Introduction
Background
Pancoast tumors are neoplasms of pulmonary origin located at the apical pleuropulmonary groove (superior sulcus).
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.
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.
By direct extension, Pancoast tumors 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).1 Small cell carcinomas are seen in fewer than 5% of cases.2
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 M1.3
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 study by an M.D. Anderson group reported the following findings4 :
- 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,5,6,7 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 have been higher in 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.
Presentation
The most common initial symptom is shoulder pain.8 This is usually due to tumor extension into any of these adjacent structures: brachial plexus, parietal pleura, endothoracic fascia, vertebral bodies, and first 3 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.9
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).10,11
Histologic diagnosis is made in 95% of the cases by means of percutaneous transthoracic needle biopsy with fluoroscopic, ultrasonographic, or CT localization.8,12
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.
Differential Diagnoses
Other Problems to Be Considered
Mesothelioma
Lymphoma
Plasmacytoma
Metastatic malignancies (thyroid, larynx)
Lymphomatoid granulomatosis
Cervical rib syndrome
Tuberculosis
Fungal infections
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References
Davis GA, Knight SR. Pancoast tumors. Neurosurg Clin N Am. Oct 2008;19(4):545-57, v-vi. [Medline].
Fontinele e Silva J, Barbosa Mde P, Viegas CL. Small cell carcinoma in Pancoast syndrome. J Bras Pneumol. Feb 2009;35(2):190-3. [Medline].
Deslauriers J, Gregoire J. Clinical and surgical staging of non-small cell lung cancer. Chest. Apr 2000;117(4 Suppl 1):96S-103S. [Medline].
Komaki R, Roth JA, Walsh GL, et al. Outcome predictors for 143 patients with superior sulcus tumors treated by multidisciplinary approach at the University of Texas M. D. Anderson Cancer Center. Int J Radiat Oncol Biol Phys. Sep 1 2000;48(2):347-54. [Medline].
Arcasoy SM, Jett JR. Superior pulmonary sulcus tumors and Pancoast''s syndrome. N Engl J Med. Nov 6 1997;337(19):1370-6. [Medline].
Ginsberg RJ, Payne DG, Shamju FS. Superior sulcus tumors. In: Comprehensive Textbook of Thoracic Oncology. 1996:375-87.
Kraut MJ, Vallieres E, Thomas CR. Pancoast (superior sulcus) neoplasms. Curr Probl Cancer. Mar-Apr 2003;27(2):81-104. [Medline].
Gofeld M, Bhatia A. Alleviation of Pancoast's tumor pain by ultrasound-guided percutaneous ablation of cervical nerve roots. Pain Pract. Jul-Aug 2008;8(4):314-9. [Medline].
Lado Castro-Rial M, Morano Amado LE. [Pancoast tumor with acropaquia and Claude-Bernard-Horner syndrome]. Med Clin (Barc). Oct 25 2008;131(14):560. [Medline].
Heelan RT, Demas BE, Caravelli JF, et al. Superior sulcus tumors: CT and MR imaging. Radiology. Mar 1989;170(3 Pt 1):637-41. [Medline].
Webb WR, Gatsonis C, Zerhouni EA, et al. CT and MR imaging in staging non-small cell bronchogenic carcinoma: report of the Radiologic Diagnostic Oncology Group. Radiology. Mar 1991;178(3):705-13. [Medline].
Yang PC, Lee LN, Luh KT, et al. Ultrasonography of Pancoast tumor. Chest. Jul 1988;94(1):124-8. [Medline].
Kuraishi H, Yamashita J, Tsuchiya Y, Kokubu F, Takizawa K. [A case of lung adenocarcinoma of pancoast type successfully treated with concurrent chemoradiotherapy]. Gan To Kagaku Ryoho. Feb 2009;36(2):291-3. [Medline].
Detterbeck FC. Changes in the treatment of Pancoast tumors. Ann Thorac Surg. Jun 2003;75(6):1990-7. [Medline].
Holmes EC, Livingston R, Turrisis A. Neoplasms of the thorax. Cancer Medicine. 3rd ed. 1993:1285-337.
Lands RH, Patel N, Maran S, Karnad A. Small cell lung cancer presenting as a Pancoast tumor. J Tenn Med Assoc. Mar 1991;84(3):113-4. [Medline].
Further Reading
Clinical guidelines
Special treatment issues in lung cancer: ACCP evidence-based clinical practice guidelines. (2nd Edition)
American College of Chest Physicians - Medical Specialty Society. 2003 Jan (revised 2007 Sep). 16 pages. NGC:005938
Neck and upper back (acute & chronic).
Work Loss Data Institute - Public For Profit Organization. 2003 (revised 2008 May 7). 283 pages. NGC:006563
ACR Appropriateness Criteria® staging of bronchogenic carcinoma. American College of Radiology - Medical Specialty Society. 1996 (revised 2005). 9 pages. [NGC Update Pending] NGC:004641
Clinical trials
Randomized, Multi-Center, Open-Label, Study of Docetaxel Versus Docetaxel/PR104 in Non-Small Cell Lung Cancer (NSCLC)
Chemoradiotherapy Followed By Surgery and Docetaxel in Treating Patients With Pancoast Tumors
Chemotherapy, Radiation Therapy, and Surgery in Treating Patients With Stage III Non-Small Cell Lung Cancer
Related eMedicine topics
Pancoast Tumor (Thoracic Surgery)
Pancoast Syndrome
Neoplastic Brachial Plexopathy
Neoplasms, Lung
Horner Syndrome
Keywords
Pancoast tumor, 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
