Malignant Spine Presentations

Authors: Sabia Rashid, Nick Glascow, Charlotte Davies / Editor: Liz Herrieven /  Codes: CAP20, HAP19, HAP2, HAP21, HAP6, OncC1, OncP1, SLO3 / Published: 24/08/2021

We know symptom control is important – and palliative care doctors are expert at treating constipation, pain, agitation, nausea and vomiting, and oncologists are expert at treating…well, cancer. Here are two cases of oncological and palliative care emergencies that might present to the emergency department, that we should know how to treat. Even if the patient is approaching the end of their life with a palliative level of treatment, these conditions should be investigated and treated as we can still make a difference. We’ve covered palliative care well in our induction modules here.

1.Metastatic Spinal Cord Compression 

A 68-year-old with a history of prostate cancer attends the emergency department feeling weak, with constipation and back pain. Examination is difficult due to pain, but you identify some weakness of hip flexion bilaterally. What’s going on? 

The first and easiest thing to rule out is hypercalcaemia, covered in a reference page, with two RCEMLearning modules – here and here.  

Next, you need to consider whether this could be metastatic spinal cord compression, which is a rare but important presentation. There are an estimated 4000 cases per year, or >100 cases per cancer network each year. This might even be the first presentation of a cancer. 

The red flag symptoms that require urgent MRI are neurological symptoms – radicular pain, limb weakness (in 60 – 85% – unsteady gait, difficulty walking or transferring, reduced functional ability), sensory loss (less common), bladder or bowel dysfunction. The urinary symptoms are interesting – unlike cauda equina where the concerning incontinence is caused by overflow, in MSCC the site of the nerve damage to sensory and motor tracts of the spinal cord might cause true urinary incontinence, or retention, or both. If the MSCC is above T12, the patient will have an upper motor neurone (spastic) bladder – incontinence, no voluntary control of bladder emptying  If the MSCC is below T12, the patient will have a lower motor neurone (flaccid) bladder – urinary retention, dribbling incontinence when the bladder is overfilled, large post-voiding residual volumes. Some patients may have a ‘mixed’ bladder when there is only partial compression of the spinal nerves. 

The other worrying symptoms that need an MRI within 24 hours are back pain with autonomic dysfunction, progressive severe unremitting pain, pain aggravated by straining, localised spinal tenderness and nocturnal spinal pain.  

The majority of MSCC occurs in either the thoracic (60-70%) or lumbar spine (20-30%). However, a small but no less important proportion of MSCC can present in the cervical spine (roughly around 5% of cases).

Consider the following:

An 82-year-old male with a known history of prostate cancer presents to ED with intractable neck pain for the last week. There are no focal neurological symptoms found on examination and no sphincter disturbance.

It is important to consider that in spite of a normal neurological examination, pain that is severe or unremitting, nocturnal in nature, or aggravated by straining is still a red flag in the context of MSCC. By extension, these patients need an urgent MRI within 24 hours to exclude MSCC.

Assessment:

– Full neurological examination including PR.
– Full musculoskeletal examination of the painful part. 

Treatment:

– 16mg dexamethasone loading dose as soon as possible after assessment.
– Blood glucose monitoring and gastric protection.
– Observe for signs of autonomic dysreflexia.
– Assess spine and neurology regularly.
– Nurse flat, and log roll until bony and neurological stability ensured.
– Consider a catheter as an unstable spine might not allow for a bed pan.
– Bowel management.
– Pain management.
– Thromboprophylaxis.
– Consider nil by mouth if surgery is a possibility.
– Further treatment dependent on MRI result. 

Investigation: 

Blood tests are useful as, if the MRI is normal, other things can be considered. Do a FBC to look for anaemia, U&E to check renal function and to guide prescribing options, LFTs to check for any derangement that might indicate liver metastasis, CRP to consider spinal infection, Calcium, blood glucose. 

MRI of the whole spine

Be aware of possible autonomic dysreflexia: a stimulus below the level of the spinal lesion (for example a full and irritated bladder) causes an uncoordinated autonomic response, leading to potentially life-threatening hypertension.

The image shows compression of the cord, followed by images used to plan radiotherapy. With thanks to Dr Vivek.

 

2. Another Malignant Spine

A 45-year-old man presents with thoracic back pain for the last 2 months, worse over last week with some numbness in his feet. He also noticed worsening fatigue and new onset of constipation for the last two weeks. Fell off his cycle and sustained an injury to his ankle, hence the visit to ED. FBC shows Hb 100, WCC 3.5, Platelets 134.

