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The superior sulcus tumor, due to its location in the bony confines of the thoracic inlet and superior costovertebral gutter, provides the thoracic surgeon with a unique challenge. It is a peripheral T3 or T4 upper lobe tumor that invades the chest wall. It may also invade the vertebral bodies and structures of the thoracic inlet. Although earlier described as a squamous cell carcinoma, adenocarcinomas are more common in recent reports. They are relatively rare, accounting for less than 5% of all lung carcinomas.
Pancoast, a radiologist, was a pioneer in radiation therapy for this lesion. In the last century, the management of the superior sulcus tumor has changed from inoperability and incurability to the current regimen of preoperative chemoradiation therapy, with an attempt at complete resection.
The posterolateral thoracotomy has certain advantages over the anterior approach. It is ideal for posterior tumors, particularly those that invade the vertebral bodies and the brachial plexus. If, on operative exploration, invasion of the subclavian artery is found, this approach can still be used. Proximal and distal control of the artery must be obtained and the position of the patient may even need to be changed. Once well exposed, the tumor may be freed through a subadventitial plane, or if necessary (for clear margins), the artery may be resected. Revascularization can be done with a polytetrafluoroethylene graft or end-to-end anastomosis. Despite the feasibility of dealing with unforeseen vascular invasion through the posterior approach, the anterior approach is better suited to that particular problem.
There is probably no benefit to incomplete resection, even when combined with postoperative radiation.
Therefore, to achieve complete resection, it is important that the surgeon be familiar with a combined approach. Contraindications to surgical resection are mediastinal lymph node metastases, extrathoracic metastases, pleural metastases, and spinal canal involvement. Invasion of the brachial plexus above the T1 nerve root (C8, C7, etc) is also a contraindication. Vertebral body involvement is not a contraindication if a complete resection can be achieved, but may require a neurosurgeon’s assistance. Similarly, invasion of the subclavian vessels is not a contraindication as long as a complete resection can be achieved.
The presentation of these tumors often involves shoulder and/or arm pain. The “Pancoast–Tobias” syndrome includes a triad of shoulder pain, Horner’s syndrome, and atrophy of the hand.
Tumors invading either the brachial plexus, the vertebral bodies, and the first and second ribs tend to produce a pain often following the distribution of the ulnar nerve. Invasion of the stellate ganglion causes a Horner’s syndrome (ipsilateral ptosis, miosis, and anhydrosis). More anterior tumors can cause unilateral upper extremity edema as a presenting symptom, or even hemorrhage. Performing a thorough neurological examination to assess for brachial plexus involvement is important. Pain or weakness on the ipsilateral hand may distinguish between C8 and T1 nerve root involvement. Weakness of the intrinsic hand muscles suggests C8 involvement, as opposed to T1 invasion, which is more consistent with ulnar forearm pain. Resecting the C8 nerve root for a clean margin would likely lead to a functionless hand.
With regard to imaging, chest radiograph, computed tomography (CT), and a metastatic work-up are necessary. These tumors are often missed on routine radiograph, obscured by the clavicle and first rib. CT is ideal for identifying the lesion. Biopsy before definitive surgery is recommended. The differential diagnosis of these apical chest “tumors” includes infectious etiologies such as staphylococcal pneumonia, tuberculosis, as well as neoplasms such as lymphoma. Lymphoma and tuberculosis, in particular, can present with lesions that invade the chest wall or cause rib destruction. Magnetic resonance (MR) is a useful adjunct when suspicious of significant brachial plexus (C8 nerve root) involvement or vascular invasion.
A brain MR, and possibly a positron emission tomography (PET) scan, should be performed to rule out metastatic disease. Because preoperative chemoradiation has become more prevalent, re-imaging before surgery is necessary. This is done not only to restage, but also to rule out disease progression. When there has been an unusual delay before surgery, the brain should be re-imaged as well. If there is any question involving the extent of tumor involvement (eg, pleural studding), the surgeon can always begin the procedure with thoracoscopy.
Other preoperative issues include the role of mediastinoscopy. Mediastinoscopy is important for prognostic reasons. N2 involvement or greater is associated with a dismal prognosis. However, a controversial area is that of ipsilateral supraclavicular node involvement (N3), which may be better classified as N1 disease in these tumors.
Finally, the surgeon should engage the patient in a lengthy discussion addressing the potential morbidity of loss of hand function. This is a problem, if, intraoperatively, it is found that the tumor has invaded the brachial plexus significantly. Generally, involvement of C8 means the resection will be incomplete. Furthermore, the patient should quit smoking several weeks before the surgery and begin respiratory conditioning to better tolerate the distortion of the chest wall postoperatively.
Because of the rarity of tumors that meet the specific “superior sulcus” definition, there is a lack of large prospective studies. In the North American intergroup trial 0160, a prospective multi-institutional study of non-small cell lung cancer of these tumors, the 5 year-survival rate was 41% (Table 1). Contributing factors to survival were pathologic response to chemoradiation and complete resection. The local recurrence rate was 12% but distant recurrence was significantly higher.
With regard to morbidity, there are several problems that can easily be avoided with careful intraoperative attention and postoperative care. Overall morbidity can be over 50% with most of these being minor complications. Common complications in the intergroup trial were atelectasis and pneumonia at 14.5 and 12%, respectively. There were also two (2.4%) postoperative deaths secondary to respiratory failure.
Other potential complications include frozen shoulder and chest wall motion issues that affect respiratory function and contribute to postoperative atelectasis. With meticulous muscle reapproximation, and coverage of both large defects and/or scapular tip defects with mesh, the risk of this occurring is decreased. Attention should be paid to preventing entrapment of the tip of the scapula in this reapproximation to protect shoulder girdle motion. Despite this, aggressive postoperative physical therapy most effectively prevents chest wall respiratory and frozen shoulder problems.
The superior sulcus tumor is a rare tumor posing a unique challenge to thoracic surgeons. The current regimen of preoperative chemoradiation with complete surgical resection leads to reasonable long-term survival. However, it is important to put these patients in trials to further improve outcomes. The posterolateral approach, when compared with the anterior approach, gives the surgeon the most latitude in resecting these tumors.
Influence of surgical resection and brachytherapy in the management of superior sulcus tumour.