AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

In 1932, Pancoast defined a superior pulmonary sulcus tumor as a mass growing at the thoracic inlet that produces a constant and characteristic clinical presentation of pain in an eighth cervical or first and second thoracic trunk distribution.¹

Pancoast tumors are a subset of lung cancers that invades the apical chest wall. (For information on various lung cancers, see Medscape’s Lung Cancer Resource Center.) Because of their location in the pleural apex, they invade adjoining tissue. Although various other tumors may produce a similar clinical presentation because of their location at the thoracic inlet, the most common cause is generally believed to be a bronchogenic carcinoma arising in or near the superior sulcus and invading the adjacent extrathoracic structures by direct extension. The location of the tumor, rather than its pathology or histology of origin, is significant in producing its characteristic clinical pattern.¹

The bulk of a true Pancoast tumor is extrathoracic, originating in an extreme peripheral location with a plaquelike extension over the lung apex and principally involving the chest wall structures rather than the underlying lung parenchyma. Bronchogenic carcinomas occurring in the narrow confines of the thoracic inlet invade the lymphatics in the endothoracic fascia and include, by direct extension, the intercostal nerves, the lower roots of the brachial plexus, the stellate ganglion, the sympathetic chain, and adjacent ribs and vertebrae.

Carcinomas in the superior pulmonary sulcus produce Pancoast syndrome, thus causing pain in the shoulder and along the ulnar nerve distribution of the arm and hand.² (These carcinomas also cause Horner syndrome.) These apical lung tumors tend to be locally invasive early. In the absence of metastases and regional nodal involvement, these apical cancers can be successfully treated.

Careful assessment and appropriate staging is performed prior to surgery, and selected patients are administered preoperative irradiation of 30 Gy over 2 weeks. After an interval of 2-4 weeks, surgical resection of the chest wall and lower brachial plexus and en bloc lung resection produces a 5-year survival rate of 30%. Contraindications to surgery include an extension of the tumor into the neck or vertebrae, the presence of substantial mediastinal lymph nodes, and peripheral tumor dissemination. Newer protocols that use combinations of irradiation, chemotherapy, and surgery are currently being studied to determine the best therapy.

History of the Procedure

Henry Pancoast first described the clinical features of an apical lung mass in his presidential address to the American Medical Association in 1932. He described this apical tumor as being associated with rib destruction, Horner syndrome, and atrophy of the hand muscles.

For the next 20 years, all attempts at curative resection of this tumor failed, and the tumor was determined to be inoperable. However, in the late 1980s, the best management of this tumor was believed to consist of preoperative radiation followed by surgical resection. The operation consisted of en bloc resection of the chest wall (including the ribs and the transverse process), intercostal nerves, and the lower trunk of the brachial plexus together with a lung resection. This combined modality resulted in 5-year survival rates of 30-35% in selected patients.

Problem

In 1932, Pancoast defined superior pulmonary sulcus tumors as masses growing at the thoracic inlet, producing a constant and characteristic clinical presentation of pain in an eighth cervical or first and second thoracic trunk distribution. Pancoast tumors are a subset of lung cancers that invades the apical chest wall.

Frequency

Pancoast tumors are much less common than other lung cancers. In past series, the rate of Pancoast tumors varied from 1-3% of all lung cancers. A major issue with Pancoast tumors is the delay in diagnosis. The apical lung cancer may not be visualized on an initial chest radiograph, and, by the time the patient presents with symptoms, the tumor has almost always invaded nearby structures. In addition, the symptoms produced by the disorder can be mimicked by numerous neurological or musculoskeletal disorders, thus delaying diagnosis.

Etiology

The risk factors for almost all lung cancers are similar. These include prior prolonged asbestos exposure, exposure to industrial elements (eg, gold, nickel), smoking, and secondary smoke exposure.

