Multiple endocrine neoplasia type 1 surgery: Difference between revisions
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* The type of surgery for gastrinoma depends on many factors. Duodenectomy with intraoperative palpation and/or ultrasound to locate and excise duodenal tumors and peri-pancreatic lymph node dissection are performed. | * The type of surgery for gastrinoma depends on many factors. Duodenectomy with intraoperative palpation and/or ultrasound to locate and excise duodenal tumors and peri-pancreatic lymph node dissection are performed. | ||
* Glucagonomas, VIPomas, and somatostatinomas are treated with aggressive surgery.<ref name="pmid19836484">{{cite journal| author=Akerström G, Stålberg P| title=Surgical management of MEN-1 and -2: state of the art. | journal=Surg Clin North Am | year= 2009 | volume= 89 | issue= 5 | pages= 1047-68 | pmid=19836484 | doi=10.1016/j.suc.2009.06.016 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19836484 }} </ref> | * Glucagonomas, VIPomas, and somatostatinomas are treated with aggressive surgery.<ref name="pmid19836484">{{cite journal| author=Akerström G, Stålberg P| title=Surgical management of MEN-1 and -2: state of the art. | journal=Surg Clin North Am | year= 2009 | volume= 89 | issue= 5 | pages= 1047-68 | pmid=19836484 | doi=10.1016/j.suc.2009.06.016 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19836484 }} </ref> | ||
* Carcinoid tumor is treated with resection of the tumor. | |||
===Pituitary Tumor=== | ===Pituitary Tumor=== | ||
* Acromegaly is treated with transsphenoidal surgery. | * Acromegaly is treated with transsphenoidal surgery. | ||
Revision as of 15:04, 9 September 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
Surgery
- Surgical management of MEN1 is complex and controversial, given the multifocal and multiglandular nature of the disease and the high risk of tumor recurrence even after surgery. Establishing the diagnosis of MEN1 prior to making surgical decisions and referring affected individuals to a surgeon with experience in treating MEN1 can be critical in preventing unnecessary surgeries or inappropriate surgical approaches.
Parathyroid Tumors
- Once evidence of parathyroid disease is established biochemically, the recommended course of action is surgical removal of the parathyroid glands.
- However, the rate of recurrence is quite high (55%–66%), and reoperation is often necessary.
- Total parathyroidectomy with autotransplantation of parathyroid tissue to the forearm is also an option. A benefit of this approach is the easier removal of recurrent disease from the forearm than from the neck. Although the likelihood of recurrence is lowered by more extensive surgery, this must be weighed against the risk of rendering the patient hypoparathyroid.
Duodenopancreatic Neuroendocrine Tumors
- The role of surgery for pancreatic NETs in MEN1 is controversial, given postoperative morbidity, long-term complications, and low cure rates. The timing and extent of surgery depend on many factors, including severity of symptoms, extent of disease, type and location of tumor, risk of metastasis, and patient preference.
- The primary goal of surgery is to improve long-term survival by reducing symptoms associated with hormone excess and lowering the risk of distant metastasis.
- The mainstay of treatment for insulinoma is surgery. Surgery can range from enucleation of single or multiple large tumors to partial pancreatic resection, or both,[1] to subtotal or total pancreatectomy.
- The type of surgery for gastrinoma depends on many factors. Duodenectomy with intraoperative palpation and/or ultrasound to locate and excise duodenal tumors and peri-pancreatic lymph node dissection are performed.
- Glucagonomas, VIPomas, and somatostatinomas are treated with aggressive surgery.[2]
- Carcinoid tumor is treated with resection of the tumor.
Pituitary Tumor
- Acromegaly is treated with transsphenoidal surgery.
References
- ↑ Crippa S, Zerbi A, Boninsegna L, Capitanio V, Partelli S, Balzano G; et al. (2012). "Surgical management of insulinomas: short- and long-term outcomes after enucleations and pancreatic resections". Arch Surg. 147 (3): 261–6. doi:10.1001/archsurg.2011.1843. PMID 22430908.
- ↑ Akerström G, Stålberg P (2009). "Surgical management of MEN-1 and -2: state of the art". Surg Clin North Am. 89 (5): 1047–68. doi:10.1016/j.suc.2009.06.016. PMID 19836484.