Pancoast syndrome is an important clinical presentation of several disorders with different etiologies that develop in the apex of the lungs [1] [2] [3] [4]. Bronchogenic carcinoma, however, is by far the most common cause of this clinical syndrome characterized by one or more of the following symptoms [5]:

• Shoulder pain - Proliferation of a mass from the lung apex eventually compresses the brachial plexus, parietal pleura, and the surrounding blood vessels, leading to severe ipsilateral and progressive pain in the shoulder [2] [3]. The pain can spread to the neck, anterior chest wall, and axilla, or progress down to the arm and hand, simultaneously producing weakness and atrophy of the muscles innervated by nerves stemming from C8-T1 roots of the brachial plexus (the ulnar nerve being predominantly affected) [2] [3] [4]. In some cases, blood vessel occlusion may lead to unilateral edema that further contributes to muscle atrophy [2] [6].
• Horner syndrome - Enophthalmos, anhidrosis, ipsilateral miosis and ptosis comprise this neurological syndrome, which is encountered in up to 50% of patients with superior sulcus tumors, and the pathogenesis stems from a mechanical damage of the sympathetic plexus by a growing mass [2].

Although bronchogenic carcinomas are responsible for the development of Pancoast syndrome in the majority of cases (hence the terms Pancoast tumors or superior sulcus tumors), other conditions are neoplastic diseases (non-Hodgkin lymphomas, multiple myelomas, metastatic cancers of the liver and uterus), fungal infections (hydatid cysts as a result of Echinococcosis, histoplasmosis, aspergillosis, and mucormycosis) and pulmonary tuberculosis, whereas bacterial infections and other benign pathologies rarely induce Pancoast syndrome [1] [3] [6].

• Arm Pain

  Shoulder and arm pain (from the brachial plexus invasion C8-T1) with wasting of the intrinsic hand muscles and paraesthesiae in the medial side of the arm. [patient.info]

  Pancoast's syndrome is a constellation of characteristic symptoms and signs that includes shoulder and arm pain along the distribution of the eighth cervical nerve trunk and first and second thoracic nerve trunks, Horner's syndrome, and weakness and atrophy [nejm.org]

  Pancoast syndrome is a term used to describe unilateral shoulder and arm pain, weakness, and atrophy of hand muscles and Horner's syndrome as a result of a mass in the lung apex compressing blood vessels and nerve trunks. [symptoma.com]

  Reference work entry DOI: https://doi.org/ 10.1007/978-3-540-85516-3_525 Definition The classic Pancoast syndrome presents symptoms and signs that include shoulder and arm pain along the distribution of the eighth cervical nerve trunk and first and second [link.springer.com]

  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. [ncbi.nlm.nih.gov]

• Enophthalmos

  Horner syndrome - Enophthalmos, anhidrosis, ipsilateral miosis and ptosis comprise this neurological syndrome, which is encountered in up to 50% of patients with superior sulcus tumors, and the pathogenesis stems from a mechanical damage of the sympathetic [symptoma.com]

  •Horner’s Syndrome: miosis, ptosis, enophthalmos and anhidrosis. •Lower brachial plexus injury (C8-T1): Klumpke’s palsy. •Paresthesia of the upper extremity due to compression of subclavian artery & vein. [abcradiology.blogspot.com]

  Clinical This is accompanied by: 1) severe pain in the shoulder region radiating toward the axilla and scapula along the ulnar aspect of the muscles of the hand; 2) atrophy of hand and arm muscles; 3) Horner syndrome (ptosis, miosis, hemianhidrosis, enophthalmos [pallipedia.org]

  Presentation This syndrome results from the invasion of a number of structures and tissues around the thoracic inlet and may be characterised by: Ipsilateral invasion of the cervical sympathetic plexus, leading to Horner's syndrome (miosis, enophthalmos [patient.info]

  […] 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 [onclive.com]

• Shoulder Pain

  […] rheumatology Background Shoulder pain is naturally thought to have an orthopaedic or rheumatological cause. [casereports.bmj.com]

  •Shoulder pain: due to involvement of upper ribs and intercostal nerves. [abcradiology.blogspot.com]

  A 61-year-old patient had the clinical findings of Pancoast syndrome with shoulder pain, weakness and atrophy of band muscles, and an opacified area in the apical lung field. [ncbi.nlm.nih.gov]

