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. 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.
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. [1] [2]
- Angiography and endovascular procedures, such as trans-arterial chemo-embolization (TACE), can be used to treat hepatic metastases.
Parathyroid Tumors
- Surgery is the mainstay of treatment for hyperparathyroidism.[3][4][1]
- Symptomatic hyperparathyroidism is an indication for surgery.[5]
Minimally invasive parathyroidectomy
- Most commonly done surgery for hyperparathyroidism is minimally invasive parathyroidectomy (MIP).[6]
- MIP is may done in loco-regional anesthesia or general anesthesia.
- Various techniques for MIP includes:[7]
- Open minimally-invasive parathyroidectomy (OMIP)[8]
- Minimally-invasive radio-guided parathyroidectomy (MI-RP)
- Endoscopic parathyroidectomy (EP)[9]
- Minimally invasive video-assisted parathyroidectomy (MIVAP)[10]
- Video-assisted parathyroidectomy through a lateral approach (VAP-LA)[11]
- Minimally-invasive radio-guided parathyroidectomy[12]
- Open minimally-invasive parathyroidectomy (OMIP) is the most commonly used minimally invasive parathyroidectomy.[13]
- MIP increases safety and cost-effectiveness in patients with preoperative localization of hyper-functioning parathyroid glands.
- Hyper-functioning parathyroid glands are excised and operative cure is confirmed by rapid intraoperative PTH assay.[14][15]
- MIP provides excellent post-operative cure rates comparable to bilateral neck exploration. This is due to precise pre-operative localization of hyper-functioning parathyroid gland and use of intra-operative parathyroid hormone (IOPTH) monitoring for predicting post-surgical success (post-operative normo-calcemia).[16][17]
Duodenopancreatic Neuroendocrine Tumors
Duodenopancreatic neuroendocrine tumors are treated as follows:[18][19]
- 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. [20] [21] [22]
- The type of surgery for gastrinoma depends on many factors. Duodenectomy with intra-operative 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 Adenoma
Endonasal transsphenoidal surgery
Most of the patients with acromegaly due to pituitary adenoma undergo transsphenoidal surgery. Although it is a challenging operation due to the anatomical location of the pituitary gland, the rate of complete successful resection in patients with adenomas smaller than 10 cm and GH level below 40ng is very high. It is important to remove the pituitary masses for this reasons:
- They may invade the cavernous sinus.
- They may be associated with microaneurysms.
MRI and CT imaging are used in guidance during the surgery and they have been linked with high safety and effectiveness of the surgery.[23]
In case the surgery is performed successfully, the acral features of acromegaly will improve within days.
Although it is very rare, some complications may occur. These complications include the following:[24][25]
- Local hemorrhage
- CSF leakage
- Diabetes insipidus
- Infection
- Meningitis
- Damage of the normal pituitary tissue
Post-operatively, somatostatin analogs and radiotherapy are recommended in case of remaining excess of growth hormone.
Indicators of successful surgery:[26]
- GH level falls to the normal level within few hours.
- IGF-1 falls to the normal level within few days.
A video showing the procedure of endo-nasal trans-sphenoidal surgery:{{#ev:youtube|v=xllKSOXDuNM|}}
Zollinger-Ellison syndrome
The feasibility of surgery depends on the stage of gastrinoma causing Zollinger-Ellison syndrome at the time of diagnosis. However, all patients diagnosed with Zollinger-Ellison syndrome with no metastasis should be offered surgical exploration and resection.
References
- ↑ 1.0 1.1 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.
- ↑ Powell AC, Alexander HR, Pingpank JF, Steinberg SM, Skarulis M, Bartlett DL; et al. (2008). "The utility of routine transcervical thymectomy for multiple endocrine neoplasia 1-related hyperparathyroidism". Surgery. 144 (6): 878–83, discussion 883-4. doi:10.1016/j.surg.2008.08.031. PMC 2625284. PMID 19040992.
- ↑ "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.
- ↑ Bilezikian JP, Brandi ML, Eastell R, Silverberg SJ, Udelsman R, Marcocci C; et al. (2014). "Guidelines for the management of asymptomatic primary hyperparathyroidism: summary statement from the Fourth International Workshop". J Clin Endocrinol Metab. 99 (10): 3561–9. doi:10.1210/jc.2014-1413. PMC 5393490. PMID 25162665.
- ↑ Miccoli, P.; Monchik, J. M. (2000). "Minimally invasive parathyroid surgery" (PDF). Surgical Endoscopy. 14 (11): 987–990. doi:10.1007/s004640000192. ISSN 0930-2794.
- ↑ Bellantone R, Raffaelli M, DE Crea C, Traini E, Lombardi CP (2011). "Minimally-invasive parathyroid surgery". Acta Otorhinolaryngol Ital. 31 (4): 207–15. PMC 3203720. PMID 22065831.
