Pancoast's Syndrome

Synonyms: Tobias syndrome, Ciuffini-Pancoast-Tobías syndrome

Classically caused by an apical (superior pulmonary sulcus) malignant neoplasm of the lung. The neoplasm is usually bronchogenic in origin (most commonly squamous cell carcinoma, sometimes adenocarcinoma and large-cell carcinoma).

It invades the surrounding tissues and produces:

• An ipsilateral invasion of the cervical sympathetic plexus leading to Horner's syndrome (miosis, enophthalmos, ptosis; only in 20% of patients)
• Ipsilateral reflex sympathetic dystrophy may occur
• Shoulder and arm pain (brachial plexus invasion C8-T2) leading to wasting of the intrinsic hand muscles and paraesthesiae in the medial side of the arm
• Less commonly unilateral recurrent laryngeal nerve palsy producing unilateral vocal cord paralysis (hoarse voice ±bovine cough), and/or phrenic nerve involvement
• There may be arm oedema secondary to the compression of blood vessels

Superior vena cava syndrome may also occur.

• Can also occur with other tumours - eg. breast cancer, mesothelioma, plasmacytoma or lymphoma; or metastatic carcinoma (eg from larynx, cervix, bladder or thyroid)
• Non-neoplastic causes of Pancoast's syndrome are very rare - but there have been reported cases due to bacterial pneumonia (staphylococcal or pseudomonas), TB, hydatid disease,¹ mycotic aneurysm, disseminated nocardiosis and plasma-cell granulomas. It has also been reported as due to cervical rib and pulmonary amyloidosis.²

This is usually as for other bronchogenic carcinomas i.e. tumour, node and metastasis (TNM) classification.³ Most Pancoast tumours are T3 at presentation as there is invasion of pleura and the brachial plexus.⁴

This is similar to other lung cancer emphasizing imaging including CXR and CT scan of the lungs, abdomen and also possibly PET. MRI is the imaging of choice to assess structures at the thoracic inlet prior to surgery. Brain CT or MRI is required as it is the commonest site of metastases. Good biopsy results are usually achieved by percutaneous methods.^5,6

Originally Pancoasts tumour was fatal as involvement of vital structures at the thoracic inlet. However this has improved with multimodality treatment including induction chemoradiotherapy (usually cisplatin based) followed by resection. Resection may involve a wedge resection or a lobectomy.^5,6

Involvement of vertebra, cervical plexus and lymph nodes are all associated with poorer outcomes. Historically 5 year survival was 30 - 40 % with complete resection and no lymph node involvement and < 10% for all other groups.⁶ Two thirds of patients experience a recurrence - however data regarding survival rates with induction chemoradiation and resection are much better and 5 year survival rates of approximately 70% have been reported.^4,6 Mediastinal lymph node involvement is associated with a particularly worse prognosis.

Although described separately by Pancoast⁷ and Tobias⁸ in 1932, it was first described by the British surgeon Edward Selleck Hare in 1838.^9,10