I certainly didn’t think my diagnosis would be low grade mucinous appendiceal neoplasm.
“The radiologist thinks you have a ruptured appendix and we know that can’t be right”
Dr. Robertson told me looking concerned after the results came back from the CT scan. Dr. Robertson is no relation to me or my husband even though we share the same last name. I was having trouble with a dull ache in my right side that had recently moved into my right pelvic area in my back. I was not in severe pain like you would expect for appendicitis. “Are you sure you feel ok?” he asked again. He told me that he had arranged for me to see two surgeons, a colorectal surgeon for the appendix and an ob/gyn surgeon for the endometriosis that had been previously diagnosed 36 years ago on my right ovary. He felt that the appendix definitely needed to be removed and he was unsure if the ovary was a problem, too.
I did eventually get the “appendicitis” pain two weeks later which was before I went to the colorectal surgeon. Of course, no one believed the radiologist’s report which included the ruptured appendix that I carried with me to the emergency room. I didn’t have the CD-ROM with the CT scan on it for the ER doctors to look at and form their own opinion. A second CT scan at the ER showed an enlarged appendix, instead of a ruptured appendix, which didn’t qualify for an emergency appendectomy.
I was in extreme pain that day for 12 hours in the ER with no fever, no nausea except what was caused by the pain medicine, and no elevated white blood cell count. Even though I didn’t have other symptoms, the severe pain made up for it. It felt like a knife had been plunged into the right lower quadrant of my abdomen and went straight through my pelvis. I could barely walk I was in so much pain. It was unrelenting. The young surgeons who saw me that day told me I didn’t have appendicitis and read off the symptoms as if recited from the textbook. I knew that I didn’t have textbook appendicitis but does anyone really have “textbook appendicitis”?
The ER doctor came in to release me since no one would do surgery. She told me that she was going to send me home with straight oxycodone (no acetaminophen) and if I needed to come back, she would have standing orders of morphine every two hours until someone would operate on me. She had put down my diagnosis as “chronic appendicitis”. So I went home.
Still in extreme pain at home, I called the on-call doctor for the colorectal surgeon I was scheduled to see. The on-call doctor was the surgeon I was supposed to see. I told him about my pain and he told me that my pain should have occurred two weeks ago when the CT scan showed a ruptured appendix. He said I should go back to the ER if the pain continued which is what I did.
I went back to the ER again at 4:00am the next morning and to the standing order of morphine every two hours. Finally, at 7:30am I was told my surgery would be set up for that afternoon. Just knowing that something was going to be done made me feel better. I was going to have an appendectomy on my 56th birthday, March 19, 2013. Considering the circumstances, this was the perfect birthday present.
“Your diagnosis is low grade mucinous appendiceal neoplasm”, said the young resident who was working with the colorectal surgeon who had done my surgery when I returned to the office for my post-surgical exam. He was describing what they had found during my surgery while I was having an out of body experience — not really feeling like it was me he was talking to or about. He said they had found this sticky, green mucous in my abdomen that was thick and hard to remove.
Oh, no, I was thinking, I’ve been “slimed” like in the “Ghostbusters” movie. Do I have cancer, I thought? This couldn’t be me. Finally, he said it was cancer. Then he said it wasn’t. After that, he admitted that he had never seen this before and he didn’t know if it was cancer or not. He said he would ask my surgeon to explain the findings.
The surgeon came back in with the resident and confirmed that it was indeed cancer since the appendix had ruptured and there was spread to the abdomen. He assured me that there were no other “tumours” in my abdomen and he had taken steps to thoroughly clean all of the mucin out. He explained the next step could be a major surgery to remove part or all of my colon or other organs; or if I wanted to, I could choose “watch and wait” which sounded better than the former to me. In any case, he wanted to refer me to an oncologist because he was certain that it would come back and that monitoring was necessary. He also told me that he would present my case to the tumour board because it was very rare and ask for their recommendations on my next step.
Me trying to do a selfie in the hospital bed at Wake Forest Baptist Hospital with my favorite nursing assistant and the medical student who took such great care of me.
I cried as I was driving home from my doctor’s appointment. Then I picked myself up when I got home and googled “low grade mucinous appendiceal neoplasm” because I needed to know everything about this new enemy. That’s when I found the term pseudomyxoma peritonei. I had actually heard of this term before while in school in the early 80s and I had learned that it was usually fatal. I was devastated.
I found details online of other survivors who had had the major surgery mentioned by my surgeon, called cytoreductive surgery, in which all visible signs of disease are removed and hyperthermic intraperitoneal chemotherapy (HIPEC). The chemotherapy is pumped into the abdomen after the surgery and sloshed around (shake and bake) to rid the abdomen of microscopic disease that cannot be seen or removed by the surgeon.
This surgery is also called the Sugarbaker Procedure named after Dr. Paul Sugarbaker who developed this aggressive approach. At first it scared me. The thought of a 10-hour surgery in which half of the colon, gallbladder, spleen, uterus, fallopian tubes, ovaries, and omentum are removed is hard to comprehend.
I decided to have this surgery and sought out the advice of an appendix cancer specialist, Dr. Edward Levine of Wake Forest Baptist Hospital, who had more than 20 years of experience and had done more than a thousand cytoreductive surgeries. I knew that going into this surgery I had very little visible disease and my surgery should not be as bad as some of the ones I had read about. My surgery lasted five hours which is not long considering the average is 10 hours. My omentum, left ovary, and a short section of bowel surrounding the appendiceal stump were removed. I recovered with very few problems. I did have an incisional hernia repaired in June, 2014. Now after my 18 months post-op CT scan, I have no evidence of disease.
Appendix cancer has changed my life and made me aware that there are things that I want to do and see before I die. My bucket list has consisted of mainly travel items thus far including trips to Israel and Italy. It has given me a new sense of purpose. I also want to be involved in helping those who have been recently diagnosed.
– Emily
Emily
Emily has been working as an Environmental Health Program Specialist (Health Inspector) for the Pitt County Health Department in Greenville, NC for over 25 years. Her work is in the Children’s Environmental Health Branch which involves child care and school inspections; lead investigations; indoor air quality consultation; and meth lab remediation. She graduated from East Carolina University, which is also in Greenville, NC, with bachelor degrees in biology and computer science. She also attended medical school for over two years. She has been married to the love of her life for almost 34 years and together they have two grown children. Her hobbies include singing in the choir, playing the piano, and participating in church activities. She is a pseudomyxoma peritonei survivor
In case you missed it...
Jennifer tells us about Jim’s diagnosis
Jim's surgeon's secretary (for his appendectomy) called him and told him they needed to make another appointment because they "had found something". Thank G-d I decided to go to that appointment! The surgeon came in and told Jim, "You have appendix cancer and I don't...
I’ve gone from 0km to 10km in just over 6 months!
In 2015, I had cytoreductive surgery and HIPEC for pseudomxyoma peritonei. To thank Pseudomyxoma Survivor for all the support I was given and all the friends I have made through the charity, I’m doing the Southend 10K to raise funds for the charity. I would have been so alone and desperate without the charity and I cannot thank you enough x
A grant from Pseudomyxoma Survivor helped me
I am so grateful for the grant from Pseudomyxoma Survivor which I received. It has helped me financially during such a difficult time.
Thank you for sharing. I’m new to this and learning all that I can.
HI Tiffany, glad you’ve found Emily’s story useful. Please do not hesitate to get in touch if you would like a buddy or to join our Facebook group.
I was just diagnosed with mixed goblet cell adenocarcinoma of the appendix on 6/19/2015. My appendix had not ruptured. I have no one who knows about this cancer and only read articles on line. My right hemicolectomy and lymph node harvesting is 8/5/2015. I am scared and this totally caught me by surprise. I went to the ER with right sided pain and ended up with a high white count and an emergency appendectomy. I was told 5 days later it was cancer. I have no idea if this has spread or if I need to ask to have my ovaries out as this is where I read it likes to spread. Any information would be appreciated.
Vicky, please let us know if you would like to join the Facebook group or to have a buddy.
My sister has this cancer. She is 42 and has 6 children. They removed her appendix. Two weeks later removed part of the bowel. The oncologist doesnt know anything really about this cancer, by his own admission. No Chemo was given. We are very scared that enough has not been done. We are in Brisbane, Qld . Australia. We are having troublefinding a specialist who knows this cancer.
Jaquie, I’m sorry to hear about your sister’s diagnosis. Has she been in touch with the team at The Princess Alexandra Hospital (PAH)?
Jaquie,
My Name is Stacey Jaundrell, and you can find my story here: https://www.pseudomyxomasurvivor.org/staceys-story/
I am 33yo mother of 5 children, I was treated in Brisbane Australia, by the Princess Alexandra Hospital under the care of Dr Nick Lutton & Brian Meade, my surgery and ordeal was very complex but in 2013 I had my surgery and 10 hours long it was called a peritonectomy which is a very lengthy and risky procedure. along side the surgery I had HIPEC.
the Princess Alexandra is who you need to see for further assessment and maybe even treatment,
ill give you the details for the surgeon you need to below here:
Dr Nicholas Lutton
(Public Clinic)
The Princess Alexandra Hospital (PAH)
199 Ipswich Rd,
Wooloongabba QLD 4102
Phone: (07) 3176 2111
Dr Brian Meade
(Public Clinic)
Brian sees Public patients through the Public Hospital in Brisbane The Princess Alexandra Hospital (PAH)
199 Ipswich Rd,
Wooloongabba QLD 4102
T: (07) 3176 2111
you now need your sister to obtain a referral and follow-up care with regard to pseudomyxoma peritonei (PMP) At the princess Alexandra Hospital in Brisbane Australia. Get it referred to via Dr Nick Lutton and make sure your sister states the condition and include all blood work and scans etc. this will make it easier and also keep copies..
Please if your sister wants to talk, I am happy for her to get in touch with me.
let me know
Stacey Jaundrell.
Hi Stacey and Angela,
I have only just found your replies. Thank you both very much I will pass this on to my sister. I will take her to the PA myself if need be. I don’t know why her doctors at Redcliffe hospital have not sent her there already.
Kind Regards
Jaquie
Hi Jaquie,
How is your sister? Did you get see any of the specialists mentioned above? My husband has just been diagnosed. We are on Townsville.
Hi Jacquie. I have just been diagnosed with mucocele of the appendix. I’m in Brisbane. My GP has no idea about this disease. How is your sister doing? May I ask which surgeon you used and any information you have will be grateful. Not much info out there!! Thanks in advance. Fiona
Thank you for sharing your story. It sounds very similar to mine so far. I too have the low grade version. My pain turned out to be a torting ovarian mass and during the hysterectomy they found pmp. I’m having the big surgery at the end of the month with Dr Paul Moroz, a PMP specialist.
My daughter in Law has just been giving the DX of PMP. She had surgery last week where they removed her appendix, right colon, fallopian tubes and did a complete hysterectomy. We had this done at Duke University in NC. She is 37 years old. They have made us an appointment to see a cancer specialist in this field. Where do you recommend would be the best place to go. She has not had chemotherapy yet.
Thanks,
Tammy, which specialist has you daughter-in-law got an appointment with? Have you had a look at our specialists’ list?
https://www.pseudomyxomasurvivor.org/pmp-specialists/surgeons-and-specialists-usa/
Thank you for sharing your storie my mom has this rare cancer and told there is nothing that can do for her do to it has spread threw out different areas just some chemo this gives me faith to look in to different opinions it feels like a bad dream nobody seems to know to much about so my question again thank you please keep my mom in your prayers
HI, my name is Jasmine and after reading this I am wondering if anyone can offer advice? I recently had my appendix removed as it was clear on ultrasound that I had a mucocele of some sort, my diagnosis was LAMN with no evidence of perforations or pmp (I had appendix removed only).. my surgeon referred me to Christies in the UK but Christies can not help without prior funding and suggested they review the pathology again and then possible surgery and chemo, my surgeon says he is certain it is LAMN and says he will now monitor me instead with ct scans etc… my worry is: how can he be certain and im in an enormous amount of pain in my left lower back and despite calling repeatedly his secretary is just rude to me and says “go to a&e” where do I turn?
Where are you based, Jasmine?
I am in Wales. Uk.
Jasmine, have a look at this page for the funding situation in Wales.
https://www.pseudomyxomasurvivor.org/pmp-specialists/specialists-uk/
Hello, I need help finding a doctor who wants to take a chance on me, I’ve had pmp since 1987 I believe. I was misdiagnosed twice. I’ve had 7 surgeries and the last three not very helpful on removing tumors. Two years ago on last visit to a new doctor I was told to go live my life, I’ve certainly earned it. Well I’ve always lived my life and a pretty happy one. Now I’m worried…my tumors have calcified. If ya’ll know of a doctor that is interested in what they call the longest living person with the cancer (pmp) I would appreciate your help. I’m in Texas. Thanks! I’m so hungry! 🙁
Thank so much for this story as its very similar to mine! And strangely enough we share the same birthday march 19th!! I presented at hospital with pain that kept moving starting in the upper admoninal to then mid right section and the dull ache at my back they sent me home with endone then I had an ultrasound in which they found a 3 cm mass near my appendix.
