Lipase hypersecretion syndrome: A rare cutaneous manifestation of advanced pancreatic acinar cell carcinoma

Abstract

Careful recognition of cutaneous lesions in patients with malignancies may aid in avoiding additional morbidity during end of life care. K E Y W O R D Sacinar cell carcinoma, lipase hypersecretion syndrome, neoplastic syndrome, pancreatic panniculitis

Curative remnant total pancreatectomy for recurrent pancreatic acinar cell carcinoma: A case report

“…ACC appears to occur as larger tumours in younger patients but causes fewer symptoms such as obstructive jaundice compared with pancreatic ductal adenocarcinoma (PDAC) [9] , [10] . Lipase hypersecretion syndrome with cutaneous manifestations often appears and is characteristic in ACC [11] . Commonly, the mean survival time with ACC is 18–47 months, and the prognosis is thought to be better than that for PDAC [9] , [12] .…”

A rare case of lipase hypersecretion syndrome

“…The condition typically occurs in the sixth decade of life and is more common in men (male/female ratio of 3.6 : 1), 2 although paediatric cases have also been reported 1 . The incidence of pACC‐related LHS is < 15%, 3 with approximately 50% of patients are also diagnosed with metastatic liver disease 5 . Prognosis is poor, with a median survival of 14 months and a 5‐year survival of < 10% 6 …”

“…Histology usually reveals anucleate and calcified ‘ghost cells’ surrounded by fat necrosis, neutrophils and haemorrhage 5 …”

“…1 The incidence of pACC-related LHS is < 15%, 3 with approximately 50% of patients are also diagnosed with metastatic liver disease. 5 Prognosis is poor, with a median survival of 14 months and a 5-year survival of < 10%. 6 The underlying pathological process of LHS is high levels of proteolytic enzymes (predominantly lipase > 10 000 U/dL) 6 being secreted directly into the systemic circulation by malignant cells, 3 causing lipolysis that results in fat necrosis/inflammation.…”

“…6 The underlying pathological process of LHS is high levels of proteolytic enzymes (predominantly lipase > 10 000 U/dL) 6 being secreted directly into the systemic circulation by malignant cells, 3 causing lipolysis that results in fat necrosis/inflammation. 5 The LHS triad primarily occurs in metastatic disease and can be treated with angiographic embolization of the liver metastases, resulting in a significant reduction in symptoms. 3 Panniculitis usually presents as raised, painful, illdefined, erythematous/brown nodules typically affecting the legs, although trunk and scalp involvement has been reported.…”