Pathophysiology

The mass in the superior sulcus is an extension of a lung tumor; most of it lies outside the lung and involves the chest wall, nerve roots, lower trunks of the brachial plexus, sympathetic chain, stellate ganglion, ribs, and bone. Most Pancoast tumors are squamous cell carcinomas or adenocarcinomas. Only 3-5% are small cell carcinomas. Squamous cell carcinoma occurs more frequently, although large cell and undifferentiated types are also common. Adenocarcinoma is sometimes found in this location and can even be metastatic. Involvement of the phrenic or recurrent laryngeal nerve or superior vena cava obstruction is not representative of the classic Pancoast tumor.

Clinical

Symptoms

The symptoms are typical of the location of the tumor in the superior sulcus or thoracic inlet adjacent to the eighth cervical nerve roots, the first and second thoracic trunk distribution, the sympathetic chain, and the stellate ganglion. Initially, localized pain occurs in the shoulder and vertebral border of the scapula. Pain may later extend along an ulnar nerve distribution of the arm to the elbow and, ultimately, to the ulnar surface of the forearm and to the small and ring fingers of the hand (C8). If the tumor extends to the sympathetic chain and stellate ganglion, Horner syndrome and anhidrosis develop on the ipsilateral side of the face and upper extremity. Pain is frequently relentless and unremitting, often requiring narcotics for relief. The patient usually supports the elbow of the affected arm in the hand of the opposite upper extremity to ease the tension on the shoulder and upper arm.

The hand muscles may become weak and atrophic, and the triceps reflex may be absent. The first or second rib or vertebrae may be involved by tumor extension and intensify the severity of pain. The spinal canal and spinal cord may be invaded or compressed, with subsequent symptoms of spinal cord tumor or cervical disk disease. Confusion with thoracic outlet syndrome and cervical disk disease is common in the early clinical course. Careful neurologic examination, electromyographic studies, and ulnar nerve studies are performed to verify the precise diagnosis.

Infrequently, a patient with a Pancoast tumor may also have features of a paraneoplastic syndrome. Most of the metabolic manifestations are the result of the secretion of endocrine chemicals by the tumor. Manifestations encompass Cushing syndrome, excessive antidiuretic hormone secretion, hypercalcemia, myopathies, hematological problems, and hypertrophic osteoarthropathy. The presence of paraneoplastic syndromes does not connote unresectability, but most of these are associated with small cell cancer. Brain metastasis may be relatively common at the point of diagnosis. The brain is the frequent site of failure for superior sulcus tumors. Brain imaging studies prior to surgery are highly recommended in patients who are receiving induction therapy for the primary tumor.³

Differential diagnoses

Differential diagnoses of masses in the apical chest include primary tumors of the thyroid, larynx, and pleura. Other causes may include infectious disorders of the lung, aneurysms of the subclavian vessels, amyloid of pleura, and multiple myeloma. The differential diagnoses of arm and shoulder pain are extensive; however, primarily, thoracic outlet syndrome and cervical disk disease must be excluded.

Diagnosis

In the very rare patient, sputum cytology has been helpful. Initially, most Pancoast tumors are diagnosed histologically based on transthoracic needle biopsy results. Diagnosis via bronchoscopy is less helpful because most of these tumors are peripherally located. The flexible scope is more useful than the rigid scope in obtaining bronchoscopic aspirates and brush biopsy specimens.

Liver, bone, and brain scans are performed to determine the presence of any metastatic disease. Although more than 90% of patients can be correctly diagnosed based on clinical and radiological findings alone, open biopsy of the tumor for pathological validation may be performed through a supraclavicular incision. Results from a needle biopsy through the supraclavicular or posterior triangle are also successful in confirming the diagnosis and in delineating the cell type prior to treatment. Even though clinical diagnosis is relatively simple, performing a tissue biopsy is still necessary.

Staging

Staging is determined by the location of the lesion and its metastases. The true Pancoast tumor is usually T3, which describes the extension of the tumor through the visceral pleura into the parietal pleura and the chest wall. Pancoast tumors are classified as T4 when mediastinal invasion, cervical invasion, or both have occurred. Peripheral metastases signal a poor prognosis, and surgery is contraindicated in such cases.

