A Doctor's case of Epithelioid hemangioendothelioma: Hopes and questions about future recovery now after this surgery

This is a HIPAA de-identified open-online-patient-record with initial information in patient's voice, posted here after collecting informed patient consent (form downloadable Click Here) by BMJ Elective Student.

January 2010: I had a bike accident but at that time I had no serious injuries, I just had fallen down from bike. Only my right great toe was injured.

December 2010: During winter, I started playing badminton game and I started suffering from shoulder pain radiating from the scapular region to my arm. I thought the pain was due to playing games. So whenever I had pain I took a painkiller. The pain remained even after I stopped playing. I consulted with an orthopaedic surgeon in Kushtia, Bangladesh (my hometown). I did an x-ray of the cervical spine, the disc spaces between c4, 5, 6 were mildly reduced. So he thought that the pain was due to nerve root compression. He prescribed me muscle relaxants and cervical collar for 2 wks. But there was no improvement and the pain started to occur every 2-3 days apart… because I have to give MBBS entrance exam test so I concentrated on the study not giving so much significance to the pain for many months…after getting admitted into Rajshahi medical college I consulted with an orthopaedic surgeon of RMCH because gradually my shoulder mobility was decreasing due to the pain.

August 2012: The doctor suggested me an MRI of cervical spine … the MRI was normal.. the nerve roots were fine… then I was suspected to have frozen shoulder and advised to do an x-ray of the shoulder joint. The x-ray was normal too… so the Doctor thought it was musculoskeletal pain… so he also prescribed me a painkiller and muscle relaxant … again there was no improvement…days passed away… but my shoulder pain remained all the time…. I concentrated on my 1st professional exam of medical college because no one could give me a solution for the pain. I used to take Etoricoxib 90 mg whenever I had severe pain. I passed 1^st Prof Exam and promoted to 3^rd year.

January 2014: I had a bike accident again and this time I had a head injury,, my left temporal bone was fractured and there were a 4mm extradural hematoma and CSF otorrhea for 7 days…I was conservatively treated and got well after about 3-4 months.. Then I continued my study.

November 2014; As it was winter I used to play badminton despite my pain at the shoulder. Suddenly one day at morning when I wake up I realized that I could not extend my fingers of right hand… I consulted with associate professor of the neuromedicine department. RMCH,, he examined my right hand and told me that it might be posterior interosseous nerve compression because there was no sensory involvement and wrist drop…so he prescribed me an oral steroid for 10 days and told me to take physiotherapy… but no improvement occurred… then he told me to do an NCS of the right upper limb from National Institute of neuroscience, Bangladesh. I did… the report revealed “complete radial nerve conduction block at the level of spiral groove”…but clinically my wrist was ok and no sensory involvement. As there was no improvement I was advised to go to India.

December 2014; I did not go to India at first….rather I consulted with a neurosurgeon at GH, Dhanmondi, Bangladesh. He was ex-head of the department of neurosurgery dept. of Dhaka Medical College Hospital. He clinically examined me again told me to do an MRI of the brain and cervical spine… MRI reports were normal … he saw the films…I showed him the NCS report. then he told me that NCS study done at NINS might be wrong because clinically there was no sensory involvement and wrist was also ok…so he also told that it was posterior interosseous nerve entrapment and he would like to do a decompression surgery… and he did the PIN decompression surgery after 7 days… then I again took physiotherapy… but there was no improvement after 3-4 months rather gradually my wrist was dropped after 4 months and sensory supply was also involved gradually…

April 2015; I went to DR. SD, a neurophysiologist at IS medical centre, Dhaka. She did an NCS/EMG of my both upper limbs. The report was (chronic brachial plexopathy and there is an injury at the distal end of the posterior cord of brachial plexus from where the radial nerve arises). Then I decided to go to India. I gave my 2nd professional exam with wrist drop with so many difficulties…I wrote my exam papers with a fully flexed hand… Then I went to India after my 2nd Prof Exam.

