Superior sulcus tumors make up less than 5% of non–small cell lung cancers (NSCLC) but are associated with a number of debilitating symptoms that are particularly challenging to treat.
Erica M. Palma, RN, BSN, OCN, is a graduate student in the School of Nursing at the University of Pennsylvania and a clinical nurse at Memorial Sloan-Kettering Cancer Center.
Superior sulcus tumors make up less than 5% of non—small cell lung cancers (NSCLC) but are associated with a number of debilitating symptoms that are particularly challenging to treat.1 Radiologist Henry Pancoast first described these tumors in 1924, and they are still referred to as Pancoast tumors. These tumors occur in the apex of the lung and invade the structures of the apical chest wall including the brachial plexus, subclavian vessels, spine, or epidural space, causing a constellation of signs and symptoms known as Pancoast’s syndrome.1.2 These include severe shoulder and arm pain, Horner’s syndrome, and weakness and atrophy of the hand.1.2 Other primary or metastatic tumors can also occur in the lung apex causing similar symptoms which are referred to as Pancoast’s syndrome.3
The debilitating pain associated with Pancoast tumors involves both nociceptive and neuropathic pain pathways. Treating the tumor itself is the preferred pain management technique.3 Advanced stage and the invasion of various regional structures make resection of Pancoast tumors more difficult than resection of other NSCLC of similar stages. Only 30% to 40% of patients with Pancoast tumors are eligible to receive preoperative concurrent chemoradiotherapy followed by complete resection.4,5 The survival rate with this ideal treatment is approximately 54% at 5 years.3,6 Unresectable tumors can be treated with chemoradiation for curative intent; however, no data exist on survival rates for this treatment.3 Treatment with radiation alone has achieved overall survival rates of only 5% to 23%.3 Palliative radiation has achieved good pain relief in about 75% of patients.3,7
Even patients who have a complete tumor resection can experience lingering neuropathic pain. Unfortunately, the literature lacks supportive care guidelines specific to pain management in these patients.
Historically, surgical procedures that disrupt nerve transmission of pain impulses were used to manage Pancoast tumor pain. A number of studies in the 1970s and 1980s reported that neurosurgical procedures, such as cordotomy and selective posterior radiculotomy, provided good to excellent relief from Pancoast tumor pain.8.9 Other surgical procedures used in these historical studies include decompression laminectomy, phenol block, transdermal stimulation, and stellate ganglion block.
In current practice, these procedures are infrequently performed. The introduction of the WHO 3-step pain ladder, and the subsequent development of cancer pain management guidelines, contributed to this decline. Guidelines such as the NCCN Adult Cancer Pain guideline10 focus on opioid use and adjuvant medications, and surgical interventions are recommended only if medication options have been exhausted.
Introduction of nondestructive techniques such as intrathecal opioid administration, also contributed to the decline in the utilization of surgical procedures. These nondestructive techniques were being developed at the same time that higher standards for evaluating medical literature were evolving. The few studies that supported surgical interventions were older and did not meet the new standards. Because evidence supporting the use of many of these procedures was lacking, fewer procedures were performed, resulting in decreased opportunities for medical training and fewer providers available to perform these procedures.11
Opioids and other analgesics have well-known limitations in the management of cancer related pain. Side effects such as constipation and sedation can negatively affect quality of life. Physical dependence, tolerance, breath depression, hyperalgesia, and immunologic dysfunction can also occur and lead to termination of opioid use.12 System-related barriers include cost of analgesics and a lack of access to them, especially in minority and low socioeconomic status communities.3
Furthermore, some evidence demonstrates that the development of opioid-induced pain sensitivity is mediated through neural mechanisms that may interact with the mechanisms underlying the development of neuopathic pain.13 This suggests that Pancoast tumor pain specifically could be improved with the use of fewer opioids and more interventional strategies.
Several authors have argued that specific surgical procedures are very useful in the treatment of Pancoast tumor pain and should not be thought of as options of last resort. In 2011 a review of literature from 1966-2009 on the use of destructive surgical techniques to treat cancer pain was conducted.11 Cordotomy was the most studied procedure. It was cited in 47 papers and used to treat 3601 cancer patients. While many of the studies were dated, retrospective, and difficult to grade, pain was assessed 6 months post-procedure in over 100 patients and three of the studies assessed patients until death. The vast majority of the patients reported excellent pain relief postcordotomy. The review included one prospective study that demonstrated statistically significant improvement in pain, performance status, activities of daily living, and total sleeping hours postcordotomy.