Mediastinoscopy is used for staging to delineate the metastases to mediastinal lymph nodes. Cervical mediastinoscopy is indicated for right pulmonary lesions; a Chamberlain procedure (left second interspace mediastinoscopy) is indicated for left pulmonary lesions. Generally, mediastinoscopy is performed if the lymph nodes appear larger than 1 cm in diameter on a CT scan because the accuracy of CT scan results for predicting metastatic involvement in enlarged lymph nodes is only 70%. Conversely, if the CT scan does not reveal any enlarged lymph nodes, the patient is deemed operable. If the nodes in the mediastinum are positive, the prognosis is poor. The exception to this rule is an upper lobe lesion with positive nodes on the right side of the trachea only. If these are internodal, spread is considered local, and the tumor may still be resectable.

INDICATIONS

Section 3 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Pancoast tumors were once considered universally fatal. However, improvements in combined modality therapy and the development of new techniques for resection have made curative treatment possible for these tumors.⁴ Patients with the diagnosis of superior sulcus pulmonary carcinoma should be considered for surgery after the appropriate diagnostic evaluation is concluded. The perfect candidate has a carcinoma restricted to the chest with T3N0M0 staging. A rare exception is made for a right upper lobe lesion with intranodal mediastinal metastases and T3N2M0 staging. However, surgery alone is not the prevalent course of treatment. Surgery is indicated in patients who have very localized early disease. The occasional inoperable patient with severe pain after irradiation therapy may be selectively considered for palliative resection.

RELEVANT ANATOMY

Section 4 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

In 1932, Pancoast defined superior pulmonary sulcus tumors as masses growing at the thoracic inlet that produce a constant and characteristic clinical presentation of pain in an eighth cervical or first and second thoracic trunk distribution.

Pancoast tumors are a subset of lung cancers that invades the apical chest wall. Because of their location in the pleural apex, they invade adjoining tissue. Although various other tumors may produce a similar clinical presentation by virtue of their location at the thoracic inlet, the most common cause is generally believed to be a bronchogenic carcinoma arising in or near the superior sulcus and invading the adjacent extrathoracic structures by direct extension. The location of the tumor, rather than its pathology or histology of origin, is significant in producing its characteristic clinical pattern.

CONTRAINDICATIONS

Section 5 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Contraindications to surgery include (1) extensive invasion of the neck, brachial plexus, or vertebrae; (2) perinodal mediastinal extension; and (3) peripheral metastases. These include extrathoracic metastatic disease and positive mediastinal nodes. Complete upper and lower brachial plexus invasion is a relative contraindication, provided complete surgical excision can be performed. Vertebral body involvement should not be a contraindication unless invasion of the cortex is confirmed.

Patients with poor respiratory function and ischemic coronary artery disease need a proper workup prior to surgery. Heart failure, recent myocardial infarction, and unstable angina are contraindications for surgery. Although many patients are elderly (the age group with the highest risk of complications), age alone is relatively unimportant if the patient is in otherwise good health.

Contraindications to surgery can be summarized as follows:

• Extensive involvement of brachial plexus
• Involvement of paraspinal region
• Involvement of lamina of vertebrae body
• Involvement of mediastinal lymph node
• Subclavian venous obstruction

