Epidermoid Cyst in an Intrapancreatic Accessory Spleen: Three Case Reports and Review of the Literatures

Abstract

The development of an epidermoid cyst in an intrapancreatic accessory spleen is an extremely rare lesion, with only 17 cases being reported in the English literature. All such cases were located in the pancreatic tail, some of which showed carbohydrate antigen 19-9 (CA19-9) immunoreactivity in the lining of the epithelium. A few of them indicated an elevation of the serum CA19-9 level. Here we report three cases of an epidermoid cyst in an intrapancreatic accessory spleen. Cases 1 and 2 were 57-year-old and 70-year-old women, while case 3 was a 37-year-old man. All three cases were asymptomatic. Serum CA19-9 levels showed within normal limits (case 1), slightly elevated (case 2), and clearly elevated (case 3). They underwent a distal pancreatectomy with splenectomy (cases 1 and 2) and without splenectomy (case 3). Grossly, the surgical specimen was a well-demarcated, multiple (case 1) or solitary (cases 2 and 3) cystic mass in the pancreatic tail. A high level of fluid CA 19-9 was detected in case 1. Microscopically, the cystic walls were lined with squamous and cuboidal epithelium, which were surrounded by normal splenic tissue and hyalinized fibrous tissue. The lining squamous epithelium was revealed as nonkeratinizing (Cases 1 and 2) or keratinizing (Case 3). Immunohistochemically, CA19-9 was positive in the monolayer and surface layer of the cuboidal epithelium, but negative for the keratinizing squamous epithelium. As for the histogenesis, it is suggested that the cystic lining of the epithelium may derive from the pancreatic duct which protrudes into the accessory spleen.

Epidermoid cyst of an intrapancreatic accessory spleen: a case report and literature review

“…However, the exact incidence of ECIPAS is difficult to determine as over 50% of the cases were incidentally detected [8,9,12,14,15,18,19,22-24], [26,27,30,32,34]. The mean age of the patients was 46.1 years (range 12 to 70 years), with a female preponderance (58.3%) [6,7,10-13,16,17,19-21,24-26,28], [31,33,34,36]. It is noteworthy that the majority of patients were of Asian origin (28/36 patients, 77.7%), suggesting possibly the presence of a racial factor [26,27].…”

A Splenic Epithelial Cyst: Increased Size, Exacerbation of Symptoms, and Elevated Levels of Serum Carcinogenic Antigen 19-9 after 6-year Follow-up

“…In the present case, the splenic epithelial cyst, the origin of which was difficult to identify preoperatively, was associated with exacerbation of symptoms, elevated serum CA19-9 levels, and an increased size after 6 years of follow-up. Concerning the preoperative diagnosis of the current case, an epithelial cyst (10), including epidermoid (8) or lymphoepithelial (9, 11) cysts, was strongly suspected due to the extremely high level of serum CA19-9 (2,5,8,9).…”

“…Epithelial cysts are not specific to the spleen. They can also develop at the pancreatic tail, with similar characteristics regarding age, image findings, and elevated serum tumor markers (8)(9)(10). We herein describe a patient with a large splenic cyst, which was difficult to differentiate from a cyst of pancreatic origin, who was followed up for 6 years.…”

Epidermoid cyst arising from an intrapancreatic accessory spleen: A case report and review of the literature

“…Thirdly, a derivation from the pancreatic duct may protrude into the accessory spleen (10). In a case described by Kadota et al (23), there were pancreatic ducts in the fibrous tissue surrounding the accessory spleen tissue, and the squamous and cuboidal epithelia indicated a transitional appearance from one form to the other. Additionally, immunohistochemical analysis demonstrated that the staining results of the cystic epithelial lining were identical to those of the pancreatic duct.…”

“…The histogenesis of an epidermoid cyst in an IPAS may be identical to that of a splenic epidermoid cyst (23). There are three hypotheses concerning the histogenesis of an epidermoid cyst in an IPAS (10).…”