Report of a case combining solitary Peutz-Jeghers polyp, colitis cystica profunda, and high-grade dysplasia of the epithelium of the colon

Abstract

BackgroundColitis cystica profunda is a rare nonneoplastic disease defined by the presence of intramural cysts that contain mucus, usually situated in the rectosigmoid area, which can mimic various malignant lesions and polyps. Its etiology still remains not fully elucidated, and several mechanisms such as congenital, post-traumatic, and infectious have been implicated in the development of this rare entity.Case presentationHerein, we describe a unique case of colitis cystica profunda in the setting of Peutz-Jeghers-type polyp of the sigmoid colon, associated with high-grade dysplasia of the overlying epithelium in a 48-year-old female patient, who presented to the emergency room with signs of intestinal obstruction. To the best of our insight, this is the first manifestation ever reported in the literature regarding the coexistence of solitary Peutz-Jeghers-type polyp, colitis cystica profunda, and high-grade dysplasia of the epithelium of the colon.ConclusionsThe purpose of this case report is to highlight colitis cystica profunda and its clinical significance. An uncommon nonneoplastic entity, many times masquerading as malignant lesion of the rectosigmoid area of the colon. Clinicians and pathologists should be aware of this benign condition that is found incidentally postoperatively in patients undergoing colectomies, leading to unnecessary increase of morbidity and mortality in these patients, who otherwise could have been cured with conservative treatment only.

Colitis cystica profunda: A report of two cases

“…CT images show a noninfiltrating submucosal mass with well‐defined borders and an unenhanced cystic lumen of variable size. The perirectal adipose tissue was absent, and the levator anus muscle was thickened 2,15 . MRI showed a homogeneous low signal on T1WI and a submucosal high signal nodule on T2WI, with no significant enhancement on contrast‐enhanced images, high signal on DWI, no diffusion restriction on ADC, and significant high signal on T2WI, suggesting the presence of mucin in the lesion 4,16 .…”

“…In 1863, Virchow named it colitis cystica polyposa for multiple polypoid cystic submucosal lesions. In 1957, Goodall and Sinclair renamed colitis cystica polyposa to colitis cystica profunda (CCP), first giving the name and detailed description of the disease 1,2 . CCP is a rare benign disease occurring in the rectum or colon, most commonly in the anterior wall of the rectum.…”

“…In 1957, Goodall and Sinclair renamed colitis cystica polyposa to colitis cystica profunda (CCP), first giving the name and detailed description of the disease. 1 , 2 CCP is a rare benign disease occurring in the rectum or colon, most commonly in the anterior wall of the rectum. The main pathological characteristics of CCP are mucus‐containing cysts reaching deep into the submucosa, muscular layer or even the serous layer.…”

Colitis cystica profunda: a two-patient case series

“…CT images show a non-infiltrating submucosal mass with well-defined borders and an unenhanced cystic lumen of variable sizes. The perirectal adipose tissue was absent and the levator anus muscle was thickened [2,15] . MRI showed a homogeneous low signal on T1WI and a submucosal high signal nodule on T2WI, with no significant enhancement on contrast-enhanced images, high signal on DWI, no diffusion restriction on ADC, and significant high signal on T2WI suggesting that the presence of mucin in the lesion [4,16] .…”

“…In 1863, Virchow named it colitis cystica polyposa for multiple polypoid cystic submucosal lesions. In 1957, Goodall and Sinclair renamed colitis cystica polyposa to colitis cystica profunda (CCP), giving the name and detailed description of the disease firstly [1,2] . CCP is a rare benign disease occurring in the rectum or colon, most commonly in the anterior wall of the rectum.…”