Bronchial Carcinoid Tumours in Children – A Review

European Oncology & Haematology, 2011;7(3):196-9

Abstract:

Bronchial carcinoids (BCs) are rare, well-differentiated malignant neuroendocrine tumours that account for 2–5 % of all lung neoplasms in adults. In paediatric patients, carcinoids represent the most frequent primary lung cancer. Although BCs in childhood often have an endobronchial location causing airway obstruction, they are frequently misdiagnosed as benign conditions, resulting in a delay in definitive diagnosis and treatment. Surgery represents the treatment of choice for BCs, and lung-sparing resections (sleeve or bronchoplastic procedures) are recommended in central carcinoid tumours; pneumonectomy should be avoided, particularly in childhood. If promptly diagnosed and radically treated, BCs in children have an excellent prognosis. Relapses can occur many years after a radical resection, highlighting the necessity for long-term follow-up.
Keywords: Bronchial carcinoid tumours, paediatric lung neoplasm, typical carcinoid, lung surgery, bronchoscopy, sleeve resection, bronchoplasty
Disclosure: The authors have no conflicts of interest to declare.
Received: May 24, 2010 Accepted July 26, 2010 Citation European Oncology & Haematology, 2011;7(3):196-9

Carcinoid tumours are rare, malignant neuroendocrine neoplasms first described in 1888 in the ileum1 and called ‘Karzinoide’ by Oberndorfer in 1907.2 Neuroendocrine cells were originally called clear cells and later amine precursor uptake and decarboxylation (APUD) system cells.3 The term neuroendocrine was introduced with the finding that these cells are capable of producing bioactive amines and that a number of these cells are identical to those of the nervous system. Neuroendocrine tumours of the lung arise from bronchial mucosal cells known as enterochromaffin cells or Kulchitsky cells, which are part of a diffuse neuroendocrine system. For many years the carcinoid tumour of the lung was called bronchial adenoma, which comprised other bronchial tumours with benign behaviour. Today, bronchial carcinoids (BCs) are classified as well-differentiated malignant neuroendocrine tumours in two distinctive forms – typical carcinoid (TC) and atypical carcinoid (AC) – with different histological features, clinical course and prognosis. The differences in histological criteria between TC and AC were first described by Arrigoni et al.4 and later modified by Travis et al.5 They were fixed in 1999 by the World Health Organization (WHO).6

Pathology
TC is a variant of neuroendocrine tumours with a low-grade histological malignancy profile (<2 mitoses/10 high-power field [HPF], nuclear pleomorphism and absence of necrosis) that rarely metastasises.7,8 AC is considered to be an intermediate grade of malignancy; it presents with ≥2 but <10 mitoses/HPF and/or coaugulative necrosis.5 Several immunohistochemical markers have been considered for the histological assessment and risk stratification of carcinoids. In terms of tumour cell commitment, carcinoids are consistently associated with the immunohistochemical expression of neuroendocrine markers (chromogranin A, neuron-specific enolase [NSE], synaptophysin, Leu7).9 Mib1 and Bcl2 expression is an independent variable associated with tumour prognosis (with no statistical interaction between the two). In the future, such a biologically plausible immunohistochemical pattern could also be suitable in the routine histological assessment of BC.10

Recently, researchers have studied the importance of genetics in BC. A recent paper reports that in TC and AC DNA, under-representations of 11q are frequent and that in AC there are also frequently losses of 10q and 13q, as in high-grade neuroendocrine malignant tumours (large- and small-cell lung cancer). Losses of 10q and 13q probably suggest a more aggressive behaviour of AC.11

