cancer/Other Thoracic Malignancies (ID 718)
- Event: WCLC 2017
- Type: Poster Session with Presenters Present
- Track: Thymic Malignancies/Esophageal Cancer/Other Thoracic Malignancies
- Presentations: 1
- Moderators:
- Coordinates: 10/17/2017, 09:30 - 16:00, Exhibit Hall (Hall B + C)
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P2.17-002 - Pulmonary and Mediastinal Paragangliomas: Rare Endothoracic Malignancies with Challenging Diagnosis and Treatment (ID 9112)
09:30 - 09:30 | Author(s): F. Di Gennaro
- Abstract
Background:
Tumors originating from chromaffin cells are located in 90% of cases in the adrenal gland and called pheochromocytomas, while in 10% of cases have an extra-adrenal origin (paraganglionic cells scattered throughout the body) and are termed paragangliomas. Endothoracic paragangliomas can arise in the lung and mediastinum, may have neuroendocrine activity, secreting catecholamines, or be non-functional, incidental, in asymptomatic patients or causing mass effect symptoms. Even if rare and with low grade of malignancy, they can present an aggressive behaviour, developing local infiltration of surrounding organs and distant metastases. A correct pathological diagnosis and radical surgical treatment are fundamental to obtain clinical recovery. We report our experience with three cases of endothoracic paragangliomas, in order to point out difficulties in diagnosis and problems related to surgical treatment.
Method:
From January 2009 to December 2016, we treated 3 patients (2 women, 1 man), mean age 67 years, with histological diagnosis of paraganglioma: 2 pulmonary, 1 mediastinal. All patients were asymptomatic for catecholamine-secreting syndromes; the two cases with pulmonary localization showed no other symptoms, while the mediastinal one had cough and dyspnea, due to compression by the mass, and blepharospasm. Imaging diagnosis was based on chest CT scan in pulmonary cases, chest CT and MRI scan for the mediastinal mass, which allowed to define vascularization and relationships with adjacent structures. No patient had preoperative histological diagnosis. Intraoperative pathological examination of the two pulmonary forms was suggestive for malignancy (extensive necrosis, high proliferative index, hypervascularization), thus a right pulmonary wedge resection was performed in one case, a right upper lobectomy in the other one. The hypervascularized mediastinal lesion, located in the middle mediastinum, tenaciously adherent to the superior vena cava, the aortic arch, the right branch of the pulmonary artery and the trachea, was completely removed after challenging isolation and section of numerous vascular pedicles.
Result:
Postoperative course was uneventful in all cases. No patient received adjuvant treatments. At a median follow-up of 35 months (range: 6-90 months), two patients are alive, without local or distant recurrence; one patient (with pulmonary involvement) died 6 months after surgery, due to disease progression.
Conclusion:
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. After complete excision, long-term prognosis is generally good. However, even after surgical removal, a close and periodical follow-up is mandatory.
