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Author: Melanie Guerrero, MD, Fellow, Pulmonary and Critical Care Medicine, Walter Reed Army Medical Center

Melanie Guerrero is a member of the following medical societies: American College of Chest Physicians and American College of Physicians-American Society of Internal Medicine

Coauthor(s): Scott C Williams, MD, Section Chief, Nuclear Medicine Associate Attending Radiologist, Advanced Radiology Consultants, Bridgeport Hospital

Editors: Judith K Amorosa, MD, FACR, Clinical Professor and Program Director, Department of Radiology, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School; Consulting Staff, Department of Radiology, Robert Wood Johnson University Hospital; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Eric J Stern, MD, Director of Thoracic Imaging, Professor of Radiology and Medicine, Departments of Radiology and Internal Medicine, Harborview Medical Center, University of Washington School of Medicine; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center

Author and Editor Disclosure

Synonyms and related keywords: superior sulcus tumor, superior pulmonary sulcus tumor, apical pleuropulmonary groove neoplasm, non–small cell carcinomas, NSCCs, squamous cell carcinomas, SCCs, adenocarcinomas, large cell carcinomas, LCC, lung cancer, cancer of the lung

Background

Pancoast tumors are neoplasms of pulmonary origin located at the apical pleuropulmonary groove (superior sulcus). By direct extension, they typically involve the lower trunks of the brachial plexus, intercostal nerves, stellate ganglion, adjacent ribs, and vertebrae.

Pathophysiology

More than 95% of Pancoast tumors are non–small cell carcinomas, most commonly squamous cell carcinomas (52%) or adenocarcinomas and large cell carcinomas (approximately 23% for each subtype). Small cell carcinomas are seen in fewer than 5% of cases.

Staging of Pancoast tumor involves the tumor, node, and metastasis (TNM) classification in which T indicates site and size of the primary tumor, N is related to nodal involvement according to site, and M indicates the presence or absence of distant metastasis. These tumors are, at a minimum, T3N0M0 (T3 for chest wall invasion, stage IIB), and they are considered T4 lesions if the brachial plexus, mediastinal structures, or vertebral bodies are involved at the time of presentation. When supraclavicular nodes are involved, they are designated as N3 nodes, although they may be the first nodal station involved. Metastatic tumor in the ipsilateral nonprimary-tumor lobe of the lung or metastases to other organ systems is considered M1.

Frequency

United States

Fewer than 5% of all primary lung cancers are located in the superior sulcus.

Mortality/Morbidity

Predictors of 5-year survival are weight loss, supraclavicular fossa or vertebral body involvement, stage of the disease and surgical treatment. A recent study by an M.D. Anderson group reported the following findings:

  • Five-year survival for patients with stage IIB disease is 47%, compared with 14% in those with stage IIIA disease and 16% in those with stage IIIB disease.
  • In patients with stage IIB disease, surgical treatment and weight loss are significant independent predictors of 5-year survival.
  • Among patients with stage IIIA disease, the only predictor of survival is the Karnofsky performance score.
  • In patients with stage IIIB disease, the only independent predictor of survival is a right superior sulcus location, which is associated with a 5-year survival rate that is worse than that of patients with tumors in the left superior sulcus.
  • Compared with patients with squamous cell tumors, more patients with adenocarcinoma had cerebral metastases within 5 years.

Race

The differences among race are correlated with smoking prevalence. In white male smokers, the reported incidence of lung cancer is 15-30 times higher than that in nonsmokers. Since the early 1980s, the prevalence of tobacco use has decreased among white men but not among black men, and, as a result, lung cancer mortality rates in 1990 have been higher among black men than in white men.

Sex

Lung cancer is the leading cause of death in both men and women. The ratio of men to women is 2:1. To the authors' knowledge, no studies have shown any difference in prevalence in those with Pancoast tumors.

Age

Cancer of the lung in general occurs in individuals aged 40-70 years, with a peak incidence in those aged 50-70 years. Only 2% of all cases appear in persons younger than 40 years.

Anatomy

The tumor is located at the extreme apex of either the right or left lung, in what is called the pleuropulmonary groove or superior sulcus near the subclavian vessels. It frequently invades the second and third ribs, intercostal nerves, brachial plexus, stellate ganglion superiorly, and vertebral bodies posteriorly.

Clinical Details

The most common initial symptom is shoulder pain. This is usually due to tumor extension into any of these adjacent structures: brachial plexus, parietal pleura, endothoracic fascia, vertebral bodies, and first three ribs. Weakness, atrophy, and paresthesias of the hand, arm, and forearm are resultant symptoms. In as many as 25% of patients, compression of the spinal cord and paraplegia develop when the tumor extends into the intervertebral foramina.

Horner syndrome, which is described as ptosis, miosis, and anhidrosis, is reported to occur in 14-50% of patients. This is caused by invasion of the paravertebral sympathetic chain and stellate ganglion.

Less common manifestations include phrenic nerve and recurrent laryngeal nerve involvement. Superior vena cava (SVC) syndrome, which is compression of the SVC with resulting dyspnea and facial and upper extremity edema, is also uncommon.

Preferred Examination

Compared with other examinations, MRI is more accurate in identification of the extent of tumor involvement; it is superior to CT scanning in the detection of invasion of adjacent organs (eg, vertebral bodies, brachial plexus, subclavian vessels).