September 2015; At first I went to Chennai Apollo hospital,, I consulted there with a renowned neurosurgeon… he clinically examined me,, told me to do and contrast MRI of brachial plexus and repeat the NCS/EMG test. After getting the MRI films and NCS/EMG reports he told me that my brachial plexus is fine. But there is multifocal involvement of motor and sensory nerves of right upper limb and it might be some sort of MND.. so there is no cure…when I asked the neurosurgeon that what was the cause of my shoulder pain he also told me it was musculoskeletal pain…He said that physiotherapy was the only option being hopeless I went to Mumbai. In BH, Mumbai I consulted with one of most senior hand surgeons in Mumbai. he again repeated the NCS/EMG tests… that time the report was (radial nerve palsy, axonopathy at the level of the radial groove) in Mumbai and Chennai, they all denied the report done by DR. SD at Dhaka, Bangladesh that there was an injury at the distal end of the posterior cord of brachial plexus… the hand surgeon after seeing the reports done in Mumbai prescribed me some medicines and suggested to continue physiotherapy for 3 months, but after 3 months there was no improvement.

February 2016; I went to Mumbai again.. the hand surgeon decided to do an open exploration of the radial nerve in the radial grove and after exploration as he found no pathology he did tendon transfer for radial nerve palsy…he transferred pronator teres to wrist extensors, flexor carpi radialis to finger extensors and palmaris longus to thumb extensor. After tendon transfer, my hand function was improving… but the shoulder pain never disappeared…

June 2016; after 4 months of tendon transfer I went to Mumbai for follow up, the hand surgeon said,, ‘’ my hand was fine.. and the function would improve more and more gradually..’’

August 2016; after I returned to my country, I felt that there was a burning and tingling sensation along the ulnar nerve distribution… I became scared… why the ulnar nerve this time!!! The burning and tingling sensation was increasing day by day and sensation was gradually decreasing along the ulnar nerve supply…

September 2016: I consulted with Hand surgeon DR R.R. K sir at GL Hospital, Dhaka because it was very tough to go Mumbai again and again.… he clinically examined me and said that I have problems in my right brachial plexus. So he taught me some exercises and gave me some medicines and told me that the ulnar nerve symptoms would be ok with time. I asked him what is the cause of my shoulder pain, he also replied, it is musculoskeletal pain. I asked him to advise me an MRI of shoulder joint to exclude the causes of shoulder pain but he denied. I followed his advice but the ulnar nerve symptoms were the same … Because my MBBS final professional exam was nearby so I decided to go to Mumbai to my hand surgeon again after giving my final Prof Exam.

January 2017: When I was attending my final prof exam… one day a thought came into my mind that why my shoulder pain never decreases… every doctor I consulted with told me that there was no relation between my shoulder pain and my nerve symptoms…so I thought that “do I have any injury within my shoulder joint like rotator cuff injury or any tear in capsule, because I used to play badminton before....’’ so just for my curiosity I wanted to do an MRI of right shoulder joint.. so I told one of my sir to advise me a contrast MRI of right shoulder joint at PDC in Rajshahi, Bangladesh… at the time of MRI, someone called me and asked what was the problem.. I told him that I had severe right shoulder pain for many years, and previously I had radial nerve exploration and tendon transfer surgery for radial nerve palsy… then he told me to do an x-ray of the right shoulder joint and give him the film… I did that the MRI report came as normal, and when I saw that it was signed by our Head of the department of radiology I believed that it was correct… as I said I wanted to do the MRI of my right shoulder joint just for my curiosity it meant that there was actually no referring clinician of that MRI…and because I thought as it was checked by a Consultant Radiologist so it was correct…so I didn’t even take the films in India later.

April 2017: After finishing my final Exams I went to Mumbai again… that time the hand surgeon after seeing my condition referred me to a renowned neurologist DR S.V. K of BH, Mumbai, the neurologist repeated the NCS/EMG study, and contrast MRI of brachial plexus. The ncs/emg report showed that I had multifocal involvement of motor and sensory nerves mostly affecting the c8 and t1 fibres. The MRI of brachial plexus revealed that posterior and medial cords and divisions are being hypertrophied but the roots and trunks are normal… so the neurologist told me that it was a rare disease suspected to be immune-mediated hypertrophic brachial plexopathy… he used to have such patient 1-2 in a year…He did not give me further investigation and I was prescribed high dose oral prednisolone for 3 months… but there was no improvement.

