Diagnosed 2000. Mid Gut NET.
About 20 years before diagnosis, I had decided to try to understand why I was continually flushing. I had started flushing deeply around 1980, in my early thirties. Around that time, when faced with a surprise or shock, I would flush a very deep red all over my face and neck, followed about 20 seconds later by a sensation of extreme facial tightening, and when I would look in the mirror I would notice that my face had turned pure white and looked shrivelled. I would also feel totally drained, woozy, and weak. Initially this event would last a couple of minutes, but by the late nineties it was lasting longer. For years I had wondered if there was something seriously wrong with me. I had no other noticeable symptoms except perhaps some intermittent diarrhea.
I was sent to a psychiatrist to see if he could help with my flushing. I was put on a course of Beta Blockers. I started with one pill, with some success, but after some years, even after taking four pills, I would have breakthrough flushing. By 1999 I was regularly flushing, unprovoked, from head to foot. A piece of cake would prove to be an instant trigger.
I saw a Chinese Medicine practitioner in 1999 who gave me a series of ‘laser’ treatments, which involved lying on a hard table with wires connected to me for an hour. I did not improve. I decided to be tested for allergies. After extensive testing no allergies were discovered.
My chiropractor was convinced I had Reynaud’s Disease, which is characterized by a red nose and red cheeks.
I arrived at my GP’s office in August 2000 for my annual physical, preoccupied with the flushing, and mentioning it to him, as I always did. He decided to send me for an abdominal ultrasound. Soon after, I was called in to his office. Spots had been found on my liver, and the radiologist had suspected something very rare called Carcinoid Syndrome, which was kind of, and I quote, ‘cancer like’ but I was not to worry as it was reputed to be very slow growing, and I could have it for the next 30 years. I was told I would most likely die of something else.
In the months that followed I went through a series of tests and scans; an octreotide scan, a nuclear scan, a 5HIAA 24 hour urine test (with a score of 136), a bone scan, a heart scan, a brain scan, and a liver biopsy. I was assigned to a medical oncologist, who put me on Sandostatin Long Acting Release 20mg, with no prior subq, which I started taking around February 2001.
My flushing stopped completely at that point.
I learned I was my oncologist’s only Carcinoid patient, and I realized that he was not actively engaged with other Carcinoid doctors. In late 2001, he did not see the need for a Chromogranin A (CgA) test, a state of the art diagnostic test.
I became determined to have a CgA test. I asked my oncologist if it was OK with him if I discussed my case with a second oncologist, and he agreed, so in mid-2002 I had a consultation with another doctor, and also had a successful CgA test, which fortunately had a low score, under 10.
I had CT scans every 4 months, which showed many small tumours on my liver, with three tumours bigger than the rest. I wondered if something should be done about those 3 by 4 centimetre and 2 by 3 centimetre tumours. My oncologist felt that it was fine to just keep taking the Sandostatin. I made an appointment to see a renowned Carcinoid doctor in New York in November, 2002.
The doctor wanted my tumour slides redone by his technician. He asked me questions about my primary tumour. It had not been found. He felt it was important to locate the primary tumour because it would determine the nature of my treatment. If it was in the small intestine I would have one treatment, and if it was in the tail of the pancreas I would have another treatment. The doctor wrote an extensive report, recommending a radio capsule endoscopy to help locate the primary tumour. One of his other recommendations was to possibly take alpha interferon as well as Sandostatin, depending on the location of the primary tumour.
I was not able to arrange a radio capsule endoscopy or go on alpha interferon.
In 2003 I went to a centre which focused on Carcinoid-NET. In October 2003, February 2004, and October 2004, I had 3 Hepatic Lipiodol 131 embolizations which were reversible in nature. The hepatic artery would initially be sealed during the embolization but after a while would open again. I was told I was inoperable.
The rationale of the embolizations was to reduce the size of the tumours, and to cut off the blood flow from the hepatic artery to the liver, ensuring that the hormones going into the liver would be lessened considerably, plus the tumours would be killed by the Lipiodol cocktail. It was better to catch the tumours when they were small, it was explained, before they got out of hand and did more damage. In March 2004 a thrombosis in a branch of the portal vein was observed by CT scan. I was put on 4 MG Coumadin working up to this after some subq shots. My third Lipiodol embolization of October 2004 went ahead successfully after an ultrasound and CT scan showed that much of the thrombosis had dissipated. I was given substance K to allow my blood to normalize.
