Lary A. Robinson, MD
Senior Member, Thoracic
Moffitt Cancer Center, Tampa, FL
ï»¿One of the most distressing and vexing presentations of lung cancer is that of the superior sulcus tumor. In 1932 when Henry Pancoast, the first chairman of radiology at the University of Pennsylvania, initially described this tumor that bears his name, he wrongly thought that it started from embryonic rests that invaded the lung. Later, it was determined that this cancer started primarily in the upper lobe of either lung and involved the apex of the pleural cavity (the superior sulcus). The tumor causes severe, painful symptoms by invading the brachial plexus, ribs, vertebrae, subclavian vessels, and occasionally the stellate ganglion.
Unfortunately the presenting arm and shoulder pain and neurologic symptoms may be misleading, often delaying the diagnosis for months while mistaken benign orthopedic causes such as cervical spine disease are investigated. The characteristic Pancoast symptoms include some or all of the following: (1) dysesthesias, paresthesias, and weakness in the arm and hand in the C-8, T-1, and T-2 distribution; (2) subclavian (vein or artery) impairment to the involved upper extremity; (3) pain in the shoulder, upper arm, scapula and/or shoulder; (4) Horner’s syndrome (ptosis, miosis, anhidrosis, and apparent enophthalmos due to the ptosis). The diagnosis requires symptoms plus a lung cancer located in the apex of chest (superior sulcus).
An estimated 3% to 5% of non-small cell carcinomas present with a symptomatic Pancoast tumor. With 17,860 new lung cancers in Florida yearly (2012 data), approximately 550 of these are Pancoast tumors and deserve evaluation for potential curative, multimodality therapy. Squamous cell carcinoma represents 52% of Pancoast cases, with the rest comprised of 23% adenocarcinomas, 20% large-cell carcinomas, and only 5% small-cell carcinomas.
Lung masses with a Pancoast tumor presentation require the same initial evaluation as other lung masses (chest CT with contrast and PET scan). To assess the extent of chest wall involvement and to further stage the tumor, an MRI with contrast of the brachial plexus and thoracic inlet (which includes the upper thoracic vertebrae) is mandatory. Once a Pancoast tumor is suspected, a needle biopsy is needed for a histologic diagnosis since induction therapy is recommended. If the tumor is deemed potentially resectable, then pulmonary function tests and possibly a cardiac evaluation are recommended.
Prior to 1950, this was a uniformly fatal tumor. In 1961, Shaw, Paulson, and Kee described neoadjuvant radiotherapy followed by surgery for Pancoast tumors, and they had some long-term survivors. Forty years later in 2001, the phase II Intergroup Pancoast tumor trial added chemotherapy to the induction regimen (two cycles of induction cisplatin/etoposide chemotherapy with concurrent 4500 cGy radiotherapy followed by resection), reporting an excellent 41% 5-year survival in the 83 of 111 patients who subsequently underwent surgery. This regimen has been adopted as the current standard of care.