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• W2997403397 abstract "The patient was a 76-year-old Japanese man with a red massive lesion on the right side of the genital region appeared about 2 years ago (Figure 1A). In the biopsy specimen, tumor cells with bright cytoplasm were forming nests and infiltrating from the thick epidermis to the deep layer of the dermis. These tumor cells were positive for PAS, CEA, GCDFP-15, Cam5.2, and negative for S-100. Computed tomography showed bilateral inguinal lymphadenopathies. Positron emission tomography (PET) showed one accumulation that seems to be bone metastasis observed in the eighth thoracic vertebrae (Figure 1C) and some accumulations in abdominal aortic lymph nodes. Serum CYFRA was increased (5.2 ng/mL). Consequently, the patient was diagnosed as extramammary Paget disease (EMPD), T2N2M1 stage IV (TNM staging proposed by Ohara et al1). Although it was thought that there is no curative surgical indication, resection of the primary tumor at 1 month after the diagnosis was planned for local control, and low-dose 5-fluorouracil/cisplatin (FP) therapy was performed prior to surgery. In low-dose FP therapy, 5-FU 500 mg/body was administered for 7 days/week and cisplatin 10 mg/body was administered for 5 days/week in 1 cycle. After consecutive 4 cycles of low-dose FP therapy, remarkable tumor shrinkage was observed (Figure 1B). Lymphadenopathies were also improved. Resection of the primary tumor with 1 cm margin from the erythema area and 3 cm margin for the unclear border area was performed. One right superior inguinal lymph node was taken out for staging and evaluation of treatment effect. The reconstruction was performed with split thickness skin graft. Histopathological examination showed that tumor cells metastasized to the extracted lymph node. Low-dose FP therapy was restarted 1 month after surgery and continued on weeks 1, 2, 3 or 1, 2 every 4 weeks. The clinical image at 2 months after surgery is shown (Figure 1E). Fluorodeoxyglucose accumulation in the eighth thoracic vertebrae and abdominal aortic lymph nodes have disappeared in PET performed after 10 cycles of low-dose FP therapy (Figure 1D). Low-dose FP therapy have been continuing for 1 year after surgery, 24 cycles. No recurrence was observed. Low-dose FP therapy has been reported to be effective for advanced EMPD.2, 3 Tokuda et al reported low-dose FP therapy for 22 patients.4 They used a dose of 600 mg/m2 of 5-fluorouracil and the median overall survival was 12 months. According our recent report using same regimen as this case, the median progression-free survival was 25 weeks, and the median overall survival was 77.4 weeks.3 Tokuda et al's regimen and Kato et al's regimen differ in dosage as well as dosing interval. Both regimens were reported to have low risk and high effect. As for neoadjuvant chemotherapy, Kobayashi et al reported low-dose FP therapy (5-FU 500 mg/body ×10, CDDP 10 mg/body ×10) for advanced breast esophageal cancer and a pathological complete response was obtained.5 Low-dose FP therapy prior to surgery may be one option for the treatment of advanced EMPD. The authors declare no conflicts of interest." @default.
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• W2997403397 date "2019-12-23" @default.
• W2997403397 modified "2023-10-12" @default.
• W2997403397 title "Low‐dose 5‐fluorouracil/cisplatin therapy as conversion chemotherapy for advanced extramammary Paget's disease" @default.
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• W2997403397 doi "https://doi.org/10.1002/kjm2.12168" @default.
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