Shahid Gilani, MD Certified Oncologist and Radiation oncologists from the University Hospital Of North Midlands discusses the study A Sustained Response of Maintenance Therapy in Pancreatic Acinar Cell Carcinoma (PACC): A Case Report and Literature Review is written with co-author Dr. Apurna Jegannathen.
Study:
Maintenance Therapy; Pancreatic Acinar Cell Carcinoma (PACC); Gemcitabine; Oxaliplatin; CapecitabineWe identify a male patient 50 years of age who has been histologically diagnosed with a pancreatic acinar cell tumor. Initially, he had localized cancer of the pancreas. He underwent distal pancreatectomy with splenectomy, con-anaphylaxis, for pT3pNxpMxR1 disorder. Oxaliplatin + capecitabine was initiated upon disease recurrence but stopped after 5 cycles due to disease progression. However, oddly, later CT scans after 3 months revealed a disease response that persisted without any treatment for nearly 6 months. We assume that this was an unusual late reaction. But sadly, soon after this, his illness worsened again. We re-challenged him at that stage with the same chemotherapy. This patient on maintenance therapy demonstrated more clinical and radiological progress in his disease more than four years after his initial diagnosis.
Introducing
8463 new cases of pancreatic cancer in the UK are diagnosed each year. Over the past two decades, the prevalence of pancreatic cancer in the UK has remained the same, affecting similar proportions of men and women, accounting for 3% of all new cancers. The incidence of pancreatic cancer would rise because of the longevity of the population. Either the endocrine or exocrine portion of the pancreas can cause pancreatic tumors. Nearly 95% of these cancers come from the ductal portion and are exocrine in origin. A rare tumor consisting of 1 to 2 percent of exocrine pancreatic cancers is pan-creatic acinar cell carcinoma (PACC). PACC is distinct genomically from other cancers of the pancreas.
Context
Acinar cell carcinoma is a rare type of pancreatic malignancy that accounts for just 1-2% of all cancers of the pancreas. They’re tending more will occur in men at the age of seventy. Symptoms of presentation are typically non-specific. It is also associated with elevated levels of lipase in the serum. We’re reporting on a young man who submitted at the age of fifty years with general ill health and weight loss. Not only did his illness respond well to the initial chemotherapy,
But on maintenance chemotherapy, they still stayed stable. After his initial diagnosis, he lived for 48 months.
Report
A 50-year-old male reported weight loss and general fatigue. He had no prior medical background or family history that was significant. The output status of his WHO was zero. Picked-up CT scans a left hypochondrial mass that was histologically confirmed later as adenocarcinoma. He underwent a distal pancreatectomy and splenectomy. As well as differentiated acinar, final histology came back pancreas cellular carcinoma (pT3 pNx pMx R1). Adjuvant chemotherapy with Gemcitabine was initiated but abandoned due to anaphylactic reaction. A CT surveillance scan three months later increased concerns of local recurrence and potential liver metastases. The PET CT scan confirmed these results. Therefore, in palliative environments, the Oxaliplatin plus Capecitabine protocol was initiated. His condition appeared to worsen after 5 cycles.
In your liver. He refused to engage in a clinical trial or to undergo additional treatment with chemotherapy. Three months later, he had pain in his abdomen. Surprisingly, at the time, CT scanning.
There was shrinkage of the pancreatic tumour and liver lesions disappearing. A manifestation of late reaction to previous chemotherapy maybe this phenomenon. But sadly, six months later, his condition worsened again, which was supported by CT and MRI scans. A re-challenge decision was made with Oxaliplatin plus Capecitabine. A remarkable response was seen again after 4 more cycles. Due to neurotoxicity, we had to avoid Oxaliplatin, but we continued maintenance with the single agent Capecitabine. Further imaging revealed a persistent response without any further development, also 48 months after its initial progression.
With diagnosis. For four years from his initial diagnosis, this patient operated on maintenance therapy with good success status.
Continued disease response to maintenance treatment with capecitabine was observed. Before his illness progressed, he was tracked with routine surveillance CT scans.
Discussion
Acinar carcinoma is more common in males than in females between the mean age of 60 and 70 years. Weight loss, nausea, and abdominal pain are usually present.
In certain patients manifesting Schmid’s triad consisting of polyarthritis, eosinophilia, and fat necrosis, serum lipase level may be elevated. Endocrine defects can also occur in rare acinary cell carcinoma. In this sense, our case is unusual in that the patient is relatively young and he has symptoms that are known to occur in this form of cancer, i.e. weight loss and constitutional symptoms.
The prognosis for most pancreatic cancers is low. The mean survival for advanced pancreatic cancers remains 4 to 8 months.
There are no different guidelines for the treatment of acinar cell tumours. This unusual form of malignancy is currently being treated in much the same manner as other forms of pancreatic exocrine tumors. For several years, gemcitabine has been the mainstay of care for advanced cancer of the pancreas (APC). Unfortunately, several earlier studies did not demonstrate survival benefits when gemcitabine was applied to various chemotherapeutic agents. But two meta-analyses later showed that the combination of gemcitabine with either capecitabine or oxaliplatin resulted in a significantly longer overall survival. A triplet combination regimen (Gemcitabine, Oxaliplatin, and Capecitabine) has recently been studied.
Some APC benefits increase the median time to 4.3 months of progression. But this comes with a toxicity price and rapid resistance growth.
Monique Antoine, et al., who survived for 37 months after treatment with several chemotherapeutic agents, reported a similar case of pancreatic acinary cell carcinoma in 2007. But in this respect, our case is special in that it survived for much longer after only undergoing a single oxaliplatin + capecitabine regimen followed by maintenance chemotherapy with capecitabine alone.
In Conclusion
There may be a late manifestation of response and long-term stabilization of the disease in certain patients, maintenance chemotherapy may be possible. This may be due to biological and genetic underlying factors that need to be further investigated by genomic profiling. For instance, RAF gene fusions and mutually exclusive inactivation of DNA repair genes are novel potential therapeutic targets altered in more than two-thirds of these tumors.