Pancoast Syndrome


Article Author:
Vipin Villgran


Article Editor:
Sujith Cherian


Editors In Chief:
Juan Batlle
Jeffrey Miller


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Kyle Blair
Trevor Nezwek
Radia Jamil
Erin Hughes
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Navid Mahabadi
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Abbey Smiley
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Daniyal Ameen
Altif Muneeb
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes
Komal Shaheen
Sandeep Sekhon


Updated:
3/3/2019 9:34:59 AM

Introduction

Superior pulmonary sulcus tumors or Pancoast tumors arise from the apical pleuro-pulmonary groove located superior to the first rib. When these tumors involve the surrounding structures such as the brachial plexus, cervical paravertebral sympathetic nervous system, and stellate ganglion, they cause a group of signs and symptoms that are collectively called Pancoast syndrome. This is characterized by ipsilateral shoulder and arm pain, paresthesias, paresis and atrophy of the thenar muscles of the hand, and Horners syndrome (ptosis, miosis, and anhidrosis).[1][2][3]

Etiology

Tumors in the superior sulcus of the lung, typically non-small cell lung cancer cause Pancoast syndrome. Among all lung cancers, non-small cell lung cancer accounts for the majority with up to 80% to 85% of all cases; whereas, Pancoast tumors by themselves account for roughly 3% to 5% of all lung cancers. More than 95% of Pancoast tumors are NSCLC, and among NSCLC, squamous cell carcinoma used to be the most common histologic type; however, lately, studies have been showing a predominance of adenocarcinoma.

Pancoast syndrome is also caused by non-NSCLC tumors, typically small cell lung cancer. Other malignancies such as primary adenoid cystic carcinomas, thyroid carcinomas, lymphomas, or metastasis from any primary carcinoma or even benign tumors occupying the superior pulmonary sulcus are known to cause Pancoast syndrome. Even apical lung infections or abscesses can also cause Pancoast syndrome if they involve the chest wall and surrounding structures. [4][5]

Epidemiology

Lung cancer is the second most common cancer and is the main cause of oncological mortality in either gender, both in the United States and globally. There were more than 200,000 cases of lung cancer in 2017 and with a 5-year survival rate of 18%, more than 150,000 died from lung cancer in 2017. Among all lung cancers, non-small cell lung cancer accounts for the majority with up to 80% to 85% of all cases; whereas, Pancoast tumors by themselves account for roughly 3% to 5% of all lung cancers.[6]

Pathophysiology

Pancoast or superior sulcus tumors, when they compress or invade the surrounding structures, cause a group of symptoms collectively called Pancoast syndrome. They typically involve the brachial plexus first and cause shoulder and arm pain in almost all patients. They can also involve the parietal pleura, ribs, or vertebral bodies and cause pain. Since pulmonary symptoms occur late in these patients, initial symptoms are often misdiagnosed as musculoskeletal, and diagnosis is often delayed.

When the tumor extends further, it can also cause C8 through T1 radiculopathy with pain and paresthesia of the medial half of the fourth finger, fifth finger, and medial side of the hand, forearm, and arm. The weakness of intrinsic hand muscles affects fine motor skills and hand grip.

When the tumor involves the sympathetic trunk and the cervical ganglion, initially patients can develop ipsilateral facial flushing and sweating due to irritation. Later with invasion, Horner syndrome with ipsilateral ptosis, miosis, and anhidrosis can be seen along with sometimes contralateral flushing and sweating as well. This happens due to a hyperactive contralateral sympathetic reaction called Harlequin syndrome.[7][8]

Histopathology

Squamous cell carcinoma historically has been the most common subtype associated with Pancoast tumors. However, adenocarcinoma has been predominant in recent times. Overall, the overwhelming majority of Pancoast tumors are non-small cell lung cancer (NSCLC).

History and Physical

Pancoast syndrome refers to the collective group of symptoms associated with these tumors, which is secondary to the involvement of brachial plexus and associated chest wall, vertebral, or rib pain. Shoulder or arm pain with associated paresthesias along the medial half of the fourth, fifth fingers and medial side of the hand, arm, and forearm due to C8 through T1 radiculopathy are usually primary symptoms associated. Since they are apically located, pulmonary symptoms of shortness of breath do not develop until later, as the tumor begins to involve more of the lung.

