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Cytoreductive Surgery Plus Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei Arising from Urachus

  • Urologic Oncology
  • Published:
Annals of Surgical Oncology Aims and scope Submit manuscript

Abstract

Background

Pseudomyxoma peritonei (PMP) is a rare locoregional disease characterized by disseminated intraperitoneal mucinous tumors. However, little is known about PMP from urachal neoplasm as a result of its rarity.

Methods

A total of 9 patients with PMP of urachal origin were treated by cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) in our institution. All specimens of surgeries were submitted for pathologic examination. Representative slides of tumors and normal urachus were submitted for immunohistochemical staining.

Results

Four patients were men; the median age was 48 years (range 27–65 years). Initial radiologic examination of all patients showed a cystic tumor located between posterior aspect of umbilicus and the dome of urinary bladder, with or without leaking mucin. Complete CRS and HIPEC were performed in all patients. Until the latest follow-up, local recurrence occurred in 1 patient. Other 8 patients had a median disease-free survival of 27.5 months. Primary urachal tumors of 9 cases were all mucinous adenocarcinoma. Six patients had low-grade mucinous carcinoma peritonei, and 3 patients had high-grade mucinous carcinoma peritonei. Signet ring cells were noted in 4 patients. All tumor specimens of 9 patients were diffuse positive for CK-20, CDX-2, MUC-2, and MUC-5AC, and were variant positive for CK-7.

Conclusions

PMP arising from urachus comes from neoplastic cells with development of intestinal-type mucinous neoplasm. It shares a similar pathophysiology as PMP from appendix. CRS including total urethrectomy, partial cystectomy, and peritonectomy plus HIPEC can be considered as a new option of treatment for PMP originating from urachus.

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DISCLOSURE

Yang Liu, Haruaki Ishibashi, Masamitsu Hirano, Kazuyoshi Takeshita, Akiyoshi Mizumoto, Masumi Ichinose, Eisei Nishino, Ippei Kashu, Yoshihiro Yamamoto, Paul H. Sugarbaker, Yutaka Yonemura there is no conflict of interest to disclose.

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Authors and Affiliations

  1. NPO to Support Peritoneal Surface Malignancy Treatment, Osaka, Japan

    Yang Liu MD, Haruaki Ishibashi MD, PhD, Kazuyoshi Takeshita MD, PhD & Yutaka Yonemura MD, PhD

  2. Peritoneal Dissemination Center, Kishiwada Tokushukai Hospital, Kishiwada, Japan

    Yang Liu MD, Haruaki Ishibashi MD, PhD, Kazuyoshi Takeshita MD, PhD & Yutaka Yonemura MD, PhD

  3. Department of Surgery, Kusatsu General Hospital, Shiga, Japan

    Masamitsu Hirano MD, PhD, Akiyoshi Mizumoto MD, PhD, Masumi Ichinose MD, PhD & Yutaka Yonemura MD, PhD

  4. Department of Pathology, Kishiwada Tokushukai Hospital, Kishiwada, Japan

    Eisei Nishino MD, PhD

  5. Department of Pathology, Kusatsu General Hospital, Shiga, Japan

    Ippei Kashu MD, PhD & Yoshihiro Yamamoto MD, PhD

  6. Center for Gastrointestinal Malignancies, Program in Peritoneal Surface Oncology, MedStar Washington Hospital Center, Washington, DC, USA

    Paul H. Sugarbaker MD, FACS, FRCS

  7. Grand Court Room 903, Kyoto, Japan

    Yutaka Yonemura MD, PhD

Authors
  1. Yang Liu MD
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  2. Haruaki Ishibashi MD, PhD
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  3. Masamitsu Hirano MD, PhD
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  4. Kazuyoshi Takeshita MD, PhD
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  5. Akiyoshi Mizumoto MD, PhD
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  6. Masumi Ichinose MD, PhD
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  7. Eisei Nishino MD, PhD
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  8. Ippei Kashu MD, PhD
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  9. Yoshihiro Yamamoto MD, PhD
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  10. Paul H. Sugarbaker MD, FACS, FRCS
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  11. Yutaka Yonemura MD, PhD
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Corresponding author

Correspondence to Yutaka Yonemura MD, PhD.

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Supplementary Fig. 1

immunohistochemical staining results of mucinous tumor in patients with pseudomyxoma peritoneifrom urachus. (a) CDX2, (b) CK7, (c) CK20, (d) MUC2, (e) MUC5AC, (f) Ki-67 (TIFF 4658 kb)

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Liu, Y., Ishibashi, H., Hirano, M. et al. Cytoreductive Surgery Plus Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei Arising from Urachus. Ann Surg Oncol 22, 2799–2805 (2015). https://doi.org/10.1245/s10434-014-4336-8

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  • DOI: https://doi.org/10.1245/s10434-014-4336-8

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