Will cavernous hemangiomas recur after total resection of difficult locations such as brainstem, cervical medullary, basal ganglia, and ventricles?

The brainstem is the center of human life, including the midbrain, pons, and medulla oblongata, and cavernous hemangiomas of the brainstem can cause brainstem hemorrhage, causing patients to appear paralyzed, comatose, and even life-threatening
.
In recent decades, with the development of neurosurgical technology and the application of high-tech surgical equipment such as intraoperative neuronavigation, MRI, neuroelectrophysiological monitoring, etc.
, the "forbidden area" of brainstem surgery is gradually being crossed and broken, surgery has become the preferred treatment strategy for brainstem cavernous hemangioma, if the recurrence rate after total resection is rare, incomplete resection or radiotherapy is easy to recur tumor residue is high
.
This treatment strategy is not only for the brainstem, but also for cavernous hemangiomas in difficult locations such as cervical medulla, basal ganglia, and ventricles
.

It is not an incurable disease, the best treatment in the world can be cured by advanced surgery, the key and difficult point is total resection of vascular malformation without causing surgery-related nerve damage
.
At present, these difficult locations of cavernous hemangiomas are still very challenging for doctors, and only a few neurosurgical experts can truly achieve safe and complete surgical resection, no sequelae, and no recurrence curative treatment
.

Will the brainstem cavernous hemangiomas recur after total resection? How did Professor Kanba evaluate?

1.
What is the probability of recurrence after surgery for brainstem cavernous hemangiomas complicated with venous malformations?

35-year-old Xiaoling went to the local hospital for 1 month due to dizziness, nausea and vomiting, unstable walking center of gravity, tongue and facial numbness, cranial CT and MRI showed left pontine brain, left fourth ventricle high-density opacity, right cerebellar old hemorrhage, considering cavernous hemangiomas combined with venous malformations, doctors told that the location of cavernous hemangiomas is difficult and the operation is very
risky.
Although the symptoms have been temporarily relieved, Xiaoling and her family know that the threat of brainstem cavernous hemangiomas being removed without surgery and causing more serious symptoms due to re-bleeding still exists, and seeking the opportunity for surgery can truly solve the root problem
.
After many inquiries, the family learned on the Internet that the world's brain tumor master INC Professor Bartlanfeld of Germany was about to come to China in November for difficult demonstration surgery, so they immediately decided to consult whether the professor could perform surgery
for himself in China.

Professor Ba replied

"For me, cavernous hemangiomas in this location, I can remove 100 percent, so the risk of recurrence is very low
.
But it is important to know that these two, venous malformations and cavernous hemangiomas, have genetic causes, and in rare cases, cavernous hemangiomas can recur, even in different locations
.
”

2.
Is a 12-year-old boy with brainstem pontine cavernous hemangiomas with hemorrhage suitable for conservative treatment? Will it return to normal after surgery?

Xiaoyu, a 12-year-old boy, developed headache and nausea without obvious cause one year ago, and presented to the local hospital with magnetic resonance examination showing cavernous hemangiomas
of the brainstem.
After visiting other hospitals, because there are no other symptoms, the doctor recommends conservative observation
.
In June this year, Xiaoyu felt weakness in his legs, and the hospital examination showed that the hemangioma's hemangioma's bleeding was small, and the symptoms disappeared
after conservative treatment.
In October, Xiaoyu suddenly felt weakness in his left hand and underwent magnetic resonance examination, which showed that the hemangioma's hemangioma was bleeding
again.
The hospital recommended surgery, but when asked about the risks of surgery, the doctor hesitated, saying that brainstem surgery is extremely risky and is likely to damage cranial nerves
.
Xiaoyu has already had 2 bleedings, and the risk of re-onset symptomatic bleeding is very large, and if it is repeated attacks, it will even have devastating consequences, and the potential threat to patients is huge
.

Professor Ba replied

The recurrence rate is approximately 1%.

Of course, we must have a mental account of this 1% risk, which means that out of 100 patients with cavernous hemangioma, 99 will not recur.

Only one patient will relapse, possibly within
10 years.
Therefore, I recommend magnetic resonance examination for patients with cavernous hemangioma
.
First once a year, then every two or three years, just to make sure nothing goes wrong
.

3.
Can C4-5 intramedullary cavernous hemangioma cavernous hemangiomas recover after surgery? What is the probability of recurrence after surgery?