August 2017: I went to Mumbai to follow up… as there was no improvement the neurologist told me to stop oral steroid by tapering the dose and said that as the condition didn’t respond to steroid it would not respond to any other drugs…so he just told to take physiotherapies. So being hopeless I returned to Bangladesh… After 20-25 days my right triceps muscle was totally paralysed, I rushed to Mumbai again in the last week of September 2017.

September 2017: the neurologist again repeated the NCS/EMG test. That time the reports said that my disease is progressing affecting C7-C8-T1 fibres. So he discussed with some other doctors about my condition and told me to take IVIG which was the last hope according to them.

October 2017: After returning to Bangladesh, I went to NINS to consult with DR QDM, the most renowned neurologist of Bangladesh.… he had formed a medical board to discuss my condition… at the time of discussion, they clinically examined me and saw all the reports and agreed that the condition was immune-mediated… they explained as I had the history of Road Traffic Accident,, maybe at that time the affected cords had been stretched and injured and gradually being hypertrophied due to chronic immune reaction… it might respond to IVIG they added.. So I took the loading dose of IVIG for 5 days under the observation of DR AU (ex-chairman of neuromedicine dept) as he had experience of dealing with the risks during taking IVIG… DR AU also prescribed me to take low dose methylprednisolone and azathioprine daily..… 8-10 days after taking loading dose of IVIG I started to feel an improvement in my right triceps function… after 1 month there was 50% improvement in right triceps but below the elbow, no improvement was seen.

December 2017: I took the 1st maintenance dose of IVIG… my right triceps was improved up to 70% but still, there was no improvement below the elbow.

February 2018: I took the 2nd maintenance dose of IVIG, then I went to Mumbai for follow up… the neurologist was happy that my condition has responded to IVIG. But he told me that as IVIG is hugely expensive so he wanted to try with methylprednisolone pulse therapy and prescribed me that…. After returning to Bangladesh I felt that my right triceps which was improved up to 70% started to deteriorate again and the condition below the elbow was worsened rapidly..… and again my right triceps was paralysed… so at that time, the question was whether my condition was really immune-mediated or the diagnosis was wrong!!… so I decided to go to Queens Square London to consult with DR HM (consultant neurologist and writer of Oxford handbook of neurology)… I got an appointment. I went to London on 3rd April 2018.

April 2018: Dr HM saw me…took a detailed history, did all the neurological examinations and saw all the previous reports…He told me that it was surely brachial plexopathy and the reason was inflammatory but he had to find out the cause of the inflammation. He gave me some blood tests to see the ganglioside antibodies and other antibody profiles. He also repeated the NCS/EMG study of upper limbs… contrast MRI of brachial plexus and cervical spine… I did all the tests…the NCS/EMG study revealed that all 3 nerves (radial, ulnar, median) of the right upper limb are affected… MRI of brachial plexus showed hypertrophy same as the MRI done in Mumbai. But then he informed me that he wanted to do an MRI of the right upper arm… I did…. And this MRI of right upper arm revealed the cause of my sufferings during the last 3.5 years and the cause of shoulder pain for the last 7.5 years… I had a round shaped lesion (1-inch diameter, speculated appearance) just medial to proximal shaft of the humerus and behind the short head of biceps, very near to brachial artery and at the distal end of brachial plexus involving all three terminal branches and also narrowing the axillary artery… there is calcification point inside the lesion… then I was told to do a PET-CT scan from vertex to knee to see if there any lesion at any other site of the body… the report revealed no other lesion at any other site except that.. the differential diagnoses were desmoid fibromatoses, neoplastic, leprosy… and the diagnoses had to confirmed by open biopsy… Dr HM then told me that I could undergo the open biopsy procedure in India as it was very costly in London.

May 2018: I returned to Bangladesh, completed my internship…one day it came into my mind that I should see the films of MRI of my right shoulder joint which I did in January 2017 just for my curiosity… so I came home and checked that films… I was shocked with grief after seeing the MRI films because the lesion was clearly visible in that MRI of right shoulder joint… maybe it was my bad luck… that no specialized clinicians or surgeon never told me to do an MRI of right upper arm/shoulder joint… I did it on my own curiosity 1.5 years back… and I had no idea that a consultant radiologist could overlook such large lesion in a specified region with a given history of symptoms… I trusted his reporting so I never showed the films to anyone… I just regret if that MRI report was given correctly, I didn’t have to take IVIG by spending 30 lakh taka (about $35000 USD) … my right-hand condition had been worsened severely in the last 1 year… if the MRI was reported correctly,,I would have taken the right treatment and the nerves would have recovered. Whatever then I contacted with my hand surgeon in Mumbai because he is also a brachial plexus surgeon. (Past president of brachial plexus surgery society of India). He decided to do the open biopsy.

