Hepatic metastasis from perianal Paget\u2019s disease without identified underlying carcinoma: a case report

Yi-Sheng Cao,Shu-Yan Wang

doi:10.1186/s12957-021-02439-4

Abstract

BackgroundPerianal Paget’s disease (PPD) is a rare malignancy, often associated with an underlying adenocarcinoma and a poor prognosis.Case presentationA 69-year-old female was presented with a history of perianal pruritus for 6 months and enlarged inguinal lymph nodes in the left side. Paget cells were confirmed by pathology after a wide excision of perianal skin. Radiotherapy was performed covering the bilateral inguinal lymphatic drainage area. Hepatic metastasis was found 8 months after surgery. Hepatic artery embolization (HAE) and high-intensity focused ultrasound therapy (HIFU) were performed successively. However, hepatic metastasis happened again 3 months later. Ultrasound-guided percutaneous radiofrequency ablation (PRFA) was carried out and various means of inspection could not identify the primary tumor. In the case of rapid progression of the tumor, we gave the patient chemotherapy regimens of XELOX. After 4 cycles of chemotherapy, the tumor marker went down continuously and the hepatic metastasis stayed stable.ConclusionsHepatic metastasis from perianal Paget’s disease without identified underlying carcinoma may benefit from XELOX on the basis of adenocarcinoma.

Highlights

Perianal Paget’s disease (PPD) is a rare malignancy, often associated with an underlying adenocarci‐ noma and a poor prognosis.Case presentation: A 69-year-old female was presented with a history of perianal pruritus for 6 months and enlarged inguinal lymph nodes in the left side
We report a rare case of hepatic metastasis from PPD
In a review by Grow, PPD can be divided into three different patterns on the basis of origins, 50% of cases associated with an apocrine or eccrine carcinoma, called “original Paget’s disease (PD)”, 25% with an underlying anal/rectal adenocarcinoma or squamous cell carcinoma, called “pagetoid extension”, and 25% with no underlying malignant lesion [7]

Summary

Background

Paget’s disease was an uncommon intraepithelial adenocarcinoma named by Sir James Paget describing breast cancer patients with characteristic lesions around the nipples in 1874 [1]. Chest high-resolution CT (HRCT) reveals a 5-mm ground glass nodule with a clear boundary in the posterior segment of the upper lobe tip of the left lung (LUNG-RADs 3). Both of the gastroscopy and colonoscopy had no positive findings. Due to large skin defect after excision of the lesion, perianal flap transfer was performed After her wound healed (Fig. 1B, C), the patient began to receive radiotherapy covering the bilateral inguinal lymphatic drainage area. Hepatic artery embolization (HAE) and high-intensity focused ultrasound therapy (HIFU) were performed successively After those therapies, the patient’s tumor marker went down a bit but soon rose again. After 4 cycles of chemotherapy, the tumor markers went down continuously (Fig. 4) and MRI found the patient in the status of partial response (PR) (Fig. 3D)

Findings

Discussion

Conclusions