Solitary colonic polypoid ganglioneuroma

Abstract

This short report discusses a case of solitary colonic polypoid ganglioneuroma associated with melanosis coli in a woman with no systemic manifestations. To our knowledge this is the first ganglioneuroma reported in the literature in association with melanosis coli. The nature and significance of this event remains unclear, although this may be coincidental due to the laxative intake. Further investigation is necessary to clarify this point. The interest of this case lies moreover in the rarity of this entity and its endoscopic and histologic resemblance to sessile polyps frequent in the clinical practice. FindingsThe hereditary syndromes of the gastrointestinal tract are classified as adenomatous or hamartomatous [1]. As a part of the hamartomatous polyposes, ganglioneuroma (GN) of the gastrointestinal tract are rare tumors composed of ganglion cells, nerve fibers, and supporting cells of the enteric nervous system [1,2]. There are few reports in the literature. We present a case of polypoid GN and melanosis coli. To our knowledge this is the first case reported in literature with this association.A 48-year old woman with arthrosis underwent colonoscopy because of a family history of colon cancer (father and cousin) and an episode of lower intestinal bleeding. The patient was an anthracene-type laxatives ("sacred rind") consumer due to usual constipation. No other symptoms were related. She and her family had no known history of multiple endrocrine neoplasia or neurofibromatosis. The laboratory tests were within the normal limits, including carcinoembryonic antigen (CEA). Physical examination revealed an anal fissure. No pigmented skin lesions were identified. The colonoscopy revealed a millimetric sessile polyp in the sigmoid colon that was removed with biopsy forceps (Figure 1).The biopsy specimen consisted of three fragments. At low magnification they looked like a hyperplastic polyp. Microscopic examination of the fragments of the polyp showed a collection of nerve ganglion and stromal cells in the lamina propia that elevated the overlying elongated glands in a nodular configuration. No significant disarray of the mucosal architecture was observed. An additional finding of diffuse melanosis coli was noticed (Figure 2). Inmunohistochemical studies were performed. The ganglion cells and the spindle stromal surrounding cells demonstrated inmunoreactivity to protein S-100, and neuronspecific enolase (NSE) marked the ganglion cells ( Figure 3).

Ganglioneuroma of the uterine cervix—a case report

“…Although ganglioneuroma is mostly a well-circumscribed mass, our case was not a circumscribed mass. Some ganglioneuroma in skin and colon were not a circumscribed mass [4,9]. It is possible that ganglioneuroma, especially in the choristomatous lesion, cannot form a circumscribed mass.…”

“…They are fully differentiated tumors composed of mature ganglion cells, nerve fibers, and Schwann cells. These tumors are usually located in the posterior mediastinum, retroperitoneum, or adrenal glands [1] but also rarely occur in the presacral space, intestine, and skin [2][3][4]. We report here the first case of ganglioneuroma in the uterine cervix.…”

Spindle cell proliferations of the sigmoid colon, rectum and anus: a review with emphasis on perineurioma

“…Isolated polypoid ganglioneuromas are usually <20 mm and found incidentally during endoscopy, where they are macroscopically indistinguishable from adenomas. There is no gender predilection, and adults of all ages are affected . They characteristically expand and replace the lamina propria with a proliferation of bland spindle and ganglion cells in a fibrillary matrix .…”

“…There is no gender predilection, and adults of all ages are affected . They characteristically expand and replace the lamina propria with a proliferation of bland spindle and ganglion cells in a fibrillary matrix . Similar to perineurioma, there is separation of crypts and serrated architecture may be seen.…”

“…The transmural involvement of DG may not be appreciated in superficial biopsies. Positive staining for S100 is seen in the spindle cell component; neurone‐specific enolase (NSE), synaptophysin and neurofilament protein (NFP) highlight ganglion cells, allowing differentiation from neurofibroma …”