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Hereditäre Schilddrüsenkarzinome

Hereditary thyroid cancer

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Zusammenfassung

Hereditäre Schilddrüsenkarzinome werden in ca. 5% der differenzierten (DTC) und 25% der medullären Schilddrüsenkarzinome nachgewiesen. Sie kommen sowohl als Organkrebs innerhalb eines Tumorsyndroms vor (z. B. differenzierte Karzinome bei FAP-Gardner-Syndrom, medulläre Karzinome beim MEN2-Syndrom), aber auch als alleiniger familiärer Organtumor. Unabhängig von der Manifestationsform treten hereditäre Schilddrüsenkarzinome häufig bei jüngeren Patienten und multifokal auf. Das Resektionsausmaß muss aufgrund der vorliegenden Keimbahnmutationen grundsätzlich in der Entfernung der gesamten Schilddrüse bestehen. Teilentfernungen der Schilddrüse, wie sie für frühe Tumorstadien der sporadischen Tumoren ausreichend sein können, sind daher unabhängig vom Tumorstadium beim hereditären Schilddrüsenkrebs nicht indiziert.

Bei den meisten hereditären differenzierten Schilddrüsenkarzinomen ist die genetische Disposition unbekannt. Somit sind ein molekulargenetisches Screening und eine DNA-basierte prophylaktische Chirurgie nicht möglich. Auch bei den syndromal assoziierten DTC (FAP-Gardner-Syndrom, Cowden-Syndrom etc.) ist eine prophylaktische Chirurgie nicht begründet, da deren Prognose sich nicht von der sporadischer DTC unterscheidet.

Beim hereditären medullären Karzinom ist die genetische Ursache der Punktmutationen im RET-Protoonkogen seit 15 Jahren bekannt. Es werden jedoch stets neue krankheitsauslösende Mutationen des RET-Protoonkogens berichtet, so dass eine abschließende Wertung aller bestehenden Mutationen derzeit nicht möglich ist. Grundsätzlich besteht eine Genotyp-Phänotyp-Korrelation mit mutationsspezifischen Risikogruppen (Risikogruppe 1–3), die eine mutationsorientierte prophylaktische Thyreoidektomie möglich machen. Aufgrund der erheblichen Variationen innerhalb der einzelnen Risikogruppen ist jedoch ein streng mutationsspezifisches Vorgehen hinsichtlich Operationszeitpunkt und -ausmaß (ohne oder mit Lymphknotendissektion) nicht möglich, so dass einem kombiniert molekulargenetisch-biochemischen Konzept unter gleichzeitiger Berücksichtigung der Ergebnisse des Pentagastrinstimulationstestes der Vorzug zu geben ist.

Abstract

Hereditary thyroid carcinomas are present in about 5% of differentiated (DTC) and 25% of medullary thyroid carcinomas (MTC). They are part of a multiorgan tumour syndrome (e. g. FAP Gardner’s syndrome with DTC and MEN 2 syndrome with MTC) or confined to the thyroid gland. Hereditary thyroid carcinomas typically show multifocal growth and occur in young patients. Due to germ cell mutations as the underlying cause of disease, partial thyroidectomies that may be justified in early sporadic carcinomas are not indicated in this type of tumours. In the case of hereditary DTC, the genetic basis of the disease has been demonstrated only in syndromatic tumour variants. In most nonsyndromatic cases, specific genetic alterations have not yet been identified. In both types of hereditary DTC, prophylactic thyroidectomy is not warranted due to the favourable prognosis of tumours that do not differ from sporadic ones. Point mutations of the RET proto-oncogene have been known for 15 years to be the genetic basis of hereditary MTC. Recently several new mutations were discovered; however, final conclusions regarding their clinical significance are not possible at present. Basically it has been shown that the clinical aggressivity of tumour development follows a genotype-phenotype correlation (risk groups 1–3). However, in mutations of all risk classes there exists a wide spectrum of different stages of hereditary C-cell disease in individual risk groups. Regarding time and extent of prophylactic thyroidectomy (without or with lymph node dissection) a combined molecular-biochemical concept including the use of pentagastrin-stimulated calcitonin values is therefore recommended.