WORKUP

Section 6 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Lab Studies

• The blood workup for patients with Pancoast tumors is not specific and results are not diagnostic.
• • Lung cancers produce various substances. Elevated levels of oncofetal carcinoembryonic antigen and beta-2 microglobulins are associated with many lung cancers. Unfortunately, these findings are not diagnostic because levels of these chemicals are also elevated by other nonspecific causes such as smoking and bronchitis.
  • Tumor markers, such as bombesin, neuron-specific enolase, and other peptides, are common with small cell cancers and are related to the stage of the disease. They may aid in distinguishing differentiated from undifferentiated forms of lung cancer.
  • Various tumor oncogenes, including K-ras, c-myc, TP53, and HER-2/neu, have also been identified in patients with lung cancers. Although the presence of these oncogenes has some prognostic value, they are not important for staging of the cancer.
• Routine blood work in all patients with a lung cancer includes a CBC count, BUN/creatinine level, WBC count, and urinalysis. Coagulation parameters, such as prothrombin time, activated partial thromboplastin time (aPTT), and platelet count, are appropriate. Unless metastatic disease is evident, liver function tests are not regularly performed. Any patient deemed a surgical candidate has blood drawn for a cross match.
• Urinalysis is performed in all patients prior to surgery, and a catheter specimen is obtained in women if the initial urinalysis result suggests contamination.

Imaging Studies

• Radiographic features
• • Chest radiographs may reveal a small homogenous apical cap or pleural thickening; they may show a thin plaque at the lung apex in the area of the superior sulcus or they may reveal a large mass, depending on the stage when first diagnosed. Suggestive films should prompt the astute diagnostician to order apical lordotic views to better visualize the area.
  • Bone destruction of the posterior 1-3 ribs may sometimes be apparent. Rib invasion or vertebral body infiltration may be evident on a plain chest radiograph.
  • Mediastinal enlargement may be apparent.
• CT scanning and MRI
• • CT scanning and MRI of the neck, chest, and upper abdomen have replaced older radiographic studies. MRI is useful for evaluating resectability.
  • CT scanning helps identify invasion of the brachial plexus, chest wall, and mediastinum. Images can also reveal involvement of the vena cava, trachea, and esophagus. Contrast CT scanning is useful to assess subclavian vessel involvement.
  • MRI findings are more accurate than CT findings for assessing invasion of cervical structures and vertebral bodies. MRI has no advantage over CT scanning in the evaluation of the mediastinum. In fact, CT scanning is much better than MRI for assessing the mediastinum for lymph nodes. Rib or transverse process involvement is not a sign of inoperability; however, involvement of the vertebral body makes achieving an adequate margin of resection very difficult and reduces the odds for survival.
• Arteriogram or venogram: Rarely, arterial or venous involvement of the subclavian artery or vein occurs; thus, an arteriogram or a venogram may be helpful. This is usually accomplished in a retrograde fashion, although it can be approached from the opposite extremity or from the leg.

Other Tests

• A baseline ECG is performed on all patients for comparison to postoperative ECG tracings (if one is performed).

Diagnostic Procedures

• Bronchoscopy helps evaluate the tracheal and bronchial lumens; however, because most Pancoast tumors are peripheral, the diagnostic yield is low.
• Tissue diagnosis is obtained based on results from percutaneous needle biopsy, either under fluoroscopy or with CT-guided procedures.
• Staging is based on scalene node biopsy results from palpable nodes or mediastinoscopy findings.

Staging

The concept of classifying tumors according to tumor-node-metastasis (TNM) descriptions was elaborated in 1946. This system takes into account the characteristics of the local tumor (T), the presence or absence of regional lymph node involvement (N), and the presence or absence of distant metastases (M). Several staging systems have evolved since then. The most recent system is the 2002 revision of the International Staging System (ISS) for Non Small Cell Lung Cancer, which is unchanged from the 1997 revision. The overall stage of the tumor (stages I-IV) depends upon the particular combination of T, N, and M characteristics for the given patient. If the extent of disease cannot be assessed for any of these features, the suffix "X" is attached (ie, TX, NX, or MX).

TREATMENT

Section 7 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Medical Therapy

Medical management has only a secondary role in the treatment of lung cancers. In patients with disseminated lung cancer, medical treatment is required for palliation and treatment of symptoms arising from paraneoplastic syndromes.

Surgical Therapy

All patients with Pancoast tumors that are directly invading the parietal pleura and chest wall should undergo surgery, provided that (1) no distant metastases are present, (2) cardiopulmonary status allows surgery, and (3) no preoperative evidence of extensive mediastinal adenopathy is present. In most patients, a complete resection is performed at surgery, with subsequent prognosis dependent on lymph node status.