References:
  1. Lubarch O, Ueber den primären Krebs des ileum, nebst Bemerkungen über das gleichzeitige Vorkommen von Krebs und Tuberkolose, Virchws Arch, 1888;111:280–317.
  2. Oberndorfer S, Karzinoide Tumoren des Dunndarms, Frankfurter Z Pathol, 1907;1:425–9.
  3. Fröhlich F, Die ‘Helle Zelle’ der Bronchialschleimhaut und ihre Beziehungen zum Problem der Chemoreceptoren, Frankfurter Z Pathol, 1949;60:517.
  4. Arrigoni MG, Woolner LB, Bernatz PE, Atypical carcinoid tumors of the lung, J Thorac Cardiovasc Surg, 1972;64:413–21.
  5. Travis WD, Rush W, Flieder DB, et al., Survival analysis of 200 pulmonary neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid, Am J Surg Pathol, 1998;22:934–44.
  6. Travis WD, Colby TV, Corrin B, Histological typing of lung and pleural tumours, 3rd edition, Berlin: Springer, 1999.
  7. Fink G, Krelbaum T, Yellin A, et al., Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from literature, Chest, 2001;119:1647–51.
  8. Ducrocq X, Thomas P, Massard G, et al., Operative risk and prognostic factors of typical bronchial carcinoid tumors, Ann Thorac Surg, 1998;65:1410–4.
  9. Beasley MB, Thunnissen FB, Brambilla E, et al., Pulmonary atypical carcinoid: predictors of survival in 106 cases, Hum Pathol, 2000;31:1255–65.
  10. Rugge M, Fassan M, Clemente R, et al., Bronchopulmonary carcinoid: phenotype and long-term outcome in a singleinstitution series of Italian patients, Clin Cancer Res, 2008;14(1):149–54.
  11. Hage R, Brutel de la Riviere A, Update in pulmonary carcinoid tumors: a review article, Ann Surg Oncol, 2003;10:697–704.
  12. Wang LT, Wilkins Jr EW, Bode HH, Bronchial carcinoid tumors in pediatric patients, Chest, 1993;103:1426–8.
  13. Dishop MK, Kuruvilla S, Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital, Arch Pathol Lab Med, 2008;132(7):1079–103.
  14. Hancock BJ, Di Lorenzo M, Youssef S, et al., Childhood primary pulmonary neoplasms, J Pediatr Surg, 1993;28(9):1133–6.
  15. Al-Qahtani AR, Di Lorenzo M, Yazbeck S, Endobronchial tumors in children: Institutional experience and literature review, J Pediatr Surg, 2003;38(5):733–6.
  16. Rizzardi G, Marulli G, Calabrese F, et al., Bronchial carcinoid tumours in children: surgical treatment and outcome in a single institution, Eur J Pediatr Surg, 2009;19(4):228–31.
  17. Fauroux B, Aynie V, Larroquet M, et al., Carcinoid and mucoepidermoid bronchial tumours in children, Eur J Pediatr, 2005;164:748–52.
  18. Hartman GE, Shochat SJ, Primary neoplasms of childhood: a rewiew, Ann Thorac Surg, 1983;36:108.
  19. Bellah RD, Mahboubi S, Berdon WE, Malignant endobronchial lesions of adolescence, Pediatr Radiol, 1992;22:563.
  20. Eggli KD, Newman B, Nodules, masses, and pseudomasses in the pediatric lung, Radiol Clin North Am, 1993;31(3):651–66.
  21. Moraes TJ, Langer JC, Forte V, et al., Pediatric pulmonary carcinoid: A case report and review of literature, Pediatr Pulmonol, 2003;35:318–22.
  22. . Escalon J, Detterbeck F, Carcinoid tumours. In: Shields T, Lo Cicero Ji, Reed C, Feins R (eds), General Thoracic surgery, 7th edition, Philadelphia: Lippincott William and Wilkins, 2009:1539–54.
  23. Kaplan KA, Beierle EA, Faro A, et al., Recurren pneumonia in children: A case report and approach to diagnosis, Clin Pediatr, 2006;45:15–22.
