eMedicine Specialties > Oncology > Carcinomas of the Lung and Other Intrathoracic Carcinomas

Pancoast Syndrome

Author: Karl J D'Silva, MD, Fellow in Hematology/Oncology, Department of Internal Medicine, Breslin Cancer Center, Michigan State University
Coauthor(s): Sarah K May, MD, Consulting Staff, Department of Hematology-Oncology, Caritas Carney Hospital, Commonwealth Hematology-Oncology PC
Contributor Information and Disclosures

Updated: Aug 26, 2008

Introduction

Background

Pancoast syndrome is characterized by a malignant neoplasm of the superior sulcus of the lung (lung cancer) with destructive lesions of the thoracic inlet and involvement of the brachial plexus and cervical sympathetic nerves (stellate ganglion). This is accompanied by (1) severe pain in the shoulder region radiating toward the axilla and scapula along the ulnar aspect of the muscles of the hand, (2) atrophy of hand and arm muscles, (3) Horner syndrome (ptosis, miosis, hemianhidrosis, enophthalmos), and (4) compression of the blood vessels with edema.

In 1924, Henry Pancoast, a radiologist from the University of Pennsylvania, published his first paper describing tumors of the pulmonary apex.1 In a 1932 report, he described in greater detail the syndrome that now bears his name.2 Medical historians note that Hare published a report in 1838 that may have included the first description of tumors of the superior sulcus.3

Pathophysiology

The overwhelming majority of cases of Pancoast syndrome are non–small cell lung carcinoma (NSCLC), with more than 95% located in the superior sulcus. The most common varieties are squamous cell carcinoma or adenocarcinoma. Small cell carcinoma is observed in fewer than 5% percent of cases in most series.

Other causes include nonepithelial neoplasms (eg, lymphoid malignancies, metastatic cancers of nonpulmonary causes), infections, lymphomatoid granulomatosis, vascular aneurysms, amyloid nodules, and cervical rib syndrome. Inflammatory pseudotumor (plasma cell granuloma), mycotic subclavian artery aneurysm, and carotid pseudoaneurysm in a child (caused by a hydatid cyst) have been reported. Because of the wide variety of diseases that can produce Pancoast syndrome, a histologic diagnosis is mandatory before initiating definitive treatment.

Frequency

United States

Pancoast tumors are uncommon and comprise fewer than 5% of all lung cancers.4,5 Originally deemed universally fatal, Pancoast tumors are now amenable to curative treatment because of improvements in combined modality therapy and development of new techniques for resection.

Mortality/Morbidity

For neoplastic causes, predictors of 5-year survival are weight loss, supraclavicular fossa or vertebral body involvement, disease stage, and surgical treatment. A study by an MD Anderson group reported the following findings:6

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

Clinical

History

Patients with Pancoast tumor (Pancoast’s tumor, an uncommon subset of lung cancer) may present with referred pain over the scapula to the shoulder as the result of damage to the afferent pain fibers of the sympathetic trunk. Pain in an ulnar nerve distribution along the medial aspect of the arm to the fingers results from involvement of the lower brachial plexus, the eighth cervical nerve, and the first thoracic nerve. This may be associated with hand weakness.

Many patients are initially treated for presumed local musculoskeletal conditions such as bursitis and vertebral osteoarthritis with radicular pain. Symptoms may persist for many months before evaluation for progression reveals the cause. In a 1994 series by Maggi and colleagues, symptoms lasted 2-36 months, with a mean of 9.7 months.7 In 1997, Muscolino described plexopathy or radicular symptoms in 53% of 15 patients.8

Physical

Physical examination of patients with Pancoast tumor (Pancoast’s tumor, an uncommon subset of lung cancer) may reveal findings consistent with Horner syndrome, such as ptosis and miosis, which result from paralysis of the dilating sympathetic fibers. Supraclavicular lymphadenopathy may also be observed.

Horner syndrome (Horner’s syndrome) is the result of invasion of the lower cervical and first thoracic ganglia, which frequently fuse into a single ganglion, the stellate ganglion. Horner syndrome is observed in 20-50% of patients at presentation.4,7,8,9 Decreased sweating on the affected side and ptosis of the denervated lid may be observed. Application of topical cocaine to the miotic eye (contracted pupil) fails to cause pupil dilation, while appropriate dilation is noted in the unaffected eye.10

The tumor may invade the bony structures of the chest. The first or second thoracic vertebra or the first, second, or third ribs may be invaded. In a review of 60 patients with Pancoast tumors, Maggi and associates found radiographic evidence of rib erosion in 50%; an almost equal percentage demonstrated involvement of the first or second rib, and 20% had involvement of the third rib. One patient had involvement of all 3 ribs.7 The tumor can also invade the first or second thoracic vertebral bodies or intervertebral foramina. From this point, it can extend to the spinal cord and result in cord compression. The subclavian vein or artery may also be invaded.