Physical examination findings include ipsilateral facial flushing and sweating due to the involvement of the sympathetic trunk and cervical ganglion. Horner syndrome with ptosis, miosis, and anhidrosis may also develop with the further extent of the tumor.

Evaluation

There are no laboratory abnormalities specific to Pancoast tumor. 

A chest x-ray can be used for initial screening which shows the increased size of the apical cap or apical mass of the lung. CT scan provides additional information with regards to the extent of the tumor, satellite nodules, as well as mediastinal adenopathy, all of which are vital in the staging of these tumors. However, MRI has been suggested as more useful in these tumors, as it helps better delineate soft tissue involvement, invasion of brachial plexus, vasculature, and spinal involvement. Therefore, MRI is currently preferred on potentially surgically resectable tumors.

Due to the outer location of these tumors, CT-guided, core biopsy is the diagnostic test of choice with a reported yield of more than 90%. Bronchoscopy has been reported to be diagnostic in around 30% to 40% of the cases, which may be because of the presence of an additional endobronchial tumor. Sputum cytology and scalene node biopsies usually have a low yield.[9][10]

Treatment / Management

Standard of care is currently chemoradiation therapy followed by surgical resection.[10][11][1]

Induction chemoradiation therapy involves chemotherapeutic regimens which include Cisplatin/Etoposide or Cisplatin/Mitomycin C/Vindesine. Radiation therapy regimes usually include 45 Gy/27 fractions over 5 to 6 weeks, followed by surgery in 4 to 6 weeks after completion of chemoradiation therapy.

Contraindications to surgical resection include:

  • Presence of metastases
  • Involvement of ipsilateral or contralateral mediastinal nodes or supraclavicular nodes
  • Involvement of vertebral bodies greater than 50% 
  • Involvement of esophagus and/or/ trachea
  • Involvement of brachial plexus above T1 nerve root

Differential Diagnosis

Other malignancies either primary adenoid cystic carcinomas, hemangiopericytomas, thyroid carcinomas, lymphomas, plasmacytomas or metastasis from any primary carcinoma, or even benign tumors occupying the superior pulmonary sulcus are known to cause Pancoast syndrome.

Even apical lung infections or abscesses can also cause Pancoast syndrome if they involve the chest wall and surrounding structures.

Surgical Oncology

Resectable NSCLC superior sulcus tumors (T3 invasion, N0-1) are best treated with the trimodality therapy comprising systemic chemotherapy, radiation treatments, and surgical resection. NCCN panel advocates for neoadjuvant concomitant chemotherapy and radiation treatments accompanied by surgical resection and chemotherapy again. This practice of neoadjuvant chemoradiation before surgery has shown a 2-year survival of approximately 50 to 70% and a 5-year survival rate of roughly 40%.

For T3–4, N0–1 lesions involving chest wall, proximal airway, or mediastinum, surgery is done first followed by chemotherapy alone if surgical margins are negative. If surgical margins are positive, they receive concurrent chemoradiation followed by re-resection and chemotherapy.[12][6]

Medical Oncology

In patients with unresectable NSCLC superior sulcus tumors (T4 extension, N0–1) full dose systemic chemotherapy is given concurrently with radiation treatments. These tumors can be reassessed with CT or FDG-PET/CT scans after treatments for resectability. If the lesion remains unresectable, these patients may benefit from consolidation therapy with durvalumab which is an anti-PD-L1 monoclonal antibody (Programmed Death Ligand 1) after completion of chemoradiation.

Staging

Roughly three-quarters of Pancoast tumors are stage III tumors as they involve the parietal pleura, and the rest are regularly stage IIB and stage IV with the involvement of brachial plexus, mediastinum, great vessels, vertebral body, or with distant metastases.

According to the American Joint Committee on Cancer (AJCC) Cancer Staging Manual, Eighth Edition, stage IIB now includes any tumor regardless of its size with metastasis or direct invasion of ipsilateral peribronchial and/or ipsilateral hilar lymph nodes and intrapulmonary nodes, i.e., all N1 tumors. Stage IIB also includes T3 tumors which are greater than 5 cm but less than or equal to 7 cm in greatest dimension or directly invading parietal pleura, chest wall, phrenic nerve, parietal pericardium, or with 1 or more separate tumor nodules in the same lobe as the primary.

