Multiple endocrine neoplasia type 2 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 2. Management of multiple endocrine neoplasia type 2 patients includes thyroidectomy including cervical, central, and bilateral lymph nodes dissection for medullary thyroid carcinoma, unilateral adrenalectomy for unilateral pheochromocytoma or bilateral adrenalectomy when both glands are involved, and selective resection of pathologic parathyroid glands for primary hyperparathyroidism.
Surgery
Management of multiple endocrine neoplasia type 2 patients includes thyroidectomy including cervical, central, and bilateral lymph nodes dissection for medullary thyroid carcinoma, unilateral adrenalectomy for unilateral pheochromocytoma or bilateral adrenalectomy when both glands are involved, and selective resection of pathologic parathyroid glands for primary hyperparathyroidism.
Medullary Thyroid Cancer
Conventional Therapy
- The treatment of choice for primary medullary thyroid carcinoma, both sporadic or hereditary, is total thyroidectomy with systematic dissection of all lymph nodes of the central compartment. Total thyroidectomy is necessary as medullary thyroid carcinoma is multicentric in 65–90% of patients in multiple endocrine neoplasia type 2 and extensive central lymph node dissection has been reported to improve survival and recurrence rates compared to less aggressive procedures. Lymph node dissection of laterocervical compartments is not performed on principle but only when the neck ultrasound suggests the presence of metastatic nodes.[1][2][3]
- Endoscopic adrenal-sparing surgery has become the method of choice for the surgical therapy of pheochromocytoma.[4]
- Among patients with an asynchronous development of pheochromocytoma, the adrenal gland without pheochromocytoma can be preserved, but the patient must be aware that the probability to repeat the surgical treatment in the near future is very high. The advantage of a unilateral adrenal surgery is the possibility to avoid substitute therapy until the second surgery is performed.[4]
- The parathyroid glands are frequently found to be enlarged at the time of the thyroidectomy for medullary thyroid carcinoma and should, therefore, be carefully evaluated. The goal in multiple endocrine neoplasia type 2 patients with primary hyperparathyroidism (PHPT) is to excise the enlarged glands and to leave at least one apparently normal parathyroid gland intact. If all glands are enlarged, a subtotal parathyroidectomy or total parathyroidectomy with autotransplantation should be performed.
Prophylactic or Precocious Thyroidectomy in RET Gene Carrier
- Prophylactic thyroidectomy is advised in gene carriers to guarantee a definitive cure in these subjects.
- In 1999, during the Seventh International Multiple Endocrine Neoplasia Meeting in Gubbio, the risk of MTC has been stratified in three categories according to the mutations of c-RET as following:
| Gene | Risk | Treatment |
|---|---|---|
| Children with MEN2B and/or c-RET codon 883, 918,
922[5] |
Highest risk of aggressive medullary thyroid carcinoma | Total thyroidectomy with
central node dissection, within the first six months. |
| Children with any c-RET codon 611, 618, 620 or 634 | High risk of medullary thyroid carcinoma | Total thyroidectomy should be performed before age of
five years, with or without central node dissection. |
| Children with c-RET codon 609, 768, 790, 791, 804 | Less aggressive and slowly growing medullary thyroid carcinoma | Operated at a later stage |
- Recently, some evidences in big series of RET gene carriers demonstrated that gene carriers with undetectable levels of basal calcitonin (Ct) have an almost null risk to have already developed the medullary thyroid carcinoma.[8][9]
- Moreover, a serum Ct <30–40 pg/mL is always associated to an intrathyroidal micro-medullary thyroid carcinoma without any evidence of lymph node metastases. Moreover, a serum Ct <30–40 pg/mL is always associated to an intrathyroidal micro-medullary thyroid carcinoma without any evidence of lymph node metastases.
- The following flowchart depicts the surgical management of medullary thyroid cancer:
Post Surgery
- Thyroxine should be supplemented for patients undergoing total thyroidectomy.[10]
- Serum calcitonin and carcinoembryonic antigen doubling time (CEA DT) are measured during post surgical follow-up.
- Provacative pentagastrin or calcium test is administered and serum calcitonin level is measured.
- If there is no significant elevation in serum calcitonin level, serum calcitonin is measured every 6 months for 2-3 years and then yearly.
- If the calcitonin is below 150 pg/ml, ultrasound neck is recommended.