Cough, dyspnea, and hemoptysis, which are signs often associated with lung cancer, are not as common in individuals with Pancoast syndrome because of the peripheral location of the tumor. When present, they are associated with a worse prognosis. Also uncommon but occasionally noted are more advanced tumors with involvement of the recurrent laryngeal nerve, phrenic nerve, or superior vena cava.

See related CME at Examining the Eyes of an Older Person.

Causes

Non–small cell lung cancer is the most common etiology of Pancoast syndrome (Pancoast's syndrome); adenocarcinoma, squamous cell carcinoma, and large cell carcinoma have all been observed. Overall, Pancoast tumors are uncommon and comprise fewer than 5% of all lung cancers.4,5 While non–small cell lung cancer is the most common cause of Pancoast syndrome, the list of differential diagnoses is broad. Although quite rare, small cell lung cancer is also observed. Maggi and colleagues reported only 3 patients with small cell carcinoma in their 1994 series of 60 patients.7 More typically, small cell carcinoma manifests in a central location rather than a peripheral.

A diverse variety of unusual causes may be involved. Rarely, benign tumors such as desmoid tumors (case records of the Massachusetts GeneralHospital: weekly clinicopathological exercises, 2000) or hemangiopericytoma11 may cause the condition. Adenoid cystic carcinoma,12 metastatic carcinoma,13 lymphoma,14 and thyroid carcinoma15  have all been associated with the syndrome. Infectious processes, including bacterial16,17 and fungal infections18,19 may also be involved.

More on Pancoast Syndrome

Overview: Pancoast Syndrome
Differential Diagnoses & Workup: Pancoast Syndrome
Treatment & Medication: Pancoast Syndrome
Follow-up: Pancoast Syndrome
References
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References

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  3. Hare ES. Tumor involving certain nerves. Lond Med Gaz. 1838;1:16-18.

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Further Reading

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Keywords

Pancoast syndrome, Pancoast’s syndrome, lung cancer, Horner syndrome, Horner’s syndrome, shoulder pain, superior sulcus tumor, Ciuffini-Pancoast syndrome, Ciuffini-Pancoast-Tobías syndrome, Hare's syndrome, Pancoast's apex syndrome, Pancoast's disease, Pancoast's pain syndrome, Pancoast-Tobías syndrome, Tobías' syndrome, Bernard-Horner syndrome, ptosis, miosis, hemianhidrosis, anhidrosis, enophthalmos, non–small cell lung cancer, NSCLC, non–small cell bronchogenic carcinoma, squamous cell carcinoma, SCC, adenocarcinoma, Pancoast tumor, Pancoast’s tumor, malignant neoplasm

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Contributor Information and Disclosures

Author

Karl J D'Silva, MD, Fellow in Hematology/Oncology, Department of Internal Medicine, Breslin Cancer Center, Michigan State University
Karl J D'Silva, MD is a member of the following medical societies: Massachusetts Medical Society
Disclosure: Nothing to disclose.

Coauthor(s)

Sarah K May, MD, Consulting Staff, Department of Hematology-Oncology, Caritas Carney Hospital, Commonwealth Hematology-Oncology PC
Disclosure: Nothing to disclose.

Medical Editor

Michael Perry, MD, MS, MACP, Nellie B Smith Chair of Oncology, Professor, Department of Internal Medicine, Division of Hematology and Oncology, University of Missouri at Columbia/Ellis Fischel Cancer Center
Michael Perry, MD, MS, MACP is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society of Hematology, Missouri State Medical Association, Southern Association for Oncology, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Benjamin Movsas, MD, Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center
Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, and American Society for Therapeutic Radiology and Oncology
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD, Clinical Professor of Medicine, Division of Hematology/Medical Oncology, Department of Internal Medicine, University of Arizona College of Medicine at Tucson; Consulting Staff, Arizona Cancer Center
Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research
Disclosure: GlobeImmune Salary Consulting; Amplimed Consulting fee Consulting

 
 
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