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Synonyms: Tobias syndrome, Ciuffini-Pancoast-Tobias 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).
This syndrome results from the invasion of a number of structures and tissues around the thoracic inlet and may be characterised by:
- An ipsilateral invasion of the cervical sympathetic plexus leading to Horner's syndrome (miosis, enophthalmos, ptosis; in 14-50% 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 tumours can also result in Pancoasts's syndrome eg, breast cancer, mesothelioma, plasmacytoma or lymphoma; or metastatic carcinoma - eg, from the larynx, cervix, bladder, thyroid or colon).
Non-neoplastic causes of Pancoast's syndrome are very rare - but there have been reported cases due to bacterial pneumonia (staphylococcal or pseudomonas), tuberculosis, hydatid disease,[3, 4]mycotic aneurysm, disseminated nocardiosis and plasma-cell granulomas. It has also been reported as due to non-Hodgkin's lymphoma,cervical rib and pulmonary amyloidosis.
This is usually as for other bronchogenic carcinomas, ie tumour, node and metastasis (TNM) classification. Most Pancoast tumours are T3 at presentation, as there is invasion of pleura and the brachial plexus.
This is similar to other lung cancer emphasising imaging, including CXR and CT scan of the lungs and abdomen, and also possibly positron emission tomography (PET). MRI is the imaging of choice to assess structures at the thoracic inlet prior to surgery.[1, 7]Brain CT or MRI are required, as it is the most common site of metastases. Good biopsy results are usually achieved by percutaneous methods.[8, 9]
Originally, Pancoast's tumour was fatal due to involvement of vital structures at the thoracic inlet. This has improved with multimodality treatment, including induction chemoradiotherapy (usually cisplatin-based) followed by resection. Resection may involve a wedge resection or a lobectomy.[1, 8, 9, 10]Traditionally, the involved brachial plexus has also been resected, leading to paralysis and neuropathic pain, but this may be unnecessary.
Involvement of vertebrae, cervical plexus and lymph nodes are all associated with poorer outcomes. Historically, five-year survival was 30-40% with complete resection and no lymph node involvement and <10% for all other groups.[1, 9]Two-thirds of patients experience a recurrence. However, data regarding survival rates with induction chemoradiation and resection are much better and five-year survival rates of approximately 50-70% have been reported.[1, 9] Mediastinal lymph node involvement is associated with a particularly poor prognosis.
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Further reading & references
- Lung cancer: diagnosis and management; NICE Clinical Guideline (April 2011)
- Lung cancer - suspected; NICE CKS, July 2005
- Pancoast's syndrome; Whonamedit.com
- Foroulis CN, Zarogoulidis P, Darwiche K, et al; Superior sulcus (Pancoast) tumors: current evidence on diagnosis and radical treatment. J Thorac Dis. 2013 Sep 5(Suppl 4):S342-S358.
- 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.
- 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.
- Dao I, El Mostarchid B, Onen J, et al; Pancaost syndrome related to hydatid cyst. Pan Afr Med J. 2013 Mar 27 14:118. doi: 10.11604/pamj.2013.14.118.1754. Print 2013.
- Sarkar A, Das A, Basuthakur S, et al; Pancoast syndrome: A rare presentation of non-Hodgkin's lymphoma. Lung India. 2013 Jul 30(3):209-11. doi: 10.4103/0970-2113.116266.
- Arcasoy SM, Jett JR; Superior pulmonary sulcus tumors and Pancoast's syndrome. N Engl J Med. 1997 Nov 6 337(19):1370-6.
- Manenti G, Raguso M, D'Onofrio S, et al; Pancoast tumor: the role of magnetic resonance imaging. Case Rep Radiol. 2013 2013:479120. doi: 10.1155/2013/479120. Epub 2013 Mar 31.
- Rusch VW; Management of Pancoast tumours. Lancet Oncol. 2006 Dec 7(12):997-1005.
- Shahian DM; Contemporary management of superior pulmonary sulcus (Pancoast) lung tumors. Curr Opin Pulm Med. 2003 Jul 9(4):327-31.
- Deslauriers J, Tronc F, Fortin D; Management of tumors involving the chest wall including pancoast tumors and tumors invading the spine. Thorac Surg Clin. 2013 Aug 23(3):313-25. doi: 10.1016/j.thorsurg.2013.05.001. Epub 2013 Jul 16.
- Davis GA, Knight SR; Pancoast tumors. Neurosurg Clin N Am. 2008 Oct 19(4):545-57, v-vi.
- 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.
- José W. Tobías; Whonamedit.com
- Edward Selleck Hare; Whonamedit.com
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