Multiple endocrine neoplasia type 1 surgery
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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 is the mainstay of treatment for multiple endocrine neoplasia type 1.
Surgery
- Surgical management of multiple endocrine neoplasia type 1 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 multiple endocrine neoplasia type 1 prior to making surgical decisions and referring affected individuals to a surgeon with experience in treating multiple endocrine neoplasia type 1 can be critical in preventing unnecessary surgeries or inappropriate surgical approaches.
- Prophylactic thymectomy can be recommended to prevent complications of multiple endocrine neoplasia syndrome.
- Angiography and endovascular procedures, such as trans-arterial chemo-embolization (TACE), can be used to treat hepatic metastases.
Parathyroid Tumors
- Parathyroid tumors are treated surgically as follows[1] [2] [3]
- 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
- Duodenopancreatic neuroendocrine tumors are treated as follows[4][5]
- The role of surgery for pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 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, to subtotal or total pancreatectomy. [6]
- The type of surgery for gastrinoma depends on many factors. Duodenectomy with intraoperative palpation and ultrasound to locate and excise duodenal tumors and peri-pancreatic lymph node dissection are performed.
- Glucagonomas, VIPomas, and somatostatinomas are treated with aggressive surgery.
- Carcinoid tumor is treated with resection of the tumor.
Pituitary Tumor
- Acromegaly is treated with transsphenoidal surgery.
References
- ↑ "Hyperparathyroidism in multiple endocrine neoplasia syndrome - Surgery".
- ↑ Tonelli F, Giudici F, Cavalli T, Brandi ML (2012). "Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy". Clinics (Sao Paulo). 67 Suppl 1: 155–60. PMC 3328832. PMID 22584722.
- ↑ Waldmann J, López CL, Langer P, Rothmund M, Bartsch DK (2010). "Surgery for multiple endocrine neoplasia type 1-associated primary hyperparathyroidism". Br J Surg. 97 (10): 1528–34. doi:10.1002/bjs.7154. PMID 20629112.
- ↑ "Surgery to Cure the Zollinger–Ellison Syndrome — NEJM".
- ↑ 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.
- ↑ 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.