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).

Presentation

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.

Other causes

• Can also occur with other tumours, e.g. breast cancer, mesothelioma, plasmacytoma or lymphoma; or metastatic carcinoma (e.g. from larynx, cervix, bladder, thyroid or colon¹)
• 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,² mycotic aneurysm, disseminated nocardiosis and plasma-cell granulomas. It has also been reported as due to cervical rib and pulmonary amyloidosis.³

Staging

This is usually as for other bronchogenic carcinomas, i.e. tumour, node and metastasis (TNM) classification.⁴ Most Pancoast tumours are T3 at presentation as there is invasion of pleura and the brachial plexus.⁵

Investigations

This is similar to other lung cancer emphasising 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 are required, as it is the commonest site of metastases. Good biopsy results are usually achieved by percutaneous methods.^6,7

Management

Originally Pancoast's 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.^6,7 Traditionally the involved brachial plexus has also been resected leading to paralysis and neuropathic pain, but recent work suggests that this maybe unnecessary.⁸

Prognosis

Involvement of vertebrae, 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.⁷ 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.^5,7 Mediastinal lymph node involvement is associated with a particularly worse prognosis.

History

Although described separately by Pancoast⁹ and Tobias¹⁰ in 1932, it was first described by the British surgeon Edward Selleck Hare in 1838.^11,12

Document references

1. Lu CC, Lin HF, Lee HS, et al; Metastatic colon cancer presenting as Pancoast's disease. Am J Surg. 2009 May;197(5):e51-2. Epub 2009 Feb 13. [abstract]
2. Ozpolat B, Ozeren M, Soyal T, et al; Unusually located intrathoracic extrapulmonary mediastinal hydatid cyst manifesting as Pancoast syndrome. J Thorac Cardiovasc Surg. 2005 Mar;129(3):688-9.
3. Arcasoy SM, Jett JR; Superior pulmonary sulcus tumors and Pancoast's syndrome. N Engl J Med. 1997 Nov 6;337(19):1370-6.
4. Mountain CF; Revisions in the International System for Staging Lung Cancer. Chest. 1997 Jun;111(6):1710-7. [abstract]
5. Detterbeck FC; Changes in the treatment of Pancoast tumors. Ann Thorac Surg. 2003 Jun;75(6):1990-7. [abstract]
6. Rusch VW; Management of Pancoast tumours. Lancet Oncol. 2006 Dec;7(12):997-1005. [abstract]
7. Shahian DM; Contemporary management of superior pulmonary sulcus (Pancoast) lung tumors. Curr Opin Pulm Med. 2003 Jul;9(4):327-31. [abstract]
8. Davis GA, Knight SR; Pancoast tumors. Neurosurg Clin N Am. 2008 Oct;19(4):545-57, v-vi. [abstract]
9. Pancoast HK, Superior pulmonary sulcus tumor. Tumor characterised by pain, Horner's syndrome, destruction of bone and atrophy of hand muscles. Journal of the American Medical Association 1932, 99:1391-1396.
10. Tobías JW. Sindrome ápico-costo-vertebral doloroso por tumor apexiano: su valor diagnóstico en el cáncer primitivo pulmonar. Rev Med Latino Am 1932; 17:1522-56.
11. Hare E.S., Tumour involving certain nerves. London Medical Gazette, 2nd series, 1838/39, 1:16-18
12. Pancoast's syndrome; Whonamedit.com

Internet and further reading

• Management of patients with lung cancer, Scottish Intercollegiate Guidelines Network - SIGN (2005)
• Lung cancer - the diagnosis and treatment of lung cancer, NICE Clinical Guideline (April 2011)
• Lung cancer - suspected, Prodigy (July 2005)