- If the basal serum calcitonin is above 150 pg/ml, screening for distant metastasis is recommended.
- The following flowchart depicts the post surgical management of medullary thyroid cancer:
References
- ↑ Machens A, Hauptmann S, Dralle H (2007). "Increased risk of lymph node metastasis in multifocal hereditary and sporadic medullary thyroid cancer". World J Surg. 31 (10): 1960–5. doi:10.1007/s00268-007-9185-1. PMID 17665245.
- ↑ Russell CF, Van Heerden JA, Sizemore GW, Edis AJ, Taylor WF, ReMine WH; et al. (1983). "The surgical management of medullary thyroid carcinoma". Ann Surg. 197 (1): 42–8. PMC 1352852. PMID 6128962.
- ↑ An, Changming; Zhang, Xiwei; Wang, Shixu; Zhang, Zongmin; Yin, Yulin; Xu, Zhengang; Tang, Pingzhang; Li, Zhengjiang (2017). "Efficacy of Superselective Neck Dissection in Detecting Metastasis in Patients with cN0 Papillary Thyroid Carcinoma at High Risk of Lateral Neck Metastasis". Medical Science Monitor. 23: 2118–2126. doi:10.12659/MSM.900273. ISSN 1643-3750.
- ↑ 4.0 4.1 Walz MK, Alesina PF (2009). "Single access retroperitoneoscopic adrenalectomy (SARA)--one step beyond in endocrine surgery". Langenbecks Arch Surg. 394 (3): 447–50. doi:10.1007/s00423-008-0418-z. PMID 18784938.
- ↑ Marini, Francesca; Falchetti, Alberto; Del Monte, Francesca; Carbonell Sala, Silvia; Tognarini, Isabella; Luzi, Ettore; Brandi, Maria (2006). Orphanet Journal of Rare Diseases. 1 (1): 45. doi:10.1186/1750-1172-1-45. ISSN 1750-1172. Missing or empty
|title=(help) - ↑ Wells, Samuel A.; Pacini, Furio; Robinson, Bruce G.; Santoro, Massimo (2013). "Multiple Endocrine Neoplasia Type 2 and Familial Medullary Thyroid Carcinoma: An Update". The Journal of Clinical Endocrinology & Metabolism. 98 (8): 3149–3164. doi:10.1210/jc.2013-1204. ISSN 0021-972X.
- ↑ İmge Aydoğan, Berna; Yüksel, Bağdagül; Tuna, Mazhar Müslüm; Navdar Başaran, Mehtap; Akkurt Kocaeli, Ayşen; Ertörer, Melek Eda; Aydın, Kadriye; Güldiken, Sibel; Şimşek, Yasin; Cihan Karaca, Züleyha; Yılmaz, Merve; Aktürk, Müjde; Anaforoğlu, İnan; Kebapçı, Nur; Duran, Cevdet; Taşlıpınar, Abdullah; Kulaksızoğlu, Mustafa; Gürsoy, Alptekin; Dağdelen, Selçuk; Erdoğan, Murat Faik (2016). "Distribution of RET Mutations and Evaluation of Treatment Approaches in Hereditary Medullary Thyroid Carcinoma in Turkey". Journal of Clinical Research in Pediatric Endocrinology. 8 (1): 13–20. doi:10.4274/jcrpe.2219. ISSN 1308-5727.
- ↑ Lau GS, Lang BH, Lo CY, Tso A, Garcia-Barcelo MM, Tam PK; et al. (2009). "Prophylactic thyroidectomy in ethnic Chinese patients with multiple endocrine neoplasia type 2A syndrome after the introduction of genetic testing". Hong Kong Med J. 15 (5): 326–31. PMID 19801688.
- ↑ Prognostic Factors of Disease-Free Survival after Thyroidectomy in 170 Young Patients with a RET Germline Mutation: A Multicenter Study of the Groupe Français d'Etude des Tumeurs Endocrines. Endocrine Society (30.09,2015)http://press.endocrine.org/doi/abs/10.1210/jc.2010-1234 accessed on October, 2015
- ↑ Pacini F, Castagna MG, Brilli L, Pentheroudakis G, ESMO Guidelines Working Group (2012). "Thyroid cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up". Ann Oncol. 23 Suppl 7: vii110–9. doi:10.1093/annonc/mds230. PMID 22997443.