Pancoast Tumor
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A Pancoast tumor is a
tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
of the apex of the
lung The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side of t ...
. It is a type of
lung cancer Lung cancer, also known as lung carcinoma (since about 98–99% of all lung cancers are carcinomas), is a malignant lung tumor characterized by uncontrolled cell growth in tissue (biology), tissues of the lung. Lung carcinomas derive from tran ...
defined primarily by its location situated at the top end of either the right or left lung. It typically spreads to nearby tissues such as the
ribs The rib cage, as an enclosure that comprises the ribs, vertebral column and sternum in the thorax of most vertebrates, protects vital organs such as the heart, lungs and great vessels. The sternum, together known as the thoracic cage, is a semi- ...
and
vertebrae The spinal column, a defining synapomorphy shared by nearly all vertebrates,Hagfish are believed to have secondarily lost their spinal column is a moderately flexible series of vertebrae (singular vertebra), each constituting a characteristic i ...
. Most Pancoast tumors are non-small-cell lung cancers. The growing tumor can cause compression of a
brachiocephalic vein The left and right brachiocephalic veins (previously called innominate veins) are major veins in the upper chest, formed by the union of each corresponding internal jugular vein and subclavian vein. This is at the level of the sternoclavicular j ...
,
subclavian artery In human anatomy, the subclavian arteries are paired major arteries of the upper thorax, below the clavicle. They receive blood from the aortic arch. The left subclavian artery supplies blood to the left arm and the right subclavian artery supplie ...
,
phrenic nerve The phrenic nerve is a mixed motor/sensory nerve which originates from the C3-C5 spinal nerves in the neck. The nerve is important for breathing because it provides exclusive motor control of the diaphragm, the primary muscle of respiration. In ...
,
recurrent laryngeal nerve The recurrent laryngeal nerve (RLN) is a branch of the vagus nerve ( cranial nerve X) that supplies all the intrinsic muscles of the larynx, with the exception of the cricothyroid muscles. There are two recurrent laryngeal nerves, right and ...
,
vagus nerve The vagus nerve, also known as the tenth cranial nerve, cranial nerve X, or simply CN X, is a cranial nerve that interfaces with the parasympathetic control of the heart, lungs, and digestive tract. It comprises two nerves—the left and right ...
, or, characteristically, compression of a sympathetic
ganglion A ganglion is a group of neuron cell bodies in the peripheral nervous system. In the somatic nervous system this includes dorsal root ganglia and trigeminal ganglia among a few others. In the autonomic nervous system there are both sympatheti ...
(the
stellate ganglion The stellate ganglion (or cervicothoracic ganglion) is a sympathetic ganglion formed by the fusion of the inferior cervical ganglion and the first thoracic (superior thoracic sympathetic) ganglion, which exists in 80% of people. Sometimes, the seco ...
), resulting in a range of symptoms known as
Horner's syndrome Horner's syndrome, also known as oculosympathetic paresis, is a combination of symptoms that arises when a group of nerves known as the sympathetic trunk is damaged. The signs and symptoms occur on the same side (ipsilateral) as it is a lesion o ...
. Pancoast tumors are named for Henry Pancoast, an American
radiologist Radiology ( ) is the medical discipline that uses medical imaging to diagnose diseases and guide their treatment, within the bodies of humans and other animals. It began with radiography (which is why its name has a root referring to radiatio ...
, who described them in 1924 and 1932.


