Tumor carcinóide do timo – Caso clínico
Thymic carcinoid – case report
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
Received 28 April 2009, Accepted 08 July 2009

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.


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.

Timo, carcinóide, prognóstico
Thymus, carcinoid, prognosis
This article is only available in PDF
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
W. Travis,E. Brambilla,H. Muller-Hermelink,C. Harris
tumors of the lung, pleura thymus and heart
WHO, IARCPress, (2004)
J. Rosai
Histological Typing of Tumours of the Thymus
2nd ed., NY: Springer-Verlag, (1999)
F. Detterbeck,A.M. Parsons
Thymic tumours
Ann Thorac Surg, 77 (2004), pp. 1860-1869 http://dx.doi.org/10.1016/j.athoracsur.2003.10.001
R. Ullmann,S. Petzmann,H. Klemen
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
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
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
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
G. Economopoulos,J. Lewis,M. Lee
Carcinoid tumours of the thymus
Ann Thorac Surg, 50 (1990), pp. 58-63
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
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 http://dx.doi.org/10.1309/Q01U-60BL-VEV4-TWR1
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 http://dx.doi.org/10.1002/ijc.11099
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 http://dx.doi.org/10.1309/3PDN-PMT5-EQTM-H0CD
C. Pan,Y. Jong,Y. Chen
Comparative genomic hybridization analysis of thymic neuroendocrine tumors
Modern Pathology, 18 (2005), pp. 358-364 http://dx.doi.org/10.1038/modpathol.3800246
M. Okumura,M. Ohta,H. Tateyama
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
F. Pearson,J. Griffith,J. Cooper,J. Deslauriers
Thoracic Surgery 2nd edition
Churchill Livingstone, (2002), pp. 1682-1710
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
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 http://dx.doi.org/10.1378/chest.06-0711
J. Rosenberg
Neoplasms of the mediastinum
Cancer: Principles and practice of oncology, 4th edition, pp. 1333-1435
I. Schmidt-wolf,J. Rockstroh,H. Schuller
Malignant thymoma: current status of classification and multimodality treatment
Ann Hematol, 82 (2003), pp. 69-76 http://dx.doi.org/10.1007/s00277-002-0597-6
T. Takayama,T. Kammeya,K. Inagaki
MEN type 1 associated with mediastinal carcinoid producing parathyroid hormone, calcitonin and chorionic gonadotropin
Pathol Res Pract, 189 (1993), pp. 1090-1996 http://dx.doi.org/10.1016/S0344-0338(11)80689-8
B. Teh,J. McArdle,S. Chan
Clinicopathologic studies of thymic carcinoids in multiple endocrine neoplasia type 1
Medicine, 76 (1997), pp. 21-27
A. Walch,H. Zitzelsberger,M. Aubele
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 http://dx.doi.org/10.1016/S0002-9440(10)65653-2
M. Wick,R. Scoot,C. Li
A clinicopathologic report of seven cases with a review of the literature
Mayo Clin Proc, 55 (1980), pp. 246-251
M. Wick,J. Carney,P. Bernatz,L. Brown
Primary mediastinal carcinoid tumours
Am J Surg Pathol, 6 (1982), pp. 195-205
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 http://dx.doi.org/10.1007/s00595-005-3047-y
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 http://dx.doi.org/10.1200/JCO.2005.02.8233
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
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
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
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
F. Barata,A. Costa,células. Carcinoma do pulmão de pequenas
Estado da arte e perspectivas futuras
Rev Port Pneumol, 13 (2007), pp. 587-603
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
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


  • Impact Factor: 1.167(2014)
  • SCImago Journal Rank (SJR):0,315
  • Source Normalized Impact per Paper (SNIP):0,685

Open Access

Creative Commons License
Revista Portuguesa de Pneumologia is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

+ info