Preoperative Details

Preparation of the patient

Most postoperative complications following lung resection are cardiopulmonary. These include myocardial ischemia, pulmonary emboli, and respiratory failure. To avoid these complications, patients selected for surgery are required to undergo an evaluation of pulmonary function. High-risk patients benefit from supervised pulmonary rehabilitation accompanied by bronchodilator therapy. Prophylactic heparin and antiembolic stockings are used in all patients. Preoperative nutritional status is carefully assessed in all patients because a low albumin level is correlated to a higher morbidity rate.

All patients are encouraged to stop smoking at least 2 weeks prior to surgery. Preoperative assessment of cardiac risk factors is critical when evaluating potential candidates for lung resection. Perioperative cardiac complications can be reduced preoperatively in patients at high risk by instituting better perioperative monitoring, performing a lesser procedure, or achieving medical optimization. Consider preoperative angioplasty or coronary bypass in all patients with significant coronary disease.

Irradiation

Previously, superior sulcus tumors were considered inoperable and were not often successfully palliated with irradiation alone. The best results seem to occur when the tumor and the localized adjacent area, including the superior mediastinal nodes, are preoperatively treated with 30-40 Gy of radiation administered over 2-3 weeks. The radiation field partly includes the primary tumor, adjacent mediastinum, and ipsilateral clavicular area. The purpose of the preoperative irradiation is to shrink the tumor and to temporarily block lymphatic spread.

Preoperative treatment with more than 40 Gy may lead to poor healing following surgery. An interval of 2-4 weeks after radiation therapy allows the radiation to have maximal effect. After 4 weeks, all patients are reassessed for surgery. If no distant disease spread has occurred, then surgery is offered. The tumor is then resected en bloc with the chest wall.

The presence of Horner syndrome or ipsilateral supraclavicular node involvement is not an absolute contraindication for combined preoperative radiation and surgery. In current practice, interstitial implantation of radioisotopes (brachytherapy) is performed in association with external radiation therapy.

Chemotherapy

More recent data indicate that the traditional treatment of a Pancoast tumor with local approaches (surgery, radiotherapy, or a combination of both) leads to a poor outcome because of the high rate of recurrence and the lack of systemic control. The latest studies indicate that a trimodality treatment approach may improve local control and even survival. Studies with induction chemotherapy combined with hyperfractionated accelerated radiotherapy prior to surgery may be effective in improving long-term survival and lower recurrence rates. Empirical use of preoperative chemotherapy and radiation followed by surgical resection is being used in some centers, but no standardized protocols are available.

Intraoperative Details

A Pancoast tumor can be approached from an anterior or posterior incision. The posterior incision is made along the contour of the scapula, and the pleural cavity is entered at the third or fourth intercostal space. Dissection from below prevents injury to the subclavian vessels and the brachial plexus.

Prior to any resection, the degree of tumor invasion is assessed. The surgical technique for resection of a superior sulcus tumor is an extended en bloc resection of the chest wall, including posterior portions of the first 3 ribs, part of the upper thoracic vertebrae (including the transverse process), the intercostal nerves, the lower trunk of the brachial plexus, the stellate ganglion, and a portion of the dorsal sympathetic ganglion together with the involved lung portion. Determinants of unresectability include involvement of the subclavian artery, involvement of the vertebral body with or without cord compression, or widespread invasion of the brachial plexus.

The other approach to a Pancoast tumor is an anterior transcervical approach.⁵ Most authorities believe that injury to the subclavian vessels and the brachial plexus is much less common with this incision. With this approach, exposure of the jugular and subclavian veins is more easily accomplished and the thoracic duct is readily identified. Assessment of tumor invasion of the subclavian vessel is readily appreciated with this incision, and reconstruction of these vessels is easier. This incision is not recommended for tumors that invade the posterior aspects of the ribs and their transverse processes, the stellate ganglion and sympathetic chain, and the vertebral bodies.

