My Leptomeningeal hemangioblastomas( rarest brain tumors)

 

Hemangioblastomas of the CNS are solid or cystic vascular-rich tumours, most common in the cerebellum, less frequent in the brainstem or spinal cord and rare in supratentorial locations with meningeal involvement.

Leptomeningeal dissemination of hemangioblastomas (HB) of the central nervous system (CNS) is extremely rare. Between 1902 and 2013, approximately 132 cases were reported. Few studies have reported leptomeningeal involvement in sporadic HB or in HB associated with von Hippel­Lindau syndrome.

A Ga-DOTANOC PET-CT based SSTR imaging because VHL syndrome associated hemangioblastomas frequently express SSTR confirmed that the tumours in my brain are hemangioblastomas. With this, the true nature could be seen and the diagnosis was confirmed.

Detecting and treating the condition of leptomeningeal hemangioblastoma without delay seems to help survival, though the number of patients analyzed is small. Patients may have other underlying health issues that may affect the data.

Because no case of de novo development of disseminated HB without previous surgery has been reported, it is strongly suggested that the spillage and spread of tumour cells through the CSF space may be an origin of hemangioblastomatosis in patients with a genetic predisposition to the condition, Care should be taken to avoid tumour cell spillage during surgery.

These tumours are extremely vascular. A biopsy would make them bleed resulting in a stroke. After my first brain tumour surgery, done in 2006 I woke up with paralysis on the left side. After the craniotomy ( open brain surgery) I experienced total numbness in arms and hands and I couldn't close my fingers and make a fist. But this was sorted out by proper physiotherapy and exercise and she got back the control of my limbs but even now she doesn't have sensations in her fingertips.

I prefer getting stereotactic radio-surgery before the growing tumours start putting pressure on the brain and become symptomatic but I lost the vision of the right eye due to a tumour sitting atop the optic nerve.

Advanced radiation techniques, such as radiosurgery, are more effective than conventionally fractionated radiotherapy, but it is difficult to perform high-dose radiation therapy or radiosurgery for numerous lesions scattered throughout the brain. 

Radiosurgery appears to be safe and has prevented local recurrences in her case, with fewer sessions than conventional radiation which caused white matter changes representing chronic ischemic changes. Since she couldn't afford cyberknife and underwent conventional radiation therapy in 2017 after which this happened.

Sometimes tumours treated with radiation, on follow-up MRI imaging scans, appear stable without evidence of growth even mildly shrinking every time.

But radiation has several side-effects

Radiation Therapy Side Effects: 

Esophagitis and Mucositis.

Since it becomes difficult to swallow foodstuff, it requires to make a puree of finely chopped fresh fruits and vegetables and taking nutritious food that is easy and convenient to swallow. High- protein milkshakes are required. Local anaesthetic mouth wash and artificial saliva help. A lot of help from a good dentist and head and neck specialist helps. The real question is how I swallow my medicines? I crush a few but in the case of the immunosuppressants and anticonvulsants, I mostly choke on them. The rest I choose which I can do without for the time being because doctors are not responding during this pandemic time.

Other side effects are

Fatigue is very common with radiation treatment and tends to begin a few weeks into therapy. Fatigue typically resolves slowly over the weeks and months following treatment but with mucositis, it's hard to get back strength. Also, the two radiation therapies and two cyberknife tend to have a permanent effect but I need more which is being delayed.

Hair loss may occur where you received radiation. Hair typically starts to regrow three months or so after treatment. However,  my hair didn't grow back exactly as it was before treatment, not only hair became thinner but loss became permanent with a bald patch.

Muffled hearing: Your hearing may become muffled during treatment. This typically resolves in 2-4 weeks after finishing treatment, however after the cyberknife 17 March 2020 it hasn't resolved yet but the pain is slowly getting better and that's a blessing.

Skin irritation: The skin in the face is irritated, dry, or sensitive and full of rashes. This is negligible compared to the benefits.

The thyroid gland/Pituitary gland gets affected which causes menstrual problems which could easily have been sorted out by an endocrinologist because I have hypoparathyroidism as well as hypothyroidism. But again a communication gap and not understanding why I am not getting my blood levels tested.

A bump on the tongue appeared after the cyberknife but now it seems smaller than before without any doctor's help.

Short term memory loss- after the 2013 cyberknife there was a  short term memory loss for a long time but never happened in the following radiation treatments.

So you might say why I want a cyberknife and that too in Bombay, well I went to every hospital in Delhi looking for good treatment but all doctors wanted was a biopsy of the rare leptomeningeal hemangioblastomas. A biopsy isn't required for diagnosis as it may cause meningitis and blood loss thereby cell spillage, putting my life in danger.

I live in a special realm

where moments are cherished, 

days are numbered,

and years are celebrated.