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Pancoast Syndrome
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Pancoast Syndrome
   Pancoast tumor refers to lung cancer (usually squamous) located in the apex of the lung, extending to involve the eighth cervical and first and second thoracic nerves. This results in shoulder pain radiating in an ular distribution down the arm (Pancoast syndrome).Clinically, pain in arm and shoulder secondary to apical tumor invading brachial plexus and sympathetic chain. A Pancoast tumor may also involve the cervical sympathetic nerves Sympathetic involvement leads to ispilateral Horner's syndrome: and cause Horner syndrome (enophthalmos, ptosis and miosis).
Invasion of chest wall, ribs, spine may occur. May be difficulate to differentiate from benign apical capping on CXR. CT or MR useful for pre-operative/RT assessment. Symptoms
The usual modes of presentation of pancoast tumor are:
Arthritis" of Shoulder:
Patients often present to a Rheumatologist or an Orthopedic Surgeon because of shoulder pain. It is not uncommon for these patients to be treated as having arthritis for some time until a CXR is ordered.
Root Pain in the Ulnar Nerve Distribution:
These patients often consult a Neurologist because of nerve root pain along the distribution of the ulnar nerve.
Routine CXR Abnormalities
Occasionally, a patient is referred because of the abnormality detected on a routine chest x-ray. The second finding you should look for is rib destruction.
"Routine Lung Cancer Symptoms"
Some patients present with the classical symptoms of cough, hemoptysis, anorexia and weight loss attributable to a lung lesion.
Physical Findings
You should specifically look for the following in patients suspected to have a pancoast tumor:
 Horner's Syndrome Brachial Plexus Involvement Tenderness and Swelling of the Supraclavicular Fossa and Posterior Upper Back Loss of Mobility of the Apex with Mueller's and Valsalva Maneuvers Loss of Mobility of ApexAn infiltrative tumor at the apex will tether the soft tissue of the supraclavicular fossa and prevent mobility of this region during the Mueller's and Valsalva maneuvers
Tenderness and SwellingTenderness and swelling over the upper posterior chest and supraclavicular fossa should be sought. The superior sulcus lies over the first and second thoracic ribs and are often invaded by tumor.
 The neural structures involved by direct extension of a tumor in the superior sulcus. The shaded area indicates the region of potential involvement, usually including C8, T1, and T2 nerve roots; the lower trunk of the brachial plexus; and the sympathetic chain. Pancoast tumors cause symptoms due to their anatomic location in the superior sulcus of the lung. This clinical syndrome is due to tumor growth in the superior sulcus at the lung apex and is related to invasion and destruction of neural structures in the area . Pancoast's syndrome is commonly manifested by: pain in the shoulder or medial portion of the scapula. radicular pain with or without muscle wasting in the distribution of the ulnar nerve to the elbow (T1 distribution) and/or medial forearm and hand (C8 distribution). Horner's syndrome (ptosis, miosis, hemianhydrosis, enopthalmus). Pancoast tumors cause symptoms due to their anatomic location in the superior sulcus of the lung. The clinical syndrome was first described by Edwin Hare, however, it was named after Henry Pancoast who defined the findings in 1932. This clinical syndrome is due to tumor growth in the superior sulcus at the lung apex and is related to invasion and destruction of neural structures in the area. Pancoast's syndrome is commonly manifested by: pain in the shoulder or medial portion of the scapula. radicular pain with or without muscle wasting in the distribution of the ulnar nerve to the elbow (T1 distribution) and/or medial forearm and hand (C8 distribution). Horner's syndrome (ptosis, miosis, hemianhydrosis, exophthalmus). Although this is a lung tumor, pulmonary symptoms are rare. The most common initial presentation is pain localized to the shoulder which is found in 90% of cases. Consequently, many cases are initially misdiagnosed as shoulder arthritis. Continued tumor growth leads to sympathetic chain involvement with a Horner's syndrome in 62% of cases. Because these tumors commonly invade the chest wall, they are staged as T3. Other common findings at presentation include invasion or destruction of ribs in 45% of cases and invasion or destruction of vertebrae in 23%. The most common cell type causing Pancoast syndrome is squamous cell carcinoma (52%) with adenocarcinoma and large cell (both 23%) being less common causes. Small cell carcinoma is a rare cause of Pancoast syndrome (1%). Radiographically the tumor is often difficult to see and may only be visible as a vague cap at the lung apex, similar to pleural thickening. CT and MRI are helpful in determining the extent of involvement and invasion of adjacent structures. MRI more clearly delineates tumor extension than CT primarily because MRI offers coronal and sagittal imaging with superior definition of vascular and neural structures. These more advanced imaging techniques are the key to determining resectability which requires accurate definition of vascular, neural, and bony invasion.  