Carcinoid Lung Tumour

Carcinoid lung tumour arises from the neuro-endocrine (Kulchitsky) cells scattered all over the lungs. These neuro endocrine cells are cells originally derived from the primitive cells that developed into the cells of the brain and spinal cord.

These cells retain their ability to secret substances into the blood stream.

Overgrowth of these cells out of normal body control leads to the development of carcinoid tumour.

Carcinoid lung tumours are very rare, and accounts for about 2.5% of all lung cancers and 15 % of carcinoid tumours any where in the body.

There are two kind carcinoid lung tumours. They are differentiated on the basis of their microscopic appearance (histology) and clinical outcome or prognosis. These are the typical carcinoid lung tumour and the atypical carcinoid lung tumour.

Typical Carcinoid Lung Tumour

These are also called Kulchitsky Cell Carcinoma type 1 or KCC1. They are the classical lung carcinoid tumours, and more commonly seen than the atypical variety. These typical carcinoid lung tumours can arise from the central (Trachea, bronchi, or main bronchus) airways or from the peripheral (alveoli, terminal bronchioles) airways.

The tumours arising from the central airways are usually 2.5 – 3cm in diameter. They tend to grow within the wall of the airway (endobronchial), causing localised swellings of the bronchi. These typical lung carcinoids secretes products that in turn leads to swellings of adjacent airways, causing significant airway obstruction more than would be expected from their original size.

Products secreted by carcinoid lung tumour include calcitonin, ACTH, 5 – hydroxytryptophan, serotonin and antiduretic hormone (ADH).

Release of these substances in large carcinoid lung tumour could lead to an attack of carcinoid tumour of lung origin (Fischer et al, 2001). Such carcinoid tumours of lung origin usually leads to left sided carcinoid heart disease, unlike the right sided carcinoid heart disease that is seen with carcinoid syndrome of gastrointestinal or liver origin. So carcinoid lung tumour can be a silient cause of abdominal pain too!

The typical carcinoid tumour of the lung, unlike most other lung tumours, is not associated with cigarette smoking. Atypical lung carcinoid tumours have been reported to be linked to cigarette.

Generally, typical lung carcinoids are slow growing tumours with very little cancerous change over time. They have a 5 and 10 year survival rate of 87%.

Atypical Carcinoid Lung Tumour

Atypical lung carcinoid tumours are also called Kulchitsky Cell Carcinoma type 2 or KCC 2. They are even rarer than the typical variety, and accounts for only 1 in 4 lung carcinoid tumours. These cells are often found in the peripheral lung, and are rapidly growing tumours, with very aggressive metastatic potential.

Their 5 and 10 years survival rates are around 50 and 35 % respectively.

Cigarette smoking is highly linked with increased incidence of atypical carcinoid lung tumour. Such association with smoking is evident by an increase in smoking related genetic changes like LOH and P53 mutation in atypical carcinoid tumour of the lung, compared with their typical counterpart.

See the following pages for other common sites of carcinoid tumours apart from the lungs:

Tumour of the Appendix and Gut

Tumour of the Liver

Diagnosis, Treatment and Prognosis of Carcinoid Tumours of The Lung

The diagnosis, treatment and prognosis of carcinoid tumour of the lung is discussed below along with carcinoids of other sites.

Diagnosis

Treament

Prognosis of carcinoid lung tumour and other carcinoids

References:

Bailey & Love’s Short Practice of Surgery. 23 rd Edition.

C. D. Knox, C. D. Anderson, L. W. Lamps, R. B. Adkins, and C. W. PinsonLong-Term Survival after Resection for Primary Hepatic Carcinoid TumorAnn. Surg. Oncol., December 1, 2003; 10(10): 1171 - 1175.

Lung Cancer: Principles and Practiceedited by Harvey I Pass, David P Carbone, John D Minna, Andrew T Turrisi, David H Johnson

General Thoracic Surgery By Thomas W Shields, Joseph Locicero, Ronald B Ponn, Valerie W Rusch


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