Cancer of Pankosta
Oncologists diagnose Pancostic cancer when a malignant tumor - the primary lung carcinoma - occurs on its apex (apex pulmonis), invading any of the nearby structures or exerting pressure on them.
Professor of Radiology, University of Pennsylvania (USA) Henry Pancost, who described this neoplasm in the first third of the last century, identified it as an apical (apical) tumor of the lung.
Another name for this pathology is known - lung cancer with Pancost syndrome.
Epidemiology
Among all lung cancer, Pancostic cancer accounts for no more than 5%. It is rare in young people, and the majority of patients are of age category 40+. And, basically, it's smoking men and women.
For example, in the UK, lung cancer is diagnosed annually in approximately 44,500 people, and in the United States (according to the National Institutes of Health) - more than 200 thousand. The most common type of tumors is non-small cell, accounting for more than 80% of cases.
Causes of the cancer of Pankost
Although lung cancer can occur, and those who have never smoked, oncologists associate the key reasons for its development - no less than 85% of cases - with smoking. And they explain this by the fact that smokers regularly inhale with smoke almost two hundred toxic and more than four dozen carcinogenic substances. No less lungs suffer from so-called passive smoking, that is, someone smokes, and cigarette smoke, containing carcinogens - polycyclic aromatic hydrocarbons - inhale others.
Risk factors
Pulmonologists take into account such risk factors for the formation of pulmonary malignant tumors, as an aggressive effect on lung tissue formaldehyde, radon, airborne asbestos dust, industrial gaseous emissions, automobile exhaust, etc.
Also, malignant neoplasms in the lungs, including lung cancer with Pancost syndrome, can develop if there is a genetic predisposition (chromosomal abnormalities).
Pathogenesis
The pathogenesis of the Pancoast tumor is due to both its localization and related adjacent structures.
Appearing at the apex of the lung - at the upper thoracic aperture (upper thoracic aperture) and in the region of the upper sulcus (superior sulcus) formed by the subclavian artery, these bronchogenic tumors gradually spread to the upper ribs, periosteum, the vertebral bodies of the thoracic spine; cause compression of the sympathetic thoracic nerves, stellate ganglion of the neck (stellate ganglion), trunks and nerve roots of the brachial plexus (plexus brachialis).
Under the pressure of the neoplasm narrowed lumens of subclavian vessels and lymphatic vessels.
In terms of histology, Pancoast tumors are squamous cell carcinomas (up to 45-50% of cases), adenocarcinomas (36-38%), undifferentiated large cell carcinomas (11-13%), and small cell carcinomas (2-8%).
Symptoms of the cancer of Pankost
Such symptoms as usual with pulmonary oncology, such as cough with bloody sputum and shortness of breath (dyspnea), in Pancoast cancer - due to the peripheral location of these tumors - are not usually observed in the initial stages of the disease. And the sequence of symptoms in Pancoast cancer often leads to diagnostic errors.
The fact is that the first symptoms of a Pancoast tumor that spreads into the chest wall and brachial plexus are manifested by pains in the shoulder and elbow radiating in the forearm, neck, sternum, armpit and shoulder blade on the side of the tumor. And the sooner paresthesia of the half of the fourth and fifth fingers of the hand, the muscle weakness (atrophy) from the inside of the hand indicates the compression of the nerves by the tumor. Actually, this set of clinical signs is Pancost syndrome in lung cancer or Pancost-Tobias syndrome.
As the disease progresses and the tumor alteration of the sympathetic trunks of the chest nerves and the stellate ganglion of the neck occurs Bernard-Horner syndrome - with the partial lowering of the upper eyelid (ptosis) by narrowing the pupil of the same eye (miosis), deepening the eyeball into orbit (enophthalmos) and almost complete cessation sweating (anhidrosis) on the ipsilateral side of the face.
By the way, this syndrome is observed in 25% of patients with a pulmonary tumor localized in the mediastinum, which is diagnosed as a mediastinal form of lung cancer. But with the X-ray study clearly shows that this tumor is located in the tracheobronchial tree, which is usually the first involved in the pathological process.
Complications and consequences
Due to the fact that Pancost's cancer is often diagnosed too late, and the proliferative activity of such a tumor is high, it is simply impossible to prevent its consequences and complications - metastasis.