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Literatur

  1. Ahmed SA, Snow-Balley K, Highsmith WE et al. (2005) Nine novel germline gene variants in the RET proto-oncogene identified in twelve unrelated cases. J Mol Diagn 7: 283–288

    PubMed  CAS  Google Scholar 

  2. Alsanea O, Wada N, Ain K et al. (2000) Is familial non-medullary thyroid carcinoma more aggressive than sporadic thyroid cancer? A multicenter series. Surgery 128: 1043–1051

    Article  PubMed  CAS  Google Scholar 

  3. Bartsch DK, Hasse C, Schug C et al. (2000) A RET double mutation in the germline of a kindred with FMTC. Exp Clin Endocrinol Diabetes 108: 128–132

    Article  PubMed  CAS  Google Scholar 

  4. Baumgartner-Parzer SM, Lang R, Wagner L et al. (2005) Polymorphisms in exon 13 and intron 14 of the RET protooncogene: genetic modifiers of medullary thyorid carcinoma? J Clin Endocrinol Metab 90: 6232–6236

    Article  PubMed  CAS  Google Scholar 

  5. Bignell GR, Canzian F, Shayeghi M et al. (1997) Familial nontoxic multinodular thyroid goiter locus maps to chromosome 14q but does not account for familial nonmedullary thyroid cancer. Am J Hum Genet 61: 1123–1130

    Article  PubMed  CAS  Google Scholar 

  6. Brandi ML, Gagel RF, Angeli A et al. (2001) Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86: 5658–5671

    Article  PubMed  CAS  Google Scholar 

  7. Brauckhoff M, Gimm O, Weiss CL et al. (2004) Multiple endocrine neoplasia 2B syndrome due to codon 918 mutation: clinical manifestation and course in early and late onset disease. World J Surg 28: 1305–1311

    Article  PubMed  Google Scholar 

  8. Bülow C, Bülow S (1997) Is screening for thyroid carcinoma indicated in familial adenomatous polyposis? The Leeds Castle Polyposis Group. Int J Colorectal Dis 12: 240–242

    Article  PubMed  Google Scholar 

  9. Burgess JR, Duffield A, Wilkinson SJ et al. (1997) Two families with an autosomal dominant inheritance pattern for papillary carcinoma of the thyroid. J Clin Endocrinol Metab 82: 345–348

    Article  PubMed  CAS  Google Scholar 

  10. Canzian F, Amati P, Harach HR (1998) A gene predisposing to familial thyroid tumors with cell oxyphilia maps to chromosome 19p13.2. Am J Hum Genet 63: 1743–1748

    Article  PubMed  CAS  Google Scholar 

  11. Cavaco BM, Batista PF, Martins C et al. (2008) Familial non-medullary thyroid carcinoma (FNMTC): analysis of fPTC/PRN, NMTC1, MNG1 and TCO susceptibility loci and identification of somatic BRAF and RAS mutations. Endocr Relat Cancer 15: 207–215

    Article  PubMed  Google Scholar 

  12. Cetta F, Olschwang S, Petracci M et al. (1998) Genetic alterations in thyroid carcinoma associated with familial adenomatous polyposis: clinical implications and suggestions for early detection. World J Surg 22: 1231–1236

    Article  PubMed  CAS  Google Scholar 

  13. Chang TC, Wu SL, Hsiao YL (2005) Medullary thyroid carcinoma. Pitfalls in diagnosis by fine needle aspiration cytology and relationship of cytomorphology to RET proto-oncogene mutations. Acta Cytol 49: 477–482

    PubMed  Google Scholar 

  14. Charkes ND (1998) Letter to the editor: on the prevalence of familial nonmedullary thyroid cancer. Thyroid 8: 857–858

    PubMed  CAS  Google Scholar 

  15. Charkes ND (2006) On the prevalence of familial nonmedullary thyroid cancer in multiply affected kindreds. Thyroid 16: 181–186