  24. Detterbeck FC, Management of carcinoid tumors, Ann Thorac Surg, 2010;89(3):998–1005.
  25. Lack EE, Harris GB, Eraklis AJ, Vawter GF, Primary bronchial tumors in childhood. A clinicopathologic study of six cases, Cancer, 1983;51(3):492–7.
  26. McCaughan BC, Martini N, Bains MS, Bronchial Carcinoids. Review of 124 cases, J Thorac Cardiovasc Surg, 1985;89:8–17.
  27. Gaissert HA, Mathisen DJ, Grillo HC, et al., Tracheobronchial sleeve resection in children and adolescents, J Pediatr Surg, 1994;29(2):192–7.
  28. Spunt SL, Pratt CB, Rao BN, et al., Childhood carcinoid tumors: the St Jude Children's Research Hospital experience, J Pediatr Surg, 2000;35(9):1282–6.
  29. Rea F, Rizzardi G, Zuin A, et al., Outcome and surgical strategy in bronchial carcinoid tumors: single institution experience with 252 patients, Eur J Cardiothorac Surg, 2007;31(2):186–91.
  30. Rizzardi G, Marulli G, Bortolotti L, et al., Sleeve resections and bronchoplastic procedures in typical central carcinoid tumours, Thorac Cardiovasc Surg, 2008;56(1):42–5.
  31. . Deslauriers J, Gregoire J, Jacques LF, et al., Sleeve lobectomy versus pneumonectomy for lung cancer: a comparative analysis of survival and sites or recurrences, Ann Thorac Surg, 2004;77:1152–6.
  32. Lee GH, Dietrich RB, Pais B, et al., Pediatric case of the day. Neuroendocrine carcinoma (atypical carcinoid/Kulchitzky-cell carcinoma II), Radiographics, 1994;14(1):188–91.
  33. Neves GR, Chapchap P, Sredni ST, et al., Childhood carcinoid tumors: description of a case series in a Brazilian cancer center, Sao Paulo Med J, 2006;124(1):21–5.
  34. Curtis JM, Lacey D, Smyth R, et al., Endobronchial tumours in childhood, Eur J Radiol, 1998;29:11–20
  35. Toker A, Bayrak Y, Dilege S, et al., Bronchial sleeve resections for carcinoid tumor in the first decade of life, Interact Cardiovasc Thorac Surg, 2004;3(2):280–2.
  36. Hamad AM, Rizzardi G, Marulli G, Rea F, Nodal recurrence of pulmonary carcinoid 30 years after primary resection, J Thorac Oncol, 2008;3(6):680–1.
  37. Cavaliere S, Foccoli P, Toninelli C, Curative bronchoscopic laser therapy for surgically resectable tracheobronchial tumors, J Bronchology, 2002;9:90–5.
  38. Filosso PL, Rena O, Donati G, et al., Bronchial carcinoid tumors: surgical management and long-term outcome, J Thorac Cardiovasc Surg, 2002;123:303–9.
  39. Ferguson MK, Landreneau RJ, Hazelrigg SR, et al., Long-term outcome after resection for bronchial carcinoid tumors, Eur J Cardiothorac Surg, 2000;18:156–61.
  40. Cardillo G, Sera F, Di Martino M, et al., Bronchial Carcinoid tumors: nodal status and long-term survival after resection, Ann Thorac Surg, 2004;77:1781–5.
  41. Capovilla M, Kambouchner M, Bernier M, et al., Late cerebellar relapse of a juvenile bronchial carcinoid, Clin Lung Cancer, 2007;8(5):339–41.
  42. Andrassy RJ, Feldtman RW, Stanford W, Bronchial carcinoid tumors in children and adolescents, J Pediatr Surg, 1977;12(4):513–7.
  43. Brandt B 3rd, Heintz SE, Rose EF, Ehrenhaft JL, Bronchial carcinoid tumors, Ann Thorac Surg, 1984;38(1):63–5.
Keywords: Bronchial carcinoid tumours, paediatric lung neoplasm, typical carcinoid, lung surgery, bronchoscopy, sleeve resection, bronchoplasty
Customize This