  You may associate shoulder pain with a physical injury. Shoulder pain can also be a symptom of lung cancer, and it may be the first symptom of it. Lung cancer can cause shoulder pain in different ways. [healthline.com]

  Clinical presentation The syndrome consists of: shoulder pain C8-T2 radicular pain Horner syndrome The classical syndrome is uncommon, with Horner syndrome present in only 25%. [radiopaedia.org]

• Neck Pain

  Cancer is an uncommon cause of radicular neck pain but should be considered in the differential, particularly when constitutional complaints are also present. [ncbi.nlm.nih.gov]

  (2) atrophy of hand and arm muscles, (3) Horner syndrome (ptosis, miosis, hemianhidrosis, enophthalmos), and (4) compression of the blood vessels with edema." [6] Common presentations include: Pancoast syndrome Neck pain [7] Pain is relieved by propping [physio-pedia.com]

• Upper Back Pain

  Case presentation A 54-year-old man was sent to the emergency department by his general practitioner with a 4-week history of progressively worsening right shoulder pain radiating down his right arm and into his upper back. [casereports.bmj.com]

  National Institutes of Health, National Cancer Institute, Lung Cancer Pancoast Tumor Pictures A 53-year-old man with a 50-pack-per-year history of smoking began experiencing upper back pain for several weeks. [emedicinehealth.com]

• Right Shoulder Pain

  shoulder pain radiating down his arm, with some associated sensory loss. [casereports.bmj.com]

• Arm Swelling

  Arm pain isn't part of Horner's Sy, but it can be present simultaneously, and pancoast cancer can go with shoulder or arm pain and Horner Sy. Even hand swelling can be caused by pancoast tumour due to compression onto blood vessels. [prep4usmle.com]

As Pancoast syndrome is the likely manifestation of a malignant disease, its early recognition is vital for ensuring optimal patient outcomes. Unfortunately, tumors are already at an advanced stage when Pancoast syndrome appears (T3 or T4 stage is observed in the majority of cases) [4]. Nevertheless, the immediate workup should start with a patient history that will note the timeline of complaints and their progression, whereas a properly conducted physical examination could identify unilateral pain of the shoulder and weakness of the ipsilateral arm, as well as constitutive features of Horner's syndrome. There should be a clinical suspicion regardless of the severity of symptoms, and imaging studies should be performed promptly, as they are the cornerstone in determining the etiology. Plain chest X-rays often reveal a homogeneous shadow or opacity in the lung apex and asymmetry of the apical caps of > 5 mm, but computed tomography (CT) is the gold standard [2] [3] [4] [5]. CT is able to confirm the state of the surrounding tissues and determine whether the mass has invaded the ribs, vertebral bodies, the mediastinum, the brachial plexus, but also if blood vessel occlusion has occurred [2] [4] [5]. Furthermore, magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) can be employed to obtain an even better view of the tissues [2] [4]. Even though bronchoscopy and subsequent biopsy of the lesion yields a definite diagnosis, the hardly accessible location of the lung apex does not allow for appropriate sampling [4] [5]. For this reason, transthoracic image-guided needle aspiration biopsy has established itself as the main technique for obtaining a viable sample for histopathological examination [1] [5].

• Abnormal Bone Scan

  Bone Scan : An occasional case where abnormal bone scan is the presenting abnormality. [angelfire.com]

These tumors can be reassessed with CT or FDG-PET/CT scans after treatments for resectability. [ncbi.nlm.nih.gov]

Histological diagnosis is currently imperative prior to the initiation of treatment, and emergency radiotherapy is reserved for the treatment of exceptional cases in which no diagnosis has been made.(12,13) The standard treatment for small cell lung carcinoma [scielo.br]

TREATMENT • The best form of treatment remains the subject of controversy • curative treatment possible. • Patients with superior sulcus pulmonary carcinoma should be considered for surgery after appropriate diagnostic evaluation. • The perfect candidate [slideshare.net]

[…] films of the chest may also be helpful CT scans may assist in evaluating Bone destruction Mediastinal adenopathy Presence of other pulmonary nodules and liver Differential Diagnosis Tuberculosis Plasmacytoma Mesothelioma Subclavian artery aneurysms Treatment [learningradiology.com]

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Also, metastatic NSCLC patients eligible for treatment with targeted therapies have a better prognosis compared with those without targetable genomic variations. [ncbi.nlm.nih.gov]