    Article  PubMed  Google Scholar 

  16. Cohen R, Campos JM, Salaün C et al. (2000) Preoperative calcitonin levels are predictive of tumor size and postoperative calcitonin normalization in medullary thyroid carcinoma. J Clin Endocrinol Metab 86: 919–922

    Article  Google Scholar 

  17. Colombo-Benkmann M, Li Z, Riemann B et al. (2008) Characterization of the RET protooncogene transmembrane domain mutation S649L associated with nonaggressive medullary thyroid carcinoma. Eur J Endocrinol 158: 811–816

    Article  PubMed  CAS  Google Scholar 

  18. Da Silva AMA, Maciel RMB, Da Silva MRD et al. (2003) A novel germ-line point mutation in RET exon 8 (Gly533Cys) in a large kindred with familial medullary thyroid carcinoma. J Clin Endocrinol Metab 88: 5438–5443

    Article  CAS  Google Scholar 

  19. De Lellis, Wolfe HJ (1981) The pathobiology of the human calcitonin (C)-cell: a review. Pathol Annu 16: 25–52

    Google Scholar 

  20. Donis-Keller H, Dou S, Chi D et al. (1993) Mutations in the RET protooncogene are associated with MEN 2A and FMTC. Hum Mol Genet 2: 851–856

    Article  PubMed  CAS  Google Scholar 

  21. Dralle H, Scheumann GFW, Kotzerke J (1992) Surgical management of MEN 2. Rec Res Cancer Res 125: 167–195

    CAS  Google Scholar 

  22. Dralle H, Damm I, Scheumann GFW et al. (1994) Compartment-oriented microdissection of regional lymph nodes in medullary thyroid carcinoma. Surg Today 24: 112–121

    Article  PubMed  CAS  Google Scholar 

  23. Dralle H, Gimm O, Simon D et al. (1998) Prophylactic thyroidectomy in 75 children and adolescents with hereditary medullary thyroid carcinoma: German and Austrian experience. World J Surg 22: 744–751

    Article  PubMed  CAS  Google Scholar 

  24. Dralle H (2002) Lymph node dissection and medullary thyroid carcinoma. Br J Surg 89: 1073–1075

    Article  PubMed  CAS  Google Scholar 

  25. Dvorakova S, Vaclavikova E, Duskova J et al. (2005) Exon 5 of the RET proto-oncogene: a newly detected risk exon for familial medullary thyroid carcinoma, a novel germ-line mutation Gly321Arg. J Endocrinol Invest 28: 905–909

    PubMed  CAS  Google Scholar 

  26. Dvorakova S, Vaclavikova E, Ryska A et al. (2006) Double germline mutations in the RET proto-oncogene in MEN 2A and MEN 2B kindreds. Exp Clin Endocrinol Diabetes 114: 192–196

    Article  PubMed  CAS  Google Scholar 

  27. Eng C (2000) Editorial: familial papillary thyroid cancer – many syndromes, too many genes? J Clin Endocrinol Metab 85: 1755–1757

    Article  PubMed  CAS  Google Scholar 

  28. Evans DB, Fleming JB, Lee JE et al. (1999) The surgical treatment of medullary thyroid carcinoma. Semin Surg Oncol 16: 50–63

    Article  PubMed  CAS  Google Scholar 

  29. Frank-Raue K, Machens A, Scheuba C et al. (2008) Difference in development of medullary thyroid carcinoma among carriers of RET mutations in codons 790 and 791 Clin Endocrinol online publiziert: PMID 18248648

  30. Gimm O, Niederle BE, Weber T et al. (2002) RET proto-oncogene mutations affecting codon 790/791: a mild form of multiple endocrine neoplasia type 2A syndrome? Surgery 132: 952–959

    Article  PubMed  Google Scholar 

  31. Gimm O, Ukkat J, Niederle BE et al. (2004) Timing and extent of surgery in patients with familial medullary thyroid carcinoma/multiple endocrine neoplasia 2A-related RET mutations not affecting codon 634. World J Surg 28: 1312–1316

