Bronchial Carcinoid Tumours in Children – A Review


and prognosis are different. In adults TCs show a very favourable prognosis with a low local recurrence rate or distant metastases after radical resection, while ACs have demonstrated a poorer prognosis due to the higher oncological aggressiveness.7,11,38–40


Clinical behaviour is not as distinct between TCs and ACs in paediatric patients as it is in adults because of the limited number of cases.15,17,34 Very few ACs are described in the literature in children16,32,41


these, two had lymph-node metastases at the time of surgery but neither recurrence nor reduced survival was observed.16 presented mediastinal and cerebellar relapse 16 years later.41


Follow-up


Long-term follow-up is strongly recommended in BC. Despite low-grade malignancies, recurrences have been reported and their prompt diagnosis and treatment is important for long-term survival.


Prospective Future and, of


Another Despite


being low-grade malignant tumours, BCs can spread to lymph nodes or distantly in paediatric patients too. The percentage of N+ at the time of surgery in the major reports varies from 9 %17


to 11.8 %12 and then 20 %16


(see Table 1), but it is difficult to define the prognostic value of nodal metastasis in a such a small population.


Diagnosis of metastatic BC in children is, fortunately, very rare,25 but late


local recurrences or distant metastases may occur many years after a radical surgery.12,16,36


historical reports is 5–27 %,25,42,43


The reported rate of metastasis in children in but these papers are not reliable


because they could include bronchial adenomas and a recent histological revision has not been made.


When recurrence occurs, if technically possible, surgical treatment is recommended because it can probably prolong survival.16,36


From a meta-analysis approach, based on the survival reported by the four major studies (see Table 1), we can confirm that BCs in children, if promptly diagnosed and radically treated, have an excellent prognosis and a disease-free survival probability of 96 and 94 % at 10 and 20 years, respectively (see Figure 2).


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3. Fröhlich F, Die ‘Helle Zelle’ der Bronchialschleimhaut und ihre Beziehungen zum Problem der Chemoreceptoren, Frankfurter Z Pathol, 1949;60:517.


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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.


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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.


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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.


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Biological and molecular factors that influence the different behaviour of some carcinoid tumours have not been ascertained. There are tumours classified as TC with aggressive behaviour because they locally metastasise and have distant localisations. Various studies are in progress whose aim is to identify prognostic morphological and molecular markers that could help to define prognosis in TC and AC. Finding prognostic markers should allow the identification of TCs that are less aggressive and can benefit from endoscopic treatment. For the ACs with the worst prognosis, finding prognostic markers could lead to research for new specific drugs and biological treatments in the hope of obtaining better control over the disease. Future therapy of carcinoid tumours will be based on specific tumour biology, and treatment will be customised for each individual patient.


Conclusions


A BC tumour is an uncommon malignancy in paediatric patients. An early diagnosis is of fundamental importance because it allows prompt treatment. Operative bronchoscopy plays an important role in the diagnosis and treatment of obstruction, thus resolving symptoms, avoiding recurrent pneumonia, which could irreversibly damage the lung parenchyma and rule out a lung-sparing resection, and aiding in planning the correct operation. In experienced and skilled hands, conservative procedures with lymphadenectomy should be considered the treatment of choice for the management of paediatric BC, ensuring excellent survival and a good quality of life. A careful and prolonged follow-up is recommended. n


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2007;31(2):186–91.


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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.


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