Journal Information
Vol. 16. Issue 1.
Pages 177-185 (January - February 2010)
Share
Share
Download PDF
More article options
Vol. 16. Issue 1.
Pages 177-185 (January - February 2010)
Full text access
Tumor carcinóide do timo – Caso clínico
Thymic carcinoid – case report
Visits
1171
Lina Carvalho1, Luís Eugénio Fernandes2, Filipe Ferreira3, Filipa Januário3, Diogo Robles3, Inês Sanches3, Edgar Vaz3, Manuel Antunes2
1 Anatomia Patológica
2 Cirurgia Cardiotorácica
3 Alunos da disciplina de Oncologia – 6.° ano – Medicina – Faculdade de Medicina da Universidade de Coimbra/6th year Oncology medical students
This item has received
Article information
Resumo

Os tumores neuroendócrinos primários representam cerca de 4% do total dos tumores do mediastino anterior, incluindo o timo, afectando mais homens que mulheres numa razão de 3:1 e apresentando uma maior incidência entre os 40 e os 60 anos. Antigamente classificados como timomas, desde 1972 são considerados um grupo distinto de neoplasias tímicas, podendo ser biologicamente activos, estando sobretudo associados à síndroma NEM-1 (19 a 25% dos doentes, sendo nestes casos mais agressivos).

São descritos como tendo grande progressão local, recorrência e metástases em elevada percentagem de casos, o que determina um mau prognóstico. O estadiamento é o parâmetro mais importante para determinar a sobrevida. Tumores detectados em fase precoce e capsulados associam-se a um excelente prognóstico, enquanto em estádios avançados, localmente invasivos, têm prognóstico relativamente pobre.

A remoção cirúrgica completa, para todos os tumores tímicos, é a base da terapêutica e o factor crítico para a sobrevivência a longo prazo, independentemente do tipo histológico. São uma causa de morte importante, nomeadamente os tumores associados a NEM-1 e síndroma de Cushing, e há autores que defendem a realização de timectomia profiláctica nestes doentes.

Palavras-chave:
Timo
carcinóide
prognóstico
Abstract

Primary neuroendocrine tumours (carcinoid tumours) account for about 4% of anterior mediastinal tumours concerning thymus localization. They appear to have a male predilection (3:1 ratio) and occur mostly between 40 and 60 years of age. Classified primarily as thymomas, they have been considered a different group of thymic neoplasia since 1972. They can be biologically active, mostly associated with MEN-1 (19–25% of patients and more aggressive in these cases).

As a locally invasive disease, with recurrence and metastasis in a high percentage of cases, it correlates with a poor prognosis. Staging is the most important determinant of survival. Encapsulated tumours diagnosed in early stages have an excellent prognosis, while locally invasive tumours in more advanced stages have a relatively poor prognosis as happens with thymomas.

Complete surgical removal is the critical factor for long-term survival rates and the basis for treatment of all thymic tumours, independent of histologic type. As an important cause of death, especially in carcinoid tumours associated with MEN-1 and Cushing's syndrome, some authors advocate the need for profilactic thymectomy in these patients.