Mr.
Yang began to develop numbness from the left hand to the upper part of the left elbow joint 2 and a half years ago, when cervical MRI was performed to find abnormal signals in the cervical medullary, considered cavernous hemangioma, and suggested cervical disc herniation, conservative observation and physiotherapy
were recommended.
After physiotherapy
, the patient's symptoms did not improve significantly.
Subsequently, the patient then repeated MRI to observe the lesion, which showed a slight change
in the size of the lesion.
Some experts recommend CyberKnife treatment, others recommend that surgery is very risky, and regular follow-up
should continue.
Since this year, patients have increased the numbness
of the left and right ring fingers and index fingers.
No other discomfort.

Current situation: persistent numbness from the mouth of the left hand to the upper part of the left elbow joint, and occasional numbness of the left and right ring fingers and index
fingers.

Professor Ba replied

"Patients recover well after surgery, but it can take more than
6-8 months.
Because lesions can be completely removed, recurrence occurs only in exceptional circumstances (up to 1-2%)
.
”

Screenshot of Professor Ba's email reply

4.
Multiple cavernous hemangiomas, repeated bleeding for 16 years, will it recur after surgery?

In 2005, 2-year-old Jiajia was diagnosed with multiple cavernous hemangioma
.
In 2007, 4-year-old Jiaja underwent the first operation in China to remove cavernous hemangiomas on both sides, thinking that he could get a healthy life, who would have expected that this was just the beginning of the story.
.
.
.
In 2013, 10-year-old Jiajia suddenly fainted at school, and the re-examination found parietal hemorrhage and basal ganglia hemorrhage, and after conservative treatment, he experienced a severe basal ganglia hemorrhage, and after treatment in domestic hospitals, his hands appeared involuntarily grasping movements, the corners of his mouth twitched, blinking and other symptoms, and his condition worsened
.
In 2021, Jiajia's parents finally found Professor
Ba.

Professor Ba replied

"If we decide on surgery, the surgical removal rate will be 100%.

After complete resection, the likelihood of recurrence is extremely low, but in cavernous hemangioma, the answer is not 100% because it is a genetic disorder
.
In another place, new lesions that are now invisible may occur later (not necessarily, but possible)
due to genetic drive.
”

Screenshot of Professor Ba's reply

5.
What is the chance of recurrence after resection of brainstem cavernous hemangiomas with hemorrhage?

One day six months ago, 25-year-old new media blogger Haochen (pseudonym) was working on an idea
.
Suddenly, a slight numbness hit his right hand and foot, and he thought it was just pressed
.
I didn't expect to suddenly have ringing in the left side of the ear two days later, and there would be discomfort when the outside sound was loud
.
This was followed by dizziness and double vision
after a nap.
Out of instinct, Haochen decided to go to the hospital for a check-up
.
The results of the examination surprised him: he found that his left eye could not be abducted, CT examination showed bleeding in the left brainstem, and the doctor diagnosed it as "brainstem cavernous hemangioma" with hemorrhage, which caused him to have the above symptoms
.
The doctor said: "In a situation like yours, we can't operate, it is recommended to go home to rest and conservative treatment and observation.
.
.
" However, only 6 months later, Haochen has successfully operated on it, and the "brainstem lesion" in his brain has been completely removed
by Professor Ba.
And, now back to work and work!

Professor Ba replied

"The risk of recurrence in my patient family is 3-4% because cavernous hemangiomas are a genetically pathogenic disease and the gene drivers that lead to the emergence of new cavernous hemangiomas cannot be completely eliminated
by surgery.
" Screenshot of Professor Ba's reply

Regarding relapse, what does Professor Ba mean by "gene drive"?

Although initially thought to be congenital, cavernous hemangiomas are now known to be acquired, and many reports confirm that patients with normal MRI findings later develop cavernous hemangiomas
.
Data show that with age, the incidence of cavernous hemangiomas is increasing, which also supports acquired causes
.

Cavernous hemangiomatoma lesions

About 80% of patients are sporadic
.
Typically, patients have a single lesion, often accompanied by developmental venous abnormalities (DVA).

Some data support the potential role
of DVA in influencing bleeding risk.
Approximately 30% of patients with sporadic cavernous hemangiomas will have associated developmental venous abnormalities
on standard MRI sequences.

Up to 20% of patients are familial
.
The three known protein-coding genes that cause familial cavernous hemangiomas are: KRIT1 (CCM1), Malcavernin (CCM2), and PDCD10 (CCM3) (see Figure 3).

These genes regulate signaling pathways
involved in endothelial cell tight junction stability, cell proliferation, and angiogenesis.
Familial cavernous hemangiomas are usually characterized by multiple cavernous hemangiomas without associated developmental venous abnormalities, but there are rare cases
of familial cavernous hemangiomas associated with developmental venous abnormalities.

In his paper, Professor Ba mentioned that cavernous hemangiomas (CCMs) are vascular lesions associated with
loss-of-function mutations in one of the three genes encoding KRIT1 (CCM1), CCM2 and PDCD10.