Signs and symptoms

Aside from
constitutional symptoms Signs and symptoms are the observed or detectable signs, and experienced symptoms of an illness, injury, or condition. A sign for example may be a higher or lower temperature than normal, raised or lowered blood pressure or an abnormality showin ...
of
cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...
such as malaise, fever, weight loss and fatigue, Pancoast tumor can include a complete Horner's syndrome in severe cases:
miosis Miosis, or myosis (), is excessive constriction of the pupil.Farlex medical dictionary
citing: ...
(constriction of the pupils),
anhidrosis Hypohidrosis is a disorder in which a person exhibits diminished sweating in response to appropriate stimuli. In contrast with hyp''er''hidrosis, which is a socially troubling yet often benign condition, the consequences of untreated hypohidrosi ...
(lack of sweating), ptosis (drooping of the eyelid), and pseudoenophthalmos (as a result of the ptosis). In progressive cases, the
brachial plexus The brachial plexus is a network () of nerves formed by the anterior rami of the lower four cervical nerves and first thoracic nerve ( C5, C6, C7, C8, and T1). This plexus extends from the spinal cord, through the cervicoaxillary canal in th ...
is also affected, causing
pain Pain is a distressing feeling often caused by intense or damaging stimuli. The International Association for the Study of Pain defines pain as "an unpleasant sensory and emotional experience associated with, or resembling that associated with, ...
and
weakness Weakness is a symptom of a number of different conditions. The causes are many and can be divided into conditions that have true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, i ...
in the muscles of the
arm In human anatomy, the arm refers to the upper limb in common usage, although academically the term specifically means the upper arm between the glenohumeral joint (shoulder joint) and the elbow joint. The distal part of the upper limb between th ...
and
hand A hand is a prehensile, multi-fingered appendage located at the end of the forearm or forelimb of primates such as humans, chimpanzees, monkeys, and lemurs. A few other vertebrates such as the koala (which has two opposable thumbs on each "h ...
with a symptomatology typical of thoracic outlet syndrome. The tumor can also compress the
recurrent laryngeal nerve The recurrent laryngeal nerve (RLN) is a branch of the vagus nerve ( cranial nerve X) that supplies all the intrinsic muscles of the larynx, with the exception of the cricothyroid muscles. There are two recurrent laryngeal nerves, right and ...
and from this a
hoarse voice A hoarse voice, also known as dysphonia or hoarseness, is when the voice involuntarily sounds breathy, raspy, or strained, or is softer in volume or lower in pitch. A hoarse voice, can be associated with a feeling of unease or scratchiness in the ...
and "bovine" (non-explosive) cough may occur. In superior vena cava syndrome, obstruction of the
superior vena cava The superior vena cava (SVC) is the superior of the two venae cavae, the great venous trunks that return deoxygenated blood from the systemic circulation to the right atrium of the heart. It is a large-diameter (24 mm) short length vein th ...
by a tumor (mass effect) causes facial swelling
cyanosis Cyanosis is the change of body tissue color to a bluish-purple hue as a result of having decreased amounts of oxygen bound to the hemoglobin in the red blood cells of the capillary bed. Body tissues that show cyanosis are usually in locations ...
and dilatation of the veins of the head and neck. A Pancoast tumor is an apical tumor that is typically found in conjunction with a history of smoking tobacco. The clinical signs and symptoms may be mistaken for neurovascular compromise at the level of the
superior thoracic aperture The superior thoracic aperture, also known as the thoracic outlet, or thoracic inlet refers to the opening at the top of the thoracic cavity. It is also clinically referred to as the thoracic outlet, in the case of thoracic outlet syndrome. A low ...
. The patient's smoking history, rapid onset of clinical signs and symptoms, and pleuritic pain can suggest an apical tumor. A Pancoast tumor can give rise to both Pancoast syndrome and Horner's syndrome. When the brachial plexus roots are involved, it will produce Pancoast syndrome; involvement of
sympathetic fibers The autonomic nervous system (ANS), formerly referred to as the vegetative nervous system, is a division of the peripheral nervous system that supplies internal organs, smooth muscle and glands. The autonomic nervous system is a control system th ...
as they exit the cord at T1 and ascend to the superior cervical ganglion will produce Horner's syndrome.


Diagnosis

Diagnosis of Pancoast tumor is usually made after evaluating clinical symptoms and imaging. Chest X-ray is a good screening test even though a CT scan of the chest can provide a better resolution and extent to which internal organs are being compressed.


Treatment

The treatment of a Pancoast lung cancer may differ from that of other types of non-small-cell lung cancer. Its position and close proximity to vital structures (such as nerves and the spine) may make surgical intervention difficult. As a result, and depending on the stage of the cancer, treatment may involve
radiation therapy Radiation therapy or radiotherapy, often abbreviated RT, RTx, or XRT, is a therapy using ionizing radiation, generally provided as part of cancer treatment to control or kill malignant cells and normally delivered by a linear accelerator. Radia ...
and
chemotherapy Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...
given prior to surgery (
neoadjuvant Neoadjuvant therapy is the administration of therapeutic agents before a main treatment. One example is neoadjuvant hormone therapy prior to radical radiotherapy for adenocarcinoma of the prostate. Neoadjuvant therapy aims to reduce the size o ...
treatment). Surgery may consist of the removal of the upper lobe of a lung together with its associated structures (
subclavian artery In human anatomy, the subclavian arteries are paired major arteries of the upper thorax, below the clavicle. They receive blood from the aortic arch. The left subclavian artery supplies blood to the left arm and the right subclavian artery supplie ...
,
vein Veins are blood vessels in humans and most other animals that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are the pulmonary and umbilical veins, both of which carry oxygenated b ...
, branches of the
brachial plexus The brachial plexus is a network () of nerves formed by the anterior rami of the lower four cervical nerves and first thoracic nerve ( C5, C6, C7, C8, and T1). This plexus extends from the spinal cord, through the cervicoaxillary canal in th ...
, ribs and
vertebral bodies The spinal column, a defining synapomorphy shared by nearly all vertebrates,Hagfish are believed to have secondarily lost their spinal column is a moderately flexible series of vertebrae (singular vertebra), each constituting a characteristic ...
), as well as
mediastinal The mediastinum (from ) is the central compartment of the thoracic cavity. Surrounded by loose connective tissue, it is an undelineated region that contains a group of structures within the thorax, namely the heart and its vessels, the esophagu ...
lymphadenectomy Lymphadenectomy or lymph node dissection is the surgical removal of one or more groups of lymph nodes. It is almost always performed as part of the surgical management of cancer. In a regional lymph node dissection, some of the lymph nodes in the ...
. Surgical access may be via
thoracotomy A thoracotomy is a surgical procedure to gain access into the pleural space of the chest. It is performed by surgeons (emergency physicians or paramedics under certain circumstances) to gain access to the thoracic organs, most commonly the hea ...
from the back or the front of the chest and modifications. Nonsurgical treatment may consist of radiation therapy alone or clinical trials of new combinations of treatment. Image:Pancoast_Tumor_2.jpg, CT scan showing a Pancoast tumor (labeled as P, non-small cell lung carcinoma, left lung), from a 47-year-old female smoker


References


External links

*
Pulmonary sulcus tumor
entry in the public domain NCI Dictionary of Cancer Terms {{Authority control Lung cancer Syndromes affecting the lung