As the experts note, such tumors are detected in stages T3 - IIIa or IIIb (according to TNM Classification of Malignant Tumors), and if the vertebral bodies, nerve trunks or blood vessels are involved in the pathological process, the tumor rises to stage T4.
First of all, metastases affect adjacent structures, regional lymph nodes (supraclavicular, thorax and mediastinum), bones and brain. According to some reports, cerebral metastases develop in 24-55% of cases; in 36% they are remote.
When the tumor sprouts on the vertebral bodies (which is observed in 10-15% of patients), it can lead to compression of the spinal cord and paraplegia - paralysis of the lower body and both legs.
Diagnostics of the cancer of Pankost
At an early stage, the diagnosis of Pancostic cancer is extremely difficult: the clinic and patient complaints are not characteristic for malignant lung diseases.
Instrumental diagnostics, including:
- Chest X-ray;
- computed tomography of the chest (CT);
- magnetic resonance imaging (MRI):
- Positron Emission Computed Tomography (PET-CT) of the whole body.
In the early stages - due to the lack of a clear visualization and a large number of shadows of nearby structures - it is difficult to detect Pancostic cancer on the x-ray. Radiography of the chest can reveal asymmetry of the apex of the lungs (a small area of thickening of the pleura in the apical zone of one lung); increase in tissue mass; damage to 1-3 ribs or parts of the vertebrae.
Cancer of the Pancake on the CT of the chest is determined by the presence in the upper thoracic aperture and more accurately the pulmonary furrow pathological tissue formation and its penetration into the chest wall, spine, blood vessels, nerves or space between the lungs.
But MRI is recommended to determine a more complete picture of the local spread of tumor cells and the amount of involvement of nerve endings.
To establish an accurate diagnosis, endoscopic mediastinoscopy is also used - for examination of lymph nodes. And if there is palpable adenopathy of the supraclavicular lymph nodes, conduct their fine-needle transdermal aspiration.
Analyzes for confirming the diagnosis of a tumor and for an accurate assessment of its stage are a biopsy (tumor cells) obtained by transthoracic fine needle aspiration biopsy. In some situations, endoscopic or open thoracotomy may be required for histological examination of the tumor.
Differential diagnosis
Differential diagnosis should distinguish Pancoast tumor from: lymphogranulomatosis and lymphoma, pleural mesothelioma, echinococcal cyst of the lung, carcinoma of the thyroid and adenoid cystic carcinoma, desmoid mediastinal tumors, breast cancer, as well as stair and cervical syndrome.
Treatment of the cancer of Pankost
The positive result - cessation of tumor growth and its regression, reduction of local and long-term relapses and an increase in survival rate - provides treatment with induction chemoradiotherapy and subsequent surgical treatment - resection of affected structures.
Types of chemotherapy in cancer Pankosta:
- before surgery - a combination of certain drugs for chemotherapy with radiation for 5-6 weeks;
- after the removal of part or all of the lung, affected adjacent tissues or upper ribs (which is carried out after the course of chemoradiotherapy) - the final postoperative chemotherapy.
Chemotherapy regimens use cystostatic Cisplatin (Platinotin) in combination with other antitumor drugs, in particular, Etoposide and Windesin (Eldisin).
For example, intravenously administered Cisplatin refers to platinum derivatives; The drug is effective, but like all anti-cancer cytotoxic agents cause many side effects and negative consequences. The most frequent consequences of chemotherapy in cancer of Pankost are described in detail in publications:
Regimes of radiation, that is, radiation therapy for Pancost's cancer, usually include fractions of 45 Gy / 27 for 5-6 weeks, after which (after 4-6 weeks) they undergo an operation.
In this contraindication to surgical treatment are metastases, lesions of supraclavicular and mediastinal lymph nodes, more than half
Bodies of vertebrae, trachea and esophagus.
Patients with an inoperable Pancoast tumor are given palliative care.
Forecast
As with other malignant neoplasms, the prognosis for patients with Pancostic cancer directly depends on the stage of the disease. A poor prognostic factor is the presence of symptoms of the Bernard-Horner syndrome.
Over the past few decades, survival rates of patients with lung cancer with Pancost syndrome have increased significantly.
After induction chemoradiotherapy and subsequent surgery in 33-40% of cases (to other data, 54-72%), the average life expectancy is five years, and the incidence of complications varies in the range of 10-28%.
Nearly 75% of patients continue to live for two years.