After the procedure is completed, 2 large pleural tubes are placed for drainage. One tube is placed at the apex of the chest to drain any residual air; the other tube is placed in the posterior gutter to drain fluid. Fix all drainage tubes to the skin site with a suture.

Surgical principles for curative resection of a Pancoast tumor can be summarized as follows:

• Excise the entire first rib and posterior segments of the second and third ribs.
• Excise corresponding thoracic nerve roots up to the intervertebral foramen.
• Excise portions of the upper thoracic vertebrae, including the transverse process if necessary.
• Excise the lower trunk of the brachial plexus.
• Excise part of the stellate ganglion and the thoracic sympathetic chain.
• Lung resection can be by either wedge or lobectomy.
• Radical mediastinal lymph node dissection can be performed.

Postoperative Details

These patients are cared for in the ICU and are extubated. Routine care of the chest tubes is maintained. Mortality rates from this procedure are 2-5%. After arrival in the ICU, vital signs are monitored every 15-30 minutes until the patient is stable. Urinary output, chest tube drainage, and temperature are monitored hourly. Daily chest radiographs are obtained until the drainage tubes are removed.

The morbidity arising from this surgery is solely caused by the extent of chest wall and lung resection. Atelectasis is very common with this surgery and requires aggressive pulmonary toilet, incentive spirometry, and early ambulation. An adjunct to the treatment of atelectasis is bronchoscopy, which is frequently required to suction out mucous plugs and to drain secretions. Almost all patients have severe chest wall pain, and epidural anesthesia is highly recommended. Most air leaks subside within a few days, and the drainage tubes are removed.

Follow-up

Postoperative radiotherapy

The adjuvant role for postoperative radiotherapy in superior sulcus tumors is undetermined. Radiotherapy is not indicated for patients who undergo complete resection and have no nodal metastasis. Yet, in the past, many individuals have been treated with postoperative radiotherapy in response to an incomplete resection with residual disease. To date, postoperative radiation therapy has not been shown to improve survival in patients with cancer of the lung who have had complete surgical resection without gross or microscopic residual tumor.

Some retrospective studies show benefit for postoperative radiation therapy in patients with nodal disease; however, recent oncology trials have shown no survival benefit in patients who underwent complete resection. Note that postoperative radiotherapy does decrease the frequency of local (intrathoracic) recurrence.

Postoperative radiation therapy following immediate operation and brachytherapy has been as effective as preoperative radiation therapy and brachytherapy in achieving complete resection, locoregional control, and, ultimately, cure. No studies document the usefulness of chemotherapy in the treatment of this disease.

Primary radiation therapy

Radiation therapy is used as the sole treatment only for patients with unresectable tumors or for those who are not surgical candidates. It provides excellent pain relief, but no long-term survival occurs if the primary tumor is not controlled. The most common site of recurrence after resection is the central nervous system, especially if the primary tumor is an adenocarcinoma or large cell cancer. In these situations, prophylactic cerebral radiotherapy should be administered if local control has been achieved.  

For excellent patient education resources, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article Bronchoscopy.

COMPLICATIONS

Section 8 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Atelectasis is a common complication of surgery and requires aggressive pulmonary toilet.

Patients who do not have a thoracic epidural usually report significant pain and require continuous narcotics, either intravenously or in patch form.

Spinal fluid leaks occur but are rare. They usually subside with pleural drainage. If a spinal fluid leak occurs in the presence of a pneumothorax, the air may enter the spinal cord and result in meningitis, which manifests as a severe headache. If the spinal fluid leak persists, an exploratory thoracotomy is performed and a muscle flap is used to close the area.

Permanent neurologic deficits are rare. They usually result from resection of the lower trunk of the brachial plexus, but they are not incapacitating. Horner syndrome may occur from resection of the stellate ganglion and the root of C8. Deficits are usually temporary, lasting a few months.