June & July 2018: I went to Mumbai in the last of June… a biopsy was done on 2^nd July under general anaesthesia. After my sense came back my surgeon told me about the surgery that anyone never expected… He said that he had never seen such lesion in his career. The lesion had a crab-like appearance,, entrapping all the nerves and blood vessels deep in my right axilla. The radial nerve and axillary artery were matted with each other by the lesion. All the nerves became hourglass shaped. So at that time he only took some portion of the lesion and biopsy from affected portion of ulnar and median nerve and repaired those nerves by short graft. He could not remove the whole lesion because axillary artery needed to be grafted by a vascular surgeon… 3 Specimens were sent to Pathology department, B hospital. The biopsy report revealed that the lesion was Epitheloid Hemangioendothelioma. So then I was referred to an Onco surgeon. The onco-surgeon told me to have a 2^nd opinion from the biopsy blocks by DR AB at S Diagnostics, Mumbai. 2^nd opinion was Intraneural Neurofibroma. But my surgeon and onco surgeon did not agreed. So they told me to have 3^rd opinion From TM Hospital in Mumbai. The 3^rd opinion also said that it was Epitheloid Hemangioendothelioma. Then I had another PET CT scan of the whole body to make sure that there was no metastasis. As I had no metastasis so then the decision was made to have a radical surgery. A team of surgeons including Brachial plexus surgeon, Onco surgeon and the vascular surgeon would do this surgery. As I had plasters over my arm and forearm for 6 weeks the next surgery was scheduled for 19 September.

September 2018: I went to Mumbai with a hope to be cured … I was tired of lying on OT tables again and again. I was praying just for a successful surgery this time. But my luck is never with me. The surgeons tried their best for me. They removed a residual tumour and radically resected all the structures that were at the level of tunor plane and sent all the specimens for frozen section biopsy.. According to frozen section biopsy report, the tumour extended intraneurally and perineurally along the nerve sheaths…they put about 8-inch artificial vascular graft to axillary artery and removed the axillary vein completely along with some lymph nodes. They removed the radial nerve … 15cm graft needed for ulnar nerve and 20 cm graft needed for radial nerve. The musculocutaneous nerve was also cut because it was also suspected to be a tumour infiltrated. Then they took both sural nerves from my both legs… but unfortunately, there was graft shortage. My surgeon said that he had put 3 cables (15cm each) to the ulnar nerve so the ulnar nerve repair is good. but he was able to put only 2 cables (20cm each) to median nerve so he is not sure whether the median nerve will come back.. he also did direct neurotisation to biceps muscle from median nerve by using 4 short cables…he could not repair the radial nerve as there was no more graft available and because I had tendon transfer previously so he decided to put graft toulnarr and median nerve at first.. So after the surgery, my whole right upper limb is now nonfunctioning and surgeons are not sure whether hand function will return or not… now I have returned to my country.

Next step regarding my treatment: Now I am realizing that as the radial nerve has been removed my right triceps function will never return so as my elbow extension. The ulnar nerve repair is good so the chances of ulnar nerve regeneration is more… but I am very much tensed about the median nerve… because after my tendon transfer surgery my wrist and finger extension function were conducted by the median nerve but it could not be repaired well… I want to know, can it be possible to re-repair the median nerve by taking any other nerve from my body (such as intercostal nerves or lateral femoral cutaneous nerve) or by taking allografts..…or is there any advance technology to repair my nerves like Schwan cell grafting … I also want to know what can be done to restore the elbow extension function?.. I need the most advance treatments of medical science to overcome my sufferings… despite all my struggles and sufferings for many years now I became a Doctor. I have not learnt to quit. I will fight to get some hope.

Investigations: (not inclusive of all the past records; past management in different hospital facility along with the whole journey is structured into tabulated format at the end of this post)

UK reports: (April 2018)