Tumor carcinóide do timo – Caso clínico

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.

Bibliografia

1.Rosai J, Higa E. Mediastinal endocrine neoplasm of probable thymic origin, related to carcinoid tumour. clinicalopathologic study of 8 cases. Cancer. 1972; 29:1061-75.
Medline
2.Travis W, Brambilla E, Muller-Hermelink H, Harris C. tumors of the lung, pleura thymus and heart. Lyon: WHO, IARCPress; 2004.
3.Rosai J. Histological Typing of Tumours of the Thymus. 2nd ed. New York: NY: Springer-Verlag; 1999.
4.Detterbeck F, Parsons AM. Thymic tumours. Ann Thorac Surg. 2004; 77:1860-9.
Medline
5.Ullmann R, Petzmann S, Klemen H, et al. The position of pulmonary carcinoids within the spectrum of neuroendocrine tumours of the lung and other tissues. Genes Chromosomes Cancer. 2002; 34:78-85.
Medline
6.Du E, Goldstraw P, Zacharias J, Tiffet O, Craig P, Nicholson A, Weinder N, Yi E. TTF-1 expression is specific for lung primary typical and atypical carcinoids: TTF-1-positive carcinoids are predominantly in peripheral location. Human Pathology. 2004; 35(7):825-31.
Medline
7.García-Yuste M, Matilla J, Cueto A, Paniagua J, Ramos G, Cañizares M, Muguruza I. 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. 2007; 31:192-7.
8.García-Yuste M, Matilla J, Alvarez-Gago T, Duque J, Heras F, Cerezal L, Ramos G. prognostic factors in neuroendocrine lung tumors: a Spanish multicenter study. Ann Thorac Surg. 2000; 70:258-63.
9.Economopoulos G, Lewis J, Lee M, et al. Carcinoid tumours of the thymus. Ann Thorac Surg. 1990; 50:58-63.
10.Moran C, Suster S. Spindle-cell neuroendocrine carcinomas of the thymus (spindle-cell thymic carcinoid): a clinicopathologic and immunohistochemical study of seven cases. Mod Phatol. 1999; 12:587-91.
11.Moran C, Suster S. Thymic neuroendocrine carcinomas with combined features ranging from well differentiated (carcinoid) to small cell carcinoma. Am J Clin Pathol. 2000; 113:345-50.
12.Engels E, Pfeiffer R. Malignant thymoma in the United States: demographic patterns in incidence and associations with subsequent malignancies. Int J Cancer. 2003; 105(4):546-51.
13.Moran C, Suster S. A clinicopathologic analysis of 80 cases of neuroendocrine carcinomas of the thymus. Am J Clin Pathol. 2000; 114:100-10.
14.Pan C, Jong Y, Chen Y. Comparative genomic hybridization analysis of thymic neuroendocrine tumors. Modern Pathology. 2005; 18:358-64.
15.Okumura M, Ohta M, Tateyama H, et al. The World Health Organization histologic classification system reflects the oncologic behavior of thymoma: a clinical study of 273 patients. Cancer. 2002; 94(3):624-32.
16.Pearson F, Griffith J, Cooper J, Deslauriers J, et al. Thoracic Surgery 2nd edition. Churchill Livingstone. 2002; 1682-710.
17.Masaoka A, Monden Y, Nakahara K, Tanioka D. Follow-up studies of thymomas with special reference to their clinical stages. Cancer. 1981; 48:2485-92.
18.Daniels C, Lowe V, Aubry M-C, Allen M, Jett J. The utility of fluorodeoxyglucose positron emission tomography in the evaluation of carcinoid tumors pre-senting as pulmonary nodules. Chest. 2007; 131:255-60.
19.Rosenberg J. Neoplasms of the mediastinum. En: De Vita V.T., Hellman S., Rosenberg S.A., editors. Cancer: Principles and practice of oncology. 4th edition. J.B Lippincott Company; 1993. 1333-435.
