Pancoast Syndrome Clinical Presentation

Updated: Nov 14, 2017
  • Author: Karl J D'Silva, MD; Chief Editor: Nagla Abdel Karim, MD, PhD  more...
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Presentation

History

Patients with Pancoast syndrome 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. 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.

The pain is frequently relentless and unremitting, and adequate relief often requires administration of narcotics. 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.

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 et al, 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. [24]

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Physical Examination

Physical examination of patients with Pancoast tumor 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. [18, 7, 24, 20] 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. [25]

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.

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, hematologic 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 and indicates an increased risk for treatment failure. Preoperative brain imaging studies are highly recommended in patients who are receiving induction therapy for the primary tumor in the superior sulcus. [26]

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