Penetrance of Functioning and Nonfunctioning Pancreatic Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1 in the Second Decade of Life

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art_GONCALVES_Penetrance_of_Functioning_and_Nonfunctioning_Pancreatic_Neuroendocrine_Tumors_2014.PDF (266.32 KB)
Citações na Scopus
49
Tipo de produção
article
Data de publicação
2014
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
ENDOCRINE SOC
Autores
TOLEDO, Rodrigo A.
Citação
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, v.99, n.1, p.E89-E96, 2014
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Context: Data are scarce on the penetrance of multiple endocrine neoplasia type 1 (MEN1)-related nonfunctioning pancreatic neuroendocrine tumors (NF-PETs) and insulinomas in young MEN1 patients. Apotential positive correlation between tumor size and malignancy (2-3 cm, 18%; >3 cm, 43%) has greatly influenced the management of MEN1 adults with NF-PETs. Objective: The aim of the study was to estimate the penetrance of NF-PETs, insulinomas, and gastrinomas in young MEN1 carriers. Design: The data were obtained from a screening program (1996-2012) involving 113 MEN1 patients in a tertiary academic reference center. Patients: Nineteen MEN1 patients (aged 12-20 y; 16 patients aged 15-20 y and 3 patients aged 12-14 y) were screened for NF-PETs, insulinomas, and gastrinomas. Methods: Magnetic resonance imaging/computed tomography and endoscopic ultrasound (EUS) were performed on 10 MEN1 carriers, magnetic resonance imaging/computed tomography was performed on five patients, and four other patients underwent an EUS. Results: The overall penetrance of PETs during the second decade of life was42%(8 of 19). All eight PET patients had NF-PETs, and half of those tumors were multicentric. One-fifth of the screened patients (21%; 4 of 19) harbored at least one large tumor (>2.0 cm). Insulinoma was detected in two NF-PET patients (11%) at the initial screening; gastrinoma was not present in any cases. Six of the 11 (54%) screened patients aged 15-20 years who underwent an EUS had NF-PETs. Potential false-positive EUS results were excluded based on EUS-guided biopsy results, the reproducibility of the NF-PET findings, or the observation of increased tumor size during follow-up. Distal pancreatectomy and the nodule enucleation of pancreatic head tumors were conducted on three patients with large tumors (>2.0 cm; T2N0M0) that were classified as grade 1 neuroendocrine tumors (Ki-67 < 2%). Conclusions: Our data demonstrated high penetrance of NF-PETs in 15- to 20-year-old MEN1 patients. The high percentage of the patients presenting consensus criteria for surgery for NF-PET alone or NF-PET/insulinoma suggests a potential benefit for the periodic surveillance of these tumors in this age group.
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URI
https://observatorio.fm.usp.br/handle/OPI/7460
Referências
  1. Barbe C, 2012, DIGEST LIVER DIS, V44, P228, DOI 10.1016/j.dld.2011.09.014
  2. Bassett JHD, 1998, AM J HUM GENET, V62, P232, DOI 10.1086/301729
  3. Brandi ML, 2001, J CLIN ENDOCR METAB, V86, P5658, DOI 10.1210/jc.86.12.5658
  4. Camera L, 2011, RADIOL MED, V116, P595, DOI 10.1007/s11547-011-0636-2
  5. De Vogelaere K, 2006, J LAPAROENDOSC ADV S, V16, P335
  6. Fabbri HC, 2010, ARQ BRAS ENDOCRINOL, V54, P754, DOI 10.1590/S0004-27302010000800016
  7. Fontaniere S, 2006, ENDOCR-RELAT CANCER, V13, P1223, DOI 10.1677/erc.1.01294
  8. Giraud S, 1997, J CLIN ENDOCR METAB, V82, P3487, DOI 10.1210/jc.82.10.3487
  9. Kontogeorgos G, 2001, CLIN ENDOCRINOL, V54, P117, DOI 10.1046/j.1365-2265.2001.01031.x
  10. Kouvaraki MA, 2006, WORLD J SURG, V30, P643, DOI 10.1007/s00268-006-0360-y
  11. Levy-Bohbot N, 2004, GASTROEN CLIN BIOL, V28, P1075, DOI 10.1016/S0399-8320(04)95184-6
  12. Lips CJ, 2012, EXPERT REV ENDOCRINO, V7, P331
  13. Lourenco DM, 2008, EUR J ENDOCRINOL, V159, P259, DOI 10.1530/EJE-08-0153
  14. Lourenço Delmar Muniz Jr, 2007, Clinics (Sao Paulo), V62, P465, DOI 10.1590/S1807-59322007000400014
  15. Newey PJ, 2009, J CLIN ENDOCR METAB, V94, P3640, DOI 10.1210/jc.2009-0564
  16. Oiwa A, 2002, ENDOCR J, V49, P635, DOI 10.1507/endocrj.49.635
  17. Pijpe A, 2012, BMJ-BRIT MED J, V345, DOI 10.1136/bmj.e5660
  18. SHEPHERD JJ, 1991, ARCH SURG-CHICAGO, V126, P935
  19. Thakker RV, 2012, J CLIN ENDOCR METAB, V97, P2990, DOI 10.1210/jc.2012-1230
  20. Thomas-Marques L, 2006, AM J GASTROENTEROL, V101, P266, DOI 10.1111/j.1572-0241.2006.00367.x
  21. Toledo RA, 2007, CLIN ENDOCRINOL, V67, P377, DOI 10.1111/j.1365-2265.2007.02895.x
  22. Triponez F, 2006, ANN SURG, V243, P265, DOI 10.1097/01.sla.000197715.96762.68
  23. Trump D, 1996, QJM-MON J ASSOC PHYS, V89, P653
  24. Verges B, 2002, J CLIN ENDOCR METAB, V87, P457, DOI 10.1210/jc.87.2.457
  25. Waldmann J, 2009, WORLD J SURG, V33, P1208, DOI 10.1007/s00268-009-9983-8

Coleções
Artigos e Materiais de Revistas Científicas - FM/MDR
Artigos e Materiais de Revistas Científicas - FM/MCG
Artigos e Materiais de Revistas Científicas - FM/MGT
Artigos e Materiais de Revistas Científicas - HC/ICESP
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - HC/InRad
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