Rare Anal Canal Cancer with Secondary Extramammary Paget’s Disease (Pagetoid Spread) Complicated by Squamous Cell Carcinoma of the Skin

A 91-year-old man had a node and erythema in the anal area resistant to treatment. A biopsy of the node in the anus showed atypical cells developing as Paget’s disease, and staining revealed that the cells were CK7-positive, CK20-positive, and GCDFP15-negative. Therefore, tumor invasion with pagetoid spread (PS) from the anus to the skin was suspected, and the patient was referred to our department for a close examination and surgical treatment. Lower gastrointestinal endoscopy showed edematous, hemorrhagic mucosa in the anal canal, and he was diagnosed with adenocarcinoma via a biopsy. Additionally, redness and swelling with white moss were observed on the skin around the anus. Biopsy showed that Paget cells were diffusely present in the epithelium, and an image of squamous cell carcinoma directly under the epithelium was obtained. Taken together, the patient was diagnosed with the invasion of anal canal cancer with PS to the skin, and we performed laparoscopic abdominoperineal resection and skin carcinoma resection in the perineum. The histopathological analysis showed adenocarcinoma invading the external anal sphincter and subcutaneous adipose tissue in the vicinity of the pectinate line of the anal canal. Pagetoid spread of the adenocarcinoma was observed in the epidermis, and the open portion was slightly invaded up to the rectal mucosa. The anal skin region of the adenocarcinoma partially continued to the hair follicles, and it was complicated by squamous cell carcinoma invading the dermis. There are a few reports of anal canal cancer with PS, and the coexistence of adenocarcinoma and squamous cell carcinoma, as seen in the present case, is rare. We report our case together with relevant literature.

Autopsy Study of Calretinin Immunohistochemistry in the Anorectal Canal in Young Infants and Potential Implications for Rectal Biopsy Approach in the Neonatal Period

Background Absent submucosal ganglion cells in biopsies 1-3 cm above the pectinate line establishes the pathologic diagnosis of Hirschsprung Disease (HD). Calretinin stains both ganglion cells and their mucosal neurites and has gained importance in HD diagnosis. Absent calretinin positive mucosal neurites in biopsies at the appropriate level above the pectinate line is highly specific for HD. Whether this applies to lower biopsies is uncertain. To address this, we studied anorectal canal autopsy specimens from infants. Methods We performed an autopsy study of infant anorectal canal specimens to describe calretinin staining in this region. Calretinin staining was correlated with histologic and gross landmarks. Results In all 15 non-HD specimens, calretinin positive mucosal neurites were present in glandular mucosa up to the anorectal line where neurites rapidly diminished. Age range was preterm 26 weeks to 3 months. Conclusions Calretinin positive mucosal neurites are present in glandular mucosa up to the anorectal line in young infants. This is potentially important regarding neonatal HD biopsy level and diagnosis. Positive calretinin staining at the anorectal line favors normal innervation making HD unlikely. Absent calretinin positive neurites in glandular mucosa is worrisome for HD in young infants, regardless of location.

A Case of Laterally Spreading Tumor of the Rectum Treated by Endoscope-Assisted Transanal Tumor Resection

Background: Curability and function should be considered in resection of a rectal lateral spreading tumor (LST). Methods: We performed endoscopic transanal resection for a rectal LST extending to the pectinate line to preserve anal function and avoid a colostomy in 1 case and describe our procedure below. An 80-year-old man with a group 5 adenocarcinoma tub1/2 and no metastasis was considered for transanal resection because of his refusal of a colostomy and for staging and preservation of anal function. A strategy involving endoscopic submucosal dissection for mucosal cutting at the proximal side and for submucosal dissection of the maximum area at the anal side of the tumor was chosen, considering the tumor size. With the patient in a jackknife position, the tumor was marked circumferentially with coagulation dots, after which MucoUp with bosmin was injected into the submucosa at the proximal side. Mucosal and submucosal dissection was performed using DualKnife to remove the largest possible area toward the anal side. Transanal resection was performed under direct vision using an anal retractor, wherein the mucosa was cut circumferentially by extending the existing resection line along with removal of the submucosa. Results: En bloc resection with clear margins was achieved. Postoperatively, retroperitoneal emphysema was observed on a computed tomography scan, which resolved after 1 week of fasting. The patient was discharged 20 days postoperatively. Neither recurrence nor metastasis has been observed 1 year postoperatively. Conclusions: Our procedure enabled successful resection of a rectal LST with negative margins and preservation of anal function.

Anorectal Disorders

Anorectal disorders can generate considerable patient discomfort and disability. Although mortality due to such complaints is very low, it is important for the clinician to maintain a high index of suspicion for systemic illness caused by an anorectal source. A detailed history and physical examination should be performed, and the need for imaging or procedures should be assessed. This review examines the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes for patients with anorectal disorders. Figures show the important structures of the anal canal; differences in the anatomy of the origin of internal and external hemorrhoid venous supplies; depictions of a typical anodermal linear tear; Foley catheter–assisted rectal foreign body removal technique; and pertinent anatomy related to a prolapsed rectum through the anus; and types and locations of anorectal abscesses and fistulas. Tables list common painful and painless anorectal disorders; key differences in anal canal structures above and below the pectinate line; anal symptoms mistakenly attributed to hemorrhoids; internal hemorrhoidal grading, description, and recommended treatment; Rome III criteria for the diagnosis of constipation; and a summary of anorectal conditions. This review contains 6 highly rendered figures, 6 tables, and 80 references. Keywords: functional constipation; PEG; abdominal radiographs; pediatric constipation

Anorectal Disorders

Anorectal disorders can generate considerable patient discomfort and disability. Although mortality due to such complaints is very low, it is important for the clinician to maintain a high index of suspicion for systemic illness caused by an anorectal source. A detailed history and physical examination should be performed, and the need for imaging or procedures should be assessed. This review examines the pathophysiology, stabilization and assessment, diagnosis and treatment, and disposition and outcomes for patients with anorectal disorders. Figures show the important structures of the anal canal; differences in the anatomy of the origin of internal and external hemorrhoid venous supplies; depictions of a typical anodermal linear tear; Foley catheter–assisted rectal foreign body removal technique; and pertinent anatomy related to a prolapsed rectum through the anus; and types and locations of anorectal abscesses and fistulas. Tables list common painful and painless anorectal disorders; key differences in anal canal structures above and below the pectinate line; anal symptoms mistakenly attributed to hemorrhoids; internal hemorrhoidal grading, description, and recommended treatment; Rome III criteria for the diagnosis of constipation; and a summary of anorectal conditions. This review contains 6 highly rendered figures, 6 tables, and 80 references. Keywords: functional constipation; PEG; abdominal radiographs; pediatric constipation