Surgical curability of medullary thyroid cancer in multiple endocrine neoplasia 2B: a changing perspective.
Ann Surg
; 259(4): 800-6, 2014 Apr.
Article
en En
| MEDLINE
| ID: mdl-23979292
ABSTRACT
OBJECTIVE:
This investigation aimed at exploring the suitability of nonendocrine manifestations preceding medullary thyroid cancer (MTC) for early diagnosis of multiple endocrine neoplasia type 2B (MEN 2B).BACKGROUND:
MEN 2B patients, running a high risk of metastatic MTC, must be diagnosed early for biochemical cure.METHODS:
Forty-four MEN 2B patients carrying inherited (3 patients) and de novo (41 patients) M918T RET mutations were examined for signs and symptoms prompting MEN 2B.RESULTS:
All 3 patients with inherited mutations were diagnosed before the age of 1 year and cured of their C-cell disease. Among 41 patients with de novo mutations, MEN 2B was diagnosed in 12 patients after recognition of nonendocrine manifestations [intestinal ganglioneuromatosis (6 patients), oral symptoms (5 patients), ocular ("tearless crying") (4 patients), and skeletal stigmata (1 patient) alone or concomitantly]. In the remaining 29 patients with de novo mutations, the diagnosis of MEN 2B was triggered by symptomatic MTC (28 patients) or pheochromocytoma (1 patient). The former patients, being significantly (P < 0.001) younger (means of 5.3 vs 17.6 years) and having lower calcitonin levels (means of 115 vs 25,519 pg/mL), smaller tumors (67% vs 0% were ≤10 mm) and less often extrathyroidal extension (0% vs 81%), lymph node (42% vs 100%), and distant metastases (8% vs 79%), were biochemically cured more often (58% vs 0%).CONCLUSIONS:
MTC is curable in patients with de novo mutations when nonendocrine MEN 2B components are quickly appreciated and surgical intervention is performed before patients turn 4 years old.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Tiroidectomía
/
Neoplasias de la Tiroides
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Biomarcadores de Tumor
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Neoplasia Endocrina Múltiple Tipo 2b
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Proteínas Proto-Oncogénicas c-ret
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
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Evaluation_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
/
Screening_studies
Idioma:
En
Revista:
Ann Surg
Año:
2014
Tipo del documento:
Article
País de afiliación:
Noruega