Stage III includes any tumor regardless of its size with N2 or N3 regional node involvement. These are tumors with metastasis to ipsilateral mediastinal and/or subcarinal lymph nodes (N2) and metastasis to contralateral mediastinal and/or hilar lymph nodes as well as scalene and/or supraclavicular lymph nodes regardless of laterality (N3). Stage III also includes T3 tumors with N1 lymph node involvement and any T4 tumor. T4 tumors are greater than 7 cm or invading the diaphragm, mediastinum, heart, great vessels, trachea, recurrent laryngeal nerve, esophagus, vertebral body, carina or with 1 or more tumor nodules in an ipsilateral lobe that is different from that of the primary.[13]

Prognosis

As with most other malignancies, patients with early-stage disease at diagnosis and better performance status have a good prognosis; whereas, advanced disease, poor performance status, and weight loss are indicators of poor outcomes. Also, metastatic NSCLC patients eligible for treatment with targeted therapies have a better prognosis compared with those without targetable genomic variations.

It is very important to promptly identify the symptoms and diagnose Pancoast syndrome as they are usually stage IIB or higher by the time they are diagnosed. Early detection, staging, and treatment are therefore critical for long-term survival.[14]

Complications

The complications are chiefly related to the treatments:

  • Surgery: Atelectasis, pain, chest wall deformity, frozen shoulder, CSF leak, prolonged air leak, injury to the brachial plexus
  • Chemotherapy: Side effects from the drugs
  • Radiation: alopecia, nausea, vomiting, leathery skin, poor wound healing

Consultations

  • Pulmonologist
  • Thoracic surgeon
  • Pain specialist
  • Critical care specialist

Pearls and Other Issues

  • NSCLC is the most common cancer associated with Pancoast tumor. 
  • Pancoast syndrome refers to superior sulcus tumors along with ipsilateral shoulder and arm pain, paresthesias, paresis and atrophy of the thenar muscles of the hand and Horners syndrome (ptosis, miosis, and anhidrosis). 
  • Trans-thoracic needle biopsy usually achieves a diagnosis. 
  • Preoperative evaluation will need MRI to delineate vascular involvement.
  • Treatment involves chemoradiation therapy followed by surgical resection.

Enhancing Healthcare Team Outcomes

Pancoast tumor has no cure, and the survival is abysmal. The cancer is often diagnosed late, and patients are subject to complex surgery which is not only painful but rarely results in a cure. Thus, the emphasis today is on prevention. One of the most common causes of lung cancer is smoking and nurses and pharmacists are in a prime position to educate the patients about the adverse effects of tobacco. Plus the nurse can educate the patient on the type of surgery, the potential side effects of radiation therapy, and chemotherapy. The pharmacist can also recommend aids to stop smoking. Further, the pharmacist can educate the patient on work hazards that have been linked to lung cancer. Finally, the patient must be educated on a healthy lifestyle, exercise and abstaining from alcohol. This advice has benefits beyond lung cancer- it can improve the quality of life and lower the cost of healthcare.[15][11] (Level V)

Outcomes

The prognosis for most patients with Pancoast cancer is poor. Less than 30% are alive at five years. To date, radiation therapy has made no difference in locoregional recurrence or long-term survival. For those who undergo surgery, the quality of life is very poor, and the pain from the surgery can be debilitating.[16][15] (Level III)


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Pancoast Syndrome - Questions

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Which of these cell types does not cause superior sulcus cancer?



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Involvement of which of the following structures is a contraindication to surgery for a pancoast tumor?



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Pancoast syndrome may involve all of the following except:



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Which of the following would provide the best assessment of a Pancoast tumor?



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Which is not seen in Pancoast syndrome?



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A 68-year-old active smoker with a 30 pack-year smoking history presents with impaired vision that improves with the chin-up position. He also reports occasional left shoulder pain, which he attributes to lifting heavy objects at work. The exam reveals left eyelid drooping, a warm and dry left side of the face, and mild weakness of his left-hand grip. What would be the usual next step in making a diagnosis?



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A 56-year-old smoker who presented with left arm pain was diagnosed with non-small cell lung cancer after chest x-ray and CT guided aspiration biopsy. Further evaluation with MRI showed a left apical 6 cm tumor invading the brachial plexus. Pathological lymph node evaluation showed positive hilar nodes but negative ipsilateral mediastinal and sub-carinal lymph nodes. MRI of the brain and FDG-PET CT scans were negative for any distant metastases. How should this patient be treated?



