After All Else Failed They Came to CA Care. Case of Intracranial Malignant Melanoma

Hui is a 9-year old girl. At birth she had many birth marks/moles ( medically referred to as naevus or naevi).  Over the years, these marks became itchy. Other than that Hui had no problem until September 2014, when she was 8 years old. Hui started to vomit, had headaches and seizure. MRI done on 11 September 2014 indicated “a solid mass lesion in the right thalamus measuring 40 x 42 x 43 mm.”

Where is the Thalamus?

 Thalamus

The thalamus is a small structure within the brain located just above the brain stem between the cerebral cortex and the midbrain and has extensive nerve connections to both. The main function of the thalamus is to relay motor and sensory signals to the cerebral cortex. It also regulates sleep, alertness and wakefulness. http://www.news-medical.net/health/What-is-the-Thalamus.aspx

A biopsy was done and the result showed “features are more in favour of malignant melanoma… in view of the presence of pigmented skin lesions.”

Hui underwent an operation  (crionotomy and EVD insertion) on 2 October 2014. “An attempt to debulk the tumour was abandoned as the tumour was very vascularised.” However, tumour debulking was finally done on 3 October 2014. Histopathology report confirmed malignant melanoma.

A follow up MRI on 11 November 2014 showed “no evidence of residual tumour or tumour recurrence.”

Barely 3 months later, MRI on 30 December 2014, showed tumour recurrence, “it measures approximately 3.1 x 2.3 x 3.7 cm. It appeared to be progressively increasing in size.”

At this point, the parents refused to have further medical treatment as they wanted to try alternative treatment.

On 1 February 2015, Hui had headaches again and started to vomit. She was rushed to the emergency. An urgent CT scan of the brain showed “a dense tumour measuring 5.3 x 3.5 x 5.9 cm with evidence of intratumoural hemorrhage” (internal bleeding).  A left front EVD was inserted to drain the hemorrhage. (EVD: external ventricular drain extraventricular drain; or ventriculostomy. A plastic tube is placed by neurosurgeon to drain fluid from the brain).

A skin biopsy was done over the right giant naevus (birthmark). The result showed “benign intra-dermal naevus”.

Hui underwent another operation – “right re-crionotomy and tumour debulking” on 12 February 2015.  The father said the  tumour was cut out but unfortunately the tumour expanded and the skull could not be put back. Hui was discharged and went home with her head without the “sawn off” skull. However, she was alright and was able to move around.

Unfortunately Hui developed persistent vomiting. A repeat CT scan on 27 February 2015 showed increasing hydrocephalus (fluid) and 2 VP shuntswere inserted. “A revision of the shunt was performed on 3 March 2015 as it was not properly placed.

Her doctor wrote, ” She was quite well since then,  and was discharge on 6 March 2015. We are greatly sorry for not being able to do MRI brain for her as our machine was broken down and her unstable conditioin.”

Hui was referred to KLGH for radiation. The parents were undecided whether to undergo radiotherapy or not and came to seek our advice. According to her parents, Hui was very weak.

Comments

It is indeed a hard case for us to handle. Should Hui undergo radiotherapy? We cannot provide that answer. The parents will have to make that decision.

We have seen many “disastrous” results after a brain operation.

2 Surgery-as-last-resort

4 When not to operation

Dr. Jeffrey Tobia and Kay Eaton (in Living with Cancer) wrote: As far as cure is concerned, there is no use pretending that brain tumours are truly curable.

5 Ssurgery come back again

Update:

This is a message we received on 18 August 2015, from the monk who brought Hui to see us.

On a sad note, the young girl with the brain melanoma passed away on Thursday morning after lapsing into unconsciousness for a week. After we saw you, she underwent the Gamma knife therapy in KL the following week and a couple more VP shunts. The tumours re-grew in about a week in different areas of the brain.

What to do now? Do we have to give up? Here is a sweet story that crossed our path – a melanoma case that had spread to the lungs.

Capture 1

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Comic available at: http://bookoncancer.com/productDetail.php?P_Id=73

 Information from the Internet

Metastatic Intracranial Melanoma

  • Melanoma is a malignancy of melanocytes, which are pigment-producing cells derived from the neural crest. This condition constitutes 3% of all cancers diagnosed in the United States; it is the most lethal form of skin cancer and the third most common malignancy that causes central nervous system (CNS) metastases, after lung and breast cancer. The primary tumor may occur at any location on the skin or, less commonly, on the mucus membranes or other locations. http://emedicine.medscape.com/article/1158059-overview
  • Malignant melanoma (MM) is often reported as the third most common cause of intracranial metastasis after carcinoma of the breast and lung. Most patients with advanced MM will have widespread extracranial disease, but the majority will die from intracerebral spread. http://www.ncbi.nlm.nih.gov/pubmed/7834426
  • 7% of patients diagnosed with melanoma subsequently developed intracranial metastasis. The prognosis of cerebral metastatic melanoma is dismal. Without treatment, the average survival time from the beginning of neurologic symptoms was 65 days in one study . Even with chemotherapy and radiotherapy, the survival time has only been extended to a range of 4 months to approximately 2 years. http://radiopaedia.org/articles/intracranial-metastatic-melanoma-2
  • Primary intracranial melanoma is uncommon and accounts for only approximately 1% of all cases of melanoma. http://www2.cmu.edu.tw/~mtjm/full-text/7%282%29p118-123.pdf
  • Central nervous system (CNS) metastases occur in 10 to 40% of patients with melanoma. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3197072/

Treatment

  • Disseminated metastatic disease, including brain metastases, is commonly encountered in malignant melanoma. The classical treatment approach for melanoma brain metastases has been neurosurgical resection followed by whole brain radiotherapy. Traditionally, if lesions were either too numerous or surgical intervention would cause substantial neurologic deficits, patients were either treated with whole brain radiotherapy or referred to hospice and supportive care. Chemotherapy has not proven effective in treating brain metastases.

Prognosis

  • Metastatic melanoma patients overall have a median survival of only 6–10 months and a 5-year survival of less than 10% .There has been virtually no improvement in survival of those patients in the past several decades. http://www.hindawi.com/journals/jsc/2011/845863/

 

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