Surgical complications can be summarized as follows:

• Mortality rate (approximately 3-5%)
• Atelectasis
• Severe chest pain
• Air leaks
• Spinal fluid leaks
• Horner syndrome
• Pain

OUTCOME AND PROGNOSIS

Section 9 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Clinical studies demonstrate that preoperative irradiation in doses not sufficient to cause gross regression of the tumor decreases local recurrences, prevents the growth of disseminated tumor cells, and increases survival compared with irradiation or surgery alone. In the past, superior sulcus tumors were considered inoperable and incurable because of their relative inaccessibility and extensive local invasion of the thoracic inlet. However, in selected patients, recent studies show complete eradication of local growth, pain relief, and improved survival rates.

Permanent neurologic defects resulting from resection of the lower trunk of the brachial plexus involve the distribution of the ulnar nerve but are not incapacitating. Horner syndrome and anhidrosis develop postoperatively secondary to dorsal sympathectomy. None of the defects is disabling. The 5-year survival rate after surgery is approximately 30%. Intraoperative brachytherapy has had no influence on locoregional recurrence or survival in patients with completely resected tumors. In the presence of positive mediastinal lymph nodes, the median survival rate is less than 9 months.

Adverse prognostic factors have been identified and include Horner syndrome, mediastinal adenopathy, and incomplete resection. To date, no patient with the above prognostic factors has survived for 5 years.

Significant adverse prognostic factors can be summarized as follows:

• Presence of Horner syndrome
• Involvement of mediastinal lymph nodes
• Involvement of supraclavicular lymph node
• Vertebral body invasion

Radiation therapy is recommended if patients do not have a resectable tumor identified on a CT scan. Some physicians have tried brachytherapy in patients with unresectable tumors identified intraoperatively, but outcomes have been dismal.

Palliation

Poor local control of a Pancoast tumor causes significant intractable pain. These patients die less than 2 years after diagnosis. The pain is caused by tumor invasion of the brachial plexus and nerve root compression in the intervertebral foramina, which is difficult to control.

Palliative surgery does not always provide relief from pain. However, when epidural compression is imminent, surgical maneuvers that alleviate this compression are of value. Techniques to interrupt pain pathways include cervical cordotomy, selective posterior rhizotomy, stereotactic thalamotomy, and commissural myelotomy.

Occasionally, radiotherapy is required for pain control.⁶ Radiation in doses from 40-60 Gy is administered over a period of 3 weeks, eliciting relief of pain in 90% of patients and occasionally reversing hoarseness and Horner syndrome. However, most patients die within 2 years. The most common site of metastatic disease is the brain.

FUTURE AND CONTROVERSIES

Section 10 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Note that in various series, irrespective of local control of the tumor, a considerable number of patients died from distant metastases rather than from local disease. The microscopic presence of distant metastatic disease is not presently discernible by available conventional screening methods, perhaps suggesting that more potent chemotherapy may be necessary to control the disease.

In summary, all current data indicate that the best survival rate is obtained with preoperative chemoradiotherapy followed by surgical resection in carefully selected patients.^{6, 7, 8, 9} Preoperative radiotherapy followed by surgery is a reasonable alternative in some patients. Involvement of the subclavian vessels or the vertebral column is associated with poor survival after surgery.⁸ However, a few centers have obtained decent experience with better surgical approaches to these structures and have published reasonable survival rates after surgery.

Involvement of mediastinal nodes is always associated with poor outcome after resection. At the time of surgery, a complete resection of all involved structures, including a lobectomy, is recommended. To date, no data describe appropriate treatment for patients with unresectable tumors who may be curable. However, extrapolation from the data for non-Pancoast stage III non–small-cell lung cancer (NSCLC) suggests that chemoradiotherapy is the best approach. In patients whose disease is believed incurable, radiotherapy offers good palliation of pain.