    Article  PubMed  Google Scholar 

  32. Gimm O, Heyn V, Krause U et al. (2006) Prognostic significance of disseminated tumor cells in the connective tissue of patients with medullary thyroid carcinoma. World J Surg 30: 1–6

    Article  Google Scholar 

  33. Giraudet AL, Ghulzan AA, Auperin A et al. (2008) Progression of medullary thyroid carcinoma: assessment with calcitonin and carcinoembryonic antigen doubling times. Eur J Endocrinol 158: 239–246

    Article  CAS  Google Scholar 

  34. Haggitt RC, Reid BJ (1986) Hereditary gastrointestinal polyposis syndromes. Am J Surg Pathol 10: 871–887

    Article  PubMed  CAS  Google Scholar 

  35. Harach HP (2001) Familial nonmedullary thyroid neoplasia. Endocr Pathol 12: 97–112

    Article  Google Scholar 

  36. Hazard JB, Hawk WA, Crile G jr (1959) Medullary (solid) carcinoma of the thyroid – a clinicopathologic entity. J Clin Endocrinol 19: 152–161

    CAS  Google Scholar 

  37. Hemmings CT (2003) Thyroid pathology in four patients with Cowden’s disease. Pathology 35: 311–314

    Article  PubMed  Google Scholar 

  38. Henry JF, Gramatica L, Denizot A et al. (1998) Morbidity of prophylactic lymph node dissection in the central neck area in patients with papillary thyroid carcinoma. Langenbecks Arch Surg 383: 167–169

    PubMed  CAS  Google Scholar 

  39. Jimenez C, Dang GT, Schultz PN et al. (2004) A novel point mutation of the RET protooncogene involving the second intracellular tyrosine kinase domain in a family with medullary thyroid carcinoma. J Clin Endocrinol Metab 89: 3521–3526

    Article  PubMed  CAS  Google Scholar 

  40. Kameyama K, Takami H, Miyajima K et al. (2001) Papillary carcinoma occurring within an adenomatous goiter of the thyroid gland in Cowden’s disease. Endocrine Pathol 12: 73–76

    Article  CAS  Google Scholar 

  41. Kouvaraki MA, Shapiro SE, Perrier ND et al. (2005) RET proto-oncogene: a review and update of genotype-phenotype correlations in hereditary medullary thyroid cancer and associated endocrine tumors. Thyroid 15: 531–544

    Article  PubMed  CAS  Google Scholar 

  42. Kraimps JL, Bouin-Pineau MH, Amati P et al. (1997) Familial papillary carcinoma of the thyroid. Surgery 121: 715 –718

    Article  PubMed  CAS  Google Scholar 

  43. Leboulleux S, Baudin E, Travagli JP et al. (2004) Medullary thyroid carcinoma. Clin Endocrinol 61: 299–310

    Article  Google Scholar 

  44. Lecube A, Hernandez C, Oriola J et al. (2002) V804 M RET mutation and familial medullary thyroid carcinoma: report of a large family with expression of the disease only in the homozygous gene carriers. Surgery 131: 509–514

    Article  PubMed  Google Scholar 

  45. Leprat F, Bonichon F, Guyot M et al. (1999) Familial non-medullary thyroid carcinoma: pathology review in 27 affected cases from 13 French families. Clin Endocrinol 50: 589–594

    Article  CAS  Google Scholar 

  46. Lesueur F, Stark M, Tocco T et al. (1999) Genetic heterogeneity in familial nonmedullary thyroid carcinoma: exclusion of linkage to RET, MNG1 and TCO in 56 families. J Clin Endocrinol Metab 84: 2157–2162

    Article  PubMed  CAS  Google Scholar 

  47. Liaw D, Marsh DJ, Dahia PLM et al. (1997) Germline mutations of the PTEN gene in Cowden disease, an inherited breast and thyroid cancer syndrome. Nat Gen 16: 64–67

    Article  CAS  Google Scholar 

  48. Lips CJM, Landsvater RM, Höppener JWM et al. (1994) Clinical screening as compared with DNA analysis in families with multiple endocrine neoplasia type 2a. N Engl J Med 331: 828–835

