Approximately 5% of all non-small cell lung cancers (NSCLC) are located in the extreme apex of the lung, frequently with involvement of some combination of the first and second ribs, brachial plexus, subclavian vessels, and upper thoracic vertebral bodies. This phenomenon is referred to as a superior sulcus carcinoma, indicating tumor location in the uppermost portion of the costovertebral gutter within the chest. Pancoast syndrome refers to superior sulcus tumors along with the triad of (1) shoulder and arm pain, (2) wasting of the hand muscles, and (3) ipsilateral Horner syndrome (i.e., ptosis, miosis, and anhidrosis due invasion of the stellate ganglion). Henry Pancoast was a radiologist who described these findings in 1932 but failed to recognize the pulmonary origin of these tumors. Unaware of Pancoast report, Tobias, an Argentine physician, described similar clinical findings and ascribed them to the presence of peripheral lung tumors. Pancoast–Tobias syndrome is perhaps a more appropriate eponym for this entity. Anatomically, the superior pulmonary sulcus is the area on the superior surface of the lung traversed by the subclavian vessels and encircled by the first rib and spine (Fig. 80-1). It also may be described as the thoracic outlet or thoracic inlet.
Defining anatomy of the superior pulmonary sulcus.
For the next 25 years, these tumors were considered unresectable and uniformly fatal. In 1951, during irradiation therapy, a patient of Dr. Robert Shaw developed unbearable pain that precluded continuation of his irradiation therapy. With a threat of suicide, Dr. Shaw performed an en bloc resection of the cancer, with the chest wall, and lower trunk of the brachial plexus. Dr. Shaw did not expect him to live long; however, the patient survived over 40 years (outliving Dr. Shaw).
In 1961, Shaw et al.1 described successful outcomes in 18 patients undergoing 30 Gy of radiation, followed by resection, and this became the standard of care for the next 20 to 30 years. In over 400 patients with N0 stage I, Urschel found that approximately 35% had a 5-year survival. The treatment of choice since these reports has been preoperative irradiation (3000 rads over 2–3 weeks) followed by surgical en bloc resection of the lung, chest wall, lower brachial plexus, and vertebrae at a 1- to 2-month interval. This effects approximately a 35% to 50% 5-year survival with N0 stage I patients. Recently, the Intergroup 0160 phase II trial demonstrated superior outcomes by using trimodality therapy for superior sulcus tumors, which now represents the standard of care.2
Dr. Shaw's initial successful outcome caused our group to treat several other patients in similar fashion with 3000 rads to the tumor and mediastinal lymph nodes over a period of 2 to 3 weeks, followed by a waiting period of 2 to 4 weeks. This ...