CXR Findings In patients suspected to have a pancoast lesion, you should carefully inspect both apices. Sometimes all you see is a sliver of apical density which can be mistaken for benign apical capping. The second finding you should specially look for is rib destruction. Make it a habit to compare ribs of both sides one at a time. Occasionally, the entire first rib may be destroyed and a casual perusal may fail to detect the abnormality. Drooping of the shoulder detected by CXR may be suggestive of brachial plexus involvement. In summary, the following should be specifically looked for in patients suspected to have a pancoast tumor Apical Cap Apical Mass Rib Destruction Drooping of Shoulder In some patients, additional radiological procedures are of help: Lordotic Views: If the lesion is hidden by a clavicle, a lordotic view will bring the lesion into better view. Rib Films: If the PA view does not clearly show rib destruction, rib films will be of help. Bone Scan: An occasional case where abnormal bone scan is the presenting abnormality. CT Scan: CT scan should be routinely obtained for better evaluation of chest wall extension, rib and vertebral destruction, mediastinal nodes and extent of tumor.  . Those patients staged N0 have a better prognosis than those with nodal metastases. Today, the standard of care for patients with a Pancoast tumor is radiation followed by tumor and chest wall resection when feasible. There are no definite criteria for unresectability, but the following general guidelines have been proposed:
N2 nodal disease extensive vertebral body invasion distant metastases superior vena cava syndrome. Retrospective study has suggested that radiotherapy followed by surgical resection is the best treatment available for operable patients with T3 N0 or N1 disease. For unresectable patients radiotherapy is important for palliative care, but rarely produces a long term cure (6% survival at 5 years).
treatment
 treatment
Options
Supposing that the patient has T3 No MO squamous cell carcinoma, click on the modality that is associated with the best survival.
External Radiation Only Surgical Resection Radiation Followed by Surgery Chemotherapy Pre-Op Radiation
High dose pre-operative radiotherapy using current techniques and fractionation (5,500 to 6,500 cGy) followed by en bloc resection has been reported with long term survival.
The purpose of pre-op radiation is to modify the extent of disease so the lesion is better localized and more completely resectable. The major effects of pre-op radiation are:
Limiting growth of tumor Blocks lymphatic channels Destroys malignant cells in perineural sheaths Pre-operative irradiation is commonly given over two weeks for a total dose of 3,000 rads. The interval between radiation and surgical therapy does not exceed 3-4 weeks. The three anatomical sites of the chest to which the external radiation is directed include:
Primary Tumor Adjacent Mediastinum Ipsilateral Supraclavicular Area Surgery
The surgical technique for resection of the superior sulcus tumor is an extended en bloc resection of the chest wall, usually including:
First three ribs Portions of the upper thoracic vertebrae Intercostal nerves Lower trunk of the brachial prexus Stellate ganglion Portion of the dorsal sympathetic chain Resection of the involved lung Surgery Contraindications
The following lists major contraindications for surgery:
 Tumor invading the great vessels -subclavian art and vein Phrenic or recurrent laryngeal nerve paralysis Invasion of the trachea or esophagus Extensive brachial plexus involvement Extensive vertebral body and spinal canal involvement Mediastinal lymph node involvement Surgical Morbidity
Mortality: You can appreciate that this is a major procedure from its extensive surgical intervention. The mortality of this procedure within the first 30 postoperative days is 3-5% and the associated morbidity is 13%. The usual pulmonary and infectious complications account for this major morbidity. In the usual case, nerves C8 through T3 must be sacrificed along with the stellate ganglion and upper fibers of the sympathetic chain. This produces numbness along the ulnar distribution of the hand and undersurface of the arm to the elbow and loss of fine motions of the hand leading to the loss of manual dexterity, such as writing, needle worka clinical case
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apical density (superior pulmonary sulcus)