    Article  PubMed  CAS  Google Scholar 

  49. Loh KC (1997) Familial nonmedullary thyroid cancer: a meta-review of case series. Thyroid 7: 107–113

    PubMed  CAS  Google Scholar 

  50. Lombardo F, Baudin E, Chiefari E et al. (2002) Familial medullary thyroid carcinoma: clinical variability and low aggressiveness associated with RET mutation at codon 804. J Clin Endocrinol Metab 87: 1674–1680

    Article  PubMed  CAS  Google Scholar 

  51. Lupoli G, Vitale G, Caraglia M et al. (1999) Familial papillary thyroid microcarcinoma: a new clinical entity. Lancet 353: 637–639

    Article  PubMed  CAS  Google Scholar 

  52. Machens A, Haedecke J, Hinze R et al. (2000) Hypercalcitoninemia in a sporadic asymptomatic neuroendocrine tumor of the pancreatic tail. Dig Surg 17: 522–524

    Article  PubMed  CAS  Google Scholar 

  53. Machens A, Haedecke J, Holzhausen HJ et al. (2000) Differential diagnosis of calcitonin-secreting neuroendocrine carcinoma of the foregut by pentagastrin stimulation. Langenbecks Arch Surg 385: 398–401

    Article  PubMed  CAS  Google Scholar 

  54. Machens A, Gimm O, Ukkat J et al. (2000) Improved prediction of calcitonin normalization in medullary thyroid carcinoma patients by quantitative lymph node analysis. Cancer 88: 1909–1915

    Article  PubMed  CAS  Google Scholar 

  55. Machens A, Gimm O, Hinze R et al. (2001) Genotype-phenotype correlation in hereditary medullary thyroid carcinoma: oncological features and biochemical properties. J Clin Endocrinol Metab 86: 1104–1109

    Article  PubMed  CAS  Google Scholar 

  56. Machens A, Holzhausen HJ, Nguyen Thanh P et al. (2003) Malignant progression from C-cell hyperplasia to medullary thyroid carcinoma in 167 carriers of RET germline mutations. Surgery 134: 425–431

    Article  PubMed  Google Scholar 

  57. Machens A, Niccoli-Sire P, Hoegel J et al. (2003) Early malignant progression of hereditary medullary thyriod cancer. N Engl J Med 349: 1517–1527

    Article  PubMed  CAS  Google Scholar 

  58. Machens A, Schneyer U, Holzhausen HJ et al. (2004) Emergence of medullary thyroid carcinoma in a family with the Cys630Arg RET germline mutation. Surgery 136: 1083–1087

    Article  PubMed  Google Scholar 

  59. Machens A, Schneyer U, Holzhausen HJ et al. (2005) Prospects of remission in medullary thyroid carcinoma according to basal calcitonin level. J Clin Endocrinol Metab 90: 2029–2034

    Article  PubMed  CAS  Google Scholar 

  60. Machens A, Holzhausen HJ, Dralle H (2006) Contralateral cervical and mediastinal lymph node metastasis in medullary thyroid cancer: systemic disease? Surgery 139: 28–32

    Article  PubMed  Google Scholar 

  61. 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: 1960–1965

    Article  PubMed  Google Scholar 

  62. Machens A, Dralle H (2007) Genotype-phenotype based surgical concept of hereditary medullary thyroid carcinoma. World J Surg 31: 957–968

    Article  PubMed  Google Scholar 

  63. Machens A, Ukkat J, Hauptmann S et al. (2007) Abnormal carcinoembryonic antigen levels and medullary thyroid cancer progression. Arch Surg 142: 289–293

    Article  PubMed  CAS  Google Scholar 

  64. Machens A, Hauptmann S, Dralle H (2008) Prediction of lateral lymph node metastases in medullary thyroid cancer. Br J Surg 95: 586–591

    Article  PubMed  CAS  Google Scholar 

  65. Machens A, Hauptmann S, Dralle H (2008) Medullary thyroid cancer responsiveness to pentagastrin stimulation: an early surrogate parameter of tumor dissemination? J Clin Endocrinol Metab 93: 2234–2238

