(Poster #297) Pancreatic Undifferentiated Pleomorphic Sarcoma-associated Pancreatic Panniculitis

Abstract

An 18-year-old male with no significant medical history presented to the Emergency Department with a 2-week history of polyarthralgia, fever, and weakness. On admission, the patient was noted to have numerous tender, violaceous, nondraining subcutaneous nodules on the bilateral lower extremities. A CT scan of the abdomen performed at the time demonstrated a solid mass in the pancreatic body. A punch biopsy of a lower extremity nodule showed a predominate panniculitic process involving the subcutaneous fat lobules and contained anucleate, thick-walled adipocytes surrounded by basophilic material and a mixed inflammatory cell infiltrate. At the same time, a fine needle aspiration was performed on the pancreatic body mass. Cytology of this mass revealed an undifferentiated pleomorphic sarcoma. Pancreatic panniculitis is a rare manifestation of pancreatic disorders, affecting around 2-3% of patients with pancreatic disease. It is hypothesized to result from the systemic release of pancreatic enzymes and subsequent necrosis and saponification of the subcutis. It is most often associated with acute and chronic pancreatitis, but it can also be associated with numerous other pancreatic abnormalities such as carcinoma, infection, and pancreatic pseudocysts. Histologically, it is predominately a lobular panniculitis and contains necrosis of adipocytes, creating thick-walled, anucleate cells (“ghost cells”) with a fine, basophilic, granular or homogenous material within their cytoplasm, representing calcium deposition. Treatment of the causative pancreatic disorder is the mainstay of therapy for pancreatic panniculitis. Supportive therapy with compression stockings and leg elevation may aid in symptom management. There have been reports of improvement of cutaneous disease with treatment with octreotide, a somatostatin analogue. We present this case because of its presentation and association with undifferentiated pleomorphic sarcoma in an otherwise healthy young individual.

Financial Disclosure:
No current or relevant financial relationships exist.

Published in: ASDP 59th Annual Meeting, USA

Publisher: The American Society of Dermatopathology
Date of Conference: October 17-23, 2022