Spinal myeloma disease (SMD) is present in up to 60% of patients at diagnosis which may or may not be symptomatic. SMD is most commonly indicated by the presence of lytic lesions or generalized osteopenia. Most lesions occur in the vertebral bodies but they can also be found in other parts of the vertebral column including the facets, pedicles and transverse/spinous processes. Affected vertebral bodies may become weakened with progressive bone destruction and eventually collapse, resulting in a vertebral compression fracture. Spinal cord compression can also result from direct extension of tumour into the epidural space.
This spinal cord compression (SCC),  is an oncologic emergency that can cause pain and potential paralysis, occurring in approximately 5% of all patients with myeloma often leads to disability and a profound impact on prognosis, even if myeloma is otherwise contained.

Myeloma with spinal involvement may represent with spinal cord or cauda equina compression, with or without neurological impairment. Signs and symptoms of SCC include pain (83–95%), motor defects (60–85%), sensory deficits (40–90%); and bowel and bladder dysfunction. Back pain is typically the first symptom.

Weakness is the most apparent and problematic manifestation of SCC. Sensory deficits are less common. Bowel and bladder dysfunction tend to occur late and typically match the degree of weakness.MRI or CT or PET CT of the entire spine must be performed immediately if this complication is suspected.

Prompt diagnosis and immediate treatment are critically important in the preservation of neurological function in patients with SCC. The goal of the treatment of the SCC is adequate control of pain; relief of spinal cord or cauda equina compression and maintenance of spinal stability; preserving or improving neurologic functions.

Management of patients with SCC includes the immediate administration of steroids in nearly all patients, followed by surgery, external beam radiation therapy (EBRT), or stereotactic body radiotherapy (SBRT).

Investigations

FBC- anaemia +/- thrombocytopenia
U&E- renal impairment
Liver functions- Raised total protein/globulins (albumin is usually reduced)
Bone profile- look for hypercalcemia
Serum electrophoresis- raised immunoglobulins, monoclonal protein and raised serum free light chains with abnormal SFLC ratio
Bone marrow biopsy
Imaging- Whole body MRI/PET CT/CT in the descending order of preference. If myeloma spine disease suspected- urgent MRI whole spine

In patients with neurologic symptoms directly due to cord compression, radiation therapy (RT) is given along with dexamethasone, and up to half of patients may have improvement of motor function with RT with longer fractionation schedules providing better relief.

Systemic therapy with regimens such as bortezomib, cyclophosphamide, dexamethasone or bortezomib, thalidomide, dexamethasone work rapidly and can be used instead of radiation in selected patients if there is minimal neurologic deficit. Surgical decompression is necessary only if the neurologic deficit does not improve or if the compression is due to retropulsed bone

Symptomatic treatment of SCC often begins prior to definitive therapy and consists of  pain management, bed-rest and anticoagulation. Patients with SCC have frequently severe pain, often limiting the ability to perform a thorough neurologic examination. Corticosteroids usually improve the pain within several hours, but most patients require opiate analgesics to tolerate the physical examination and necessary diagnostic studies.

Plasma cell

Plasma Cell
Image1

 

3. SVCO

A 62-year-old ex-smoker attends complaining of shortness of breath on exertion, and a swollen left arm. On examination, her chest is clear. Her left arm is swollen with non-pitting oedema, and you notice a few prominent veins. Her CXR demonstrates a right sided mass. 

Superior vena cava obstruction (SVCO) is often a complication of cancer. Its presentation can be subtle, and most cases are secondary to malignancy, that may or may not be diagnosed yet!  

SVC syndrome. 80% malignancy (right sided lung and mediastinal masses). Thrombotic 20% (indwelling line or similar)

Back to basics… the superior vena cava is formed from the right and left brachiocephalic veins, carrying venous blood from the upper body. It begins behind the first right costal cartilage and descends to drain into the right atrium at the level of the third costal cartilage. A right sided malignancy can compress it, causing obstructive features. It is unlikely to be compressed at the superior vena cava level on the left, because of the way it tucks over the aorta. 

Anatomy of the superior vena cava syndrome.
Image2

If the SVC is compressed by a mass, there may be symptoms and signs arising from the mass itself, along with symptoms and signs of SVC obstruction. These include: 

  • Shortness of breath 
  • Reduced contractility leading to pericardial effusion 
  • Neck masses
  • Distended arm, neck and chest veins 
  • Facial swelling and plethora 
  • Cough
  • Hoarseness or stridor 
  • Headaches, visual symptoms, coma, confusion, dizziness (less common)

The mediastinal venous vasculature may be blocked further along its distal route at the subclavian or axillary level. This is less likely to be due to malignancy, and more likely to be due to thrombosis or other features. 

Diagnosis in the emergency department is based on CT scanning. A CXR may show an aortic nipple due to congestion of the azygous vein, and may confirm the presence of a malignancy, if this was previously unknown. 