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A 65-year-old patient was diagnosed with a 7.5 cm right superior sulcus non-small cell lung cancer (NSCLC). FDG-PET/CT scans revealed evidence of bilateral mediastinal lymph node involvement, and this was confirmed by endobronchial ultrasound-guided transbronchial needle aspirate (EBUS-TBNA). He was subsequently started on definitive concurrent chemoradiation with no disease progression after three cycles. What would be the next step in treatment?



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Pancoast Syndrome - References

References

Kratz JR,Woodard G,Jablons DM, Management of Lung Cancer Invading the Superior Sulcus. Thoracic surgery clinics. 2017 May     [PubMed]
Zarogoulidis K,Porpodis K,Domvri K,Eleftheriadou E,Ioannidou D,Zarogoulidis P, Diagnosing and treating pancoast tumors. Expert review of respiratory medicine. 2016 Dec     [PubMed]
Marulli G,Battistella L,Mammana M,Calabrese F,Rea F, Superior sulcus tumors (Pancoast tumors). Annals of translational medicine. 2016 Jun     [PubMed]
Panagopoulos N,Leivaditis V,Koletsis E,Prokakis C,Alexopoulos P,Baltayiannis N,Hatzimichalis A,Tsakiridis K,Zarogoulidis P,Zarogoulidis K,Katsikogiannis N,Kougioumtzi I,Machairiotis N,Tsiouda T,Kesisis G,Siminelakis S,Madesis A,Dougenis D, Pancoast tumors: characteristics and preoperative assessment. Journal of thoracic disease. 2014 Mar     [PubMed]
Foroulis CN,Zarogoulidis P,Darwiche K,Katsikogiannis N,Machairiotis N,Karapantzos I,Tsakiridis K,Huang H,Zarogoulidis K, Superior sulcus (Pancoast) tumors: current evidence on diagnosis and radical treatment. Journal of thoracic disease. 2013 Sep     [PubMed]
Solli P,Casiraghi M,Brambilla D,Maisonneuve P,Spaggiari L, Surgical Treatment of Superior Sulcus Tumors: A 15-Year Single-center Experience. Seminars in thoracic and cardiovascular surgery. 2017 Spring     [PubMed]
Khadilkar SV,Khade SS, Brachial plexopathy. Annals of Indian Academy of Neurology. 2013 Jan     [PubMed]
Davis GA,Knight SR, Pancoast tumors. Neurosurgery clinics of North America. 2008 Oct     [PubMed]
Shen KR,Meyers BF,Larner JM,Jones DR, Special treatment issues in lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition). Chest. 2007 Sep     [PubMed]
Detterbeck FC,Jones DR,Kernstine KH,Naunheim KS, Lung cancer. Special treatment issues. Chest. 2003 Jan     [PubMed]
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 May     [PubMed]
McFadden PM,Wiggins LM, Assessment of Contemporary Aggressive Surgical Resection for Superior Sulcus and Pancoast Lung Tumors. Seminars in thoracic and cardiovascular surgery. 2017 Spring     [PubMed]
Jeannin G,Merle P,Janicot H,Thibonnier L,Kwiatkowski F,Naame A,Chadeyras JB,Galvaing G,Belliere A,Filaire M,Verrelle P, Combined treatment modalities in Pancoast tumor: results of a monocentric retrospective study. Chinese clinical oncology. 2015 Dec     [PubMed]
Waseda R,Klikovits T,Hoda MA,Hoetzenecker K,Bertoglio P,Dieckmann K,Zöchbauer-Müller S,Pirker R,Prosch H,Döme B,Klepetko W, Trimodality therapy for Pancoast tumors: T4 is not a contraindication to radical surgery. Journal of surgical oncology. 2017 Aug     [PubMed]
Glassman LR,Hyman K, Pancoast tumor: a modern perspective on an old problem. Current opinion in pulmonary medicine. 2013 Jul     [PubMed]
Buderi SI,Shackcloth M,Woolley S, Does induction chemoradiotherapy increase survival in patients with Pancoast tumour? Interactive cardiovascular and thoracic surgery. 2016 Nov     [PubMed]

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