Anyone have Pancreatic Acinar Cell Carcinoma?

I was diagnosed with Pancreatic Acinar Cell Carcinoma in June 2012 with the tumor 4cmx3cmx3cm on the head of the pancreas and in contact with the portal vein. The frequency of PACC is between 1-2% of all pancreatic cancers. A Whipple with portal vein resection was performed and I was restaged as IV with metastatic disease to the liver.

I did 46 cycles of chemo over 24 months every 15th day with no pause consisting of 24 cycles full dose Folfirinox of the original higher concentration and 22 cycles 5-FU/Leucovorin alternating in groups of six cycles to lessen peripheral neuropathy which was successful. At the end of the 24 months, nearly all liver Mets were gone and of two still visualized, was thought to be scar tissue.

At this point, I was the first (US) patient to enroll in the PARP-1 inhibitor Rucaparib (Rubraca) which is a biosimilar to Olaparib (Lynparza) to target the germline BRCA mutation I have. The cohort consisted of 19 having a germline or somatic BRCA1, BRCA2 or PALB2 mutation. At the conclusion of the trial, it was determined I had a complete response and the only one of the 19 to remain N.E.D. from the RucaPANC trial. I am the longest former pancreatic cancer patient in the world on Rubraca at 9.5 years. For me the PARP is well tolerated. I was on full dose for exactly 6 years before anemia manifested and I had one dose reduction. I still have mild anemia and take a pause of 6 weeks every six months to give the bone marrow a rest and recover.

Other than the anemia, it causes me a transient increase in serum creatinine and elevated eGFR. With each pause, those results return to normal. A small percentage of patients developed Myelo Dysplasia Syndrome /Acute Myelogenous Leukemia which is featal. In the patients where this happened, they were having difficulty tolerating the PARP early in into use. When I developed anemia after six years, I requested having a bone marrow aspirate done as a precaution. Only RBC’s were affected and not WBC’s or platelets. In addition, genetics showed no abnormalities of precursor cells in the BM, ruling out MDS/AML. I have an excellent quality of life and was declared N.E.D. In 2016. Many oncologists following my case are of the opinion I achieved cure in 2014 and it was the aggressive treatment with Folfirinox that is credited with wiping out minimal residual disease.

I am presented as patient #8 in the graphs of the publication that resulted from the clinical trial.

RucaPANC TRIAL
https://ascopubs.org/doi/full/10.1200/PO.17.00316

During the 24 months of Folfirinox treatment, I walked between 3-5 miles. I worked all through my treatment requiring a daily roundtrip commute of 4 hours 20 minutes minimum. At work I walked the stairwell of 6-8 stories several times a day as well as walking up the escalators and stairs of the NYC subway and an 8 block walk part of my commute.

Just prior to my diagnosis, I was riding 50 mile segments for speed and endurance 2-4 mornings each week for a total of 100-200 miles/week.

Currently walk and ride my bike in nice weather. I have a stationary bike to ride in inclement weather. The exercise helped me in tolerating the aggressive amount of chemo and the duration of treatment of 2 years.