    Article  PubMed  CAS  Google Scholar 

  66. Magalhaes PKR, de Castro M, Elias LLK et al. (2004) Polymorphisms in the RET proto-oncogene and the phenotypic presentation of familial medullary thyroid carcinoma. Thyroid 14: 848–852

    PubMed  CAS  Google Scholar 

  67. Maxwell EL, Hall FT, Freeman JL (2004) Familial non-medullary thyroid cancer: a matched-case control study. Laryngoscope 114: 2182–2186

    Article  PubMed  Google Scholar 

  68. McKay JD, Lesueur F, Jonard L et al. (2001) Localization of a susceptibility gene for familial nonmedullary thyroid carcinoma to chromosome 2q21. Am J Hum Genet 69: 440–446

    Article  PubMed  CAS  Google Scholar 

  69. Miyauchi A, Matsuzuka F, Kuma K et al. (1988) Evaluation of surgical results and prediction of prognosis in patients with medullary thyroid carcinoma by analysis of serum calcitonin levels. World J Surg 12: 610–615

    Article  PubMed  CAS  Google Scholar 

  70. Moley JF (1999) DeBenedetti MK Patterns of nodal metastases in palpable medullary thyroid carcinoma. Ann Surg 229: 880–888

    Article  PubMed  CAS  Google Scholar 

  71. Moore FD (2006) Inherited aspects of papillary thyroid carcinoma. J Surg Oncol 94: 719–724

    Article  PubMed  Google Scholar 

  72. Mulligan LM, Kwok JBJ, Healy CS et al. (1993) Germline mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A (MEN 2A). Nature 363: 458–460

    Article  PubMed  CAS  Google Scholar 

  73. Musholt TJ, Musholt PB, Petrich T et al. (2000) Familial papillary thyroid carcinoma: genetics, criteria for diagnosis, clinical features and surgical treatment. World J Surg 24: 1409–1417

    Article  PubMed  CAS  Google Scholar 

  74. Niccoli-Sire P, Murat A, Rohmer V et al. (2001) Familial medullary thyroid carcinoma with noncysteine RET mutations: phenotype-genotype relationship in a large series of patients. J Clin Endocrinol Metab 86: 3746–3753

    Article  PubMed  CAS  Google Scholar 

  75. Ong SC, Schöder H, Patel SG et al. (2007) Diagnostic accuracy of 18F-FDG PET in restaging patients with medullary thyroid carcinoma and elevated calcitonin levels. J Nucl Med 48: 501–507

    Article  PubMed  CAS  Google Scholar 

  76. Pal T, Vogl FD, Chappuis PO et al. (2001) Increased risk for nonmedullary thyroid cancer in the first degree relatives of prevalent cases of nonmedullary thyroid cancer: a hospital-based study. J Clin Endocrinol Metab 86: 5307–5312

    Article  PubMed  CAS  Google Scholar 

  77. Ponder BAJ, Ponder MA, Coffey R et al. (1988) Risk estimation and screening in families of patients with medullary thyroid carcinoma. Lancet 1: 397–401

    Article  PubMed  CAS  Google Scholar 

  78. Roh JL, Park JY Park CII (2007) Total thyroidectomy plus neck dissection in differentiated papillary thyroid carcinoma patients: pattern of nodal metastasis, morbidity, recurrence and postoperative levels of serum parathyroid hormone. Ann Surg 245: 604–610

    Article  PubMed  Google Scholar 

  79. Scollo C, Baudin E, Travagli JP et al. (2003) Rationale for central and bilateral lymph node dissection in sporadic and hereditary medullary thyroid cancer. J Clin Endocrinol Metab 88: 2070–2075

    Article  PubMed  CAS  Google Scholar 

  80. Sippel RS, Caron NR, Clark OH (2007) An evidence-based approach to familial nonmedullary thyroid cancer: screening, clinical management and follow-up. World J Surg 31: 924–933

    Article  PubMed  Google Scholar 

  81. Skinner MA, Moley JA, Dilley WG (2005) Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A. N Engl J Med 353: 1105–1113

