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01.12.2014 | Research | Ausgabe 1/2014 Open Access

World Journal of Surgical Oncology 1/2014

Diagnosis and surgical treatment of multiple endocrine neoplasia type 2A

Zeitschrift:
World Journal of Surgical Oncology > Ausgabe 1/2014
Autoren:
Kun-Long Tang, Yi Lin, Li-Ming Li
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​1477-7819-12-8) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors' contributions

KLT designed the study and drafted the manuscript, YL and LML carried out the operation and collected the data. All authors read and approved the final manuscript.

Abstract

Background

This study aims to introduce the diagnosis and surgical treatment of the rare disease multiple endocrine neoplasia type 2A (MEN 2A).

Methods

Thirteen cases of MEN 2A were diagnosed as medullary thyroid carcinoma (MTC) and pheochromocytoma by biochemical tests and imaging examination. They were treated by bilateral adrenal tumor excision or laparoscopic surgery.

Results

Nine patients were treated by bilateral adrenal tumor excision and the remaining four were treated by laparoscopic surgery for pheochromocytoma. Ten patients were treated by total thyroidectomy and bilateral lymph nodes dissection and the remaining three were treated by unilateral thyroidectomy for MTC. Up to now, three patients have died of MTC distant metastasis.

Conclusions

We confirmed that MEN 2A can be diagnosed by biochemical tests and imaging examination when genetic testing is not available. Surgical excision is the predominant way to treat MEN 2A; pheochromocytoma should be excised at first when pheochromocytoma and MTC occur simultaneously.
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