Painful lower limb nodules as first symptom of resectable pancreatic acinar cell cancer: a case report

Abstract

Background Pancreatic panniculitis is characterized by subcutaneous fat necrosis and is a rare presentation of an underlying pancreatic disease, appearing in approximately 2–3% of all patients with a pancreatic disease. The nodules usually involve the lower extremities. Pancreatic panniculitis is commonly associated with acute or chronic pancreatitis, and occasionally with pancreatic cancer, especially acinar cell carcinoma. Case presentation A 77-year-old Caucasian woman with no significant medical history was referred to our center with multiple painful, itchy, and warm red/blue cutaneous nodules on the left lower leg. These skin lesions were consistent with the clinical diagnosis of panniculitis. The skin biopsy obtained showed a predominantly lobular panniculitis with fat necrosis of which the aspect was highly suspicious for pancreatic panniculitis. Further analysis revealed high lipase serum of > 3000 U/L (normal range < 60 U/L), and on computed tomography scan a mass located between the stomach and the left pancreas was seen. Endoscopic ultrasonography-guided fine-needle biopsy confirmed the diagnosis of acinar cell carcinoma. After discussing the patient in the pancreatobiliary multidisciplinary team meeting, laparoscopic distal pancreatectomy including splenectomy and en bloc wedge resection of the stomach due to tumor in-growth was performed. The cutaneous nodules on both legs disappeared 1–2 days after surgery. No long-term complications were reported during follow-up. One year after surgery, the patient presented with similar symptoms as preoperatively. Computed tomography scan showed local recurrence and distal metastases, which were subsequently confirmed by biopsy. She started with palliative folinic acid–fluorouracil–irinotecan–oxaliplatin chemotherapy but stopped after two cycles because of disease progression. The patient died 2 months later, 13 months after surgical resection. Conclusion This case illustrates the importance of clinically recognizing cutaneous nodules and pathological recognizing the specific microscopic changes as sign of a (malignant) pancreatic disease.

Metastatic Pancreatic Acinar Cell Carcinoma: An Unlikely Culprit

“…When suspecting the diagnosis of a metastatic PACC, prompt evaluation via skin biopsy with immunohistochemistry is paramount, as patients presenting with cutaneous involvement can be the first indication of an underlying malignancy [18]. For instance, in the case reported by Haenen et al, cutaneous lesions were the initial signs of the PACC, which in turn led to an urgent pancreatectomy that prolonged the patient's life [19]. Early surgical resection of the primary pancreatic neoplasm has been documented to increase the five-year survival rate up to 66%, compared to 17% in unresected patients [20].…”