Prophylactic thyroidectomy as method of medullary thyroid carcinoma prevention in children from MEN syndrom families

Cover Page

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription Access


The article presents results of diagnostics, surgical treatment and follow-up of patients with rare hereditary-conditioned forms of thyroid cancer – medullary thyroid carcinoma in content of multiple endocrine neoplasia syndrome. Particular attention is paid to the examination and tactics of treatment of children and adolescents with family genetically confirmed Sipple syndrome. The disease is diagnosed in 4 families. Syndrome of multiple endocrine neoplasia 2a type we found in 7 (0.024%) of 29,325 children and adult patients. All the children were from families in which one of the blood relatives suffered medullary thyroid cancer. The family nature of the disease was confirmed by molecular genetic studies that revealed mutations in C634 (T1900C) in the 11 exon of the RET gene. Only in 3 out of 7 cases thyroidectomy was prophylactic. Four children were fond foci of medullary carcinoma in the removed thyroid gland. In total, 22 operations were performed for the members of 4 families suffering from the family syndrome MEN-2a. The article shows that if a patient is diagnosed Sippl's syndrome, all his blood relatives need to be checked for the mutation of the RET gene to identify familial medullary thyroid cancer, adenomas of parathyroid glands and pheochromocytomas. Early removal of the thyroid gland (in children under the age of 5 years) prevents medullary cancer, and timely diagnosis and adequate surgical removal of neoplasms of parathyroid glands and adrenal glands ensure recovery of the patients. Closest relatives should be checked for the level of calcium and calcitonin, catecholamines, vanillylmandelic acid and metanephrine, ACTH, cancer-embryonic antigen.

Full Text

Restricted Access

About the authors

Zoya S. Matveeva

St. Petersburg State Pediatric Medical University

Author for correspondence.

MD, PhD, Assistant Professor, Department of Hospital Surgery with Traumatology and Military Surgery Courses

Russian Federation, St. Petersburg

Anatoliy F. Romanchishen

St. Petersburg State Pediatric Medical University


MD, PhD, Dr Med Sci, Professor, Head, Department of Hospital Surgery with Traumatology and Military Surgery Courses

Russian Federation, St. Petersburg

Alexandr V. Gostimsky

St. Petersburg State Pediatric Medical University


MD, PhD, Dr Med Sci, Professor, Head, Department of General Medical Practice

Russian Federation, St. Petersburg

Kristina V. Vabalayte

St. Petersburg State Pediatric Medical University


MD, PhD, Associate Professor, Department of Hospital Surgery with Traumatology and Military Surgery Courses

Russian Federation, St. Petersburg


  1. Багатурия Г.О., Романчишен А.Ф. Паллиативные операции при местнораспространенном раке щитовидной железы // Педиатр. – 2015. – Т. 6. – № 3. – С. 24–28. [Bagaturiya GO, Romanchishen AF. Palliative surgery in locally advanced thyroid cancer. Pediatr. 2015;6(3):24-28. (In Russ.)]
  2. Романчишен А.Ф., Томпсон Д. Сравнительные результаты лечения детей и подростков, страдавших раком щитовидной железы, в Центре эндокринной хирургии Санкт-Петербурга и в Клинике Мейо // Педиатр. – 2015. – Т. 6. – № 1. – С. 38–43. [Romanchishen AF, Thompson GB. Comparative results of treatment of children and adolescents with thyroid cancer at St. Petersburg Endocrine Surgery Centre and Mayo Clinic. Pediatr. 2015;6(1):38-43. (In Russ.)]
  3. Романчишен А.Ф., Гостимский А.В., Матвеева З.С., Передереев С.С. Хирургическое лечение детей с синдромом Сиппла. Материалы IX Российского форума с международным участием «Здоровье детей: профилактика и терапия социально значимых заболеваний. Санкт-Петербург-2015», 19–20 мая 2015 г. – СПб., 2015. – С. 107. [Romanchishen AF, Gostimsky AV, Matveeva ZS, Peredereev SS. Surgical treatment of children with Sippl’s syndrome. (Conference proceedings) IX Rossijskiy Forum s mezhdunarodnym uchastiem “Zdorov’e detej: profilaktika i terapiya social’no-znachimyh zabolevanij. Saint Petersburg-2015”; 2015 may 19-20. Saint Petersburg; 2015. P. 107. (In Russ.)]
  4. Романчишен А.Ф., Лисовский О.В., Матвеева З.С. Наш опыт лечения больных синдромами множественной эндокринной неоплазии (глава в монографии) // Хирургия щитовидной и околощитовидных желез. – СПб.: ИПК «Вести», 2009. – С. 466–469. [Romanchishen AF, Lisovsky OV, Matveeva ZS. Our experience of treatment of multiply endocrine neoplasia patients. In: Hirurgiya shchitovidnoj i okoloshchitovidnyh zhelez. Saint Petersburg: Vesti; 2009. P. 466-469. (In Russ.)]
  5. Романчишен А.Ф., Матвеева З.С. Особенности диагностики и лечения синдромов множественной эндокринной неоплазии // Голова и шея. – 2013. – № 3. – С. 14–17. [Romanchishen AF, Matveeva ZS. Specifics of multiple endocrine neoplasia syndrome diagnostics and treatment. Golova i sheya. 2013;(3):14-17. (In Russ.)]
  6. Романчишен А.Ф., Решетов И.В., Матвеева З.С., Вабалайте К.В. Семейные формы рака щитовидной железы // Голова и шея. – 2015. – № 3. – С. 43–46. [Romanchishen AF, Reshetov IV, Matveeva ZS, Vabalajte KV. Family forms of thyroid cancer. Golova i sheya. 2015;(3):43-46. (In Russ.)]
  7. Brandi ML, Gagel RF, Angeli A, et al. Consensus. Guidelines for diagnosis and therapy of MEN type 1 and type 2. Clin Endocrinol Metab. 2001;86(12):5658-71. doi: 10.1210/jcem.86.12.8070.
  8. Dralle H, Gimm O, Simon D, et al. Prophylactic thyroidectomy in 75 children and adolescents with hereditary medullary thyroid carcinoma: German and Austrian experience. World J Surg. 1998;22(7):744-750. doi: 10.1007/s002689900463.
  9. Hofstra RM, Landsvater RM, Ceccherini I, et al. A mutation in RET protooncogene associated with multiple neoplasia type 2B and sporadic medullary thyroid carcinoma. Nature. 1994;367:375-376. doi: 10.1038/367375a0.
  10. Mathew CG, Chin KS, Easton DF, et al. A linked genetic marker for multiple endocrine neoplasia type 2A on chromosome 10. Nature. 1987;328:527-528. doi: 10.1038/328527a0.
  11. Romanchishen AF, Kuzmichev AS, Matveewa ZS. Multiple endocrine neo-oplasia syndrome treatment – our experience. 2nd Italian-Russian Congress of Surgery and Gynecology. Perugia, Italy, 2011. P. 70.
  12. Szinnai G, Meier C, Komminoth P, et al. Review of multiple endocrine neoplasia type 2 A in children: therapeutic results of early thyroidectomy and prognostic value of codon analysis. Pediatrics. 2003;111(2):32-39. doi: 10.1542/peds.111.2.e132.

Supplementary files

There are no supplementary files to display.



Abstract: 1172

PDF (Russian): 161


Article Metrics

Metrics Loading ...


Copyright (c) 2017 Matveeva Z.S., Romanchishen A.F., Gostimsky A.V., Vabalayte K.V.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies