An 81-year-old woman underwent rectal cancer excision with a transanal approach 17 years ago. The tumor was diagnosed pathologically as intramucosal, well-differentiated rectal adenocarcinoma (Fig. 1a, b). Then, she underwent two reoperations for local rectal cancer recurrence in the next year, and the year after that, she underwent primary resection. Warts frequently appeared on the vulva on several occasions, which were treated each time by liquid nitrogen ablation. The warts also appeared on the vulva 1 year ago. At that time, the tumor was diagnosed as pPD by biopsy, so she underwent perineal tumor resection. The time course of the diagnosis of events and treatment history are shown in Fig. 1c.
Pathological diagnosis with hematoxylin and eosin staining revealed that Paget cells were scattered throughout the squamous epithelium, and adenocarcinoma tissue was observed in the submucosa (Fig. 2a, b). Immunohistochemical staining of the tumor showed active immunoreactivity for cytokeratin (CK)7, CK20, and carcinoembryonic antigen (CEA), but the major marker for PD, gross cystic disease fluid protein (GCDFP)-15 was negative (Fig. 2c). Based on these pathological findings, she was diagnosed with intramucosal rectal cancer recurrence with pagetoid spread. Furthermore, the resected stump was positive for cancer cells, and additional resection was considered for her remaining anal lesion.
At the time of her initial visit to our department, she had no particular symptoms, and no specific target lesions were observed by rectal endoscopy and computed tomography (CT) (Fig. 3a, b). Considered she was 81 years old, had long clinical course, surgical invasions, and decline in her quality of life (QOL) if she will underwent radical operations, we decided to follow her progress carefully. Only 5 months later, she experienced severe anal pain. Blood biochemical tests revealed marked increases in tumor makers CEA (171.5 ng/ml) and carbohydrate antigen 19-9 (259.1 U/ml). Endoscopy showed that the anorectal mucous membranes had become rough (Fig. 3c). CT revealed thickening of the anorectal wall and bilateral inguinal lymph node metastasis (Fig. 3d). Because the tumor was growing rapidly and severe anal pain was becoming worse, she underwent abdominoperineal resection (Miles’ operation) with lymph node dissection (perirectal, sigmoid, superior rectal, and lateral without inguinal lymph nodes).
Pathology
Pathological examination of the surgical specimen revealed that the tumor lesion occupied the perianal area and rectum, with invasion of the internal anal sphincter (Fig. 4a). Many Paget cells were observed in the squamous epithelium, and there were moderately to poorly differentiated adenocarcinoma cells in the submucosal layer (Fig. 4b, c). The lateral lymph node (no. 283L) was positive for cancer cells. These pathological features were consistent with adenocarcinoma with pagetoid spread.
Postoperative course
The patient was discharged from hospital on postoperative day 20, and we explained in detail the disease and its histological and clinical conditions to the patient and her family. Because of her age (>80 years) and her performance status 2, neither the patient nor her family wanted any additional therapy. She was transferred to a hospital near her home, and 7 months after the Miles’ operation, she died from multiple metastases of cancer.