From late 2004 till 2010 I was stable with only marginal tumour growth. I continued on 20 mg Long Acting Release Sandostatin every 28 days, and 4 mg day of Coumadin between 2004 and 2009. In 2009 I was put on Sandostatin LAR 20 every 21 days as opposed to every 28 days. I remained extremely interested in where my primary tumour might be but no scan detected it. It was suggested that finding the primary was not important but that treating the symptoms was.
I continued to have twice yearly CT scans. I also had once yearly MIBG scans and Octreoscans. In the summer of 2010 my liver enzymes were suddenly elevated to an extreme degree. I had scores 10 times normal for Alkaline phosphate, ALT, AST and GGT At its highest my GGT score was 1000. There was no explanation.
My liver enzyme numbers came down quickly but after my next CT scan in the fall of 2010 it was noted that there was a pelvic growth that looked like a new cancer. The reason for this understanding by the interpreting radiologist was that the new growth did not “light up”. Also suspected was a hydronephrosis of the right kidney. Co-incident with this my labs were showing a very poor Glomular Filtration Rate of 28 or 31 and my creatinine was around 140. I was advised to return to my home centre to have the possible new cancer explored, evaluated, and treated.
Between October 2010 and March 2011 I saw, in my home centre, a urologist, gynecologist, endocrine oncologist, gynecological oncologist, pathologist, and general surgeon with a specialty in abdominal surgery.
I had a renal scan in late 2010 that confirmed the right kidney hydronephrosis and in early 2011 I had a stent implanted in the right kidney to the bladder to see if this could help with the hydronephrosis. Suspicion grew among my doctors that I had ovarian cancer.
From gynecological oncologist’s report of February 23 2011
“She was advised to have an MRI of her pelvis which was done on 7th February 2011. Large mass seen in right hemipelvis 8.4cm by 4.2 cm and this is encasing the right ureter.
At least six specialists involved at this point.
From my fax on March 28 2011 to 2 nurses and 8 specialists in three hospitals and a clinic.
1. I notice that I have a number of suspected new malignancies according to my reports. Knowing this, does this mean that I will have tissue extracted from more than one site?
2. I believe that it is expected that an octreotide protocol will be in place for the biopsy. Will an octreotide protocol be in place for me during the biopsy?
Concern: I believe that if I do have an eight centimetre tumour now wrapped around my right ureter as well as a host of other tumours, that very rapid growth must have taken place.
I feel that my case should be upgraded to extreme emergency and that a surgical team should come together sooner rather than later to decide my fate.
From report of surgeon who decided not to operate. April 13 2011
Large pelvic mass with peritoneal implants. Mass very close to pelvic sidewall. Diagnosis of ovarian cancer did not seem quite clear. So…….
Biopsy performed (March 24) and the final diagnosis positive for a …….neuroendocrine tumour. Positive for vimentin and neuron specific amylase with focal positivity for synaptophysin. Chromogranin, CD56, and keratin were all negative. Tumour poorly differentiated and has a proliferative index of approximately 20%. Mitotic index 19%. Clinically the patient is doing actually well. No symptoms of bowel obstruction and no other worrying symptoms.
Large lesion in the pelvis implicating sidewall, uterus, ovaries, ureter, and rectum with peritoneal implants and mesenteric implants. At this time, surgery would be extremely morbid involving a full pelvic extenteration leading to probable ileo-conduit and permanent colostomy as well as having to consider the pelvic sidewall involvement and all the vascular involvement on that side. Moreover she has these peritoneal implants. At this time a feasible debulking that would lead to more than 90% removal of a neuroendocrine tumour is unlikely, and therefore surgery is not recommended at this time, Chemo is to start on Monday. The gynecological oncologist agrees.
From CT report 18 April 2011
IV contrast could not be administered to this patient who has an obstructed right kidney and an elevated creatinine.
Gallbladder contracted with a small focus of high density,
No biliary dilation. Normal pancreas, spleen, and adrenals.
Left kidney malrotated with an extrarenal pelvis.
Complex solid centrally placed pelvic mass exerting mass effect on the adjacent uterine puncture.
The mass itself measures up to 9.6x 6cm centrally as compared with 8.7×5.0 on the prior MR evaluation.
There is significant mass effect on the adjacent rectosigmoid colon which is displaced and effaced. The mass is in direct contact with the rectosigmoid colon.
