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Pulmonary Pathology Online

Pathology of Neuroendocrine Tumours of the Lung

Dr Sampurna Roy MD

 

Neuroendocrine tumours form a spectrum of lesions ranging from very well differentiated neuroendocrine tumours (carcinoid tumors) to poorly differentiated malignancies with endocrine features, typified by small cell carcinoma.

Neuroendocrine differentiation is defined at the morphologic level by the presence of a typical organoid growth pattern, at the ultrastructural level by the demonstration of dense-core neurosecretory granules, and at the immunohistochemical level by the presence of immunoreactivity for neuroendocrine markers such as chromogranin A and synaptophysin, or peptide hormones such as serotonin, ACTH and bombesin.

An important clinical association of these carcinomas is the production of paraneoplastic syndromes due to aberrant expression of peptide hormones , including Cushing’s syndrome and the syndrome of inappropriate secretion of antidiuretic hormone.

The tumour usually occurs in adults but younger patients are being diagnosed more often nowadays.

The most interesting observation is that that neuroendocrine tumours of lung produce exclusively the alpha subunit of human chorionic gonadotropin (hCG).

Carcinoid Tumours:

Cytogenetically, carcinoid tumors are characterized by 11q deletions.     

Clinical manifestations depend on their size, location, and biologic activity.  

a) Central Carcinoid Tumours   are endobronchial, highly vascular and involve the main bronchus producing wheezing, cough, hemoptysis etc.

b) Peripheral Carcinoid Tumours often arise immediately beneath the pleura and are usually asymptomatic

About 12% of all carcinoids arise in the lung but less than 1% of lung tumours are carcinoids.

 

Surgical resection is curative in 90 to 95%.

 

About 10% are more aggressive exhibiting invasion or distant metastasis. 

 

Pulmonary Neuroendocrine Tumours are divided into 4 major categories:

 

- Typical Carcinoid - well differentiated carcinoma

- Atypical Carcinoid   - moderately differentiated carcinoma

- Small Cell Carcinoma - poorly differentiated  and

- Large Cell Neuroendocrine Carcinoma  - poorly differentiated .

 

Further reading:

The spectrum of neuroendocrine tumours. In: Sobin LH (ed). WHO International Histological Classification of Tumours. Histological Typing of Lung and Pleural Tumors. Springer-Verlag: Berlin, 1999, pp 7–9.

34BetaE12 expression along the whole spectrum of neuroendocrine proliferations of the lung, from neuroendocrine cell hyperplasia to small cell carcinoma.

Pulmonary neuroendocrine carcinomas. A review of 234 cases and a statistical analysis of 50 cases treated at one institution using a simple clinicopathologic classification. 

The surgical spectrum of pulmonary neuroendocrine neoplasms.

Differential distribution of the neuron-associated class III beta-tubulin in neuroendocrine lung tumors.

Carcinoid tumors of the lung. An analysis of 65 operated cases. 

Typical and atypical carcinoid tumors of the lung are characterized by 11q deletions as detected by comparative genomic hybridization. 

Analysis of p53, K-ras-2, and C-raf-1 in pulmonary neuroendocrine tumors. Correlation with histological subtype and clinical outcome.

Apoptosis-related factors p53, Bcl2, and Bax in neuroendocrine lung tumors.

Differential diagnostic patterns of lung neuroendocrine tumours. A clinico-pathological and immuno-histochemical study of 122 cases.

Bronchial carcinoid tumors: a retrospective analysis of 126 patients.

Neuroendocrine neoplasms of the lung. A clinicopathologic update.

Neuroendocrine neoplasms of the bronchopulmonary tract. A classification of the spectrum of carcinoid to small cell carcinoma and intervening variants.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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