Key-words:
Thymus
carcinoid
prognosis
Full text is only aviable in PDF
Bibliografia
[1.]
J. Rosai, E. Higa.
Mediastinal endocrine neoplasm of probable thymic origin, related to carcinoid tumour. clinicalopathologic study of 8 cases.
Cancer, 29 (1972), pp. 1061-1075
[2.]
W. Travis, E. Brambilla, H. Muller-Hermelink, C. Harris.
tumors of the lung, pleura thymus and heart.
WHO, IARCPress, (2004),
[3.]
J. Rosai.
Histological Typing of Tumours of the Thymus.
2nd ed., NY: Springer-Verlag, (1999),
[4.]
F. Detterbeck, A.M. Parsons.
Thymic tumours.
Ann Thorac Surg, 77 (2004), pp. 1860-1869
[5.]
R. Ullmann, S. Petzmann, H. Klemen, et al.
The position of pulmonary carcinoids within the spectrum of neuroendocrine tumours of the lung and other tissues.
Genes Chromosomes Cancer, 34 (2002), pp. 78-85
[6.]
E. Du, P. Goldstraw, J. Zacharias, O. Tiffet, P. Craig, A. Nicholson, N. Weinder, E. Yi.
TTF-1 expression is specific for lung primary typical and atypical carcinoids: TTF-1-positive carcinoids are predominantly in peripheral location.
Human Pathology, 35 (2004), pp. 825-831
[7.]
M. García-Yuste, J. Matilla, A. Cueto, J. Paniagua, G. Ramos, M. Cañizares, I. Muguruza.
Typical and atypical carcinoid tumours: analysis of the experience of the Spanish multi-centric study of neuroendocrine tumours of the lung.
Eur J Cardio-Thorac Surg, 31 (2007), pp. 192-197
[8.]
M. García-Yuste, J. Matilla, T. Alvarez-Gago, J. Duque, F. Heras, L. Cerezal, G. Ramos.
prognostic factors in neuroendocrine lung tumors: a Spanish multicenter study.
Ann Thorac Surg, 70 (2000), pp. 258-263
[9.]
G. Economopoulos, J. Lewis, M. Lee, et al.
Carcinoid tumours of the thymus.
Ann Thorac Surg, 50 (1990), pp. 58-63
[10.]
C. Moran, S. Suster.
Spindle-cell neuroendocrine carcinomas of the thymus (spindle-cell thymic carcinoid): a clinicopathologic and immunohistochemical study of seven cases.
Mod Phatol, 12 (1999), pp. 587-591
[11.]
C. Moran, S. Suster.
Thymic neuroendocrine carcinomas with combined features ranging from well differentiated (carcinoid) to small cell carcinoma.
Am J Clin Pathol, 113 (2000), pp. 345-350
[12.]
E. Engels, R. Pfeiffer.
Malignant thymoma in the United States: demographic patterns in incidence and associations with subsequent malignancies.
Int J Cancer, 105 (2003), pp. 546-551
[13.]
C. Moran, S. Suster.
A clinicopathologic analysis of 80 cases of neuroendocrine carcinomas of the thymus.
Am J Clin Pathol, 114 (2000), pp. 100-110
[14.]
C. Pan, Y. Jong, Y. Chen.
Comparative genomic hybridization analysis of thymic neuroendocrine tumors.
Modern Pathology, 18 (2005), pp. 358-364
[15.]
M. Okumura, M. Ohta, H. Tateyama, et al.
The World Health Organization histologic classification system reflects the oncologic behavior of thymoma: a clinical study of 273 patients.
Cancer, 94 (2002), pp. 624-632
[16.]
F. Pearson, J. Griffith, J. Cooper, J. Deslauriers, et al.
Thoracic Surgery 2nd edition.
Churchill Livingstone, (2002), pp. 1682-1710
[17.]
A. Masaoka, Y. Monden, K. Nakahara, D. Tanioka.
Follow-up studies of thymomas with special reference to their clinical stages.
Cancer, 48 (1981), pp. 2485-2492
[18.]
C. Daniels, V. Lowe, M.-C. Aubry, M. Allen, J. Jett.
The utility of fluorodeoxyglucose positron emission tomography in the evaluation of carcinoid tumors pre-senting as pulmonary nodules.
Chest, 131 (2007), pp. 255-260