No mechanical bowel obstruction is present. No ascites.
I had resigned myself to no surgery but chemo locally when I had a call from the specialised centre to say that they had reviewed my scans and reports from my local hospital and felt that surgery could be performed after all. I was elated.
The following week in late April 2011 I did not start chemo but instead showed up for a pre-surgery evaluation and was declared fit for surgery.
Surgery took place on May 3 2011
Here are some excerpts from my specialized centre’s multidisciplinary team’s surgical reports.
Preoperative Diagnosis. Metastatic neuroendocrine tumour with primary unknown.
Postoperative diagnosis. Metastatic neuroendocrine tumour with primary likely terminal ileum. Right hydronephrosis.
Operation performed. Laparotomy, Total abdominal hysterectomy. Bilateral salpingo-oophorectomy. Left ureteric stent insertion. Right nephroureterectomy. Right hemicolectomy. Anterior resection. Double primary anastomosis. Surgeons: General, gynecological, urological. Three other physicians in attendance.
Anaesthetic: General endotracheal.
Blood loss: 1 litre.
Some notes: As a result of enlarging pelvic mass, chronic renal failure. Some GI obstructive type symptoms likely tumour involvement of the rectosigmoid junction. Multiple metastatic peritoneal deposits . On running the small bowel a number of lesions on mesenteric border of many segments of small bowel. Larger lesion right in the terminal ileum-likely primary tumour. This was in fact, near obstructing. Bladder was involved with the tumour but tumour was excised from bladder.
This was ‘a total mess’ and my abdomen was totally invaded. I was in hospital for 12 days.
Two weeks later I was readmitted for uncontrollable diarrhea and was in hospital for 6 days. I was on a regimen of Sandostatin LAR 30 every 4 weeks. Tylenol 3 every 4 hours as needed, loperamide, ciprofloxacin and 5 mg morphine every 4 hours as needed. Also potassium pills.
Chemo was started six weeks later with Carboplatin and Etoposide. Regular dose. Regimen was to be 4 or six cycles of three consecutive days every three weeks. I was given Ondancetron and Potassium chloride to help with nausea.
I developed Febrile Neutropenia plus suspected onset of sepsis 10 days after the end of the first cycle and was admitted to hospital on July 2. I spent a week in hospital. High fever. Suffered temporary memory impairment. MRI of brain showed no impairment.
Three more rounds of reduced chemo were uneventful. I was started on Neupogen injections and stayed on those throughout chemo.
I have had four CT scans since surgery, and all have shown no sign of further tumour growth in my abdomen. The most recent CT scan was May 2012.
I have been on Sandostatin 60 LAR since October 2011. My oncologist recommends nothing else for now.
Medications include Tylenol 3 and Lomotil as needed. Cholestyramine as needed. 2 pills of potassium per day. Because of the onset of severe Hypertension I am now on 8 mg a day of Coversyl. This has brought my blood pressure from 180/100 to normal levels and I am not taking potassium.
I see a general cancer dietician in regard to my now low fibre diet.
My recent CgA score was about 200. I had markers such as serum serotonin and pancreastatin checked at Interscience Institute, a lab dedicated to Neuroendocrine Tumour patients, in the US along with Neurokinin A and I am within reference range for all tests. My NKA was 13 which is far below the ‘danger’ level of 40, which indicates poor prognosis.
On the suggestion of my New York doctor a biopsy slide was sent to CARIS labs in Texas for analysis. The report showed that a good drug for me is Temozolomide and Xeloda. No other tests could be performed because the tumour sample that was sent was too small to allow for further testing. My post-operative tumours were pickled and deposited, with my permission, in an anonymous tumour bank in Ontario to be used at some point for general research.
I have ongoing problems with diarrhea, gut pain, and gas.
I am now receiving B12 shots and am trying to develop a regimen of drinking black raspberry juice.
I need to find a way to increase my chances of survival. Having heard so much about the excellent clarity of the Gallium 68 scan which is used in many other countries I wonder if it would be more helpful in terms of information gleaned, than a regular CT scan.
My next focus is on my gut issues which are unpredictable and interfere with my quality of life. I am, however, delighted to be alive and enjoying family, friends, and good weather.
Saturday June 30, 2012
Sadly, Maureen lost her battle with NET cancer on August 31st, 2013.