[…] metastases are contraindications for surgery Preoperative radiation therapy followed by surgical resection is the most common form of treatment Complications Surgical complications include Atelectasis Chest pain Spinal fluid leaks Horner’s syndrome Prognosis [learningradiology.com]

Prognosis Until the 1990s bimodal treatment (pre-operative radiotherapy followed by surgery) resulted in a five-year survival at best of only 30%, and a recurrence rate of 70%[11]. [patient.info]

Pancoast syndrome is an important clinical presentation of several disorders with different etiologies that develop in the apex of the lungs. [symptoma.com]

Venography • Can give precise level of obstruction • Less information on etiology of SVCS • Requires larger contrast dose 22. [slideshare.net]

This report emphasises the importance of establishing a firm pathologic diagnosis of the etiology of Pancoast's syndrome before instituting treatment. [unboundmedicine.com]

Etiology The etiology of Horner’s syndrome varies with the patient age and site of lesion. The etiology remains unknown in 35-40% of cases. [eyewiki.aao.org]

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 [ncbi.nlm.nih.gov]

ICD-9 337.9 Epidemiology In a population based study of Horner’s syndrome in the pediatric age group, the incidence of Horner’s syndrome was estimated to be 1.42 per 100 000 patients younger than 19 years, with a birth prevalence of 1 in 6250 for those [eyewiki.aao.org]

[…] disorder resulting from compression of the brachial plexus and/or subclavian vessels in the interval between the neck and axilla treatment may be nonoperative or include surgical decompression or a vascular procedure depending on the specific etiology Epidemiology [orthobullets.com]

[…] pseudoaneurysm in a child (caused by a hydatid cyst ) Risk factors are similar for almost all lung cancers and include the following: Prolonged asbestos exposure Exposure to industrial elements (eg, gold, nickel) Tobacco smoking Secondary smoke exposure Epidemiology [emedicine.medscape.com]

Grasp the nuances of the pathophysiology of today’s major respiratory conditions—including pulmonary hypertension, COPD, asthma, environmental lung disease, sleep disorders, infections of the immunocompromised, neonatal breathing disorders, and drug-resistant [books.google.com]

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 [oncnursingnews.com]

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 [ncbi.nlm.nih.gov]

Pathophysiology Horner's syndrome results from a lesion to the sympathetic pathways that supply the head and neck, including the oculosympathetic fibers. Relevant anatomy The pupil is innervated by sympathetic and parasympathetic fibers. [eyewiki.aao.org]

Early detection and prompt treatment of paraspinal metastasis are important to relieve symptoms and prevent permanent loss of neurological functions. [ncbi.nlm.nih.gov]

Preventing Pancoast Tumor The most important preventive step is to avoid using tobacco products. Quitting tobacco also reduces the risk of lung cancer. [webmd.com]

Primary prevention Prevention strategies depend on the etiology of Horner’s syndrome. These may include prevention of trauma (especially head trauma), control of cardiovascular risk factors and smoking cessation. [eyewiki.aao.org]

However palliative radiotherapy may benefit the patient in the longer term & prevent further local symptoms. Re-evaluation will take place after 6-8 weeks and subsequent treatment may include chaemotherapy and radiotherapy'. [community.macmillan.org.uk]

1. Sarkar A, Das A, Basuthakur S, Pandit S, Das SK, Choudhury S. Pancoast syndrome: A rare presentation of non-Hodgkin’s lymphoma. Lung India. 2013;30(3):209-211.
2. Foroulis CN, Zarogoulidis P, Darwiche K, et al. Superior sulcus (Pancoast) tumors: current evidence on diagnosis and radical treatment. J Thorac Dis. 2013;5(Suppl 4):S342-S358.
3. Dao I, El Mostarchid B, Onen J, Mandour C, El Asri CA, Boucetta M. Pancaost syndrome related to hydatid cyst. Pan Afr Med J. 2013;14:118.
4. Panagopoulos N, Leivaditis V, Koletsis E, et al. Pancoast tumors: characteristics and preoperative assessment. J Thorac Dis. 2014;6(Suppl 1):S108-S115.
5. Fontenele e Silva J, Barbosa Mde P, Viegas CL. Small cell carcinoma in Pancoast syndrome. J Bras Pneumol. 2009;35(2):190-193.
6. Bansal M, Martin SR, Rudnicki SA, Hiatt KM, Mireles-Cabodevila E. A rapidly progressing Pancoast syndrome due to pulmonary mucormycosis: a case report. J Med Case Reports. 2011;5:388.