20.Schmidt-wolf I, Rockstroh J, Schuller H, et al. Malignant thymoma: current status of classification and multimodality treatment. Ann Hematol. 2003; 82(2):69-76.
21.Takayama T, Kammeya T, Inagaki K, et al. MEN type 1 associated with mediastinal carcinoid producing parathyroid hormone, calcitonin and chorionic gonadotropin. Pathol Res Pract. 1993; 189:1090-996.
22.Teh B, McArdle J, Chan S, et al. Clinicopathologic studies of thymic carcinoids in multiple endocrine neoplasia type 1. Medicine. 1997; 76:21-7.
23.Walch A, Zitzelsberger H, Aubele M, et al. Typical and atypical carcinoid tumours of the lung are characterized by 11q delections as detected by comparative genomic hybridization. Am J Pathol. 1998; 153:1089-98.
24.Wick M, Scoot R, Li C, et al. A clinicopathologic report of seven cases with a review of the literature. Mayo Clin Proc. 1980; 55:246-51.
25.Wick M, Carney J, Bernatz P, Brown L. Primary mediastinal carcinoid tumours. Am J Surg Pathol. 1982; 6:195-205.
26.Strobel P, Marx A, Zettl A, Muller-Hermelink HK. Thymoma and thymic carcinoma: an update of the WHO Classification 2004. Surg Today. 2005; 35:805-11.
27.Rossi G, Cavazza A, Marchioni A, Longo L, Migaldi M, sartori G, Bigiani N, Schirosi L, Casali C, Morandi U, Facciolongo N, Maiorana A, Bavieri M, Fabbri L, Brambilla E. Role of chemotherapy and the recptor tyrosine kinases KIT, PDGFRα PDGFRβ, and meti n large-cell neuroendocrine carcinoma of the lung. J Clin Oncol. 2005; 23(24):8774-85.
28.Davies S, Gosney J, Hansell D, Wells A, Bois R, Burke M, Sheppard M, Nicholson A. Didffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax. 2007; 62:248-52.
29.Bastos P, Magalhães A, Cruz MR, Saleiro S, Gonçalves L. Piñon Miguel. Cistos e tumores primários do Mediastino. Rev Port Pneumol. 2007; 13(5):659-73.
30.Ping H, Varticovski L, Bowman E, Fukuoka J, Welsh J, Miura K, Jen J, Gabrielson E, Brambilla E, Travis W, Harris C. Identification of carboxypeptidase e and γ-glutamil hydrolase as biomarkers for pulmonary neuroendocrine tumors by cDNA microarray. Human Pathology. 2004; 35(10):1196-209.
31.Kobayashi Y, Tokuchi Y, Hashimoto T, Hayashi M, Nishimura H, Yuichi I, Nakagawa K, Sato Y, Takahashi A, Tsuchiya E. Molecular markers for reinforcement of histological subclassification of neuroendocrine lung tumors. Cancer Sci. 2004; 95:334-41.
32.Barata F, Costa A, Carcinoma do pulmão de pequenas células. Estado da arte e perspectivas futuras. Rev Port Pneumol. 2007; 13(4):587-603.
Medline
33.Sousa B, Araújo A, Amaro T, Azevedo I, Soares M, Sousa O. Timomas malignos. a experiência do IPO do Porto e revisão de literatura. Rev Port Pneumol. 2007; 13(4):553-85.
Medline
34.Onuki N, Wistuba I, Travis W, Virmani A, Yashima K, Brambilla E, Hashleton P, Gazdar A. Genetic chan ges in the spectrum of neuroendocrine lung tumors. Cancer. 1999; 85(3):600-7.
Medline
Lina Carvalho a, , Luís Eugénio Fernandes b, Filipe Ferreira c, Filipa Januário c, Diogo Robles c, Inês Sanches c, Edgar Vaz c, Manuel Antunes b,

a Anatomia Patológica
b Cirurgia Cardiotorácica
c Alunos da disciplina de Oncologia – 6.° ano – Medicina – Faculdade de Medicina da Universidade de Coimbra/6th year Oncology medical students