Department of Pathology, Koç University Hospital, Davutpasa Caddesi No:4, Topkapi, 34010 Istanbul, Turkey.
Department of Pathology, Koç University Hospital, Davutpasa Caddesi No:4, Topkapi, 34010 Istanbul, Turkey.
Semin Diagn Pathol. 2019 Jul;36(4):240-245. doi: 10.1053/j.semdp.2019.07.001. Epub 2019 Jul 5.
Lipase hypersecretion syndrome (LHS) is a paraneoplastic syndrome seen exclusively as a result of pancreatic acinar cell carcinoma (ACC). In LHS, acinar enzymes (lipase, trypsin and chymotrypsin) which are normally secreted to the duodenum for digestive purposes, are instead released to the blood by the carcinoma cells. In a way, it is "endocrine-ization" of an "exocrine" function. These circulating enzymes, especially lipase, exerts its digestive action on other tissues, especially on the subcutaneous tissues in the pressure poins of legs, creating a picture often mistaken as erythema nodosum or rheumatic nodules. The bone and joints may also be effected, which mostly appears to be secondary to the complications and super-infection of the skin lesions. Eosinophilia also often accompanies this syndrome. The accurate diagnosis of LHS requires the identification of the pancreatic primary as well as its correct classification as acinar because a variety of pancreatic tumors can be associated with skin lesions, ranging from rare metastasis of adenocarcinoma to the necrolytic migratory erythema caused by glucagon-producing neuroendocrine tumors. Towards this differential, the diagnostic characteristics of acinar cell carcinomas that have been better elucidated in the past decade often need to be employed in increasingly smaller specimens and the liver, especially since most LHS cases also have liver metastasis (presumably due to the by-pass of the "first-pass" liver metabolism phenomenon). ACC (and LHS) occur in patients in their 60's. The pancreatic mass is often large, round, demarcated and closely resemble neuroendocrine and solid-pseudopapillary neoplasms but are more atypical/proliferative, and commonly show single prominent nucleoli and a distinctive chromophilia. Immunostaining with trypsin/chymotrypsin, negativity of beta-catenin help in the differential; as a caveat, neuroendocrine differentiation is common in ACCs. In conclusion, LHS is a rare type of paraneoplastic syndrome specific to ACC. The accurate diagnosis requires attention to their subtle diagnostic characteristics.
脂肪酶分泌过多综合征（LHS）是一种副肿瘤综合征，仅见于胰腺腺泡细胞癌（ACC）。在 LHS 中，原本为了消化而分泌到十二指肠的酶（脂肪酶、胰蛋白酶和糜蛋白酶）被癌细胞释放到血液中。在某种程度上，这是一种“内分泌化”的“外分泌”功能。这些循环酶，特别是脂肪酶，对其他组织，特别是腿部压力点的皮下组织发挥其消化作用，形成一种常被误认为是结节性红斑或风湿结节的图像。骨骼和关节也可能受到影响，这主要是由于皮肤病变的并发症和继发感染。嗜酸性粒细胞增多症也常伴有这种综合征。LHS 的准确诊断需要识别胰腺原发肿瘤，并正确分类为腺泡，因为各种胰腺肿瘤都可能与皮肤病变相关，从罕见的腺癌转移到由胰高血糖素分泌的神经内分泌肿瘤引起的坏死性游走性红斑。为了进行鉴别，过去十年中已经更好地阐明了腺泡细胞癌的诊断特征，通常需要在越来越小的标本和肝脏中使用，尤其是因为大多数 LHS 病例也有肝转移（可能是由于“第一通过”肝脏代谢现象的旁路）。ACC（和 LHS）发生在 60 多岁的患者中。胰腺肿块通常较大、圆形、边界清晰，与神经内分泌和实性假乳头状肿瘤相似，但更具非典型性/增生性，通常表现出单个突出的核仁，并具有独特的嗜铬性。用胰蛋白酶/糜蛋白酶进行免疫染色、β-连环蛋白阴性有助于鉴别；需要注意的是，神经内分泌分化在 ACC 中很常见。总之，LHS 是一种罕见的副肿瘤综合征，特异性地与 ACC 相关。准确的诊断需要注意其微妙的诊断特征。