[19.]
J. Rosenberg.
Neoplasms of the mediastinum.
Cancer: Principles and practice of oncology, 4th edition, pp. 1333-1435
[20.]
I. Schmidt-wolf, J. Rockstroh, H. Schuller, et al.
Malignant thymoma: current status of classification and multimodality treatment.
Ann Hematol, 82 (2003), pp. 69-76
[21.]
T. Takayama, T. Kammeya, K. Inagaki, et al.
MEN type 1 associated with mediastinal carcinoid producing parathyroid hormone, calcitonin and chorionic gonadotropin.
Pathol Res Pract, 189 (1993), pp. 1090-1996
[22.]
B. Teh, J. McArdle, S. Chan, et al.
Clinicopathologic studies of thymic carcinoids in multiple endocrine neoplasia type 1.
Medicine, 76 (1997), pp. 21-27
[23.]
A. Walch, H. Zitzelsberger, M. Aubele, et al.
Typical and atypical carcinoid tumours of the lung are characterized by 11q delections as detected by comparative genomic hybridization.
Am J Pathol, 153 (1998), pp. 1089-1098
[24.]
M. Wick, R. Scoot, C. Li, et al.
A clinicopathologic report of seven cases with a review of the literature.
Mayo Clin Proc, 55 (1980), pp. 246-251
[25.]
M. Wick, J. Carney, P. Bernatz, L. Brown.
Primary mediastinal carcinoid tumours.
Am J Surg Pathol, 6 (1982), pp. 195-205
[26.]
P. Strobel, A. Marx, A. Zettl, H.K. Muller-Hermelink.
Thymoma and thymic carcinoma: an update of the WHO Classification 2004.
Surg Today, 35 (2005), pp. 805-811
[27.]
G. Rossi, A. Cavazza, A. Marchioni, L. Longo, M. Migaldi, G. sartori, N. Bigiani, L. Schirosi, C. Casali, U. Morandi, N. Facciolongo, A. Maiorana, M. Bavieri, L. Fabbri, E. Brambilla.
Role of chemotherapy and the recptor tyrosine kinases KIT, PDGFRα PDGFRβ, and meti n large-cell neuroendocrine carcinoma of the lung.
J Clin Oncol, 23 (2005), pp. 8774-8785
[28.]
S. Davies, J. Gosney, D. Hansell, A. Wells, R. Bois, M. Burke, M. Sheppard, A. Nicholson.
Didffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease.
Thorax, 62 (2007), pp. 248-252
[29.]
P. Bastos, A. Magalhães, M.R. Cruz, S. Saleiro, L. Gonçalves.
Piñon Miguel. Cistos e tumores primários do Mediastino. Rev Port Pneumol, 13 (2007), pp. 659-673
[30.]
H. Ping, L. Varticovski, E. Bowman, J. Fukuoka, J. Welsh, K. Miura, J. Jen, E. Gabrielson, E. Brambilla, W. Travis, C. Harris.
Identification of carboxypeptidase e and γ-glutamil hydrolase as biomarkers for pulmonary neuroendocrine tumors by cDNA microarray.
Human Pathology, 35 (2004), pp. 1196-1209
[31.]
Y. Kobayashi, Y. Tokuchi, T. Hashimoto, M. Hayashi, H. Nishimura, I. Yuichi, K. Nakagawa, Y. Sato, A. Takahashi, E. Tsuchiya.
Molecular markers for reinforcement of histological subclassification of neuroendocrine lung tumors.
Cancer Sci, 95 (2004), pp. 334-341
[32.]
F. Barata, A. Costa, células. Carcinoma do pulmão de pequenas.
Estado da arte e perspectivas futuras.
Rev Port Pneumol, 13 (2007), pp. 587-603
[33.]
B. Sousa, A. Araújo, T. Amaro, I. Azevedo, M. Soares, O. Sousa.
Timomas malignos. a experiência do IPO do Porto e revisão de literatura.
Rev Port Pneumol, 13 (2007), pp. 553-585
[34.]
N. Onuki, I. Wistuba, W. Travis, A. Virmani, K. Yashima, E. Brambilla, P. Hashleton, A. Gazdar.
Genetic chan ges in the spectrum of neuroendocrine lung tumors.
Cancer, 85 (1999), pp. 600-607
Copyright © 2010. Sociedade Portuguesa de Pneumologia
Download PDF
Pulmonology
Article options
Tools

Are you a health professional able to prescribe or dispense drugs?