Histologic diagnosis is made in 95% of the cases by means of percutaneous transthoracic needle biopsy with fluoroscopic, ultrasonographic, or CT localization.

Among other considerations, CT or MRI of the brain is recommended in the initial evaluation, because distant metastases to the brain are not infrequent, and diagnosis of these is necessary for staging.

Limitations of Techniques

Noninvasive preoperative evaluation of the mediastinum with CT or MRI is limited by substantial false-positive and false-negative results (30-40%, depending on the criteria used to define enlarged lymph nodes and the patient population). Positron emission tomography (PET), and possibly surgical assessment of the mediastinum with lymph node sampling, should be strongly considered before curative surgery is attempted.



Other Problems to be Considered

Mesothelioma
Lymphoma
Plasmacytoma
Metastatic malignancies (thyroid, larynx)
Lymphomatoid granulomatosis
Cervical rib syndrome
Tuberculosis
Fungal infections



Findings

Posteroanterior (PA) chest radiographs show unilateral apical opacity or just asymmetry of the apices of greater than 5 mm. Local rib destruction can sometimes be observed. Lordotic chest views can be beneficial, but the findings can also be misleading.

Degree of Confidence

In the early stages, Pancoast tumors are difficult to detect on PA chest radiographs because of the difficulty in interpreting overlying shadows at the apices.



Findings

CT is best in demonstrating bony destruction. MRI appears to be superior in demonstrating chest wall invasion. The anatomy above the lung apex is better demonstrated on multiplanar MRI, because the nerves of the brachial plexus and blood vessels follow a horizontal and parallel course, meeting above the apex of the lung.

Degree of Confidence

In an older study of 31 patients with superior pulmonary sulcus tumors (Heelan, 1989), CT had a sensitivity of 60% and a specificity of 65%, with an overall accuracy of 63% in the evaluation of the extent of disease.



Findings

MRI provides superior delineation of the normal anatomy of the brachial plexus because of its multiplanar capabilities. The absence of streak artifact from bone and accurate identification of vessels are some of the advantages of MRI. It also has superior soft-tissue contrast, and it is more accurate than other methods in documenting or excluding brachial plexus involvement by the tumor.

Compared with other techniques, MRI is more accurate in the evaluation of extension to the vertebral body, spinal canal, brachial plexus, and subclavian artery. This advantage is important, because vertebral body, spinal canal, and upper brachial plexus invasion are contraindications to surgical resection.

False Positives/Negatives

In an older study of 31 patients with Pancoast tumors, MRI had a sensitivity of 88%, a specificity of 100%, and an overall accuracy of 94% (Heelan, 1989).



Findings

Some have suggested that all patients with Pancoast tumors should undergo ultrasonographic examination of the ipsilateral scalene area, with percutaneous biopsy of nodes that are larger than 1 cm in their transverse diameter. The purpose of these studies is to assist in staging of the disease. The use of a sector ultrasonographic unit with a supraclavicular approach has been useful in guiding needle aspirations, with a yield for pathologic diagnosis in 91% of cases.



Findings

PET scanning is promising in detecting distant metastases and mediastinal involvement.



Findings

On occasion, subclavian artery angiography may be indicated to rule out local invasion of these vessels by the tumor.



Preoperative radiation therapy at doses of 2000-6500 cGy, followed by surgical resection, is the most common form of treatment. However, the advantage of preoperative radiation therapy has not been definitively demonstrated, and the dose of radiation has not been clearly established. The overall 5-year survival rate in patients treated with preoperative radiation therapy and surgery is reported to be 20-35%.

Radiation therapy at a dose of 6000 cGy or greater has been used as a primary treatment modality for inoperable tumors, with successful palliation of pain in as many as 90% of patients. The reported 5-year survival rate is 0-29% in these patients, which is likely a result of extensive disease involvement at initial presentation.

The routine use of intraoperative and postoperative radiation therapy is not currently recommended, except in patients in whom unresectable tumors are found at the time of surgery.



Media file 1:  Pancoast tumor. A 53-year-old man with a 50 pack-year history of smoking began experiencing upper back pain for several weeks. PA chest radiograph shows asymmetry of the apices (superior sulcus). The right apex is more opaque than the left. When the image is enlarged, the partially destroyed second and third right posterior ribs near the costovertebral junction can be seen.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  X-RAY

Media file 2:  Pancoast tumor. Axial nonenhanced CT image of the upper dorsal spine demonstrates a soft tissue mass destroying the vertebra on the right and the right posterior elements, including the pedicle and part of the posterior spinous process.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT

Media file 3:  Pancoast tumor. Sagittal fast spin-echo T2-weighted MRI shows collapsed vertebrae and cord compression at C7, T1, and T2 caused by a soft tissue mass.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 4:  Pancoast tumor. Sagittal gradient-echo T2-weighted MRI demonstrates a soft tissue mass involving C7, T1, and T2, with collapse of the vertebrae and moderate cord compression.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 5:  Pancoast tumor. Axial T1-weighted image shows cord compression caused by a large, enhancing mass. The right subclavian artery is not involved.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI



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Pancoast Tumor excerpt

Article Last Updated: Apr 14, 2004