    Article  PubMed  CAS  Google Scholar 

  82. Sprengel O (1882) Mittheilungen über die in den Jahren 1874 bis 1878 auf der Volkmann’schen Klinik operativ behandelten 131 Fälle von Brustcarcinom. Langenbecks Arch Chir 27: 805–864

    Google Scholar 

  83. Starink TM (1984) Cowden’s disease: analysis of fourteen new cases. J Am Acad Dermatol 11: 1127–1141

    Article  PubMed  CAS  Google Scholar 

  84. Stoffer SS, Van Dyke DL, Bach JV et al. (1986) Familial papillary carcinoma of the thyroid. Am J Med Gen 25: 775–782

    Article  CAS  Google Scholar 

  85. Stratakis CA, Courcoutsakis NA, Abati A et al. (1997) Thyroid gland abnormalities in patients with the syndrome of spotty skin pigmentation, myxomas, endocrine overactivity and schwannomas (Carney complex). J Clin Endocrinol Metab 82: 2037–2043

    Article  PubMed  CAS  Google Scholar 

  86. Sturgeon C, Clark OH (2005) Familial nonmedullary thyroid cancer. Thyroid 15: 588–593

    Article  PubMed  Google Scholar 

  87. Sywak M, Cornford L, Roach P (2006) Routine ipsilateral level VI lymphadenectomy reduces postoperative thyroglobulin levels in papillary thyroid cancer. Surgery 140: 1000–1007

    Article  PubMed  Google Scholar 

  88. Tomoda C, Miyauchi A, Uruno T et al. (2004) Cribriform-morular variant of papillary thyroid carcinoma: clue to early detection of familial adenomatous polyposis-associated colon cancer. World J Surg 28: 886–889

    Article  PubMed  Google Scholar 

  89. Triponez F, Wong M, Sturgeon C et al. (2006) Does familial non-medullary thyroid cancer adversely affect survival? World J Surg 30: 787–793

    Article  PubMed  Google Scholar 

  90. Uchino S, Noguchi S, Kawamoto H et al. (2002) Familial nonmedullary thyroid carcinoma characterized by multifocality and a high recurrence rate in a large study population. World J Surg 26: 897–902

    Article  PubMed  Google Scholar 

  91. Ukkat J, Lorenz K, Hinze R et al. (2001) Importance of early screening and prophylactic thyroidectomy in asymptomatic nonindex RET germline carriers. World J Surg 25: 713–717

    Article  PubMed  CAS  Google Scholar 

  92. Vriens MR, Sabanci Ü, Epstein HD (1999) Reliablility of fine-needle aspiration in patients with familial nonmedullary thyroid cancer. Thyroid 9: 1011–1016

    Article  PubMed  CAS  Google Scholar 

  93. Wells SA, Chi DD, Toshima K et al. (1994) Predictive DNA testing and prophylactic thyroidectomy in patients at risk for multiple endocrine neoplasia type 2a. Ann Surg 220: 237–250

    Article  PubMed  Google Scholar 

  94. Williams ED, Brown CL, Doniach I (1996) Pathological and clinical findings in a series of 67 cases of medullary carcinoma of the thyroid. J Clin Pathol 19: 103–113

    Article  Google Scholar 

  95. Zbuk KM, Eng C (2007) Cancer phenomics: RET and PTEN as illustrative models. Nat Rev Cancer 7: 35–45

    Article  PubMed  CAS  Google Scholar 

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  1. Universitätsklinik für Allgemein-, Viszeral- und Gefäßchirurgie, Universitätsklinikum Halle, Medizinische Fakultät der Martin-Luther-Universität Halle-Wittenberg, Halle (Saale), Ernst-Grube-Straße 40, 06097, Halle, Deutschland

    H. Dralle, A. Machens & K. Lorenz

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  1. H. Dralle
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Dralle, H., Machens, A. & Lorenz, K. Hereditäre Schilddrüsenkarzinome. Chirurg 79, 1017–1028 (2008). https://doi.org/10.1007/s00104-008-1558-y

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