Patients with multiple lesions or a family history of single lesions should consider genetic counseling and testing
.
Familial patients may also have skin lesions (capillary vein malformations) and retinal hemangioma
.
Familial patients may develop new CM lesions at a rate of approximately once every 2 years
.
Patients with childhood-onset are at greater risk of intracerebral haemorrhage and may have other systemic conditions, including scoliosis, meningiomas, astrocytomas, and vestibular schwannomas (acoustic neuromas).

So far, none of the INC patients with cavernous hemangiomas operated by Professor Ba have recurred

Brainstem cavernous hemangioma has only "1 month of life" left, and there is no recurrence for 3 years after safe total resection

"I found experts from many big hospitals in China, and they all told me to treat
conservatively.
I was very discouraged, but I found Professor Bartlangfi and saw hope.
.
.
I am very satisfied with the results of the surgery and now live
exactly like a normal person.
It is recommended that patients with cavernous hemangioma, who have a wide brain trunk, must be treated as soon as possible, and the more delayed
, the more critical the condition.
”

Recurrent brainstem hemorrhage and severe facial paralysis for nearly 2 months, no recurrence for 3 years after safe total resection

"At that time, the domestic doctor looked at my condition, felt that the operation was risky, the sequelae would be more serious, after having the opportunity to meet Professor Ba, Professor Ba gave me treatment advice, very detailed said that he could help me do the operation, and the impact of the operation on my later life, which gave me a lot of confidence, I decided to ask Professor Ba to do this operation, very satisfied with the surgical effect, from the quality of life, got a very good recovery, Professor Ba's surgery still let me get a lot of benefits.
"
。 ”

Brainstem hemorrhage caused eye movement disorders and mild facial paralysis, and there was no recurrence for 3 years after safe total resection

Climbing, playing basketball, jumping on the bed, playing with toys.
.
.
Congcong in the video, playful and funny, with a bright smile on his face, is almost 5 years old and is in kindergarten middle class this year, like all children of the same age, he is full of vitality every day, and his body seems to have endless energy
.
However, 3 years ago, this sunny and happy little boy was a child with a fatal brainstem cavernous hemangioma, experiencing a life and death catastrophe
that made his parents still feel palpitations.

Blind paralysis caused by brainstem hemorrhage, no surgery for 13 years, no recurrence 1 year after total resection

The patient was transferred to the general ward the day after surgery, had no new neurological impairment, and could walk on the ground, and was discharged two weeks after surgery, with greatly reduced preoperative symptoms and good recovery
.
After more than 13 years of hard struggle with this fatal cerebrovascular disease, patients will usher in a normal life
that is no different from ordinary people.

6 brainstem hemorrhages in 10 years, no recurrence for more than 3 years after total resection

"My preoperative symptoms were so severe and Professor Ba gave me such a successful
surgery.
As a doctor, I had a short contact with Professor Ba and learned that the professor's superb medical ethics and spirit of technical excellence made me admire him very much
.
The morning after the surgery, the cannula was removed and I opened my eyes to see Professor
Ba.
I did it! My first words were: Hello, Professor Ba! Then I couldn't help but give a thumbs up to praise Professor Ba.
.
.
" Two and a half years ago, Director Huang sighed
sincerely after undergoing Professor Ba's surgery to completely resect the cavernous hemangioma of the brainstem.

INC Professor Bartlangfeld, Germany: 309 cases of brainstem cavernous hemangioma, surgery cases and results analysis

Professor INC Bartlang Fiy shared his more than 30 years of brainstem surgery experience and more than 300 successful cases of brainstem cavernous hemangiomas in his speech at the 2022 International Extratheological Master Course, covering the difficult and successful clinical case data of teaching brainstem cavernous hemangiomas in the world and the papers and monographs published in world-renowned neurosurgical journals, mainly including the location and imaging characteristics of 309 cases of midbrain, pons, and bulbar cavernous hemangiomas, and deciding the most suitable surgical timing and approach selection for patients Excellent surgical results
were obtained.
Among the more than 300 patients with cavernous hemangioma of the brainstem operated by Professor Ba, more than 90% of the patients achieved curative effect (safe total resection without any nerve damage) after surgery, and the probability of postoperative sequelae and rebleeding was extremely low
.

2022 International Extradivine Master Course – Lecture by Professor Bartlang Fi

Location distribution of 309 surgical cases (85 cases in midbrain, 179 cases in pontine brain, 45 cases in medulla oblongata)

Reference: Helmut Bertalanffy.
Genome-Wide Sequencing Reveals MicroRNAs Downregulated in Cerebral Cavernous Malformations.
J Mol Neurosci.
2017 Feb; 61(2):178-188.

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