Medulläres Schilddrüsenkarzinom – Besonderheiten, Diagnostik, Therapie und Nachsorge

Das medulläre Schilddrüsenkarzinom (MTC) ist ein seltener maligner Tumor der Schilddrüse, es macht vermutlich ca. 3–8 % aller Schilddrüsenkarzinome aus. Im Gegensatz zu dem Großteil der Schilddrüsenkarzinome, welche von den Thyreozyten ausgehen, hat das MTC seinen Ursprung in den parafollikulären C-Zellen. Diese synthetisieren u.a. Kalzitonin, das als sehr sensitiver Tumormarker gilt. Das MTC ist der sonst postoperativ üblichen konventionellen Radiojodtherapie nicht zugänglich, weil die C-Zellen im Gegensatz zu den Thyreozyten kein Jod aufnehmen. Dies stellt zusätzliche Anforderungen an die chirurgische Therapie. Gegenstand aktuell intensiver Forschung gilt der Behandlung von MTCs, welche auf Grund von Inoperabilität bzw. dem Vorhandensein von disseminierten Fernmetastasen einer chirurgischen Therapie nicht zugänglich sind.

Medullary thyroid carcinoma (MTC) is a rare malignant tumor of the thyroid gland, it probably accounts for about 3 – 8 % of all thyroids cancers. In contrast to the majority of thyroid cancers that develop from thyreocytes, MTC has its origins in parafollicular C cells. Among others, these cells synthesize calcitonin which thus represents a very sensitive tumor marker. MTC is not responsive to the usually employed conventional radio-iodine therapy because C cells, in contrast to threocytes, do not take up iodine. This fact places additional demands on surgical treatment. The management of MTCs that are not suitable for operative treatment because of their inoperable nature or, respectively, due to the presence of distant disseminated metastases is a hot topic of current intensive research

Key words

Thyroid cancer - medullary - C cells - therapy - follow-up

• Literatur

• 1 Marsh DJ, Gimm O. Multiple endocrine neoplasia: types 1 and 2. Adv Otorhinolaryngol 2011; 70: 84-90
• 2 Brauckhoff M, Machens A, Hess S, Lorenz K, Gimm O, Brauckhoff K, Sekulla C, Dralle H. Premonitory symptoms preceding metastatic medullary thyroid cancer in MEN 2B: An exploratory analysis. Surgery discussion 2008; 144: 1050-1043
• 3 Musholt TJ, Clerici T, Dralle H et al. German Association of Endocrine Surgeons practice guidelines for the surgical treatment of benign thyroid disease. Langenbecks Arch Surg 2011; 396: 639-649
• 4 Lenders JW, Pacak K, Walther MM et al. Biochemical diagnosis of pheochromocytoma: which test is best?. JAMA 2002; 287: 1427-1434
• 5 Machens A, Dralle H. Biomarker-based risk stratification for previously untreated medullary thyroid cancer. J Clin Endocrinol Metab 2010; 95: 2655-2663
• 6 Kloos RT, Eng C, Evans DB et al. Medullary thyroid cancer: management guidelines of the American Thyroid Association. Thyroid 2009; 19: 565-612
• 7 Barbet J, Campion L, Kraeber-Bodere F, Chatal JF. Prognostic impact of serum calcitonin and carcinoembryonic antigen doubling-times in patients with medullary thyroid carcinoma. J Clin Endocrinol Metab 2005; 90: 6077-6084
• 8 Laure Giraudet A, Al Ghulzan A, Auperin A et al. Progression of medullary thyroid carcinoma: assessment with calcitonin and carcinoembryonic antigen doubling times. Eur J Endocrinol 2008; 158: 239-246
• 9 Martinez SR, Beal SH, Chen A et al. Adjuvant external beam radiation for medullary thyroid carcinoma. J Surg Oncol 2010; 102: 175-178
• 10 Wells Jr. SA, Robinson BG, Gagel RF et al. Vandetanib in Patients With Locally Advanced or Metastatic Medullary Thyroid Cancer: A Randomized, Double-Blind Phase III Trial. J Clin Oncol 2011; 30: 134-141
• 11 Kraeber-Bodere F, Bodet-Milin C, Niaudet C et al. Comparative toxicity and efficacy of combined radioimmunotherapy and antiangiogenic therapy in carcinoembryonic antigen-expressing medullary thyroid cancer xenograft. J Nucl Med 2010; 51: 624-631
• 12 Iwase Y, Maitani Y. Octreotide-targeted liposomes loaded with CPT-11 enhanced cytotoxicity for the treatment of medullary thyroid carcinoma. Mol Pharm 2011; 8: 330-337
• 13 Miyauchi A, Matsuzuka F, Hirai K et al. Prospective trial of unilateral surgery for nonhereditary medullary thyroid carcinoma in patients without germline RET mutations. World J Surg 2002; 26: 1023-1028
• 14 Machens A, Ukkat J, Hauptmann S, Dralle H. Abnormal carcinoembryonic antigen levels and medullary thyroid cancer progression: a multivariate analysis. Arch Surg discussion 2007; 142
• 15 Gimm O. Familial endocrine conditions. In: Hubbard JG, Inabnet WB, Lo CY, (eds) Endocrine surgery: principles and practice. Springer; 2009: 567-581
• 16 Toledo SP, Lourenco Jr. DM, Santos MA et al. Hypercalcitoninemia is not pathognomonic of medullary thyroid carcinoma. Clinics (Sao Paulo) 2009; 64: 699-706
• 17 Robbins KT, Medina JE, Wolfe GT et al. Standardizing neck dissection terminology. Official report of the Academy's Committee for Head and Neck Surgery and Oncology. Arch Otolaryngol Head Neck Surg 1991; 117: 601-605