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

1. Paulson DL. Carcinomas in the superior pulmonary sulcus. J Thorac Cardiovasc Surg. Dec 1975;70(6):1095-104. [Medline].
2. Pitz CC, de la Riviere AB, van Swieten HA, Duurkens VA, Lammers JW, van den Bosch JM. Surgical treatment of Pancoast tumours. Eur J Cardiothorac Surg. Jul 2004;26(1):202-8. [Medline].
3. Shah H, Anker CJ, Bogart J, Graziano S, Shah CM. Brain: the common site of relapse in patients with pancoast or superior sulcus tumors. J Thorac Oncol. Nov 2006;1(9):1020-2. [Medline].
4. Rusch VW. Management of Pancoast tumours. Lancet Oncol. Dec 2006;7(12):997-1005. [Medline].
5. Dartevelle PG, Chapelier AR, Macchiarini P, Lenot B, Cerrina J, Ladurie FL, et al. Anterior transcervical-thoracic approach for radical resection of lung tumors invading the thoracic inlet. J Thorac Cardiovasc Surg. Jun 1993;105(6):1025-34. [Medline].
6. Anderson TM, Moy PM, Holmes EC. Factors affecting survival in superior sulcus tumors. J Clin Oncol. Nov 1986;4(11):1598-603. [Medline].
7. Detterbeck FC, Jones DR, Kernstine KH, Naunheim KS, American College of Physicians. Lung cancer. Special treatment issues. Chest. Jan 2003;123(1 Suppl):244S-258S. [Medline]. [Full Text].
8. Hilaris BS, Martini N, Wong GY, Nori D. Treatment of superior sulcus tumor (Pancoast tumor). Surg Clin North Am. Oct 1987;67(5):965-77. [Medline].
9. Stanford W, Barnes RP, Tucker AR. Influence of staging in superior sulcus (Pancoast) tumors of the lung. Ann Thorac Surg. May 1980;29(5):406-9. [Medline].
10. Beyer DC, Weisenburger T. Superior sulcus tumors. Am J Clin Oncol. Apr 1986;9(2):156-61. [Medline].
11. Detterbeck FC. Changes in the treatment of Pancoast tumors. Ann Thorac Surg. Jun 2003;75(6):1990-7. [Medline].
12. Herbert SH, Curran WJ Jr, Stafford PM, Rosenthal SA, McKenna WG, Hughes EN. Comparison of outcome between clinically staged, unresected superior sulcus tumors and other stage III non-small cell lung carcinomas treated with radiation therapy alone. Cancer. Jan 15 1992;69(2):363-9. [Medline].
13. Komaki R, Mountain CF, Holbert JM, Garden AS, Shallenberger R, Cox JD, et al. Superior sulcus tumors: treatment selection and results for 85 patients without metastasis (Mo) at presentation. Int J Radiat Oncol Biol Phys. Jul 1990;19(1):31-6. [Medline].
14. Marra A, Eberhardt W, Pottgen C, Theegarten D, Korfee S, Gauler T, et al. Induction chemotherapy, concurrent chemoradiation and surgery for Pancoast tumour. Eur Respir J. Jan 2007;29(1):117-26. [Medline].
15. Miller JI, Mansour KA, Hatcher CR Jr. Carcinoma of the superior pulmonary sulcus. Ann Thorac Surg. Jul 1979;28(1):44-7. [Medline].
16. Neal CR, Amdur RJ, Mendenhall WM, Knauf DG, Block AJ, Million RR. Pancoast tumor: radiation therapy alone versus preoperative radiation therapy and surgery. Int J Radiat Oncol Biol Phys. Aug 1991;21(3):651-60. [Medline].
17. Van Houtte P, MacLennan I, Poulter C, Rubin P. External radiation in the management of superior sulcus tumor. Cancer. Jul 15 1984;54(2):223-7. [Medline].
18. Van Houtte P, Rocmans P. Do superior sulcus tumors have a better prognosis than other lung cancer sites?. Int J Radiat Oncol Biol Phys. Sep 1990;19(3):823-4. [Medline].

Article Last Updated: Mar 5, 2008