Recently, CT-guided cordotomy was prospectively studied in 108 patients with lung cancer experiencing intractable pain.12 Ninety-eight percent of all the patients reported significantly improved pain relief and performance status following cordotomy. Specifically in the 15 Pancoast tumor patients, the median pain score on the Visual Analogue Scale decreased from 8 preoperatively to 1 postoperatively, and the mean Karnofsky Performance score increased from 40 preoperatively to 70 postoperatively (P values for each = .001).
Collectively, these authors advocate for more widespread use of cordotomy in patients who experience unilateral somatic pain below the neck that is nonresponsive to standard analgesic therapy or if patients are experiencing analgesic side effects. Cordotomy can be used in terminal cancer patients, even in the last weeks of life, to promote comfort and quality of life. The hospital stay is short, only local anesthesia is required, and overall charges are low.
Pancoast tumor pain can be an agonizing experience for patients, their families and loved ones. While the current national pain management guidelines may be appropriate for many types of cancer-related pain, the medication-based recommendations in these guidelines do not provide adequate pain relief for the many of these patients. Given the complexity of the pain pathophysiology seen in Pancoast tumors, and the positive results found in a small body of older and more recent research, surgical therapies such as cordotomy should be considered more often and earlier in the illness continuum.
More research is needed to determine which surgical interventions offer the most benefit and how they can be incorporated into relevant pain guidelines. Incorporating these interventions into medical training is critical in order to assure that there are enough providers adequately prepared to deliver these interventions.
References
1. Peedell C, Dunning J, Bapusamy A. Is there a standard of care for the radical management of non-small cell lung cancer involving the apical chest wall (Pancoast tumors)? Clin Oncol (R Coll Radiol). 2010; 22(5):334-346.
2. Jett JR. Superior sulcus tumors and Pancoast’s syndrome. Lung Cancer. 2003;42(suppl 2):S17-S21.
3. Kozower BD, Larner JM, Detterbeck FC, Jones DR. Special treatment issues in non-small cell lung cancer: Diagnosis and management of lung cancer, 3rd ed: American College of Chest Physicians evidence-based clinical practice guidelines. Chest. 2013;143(5):369-399.
4. Parissis H, Young V. Treatment of Pancoast tumors from the surgeon’s perspective: reappraisal of the anterior-manubrial sternal approach [published online November 4, 2010] J Cardiothorac Surg. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2992054/. Accessed December 8, 2015.
5. Pourel N, Santelmo N, Naafa N, et al. (2008). Concurrent cisplatin/etoposide plus 3D-conformal radiotherapy followed by surgery for stage IIB (superior sulcus T3N0)/III non-small cell lung cancer yields a high rate of pathological complete response. Euro J Cardiothorac Surg. 2008;33(5):829-836.
6. Rusch VW, Giroux DJ, Kraut MJ, et al. Induction chemoradiation and surgical resection for superior sulcus non-small-cell lung carcinomas: Long-term results of Southwest Oncology Group trial 9416 (intergroup trial 0160). J Clin Oncol. 2007;25(3):313-318.
7. Van Houtte P, MacLennan I, Poulter C. External radiation in the management of superior sulcus tumor. Cancer. 1984;54(2):223-227.
8. Batzdorf U, Brechner VL. Management of pain associated with the Pancoast syndrome. Am J Surg. 1979;137(5):638-646.,
9. Esposito, S, Bruni P, Delitala A, et al. Therapeutic approach to the Pancoast pain syndrome. Appl Neurophysiol. 1985;489(1-6): 262-266.
10. Swarm RA, Abernethy AP, Anghelescu DL, et al. Adult cancer pain. J Natl Compr Canc Netw. 2013;11(8):992-1022.
11. Raslan AM, Cetas JS, McCartney S, Burchiel KJ. Destructive procedures for control of cancer pain: The case for cordotomy. J Neurosurg. 2011;114(1):155-170.
12. Kanpolat Y, Ozdemir M, Al-Beyati E. CT-guided percutaneous cordotomy for intractable pain in what is more than a disease: lung malignancies. Turk Neurosurg. 2013;23(1):81-87.
13. Mao J. Opiod-induced abnormal pain sensitivity. Curr Pain Headache Rep. 2006;10():67-70.
2 Commerce Drive
Cranbury, NJ 08512