The patient diagnosed with stage IV pancreatic acinar cell carcinoma with polyarthralgia did not show improvement after receiving chemotherapy (multiple drugs). The patient was finally shifted to hospice care. 

The patient reported a 2-week history of pain and inflammation in his right and left ankle joints.

Clinical Diagnosis: The patient was initially presented for initial lab workup that included a complete blood count (with elevated WBC's with absolute neutrophils), ESR (118mm/hr), and CRP (8mg/dL) tests. All metabolic panels were within reasonable limits. Immunological workup includes analysis of ANA titer, positive lupus anticoagulant and dsDNA antibodies. Then an ultrasound and MRI of the right wrist was done. The results of the ultrasound revealed effusion in joints and swelling in surrounding soft tissue. Whereas MRI scan revealed composite signal and enhancement of multiple carpel bones involving the second through fifth metacarpals, subcutaneous and deep soft tissue of the wrist and proximal hard. Clinical and laboratory diagnosis states that the patient was suffering from systemic lupus erythematosus (SLE), and this rheumatological condition can be a conventional differential for polyarthralgia.

Confirmatory Diagnosis: After a few days later, physical examination showed subcutaneous nodules with a palpable, non-tender mass on the left side of the abdomen. CT of the abdomen revealed a large heterogeneous mass measuring 20.1 by 10.7 by 18.1 cm in the left quadrant of the stomach with a poorly attenuation mass measuring 2.9 by 3cm in the hepatic lobe. CT guided biopsy of the left-sided abdominal mass was non-diagnostic Multiple abnormal areas in liver concerning metastases were recorded, and pathology of resected mass revealed sheets of malignant acinar cells with no ducts/ islets. Based on biopsy and staging, it was found that the patient has stage IV pancreatic acinar cell carcinoma with polyarthralgia, but the cause of polyarthralgia was still not known.

The patient was administrated initially with adjuvant chemotherapy of leucovorin, 5-fluorouracil (5-FU) and oxaliplatin, but he had not shown any significant improvement. So, the chemotherapy was switched to capecitabine and subsequently to gemcitabine and Protein-bound paclitaxel. In spite of chemotherapy, the patient did not demonstrate any adequate response, and his clinical condition worsened. Finally, he was shifted to hospice care after discussion of goals of concern with the patient and his family, due to poor prognosis, and chemotherapy was discontinued.

Acinar cell carcinomas are a category of malignant epithelial neoplasms that occurs with an incidence rate of about 1% to 2% of pancreatic tumours. They usually happen in the solid state, but sometimes they can also be present in cystic lesions or mixed with both solid and cystic components. They can occur at any stage but are predominant in males as compared to females.  The typical symptoms are abdominal pain, weight loss, fatigue or nausea. They can be distinguished by the production of zymogen granules containing pancreatic exocrine enzymes, in the tumour cells. It can result in a rare paraneoplastic syndrome called hyperlipasemia or lipase hypersecretion syndrome. The rise in serum levels in this condition above 10,000 U/dl can result in the formation of multiple subcutaneous fat nodules and necrosis termed as pancreatic panniculitis and polyarthralgia due to sclerotic lesions and effusions in the bones. It can be the initial manifestation of the neoplasm and can be related to a poor prognosis. Histologically, these neoplasms are indicated by malignant cells with differentiation of acinar cells. They stain positive on immunohistochemical stainings such as trypsin, chymotrypsin, elastase, or lipase. Zymogen granules.  The presence of paraneoplastic syndrome can lead to worsening of prognosis and can adversely affect the quality of life.

The management modalities of hyperlipasemia and acinar cell carcinomas varies.  Among the various treatment modalities, surgical resection is one of the most therapeutic options, whereas if acinar cell carcinoma is found to be in advanced or metastatic stages, they require adjuvant chemotherapy. Acinar cell carcinomas are treated with 5-FU/leucovorin, but other chemotherapeutic agents such as irinotecan, doxorubicin, erlotinib, gemcitabine, docetaxel, cisplatin, capecitabine, oxaliplatin, sirolimus and sunitinib can also be used.

Pancreatic acinar cell cancer is a rare pancreatic malignancy often prevalent in the elderly population. It is predominated by signs of abdominal pain and less frequent jaundice. The hypersecretion subtype may present with fever, arthralgia, rash, panniculitis and hypereosinophilia. Large lesions with sharp boundaries are revealed on imaging. The tissue is necessary for diagnosis and surgery is essential in the treatment of localised injuries. Chemotherapy can be helpful for long-term survival regarding metastatic lesions. The current study states that oxaliplatin-based chemotherapy can be useful, and these malignancies carry a better prognosis as compared to pancreatic adenocarcinomas. So, it is essential to be mindful of various paraneoplastic manifestations of malignancies as they might represent the initial signs of malignancy.

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