Endocrine Tumour
 

Treatment of Asymptomatic Hypercalcitonaemia

Adjuvant therapy in MTCs
• No role of RI therapy
• I131 MIBG scan, Anti- CEA monoclonal antibody and human recombinanat alpha interferon are still investigational
EBRT- for inoperable or gross residual disease
• No role of chemotherapy
• Biological response modifiers- Somatostatin analogues – role debatable, but reported to decrease flushing and diarrheoa in symptomatic patients.

Prognosis in MTCs

Best to worst progmosis-
– Non MEN Familial MTC
– MEN IIA
– Sporadic MTC
– MEN IIB

Abstracts
13. Advances and controversies in the diagnosis and management of medullary thyroid carcinoma. Heshmati HM, Gharib H, van Heerden JA et al Am J Med. 1997 Jul;103(1):60-9.

Recent advances in the diagnosis and treatment of medullary thyroid carcinoma (MTC) have been significant, but some issues remain controversial. MTC may occur either as a hereditary or a nonhereditary entity. Hereditary MTC can occur either alone—familial MTC (FMTC)—or as the thyroid manifestation of multiple endocrine neoplasia type 2 (MEN 2) syndromes (MEN 2A and MEN 2B). These hereditary disorders are due to germline mutations in the RET proto-oncogene. Early diagnosis and treatment considerably improve the prognosis in patients with MTC. Genetic testing can identify almost all affected individuals with hereditary disease and permits early thyroidectomy in gene carriers. Plasma CT is an excellent marker for postoperative follow-up. Imaging studies help delineate recurrent or metastatic lesions. Treatment of recurrent or metastatic disease is primarily surgical, including either palliative or microdissective surgery. Radiation therapy is reserved for skeletal metastasis or nonresectable metastatic MTC. Efficacy of current chemotherapy programs is not well established. Overall, the 10-year survival rates are approximately 65%.

14. Diagnosis and therapy of sporadic and familial medullary thyroid carcinoma. Gimm O, Sutter T, Dralle H. J Cancer Res Clin Oncol. 2001;127(3):156-65.

Medullary thyroid carcinoma (MTC) is a rare thyroid malignancy. About 75% are sporadic (sMTC) while the remaining 25% are hereditary (hMTC). The treatment of choice for both sMTC and hMTC is surgery. An adequate initial operation provides the best chance of cure. Hence, the diagnosis of MTC should be made preoperatively. In sMTC, ultrasound, ultrasound-guided fine-needle aspiration cytology and measurement of calcitonin levels (basal and after injection of calcitonin-stimulating reagents, e.g., pentagastrin) are sensitive diagnostic tools. In hMTC, identification of a germline mutation in the proto-oncogene RET is sufficient for making the diagnosis. Total thyroidectomy is recommended in all patients, sporadic and hereditary. In addition, lymphadenectomy of the cervicocentral and both cervicolateral compartments should be performed. The only indication to perform a less extensive operation may be given in young patients with hMTC. Sufficient treatment of MTC beyond local disease is still non-existent. Future research should concentrate on this issue.

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