I went back into hospital and the drs could not decide wether to operate or not as I looked too well , they gave the option to me and I requested the surgery. Thank goodness i did, they removed the mass and a weird shaped structure and tested my fluid. I was in hospital for 4 days after surgery with no answers, thinking i had an appendicitis, when the results came back i was diagnosed with low grade appendices mucinous neoplasm. And the weird shaped structure was actually my fallopian tube. I was hoping to find a specialist in NSW Australia? I really would like to take my notes to someone who knows about this as the Dr’s at my public hospital have never seen it as told me like it was nothing to worry about ….Any help or referrals would be greatly appreciated xxx
Lauren
Thanks for your comment and I hope your recovery is uneventful. The specialist that you need to see in NSW is the very lovely Dr Morris. You can find details for him here:
Specialists in Australia
Angela
Because I had an extended abdomen (no pain),I’ve had surgery – removal of appendix, and all female organs but was told that I had a PRE-cancerous condition and that chemo would be useless. Have you ever heard of that?
Pseudomyxoma peritonei is a widely mis-used term but it is not considered to be a pre-cancerous condition. You need to see an experienced appendix cancer specialist for a consult. Lots of doctors misunderstand this diagnosis because the pathology report describes a benign polyp. The appendix ruptures and mucus and cancer cells are displaced from the GI tract to the abdomen. This is why it is a cancer. I know it doesn’t make sense.
I’m 61, female, and was diagnosed with advanced stage pmp with metastases to my lung, lymph nodes and bilateral pleural effusions. Rare for this to metastasize, I’m told.
Anyone out there with advanced disease like this?
Hi Patsy,
are you seeing a specialist for your PMP? I’m asking because it doesn’t seem to be that unusual to have metastases….on the facebook group there are several people with mets in assorted places, including lungs, lymph nodes, liver. My husband has multiple mets in the lungs (over 100 small mets), as well as in the liver and the lymph nodes behind the liver. He has had a pleural effusion, but it proved to be just “one of those things” when they drained it, no evidence of malignant cells in it …(ha, once in a while something easy to deal with pops up!!)
So short answer…yes, there are others out there with advanced disease.
Hi Patsy, I was very fortunate because mine was caught early and the only tumor I had was the appendix. It had ruptured and there was mucin in the abdominal cavity which means that there may have have been cancer cells released as well. My diagnosis was low grade PMP which means that the cells were mature cells which do not divide rapidly and usually do not invade other tissues such as lymph nodes. It appears that your diagnosis may be high grade PMP because your cells have metastasized to other tissues and may be cells that divide faster. In some respects, this can be good because high grade PMP is more responsive to systemic chemotherapy. There are lots of people in our support group with this form of the disease. Please join us. You are not alone in your fight against this disease.
I’m worried that I have appendicitis. My pain is in my lower abdomen, right above the groin area. I still am able to walk and eat and stuff, but I’m still worried. As per Everydayhealth, medicinenet, Abdominal pain is common in a lot of illnesses, such as irritable bowel syndrome, gastroenteritis, urinary tract infections (UTI), and pelvic inflammatory disease, an infection of a woman’s reproductive organs. My symptoms are similar, I am not pregnant for sure, My wedding is early next year…I am totally confused. I need Help?
Heather, please see your doctor. We are a patient and caregiver-led organisation and cannot give medical advice. You need to take up your concerns with a medical professional.
Hi gals!
My story starts Aug 2014 with right side pain, a 3 am visit to E.R. , 4 am CT & appendectomy by 6 am.
Seven days later I’m told I have “musconous appendiceal adenocarcinoma” . My appendix had not ruptured. Visited oncologist 90 miles away a AJ later. Had right hemicolectomy and 16 lymph nodes rmvd. No cancer.
My E.R. Doc, oncologist and surgeon had never had a patient with this rare cancer. Everyone agreed no chemo. Have had 3 colonoscopies-3 cat scans and blood work every 3 mos. This August 2019 it will be 5 yrs.
I really feel alone with all of this. I know this cancer is rare. Is there anyone out there with my story? How are you doing today. What has your follow up care been like? Do you get scare? Depressed?
I was 56 when my life changed.
I am now 61. A cancer survivor?
Good luck to all of you!!! Any reply would be wonderful??.
Cheryl from N.W. Montana
Cheryl, you might find it useful to join our Facebook group.