Oesophageal Cancer

Interview with Theresa Lowry Lehnen (GPN, RNP, PhD) – Clinical Nurse Specialist and Associate Lecturer Institute of Technology Carlow

Oesophageal cancer (OC) is the eighth most common cancer and sixth most common cause of cancer mortality worldwide. Oesophageal cancer is more common in people aged over 60. It affects more men than women. More than 500 people are diagnosed with it in Ireland every year.

The oesophagus is the neuromuscular tube that connects the pharynx with the stomach and lies in the posterior mediastinum within the thorax near the lung pleura, peritoneum, pericardium, and diaphragm. The upper and lower oesophagus are controlled by the sphincter function of the cricopharyngeus muscle and gastro-oesophageal sphincter, respectively. The functions of the oesophagus and its sphincters are to transport swallowed materials from the pharynx to the stomach, and to defend the airways and itself from the reflux of gastric contents.

Speaking about how oesophageal cancer develops, Theresa Lowry Lehnen, Clinical Nurse Specialist and Associate Lecturer Institute of Technology Carlow says, “Oesophageal cancer occurs when cells in the oesophagus develop mutations in their DNA and the cells grow and divide out of control. The accumulating abnormal cells form a tumour in the oesophagus that can grow to invade nearby structures and spread to other parts of the body. The tumour does not cause symptoms at first, but as it grows causes difficulty with swallowing. As oesophageal cancer expands, the lumen of the oesophagus narrows, and dysphagia occurs due to mechanical obstruction. Due to the muscular and expansive nature of the oesophagus, symptoms from an obstructing or stricturing lesion may only become apparent when the tumour has reached a relatively locally advanced or even metastatic stage.”

Symptoms of oesophageal cancer include:

  • Dysphagia
  • Acid reflux
  • pain in the sternum, back or throat
  • Weight loss
  • Anorexia
  • Cough

Risk factors for developing oesophageal cancer include increasing age, gender (OC is two to three times more common in males than females), gastroesophageal reflux disease (GORD), Barrett’s oesophagus, achalasia, obesity, smoking, alcohol, drinking very hot liquids, poor diet and undergoing radiation treatment to the chest or upper abdomen.

The main risk factors associated with development of oesophageal adenocarcinoma are gastrooesophageal reflux, obesity, high intake of red meat and low intake of fruits and vegetables. Oesophageal squamous cell carcinoma develops from squamous epithelial cells and is typically localised to the upper two-thirds of the oesophagus. Tobacco consumption and alcohol intake are the most notable risk factors, although their relative risk varies by region.

Theresa says, “The increase in the incidence of oesophageal adenocarcinoma has paralleled with the rise of obesity in the western world. A variety of observational studies, systemic reviews and meta-analyses have shown and confirmed association between obesity and OAC. The risk of OAC in patients with a BMI of 30 or above is approximately 16 times greater compared to those with a normal BMI.”

Furthermore, patients with Barrett’s oesophagus have been shown to have a 30 to 60-fold increase in the incidence of oesophageal adenocarcinoma, although the annual absolute risk of developing OAC is 0.12%. The incidence of Barrett’s oesophagus is two to three times higher in men than women, and male sex is an independent risk factor for malignant transformation.

“The role of HPV infection in the development of oesophageal cancer has long been suspected,” she adds. “Although HPV has been widely studied, the overall rate of HPV infection in oesophageal squamous cell carcinoma remains controversial and there is a lack of robust evidence for a definitive etiological role.

“The association between HPV and oropharyngeal SCC, and the histologic similarities between the squamous epithelium of the oral mucosa and upper oesophagus could suggest a similar association. HPV16 and HPV18 are the most frequently detected types in HPV-associated cancers. Studies have shown a significant association between HPV16 and OSCC, but not HPV18.

Diagnosis, Staging and Treatment

In 2019, new national clinical guidelines, ‘Diagnosis, staging and treatment of patients with oesophageal or oesophagogastric junction cancer National Clinical Guideline No. 19’ developed by multidisciplinary groups supported by the HSE’s National Cancer Control Programme (NCCP) and quality assured by the Department of Health’s National Clinical Effectiveness Committee (NCEC) were launched to help with the diagnosis, staging and treatment of patients with oesophageal or oesophagogastric junction (OGJ) cancer. It details advances in staging, diagnosis, and treatment that define and underpin a modern standard of care across all designated centres in Ireland.

Theresa adds, “Patients with oesophageal cancer usually present with dysphagia, prompting endoscopy and biopsy. Diagnosis incudes clinical history, examination, barium swallow, endoscopy, endoscopic ultrasound (EUS), liver ultrasound scan, biopsy. Endoscopy is the traditional gold standard for detecting oesophageal cancer, but less invasive screening methods are being developed such as Cytosponge, a cell collection device that is swallowed and retrieved with a string, followed by analysis of cells for trefoil factor 3 protein. This method aims to identify patients who warrant an endoscopy for suspected Barrett’s oesophagus.

“Tumour characteristics documented at endoscopy should include the exact site relative to the gastro-oesophageal junction, extension into the stomach and distance from the teeth, length of the lesion, circumferential involvement and presence of obstruction. Any adjacent pre-malignant lesions, i.e. squamous dysplasia or Barrett’s oesophagus should be documented and measured. Tumours of the oesophagus are conventionally described in terms of distance of the upper border of the tumour to the incisors.

When measured from the incisors via endoscopy, the oesophagus extends approximately 30 to 40 cm. The oesophagus is divided into four main segments: Cervical oesophagus (15–20 cm from the incisors); upper thoracic oesophagus (20–25 cm from the incisors); middle thoracic oesophagus (25–30 cm from the incisors) and lower thoracic oesophagus and gastroesophageal junction (30–40 cm from the incisors). Once the diagnosis is confirmed, clinical staging is the next step for which computed tomography (CT) and positron-emission tomography (PET) are the two most useful imaging tools.”