- ↑ Agarwal G, Barraclough BH, Reeve TS, Delbridge LW (2002). "Minimally invasive parathyroidectomy using the 'focused' lateral approach. II. Surgical technique". ANZ J Surg. 72 (2): 147–51. PMID 12074068.
- ↑ Naitoh T, Gagner M, Garcia-Ruiz A, Heniford BT (1998). "Endoscopic endocrine surgery in the neck. An initial report of endoscopic subtotal parathyroidectomy". Surg Endosc. 12 (3): 202–5, discussion 206. PMID 9502695.
- ↑ Miccoli P, Berti P, Conte M, Raffaelli M, Materazzi G (2000). "Minimally invasive video-assisted parathyroidectomy: lesson learned from 137 cases". J Am Coll Surg. 191 (6): 613–8. PMID 11129809.
- ↑ Henry JF, Defechereux T, Gramatica L, de Boissezon C (1999). "Minimally invasive videoscopic parathyroidectomy by lateral approach". Langenbecks Arch Surg. 384 (3): 298–301. PMID 10437620.
- ↑ Norman J, Chheda H, Farrell C (1998). "Minimally invasive parathyroidectomy for primary hyperparathyroidism: decreasing operative time and potential complications while improving cosmetic results". Am Surg. 64 (5): 391–5, discussion 395-6. PMID 9585770.
- ↑ Sackett WR, Barraclough B, Reeve TS, Delbridge LW (2002). "Worldwide trends in the surgical treatment of primary hyperparathyroidism in the era of minimally invasive parathyroidectomy". Arch Surg. 137 (9): 1055–9. PMID 12215160.
- ↑ Udelsman R, Pasieka JL, Sturgeon C, Young JE, Clark OH (2009). "Surgery for asymptomatic primary hyperparathyroidism: proceedings of the third international workshop". J Clin Endocrinol Metab. 94 (2): 366–72. doi:10.1210/jc.2008-1761. PMID 19193911.
- ↑ Fraker DL, Harsono H, Lewis R (2009). "Minimally invasive parathyroidectomy: benefits and requirements of localization, diagnosis, and intraoperative PTH monitoring. long-term results". World J Surg. 33 (11): 2256–65. doi:10.1007/s00268-009-0166-4. PMID 19763685.
- ↑ Carneiro DM, Irvin GL (2000). "Late parathyroid function after successful parathyroidectomy guided by intraoperative hormone assay (QPTH) compared with the standard bilateral neck exploration". Surgery. 128 (6): 925–9, discussion 935-6. doi:10.1067/msy.2000.109964. PMID 11114625.
- ↑ Irvin GL, Prudhomme DL, Deriso GT, Sfakianakis G, Chandarlapaty SK (1994). "A new approach to parathyroidectomy". Ann Surg. 219 (5): 574–9, discussion 579-81. PMC 1243192. PMID 8185406.
- ↑ "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.
- ↑ Tonelli F, Fratini G, Nesi G, Tommasi MS, Batignani G, Falchetti A; et al. (2006). "Pancreatectomy in multiple endocrine neoplasia type 1-related gastrinomas and pancreatic endocrine neoplasias". Ann Surg. 244 (1): 61–70. doi:10.1097/01.sla.0000218073.77254.62. PMC 1570585. PMID 16794390.
- ↑ Machado MC (2012). "Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1". Clinics (Sao Paulo). 67 Suppl 1: 145–8. PMC 3328825. PMID 22584720.
- ↑ Lasio G, Ferroli P, Felisati G, Broggi G (2002). "Image-guided endoscopic transnasal removal of recurrent pituitary adenomas". Neurosurgery. 51 (1): 132–6, discussion 136-7. PMID 12182410.
- ↑ Melmed S (2009). "Acromegaly pathogenesis and treatment". J Clin Invest. 119 (11): 3189–202. doi:10.1172/JCI39375. PMC 2769196. PMID 19884662.
- ↑ Cappabianca P, Cavallo LM, Colao A, de Divitiis E (2002). "Surgical complications associated with the endoscopic endonasal transsphenoidal approach for pituitary adenomas". J Neurosurg. 97 (2): 293–8. doi:10.3171/jns.2002.97.2.0293. PMID 12186456.
- ↑ Feelders RA, Bidlingmaier M, Strasburger CJ, Janssen JA, Uitterlinden P, Hofland LJ; et al. (2005). "Postoperative evaluation of patients with acromegaly: clinical significance and timing of oral glucose tolerance testing and measurement of (free) insulin-like growth factor I, acid-labile subunit, and growth hormone-binding protein levels". J Clin Endocrinol Metab. 90 (12): 6480–9. doi:10.1210/jc.2005-0901. PMID 16159936.