A contrast CT will be needed to confirm whether the obstruction is due to the malignancy, or due to thrombus. Getting the CT scan can be difficult if the patient is unable to lay flat. Treatment for this won’t be started in minutes or hours, so the scan is able to be delayed until working hours. In most departments, this would require an acute admission rather than an ambulatory care referral. Once the CT has confirmed SVCO, treatment depends on the type of malignancy causing the compression and may include chemotherapy or a stent. Your oncology team will need to be involved in the management of this patient. 

Here are some images from Dr Vivek of SVCO on CT, followed by a treatment regime, stent placement, and further tumour growth around a stent. 

SVCO: CXR shows aortic nipple and right sided malignancy. CT is in working hours with contrast. Treatment: oncology input. ?chemo ?stent

Thrombotic causes of obstruction can also happen and are difficult to rule out, because neither the Wells score nor the d-dimer have been validated in upper limb VTE – see here and here. The Constans score can be used to help, and reminds us that upper limb thrombosis is normally due to a foreign substance like a pacemaker. 

On first look the sensitivity and specificity doesn’t seem great – but if you compare it to the Wells Score which has a low pre-test probability specificity of 2-29% and sensitivity of 24-77%, it’s probably as good as the Wells. If you need a refresher on specificity and sensitivity have a look at our induction module on VTE available here

The subclavian vein can be compressed by:

*Thrombus: Idiopathic
Secondary to repeated trauma and exercise (eg rowing) which can cause a tight band of scar tissue. Commonly caused Paget-von Schroetter Syndrome. 

*Ribs: If there is a tight gap between the clavicle and the first rib 

*Thoracic Outlet: As the subclavian passes through the thoracic outlet found in the subclavius muscle it can be squeezed. This can also cause neurogenic compression which is painful. 

Diagnosis is by CT or by ultrasound. A CT is often used to see the extent of any thrombus, but many centres would get an ultrasound as firstline. 

So does your patient have an axillary DVT, or an SVC obstruction? Clinically, if a patient has any shortness of breath, it’s SVCO until proven otherwise. If it’s just a swollen arm, it can be treated as an axillary thrombosis until proven otherwise. The treatment is slightly different – but as cancer patients are at high risk of VTE, anticoagulants are unlikely to be harmful. 

We hope you’ve enjoyed this highlight of some of the key points of two oncological emergencies. Neutropenic sepsis is still important, and should be treated promptly, before the FBC result is back – especially in patients with blood cancers… but SVCO and MSCC are also worth knowing about. 

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Further RCEMLearning Resources

References and Further Reading

  1. NICE, Metastatic spinal cord compression in adults: risk assessment, diagnosis and management: Clinical guideline [CG75]. 26 November 2008.
  2. Prostate Cancer UK, Metastatic spinal cord compression (MSCC).
  3. RNOH, MSCC – Professional Information.
  4. SGH, Metastatic Spinal Cord Compression (MSCC).
  5. SGH, The South West London Metastatic Spinal Cord Compression (MSCC) Service SOP. JUly 2015.
  6. Al-Qurainy R, Collis E. Metastatic spinal cord compression: diagnosis and management. BMJ 2016;353:i2539.
  7. Jackson CR, Acland R. Knowledge of autonomic dysreflexia in the emergency department. Emergency Medicine Journal 2011;28:866-869.
  8. Kyle RA, Gertz MA, Witzig TE, Lust JA, Lacy MQ, Dispenzieri A, et al. Review of 1,027 patients with newly diagnosed multiple myeloma. Mayo Clin Proc. 2003; 78: 21-33.
  9. Sean Molloy, Maggie Lai, Guy Pratt, Ramasamy K, Wilson D, Quraishi N, et al. Optimizing the management of patients with spinal myeloma disease. Br J Haematol. 2015; 171: 332-343
  10. Basic-kes V, Basic-Jukic N, Kes P, Demarin V, Labar B. Neurologic sequelae of bone changes in multiple myeloma and its therapy. Acta Med Croatica. 2002; 56: 103-107.
  11. Helweg-Larsen S, Sørensen PS, Kreiner S. Prognostic factors in metastatic spinal cord compression: a prospective study using multivariate analysis of variables influencing survival and gait function in 153 patients. Int J Radiat Oncol Biol Phys. 2000; 45: 1163-1169.
  12. Rades D, Hoskin PJ, Stalpers LJ, Schulte R, Poortmans P, Veninga T, et al. Short-course radiotherapy is not optimal for spinal cord compression due to myeloma. Int J Radiat Oncol Biol Phys. 2006; 64: 1452-1457
  13. Schiff D, Brown P, Shaffrey ME. Treatment and prognosis of neoplastic epidural spinal cord compression, including cauda equina syndrome. Up to date. 2016.
  14. Image1: File:Multinucleated plasma cell — extremely high mag.jpg.” Wikimedia Commons, the free media repository. 23 Sep 2020. [Accessed: 10/08/2021].
  15. Image2: Superior Vena Cava Syndrome. About Cancer. [Accessed: 10/08/2021].

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