Treatment for oesophageal cancer includes surgery, radiotherapy and chemotherapy. Management is dependent on the patient’s characteristics and the tumour, mainly the TNM-stage.

She adds, “Tumour, node and metastasis (TNM) staging is the most common way to stage oesophageal cancer although the number staging system can also be used. A higher number, such as stage 4, means a more advanced cancer. Early tumours may be suitable for endoscopic removal, whereas more locally advanced cancers are treated with chemotherapy, chemoradiotherapy, surgical resection or combinations of these.”

There are 4 stages of tumour size in oesophageal cancer – T1 to T4.

Tumour (T)

T1: the cancer has grown no further than the submucosa. T1 is divided into T1a and T1b.

T1a: the cancer is in the inner layer (mucosa) or thin muscle layer of the oesophagus wall.

T1b: the cancer has grown into the supportive tissue (submucosa).

T2: the cancer has grown into the muscle layer of the wall of the oesophagus.

T3: the tumour has grown into the adventitia, the membrane covering the outside of the oesophagus.

T4: tumour has grown into other organs or body structures. T4 is divided into T4a and T4b.

T4a: the cancer has grown into the pleura, pericardium, diaphragm, or peritoneum.

T4b: the cancer has spread into nearby structures such as the trachea, vertebra or the aorta.

Node (N) 12

Node (N) describes whether the cancer has spread to the lymph nodes. There are 4 possible stages – N0 to N3.

N0: no lymph nodes containing cancer cells.

N1: there are cancer cells in 1 or 2 nearby lymph nodes.

N2: there are cancer cells in 3 to 6 nearby lymph nodes.

N3: there are cancer cells in 7 or more nearby lymph nodes.

Metastasis (M) 12

Metastasis (M) describes whether the cancer has spread to a different part of the body. There are 2 stages of metastasis.

M0 means the cancer has not spread to other organs.

M1 means the cancer has spread to other parts of the body.

Treatment

According to Theresa, treatment requires a multidisciplinary team approach and optimal therapy is still debated. Endoscopic therapies, including radiofrequency ablation, endoscopic mucosal resection and endoscopic sub mucosal dissection, have become the standard treatment modality for Barrett’s oesophagus and early carcinoma.

“Advances in the understanding of the role of genomic instability in Barrett’s oesophagus will facilitate identifying patients at risk for malignant transformation who would benefit from early intervention,” she adds. Multimodal treatment, which includes chemotherapy, radiation therapy followed by surgical resection or without surgical resection, in varying orders remains the main mode of treatment for most patients.

“Chemotherapy can be given before surgery to shrink the tumour and make it easier to remove or after surgery, to lower the risk of the cancer coming back. Chemotherapy may also be required both before and after surgery. This is common with adenocarcinoma. Many oesophageal cancer patients receive a combination of two or three chemotherapy drugs.

“External radiotherapy uses highenergy rays to kill or shrink cancer cells. The rays are aimed directly at the tumour. It can be given before surgery to shrink the tumour or after surgery to kill any cancer cells left behind to prevent the cancer coming back. 15

During Internal radiotherapy (brachytherapy), the radiation source is placed into the oesophagus for several minutes. Released radiation aims to kill the cancer cells but causes little or no damage to the nearby healthy tissue.

“In some patients with partial oesophageal obstruction, dysphagia may be relieved by placement of an expandable metallic stent or by radiation therapy if the patient has disseminated disease or is not a candidate for surgery.

“Surgery is the most common treatment for cancer that has not spread outside the oesophagus. Minimally invasive surgical approaches have become the standard for oesophagectomy and current literature has demonstrated similar oncological outcomes with reduced morbidity.7 As oesophageal cancer surgery is associated with considerable morbidity and changes in postoperative quality of life, careful attention to patient selection for resection is essential in order to minimise the risk of futile surgery in patients with incurable disease.”

St James’s Hospital, Dublin is the National Centre for Oesophageal and Gastric Cancer and the National Centre for Management of Early Upper Gastrointestinal Mucosal Neoplasia. The hospital manages approximately 65% of oesophageal surgical resections nationally. Cure rates are improving, with overall survival at 35%, 65% for node negative disease, and 75% for Stage I/II disease. Outcomes are consistent with best international benchmarks.

Theresa notes, “Patients with oesophageal cancer face several challenges during the course of their illness and treatment because of the uncertainty in their prognoses and complexity and side effects of the treatment. Symptoms affect patients’ quality of life on a physical, social, and emotional level.

“Oesophageal cancer patients’ quality of life is first negatively affected by the obstructing tumour and later by complex treatment. Before a diagnosis of oesophageal cancer is established a majority of patients have experienced dysphagia and appetite loss, resulting in considerable weight loss and fatigue, which influence the patients’ daily living and quality of life. Patients with advanced tumour stage may experience additional problems, e.g. odynophagia, hoarseness and coughing due to tumour overgrowth or metastatic disease.

“Health professionals establish strategies and play an important role in preparing, supporting and educating patients and their families about what to expect during and after treatment and ensure that patients’ vulnerability is addressed throughout the treatment pathway. Given the poor prognosis of OC and impact of surgery, it is important to address patients’ support needs to help them regain control, reduce their anxiety, improve compliance, create realistic expectations, promote self-care, and generate feelings of security.

“In most cases, oesophageal cancer is a treatable disease, but it is rarely curable. The 5-year relative survival rate is 19.9%. As a new approach, targeted therapies and novel drugs play an important role in the current and future treatment of cancer.”

References available on request

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