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Keywords:

  • endoscopy;
  • extramammary Paget's disease;
  • mapping-biopsy;
  • staging

Abstract

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. Acknowledgments
  7. References

Abstract  We report a 65-year-old woman with extramammary Paget's disease which recurred on the skin flap. We performed a mapping-biopsy of the urethra by ureteroscopy to select the appropriate treatment. This method is presumed to be useful for determining the margin, deciding the correct staging for extramammary Paget's disease and for making a judgement on appropriate therapy.


Introduction

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. Acknowledgments
  7. References

Extramammary Paget's disease is an uncommon intraepithelial carcinoma of the skin with mucosa usually occurring in the anogenital area and is frequently associated with subjacent or regionally proximate carcinoma. Treatment is often unsuccessful and local recurrences after surgery are common. One of the reasons extramammary Paget's disease has a high recurrence rate is the difference in the margin histologically and superficially. Extramammary Paget's disease of the vulva, as a histological disease, extends well beyond clinical margins in many cases. Therefore, it is difficult to determine the margin of an excision. Skin mapping biopsy in the present case was performed by the conventional method suggested by Pitman et al.1 However, there have been no detailed reports on biopsy and treatment of the urethra. We present a biopsy method using an endoscopic technique.

Case report

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. Acknowledgments
  7. References

A 65-year-old woman with recurrent extramammary Paget's disease after a simple vulvectomy was referred to the Department of Plastic and Reconstructive Surgery, Nippon Medical School Hospital, Tokyo, Japan by another hospital. An urogenital survey showed no other malignancy. A radical wide excision was made after performing the mapping biopsy. In the urethra, the margin was substituted for the external urethral meatus and no tumor cells were identified by biopsy in that portion. There were no tumor cells at the margin of the specimen. The defect was reconstructed with a rectus abdominal flap. Eight years after the first operation, a recurrence of the disease was found on the reconstructed flap. After further examination and skin mapping biopsy (Fig. 1), we performed an endoscopic biopsy. The mapping biopsy of the urethra was carried out by endoscopy or cystoscopy, depending on the diameter of the urethra. In the present case, because the urethra was narrow, ureteroscopy was used in the same manner as urethral biopsy and it is important to check the precise distance from the external urethral meatus. The biopsy was performed at 0, 3, 6 and 9 o’clock-positions at 1, 2, 3 and 4 cm from the external urethral meatus. A total of 17 biopsies were performed including one from the bladder (Fig. 2).

image

Figure 1. Skin mapping biopsy showing the erythema of the skin around the opening of the urethra.

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image

Figure 2. The mapping biopsy with ureter endoscopy. ⋆, mapping positions; ⋆, positive positions (five positions).

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In the present case, the external urethral meatus was not the same as the original opening because during the primary operation the external urethral meatus was resected 1 cm inward. Pathologically, endoscopic biopsy revealed tumor cells at one of the 1 cm positions (3 o’clock) and at four of the 4 cm positions. There were no tumor cells at any of the other positions. The specimen was tiny but it could be diagnosed. It showed Paget's cells in the mucous membrane and submucous invasion (Figs 3,4). Paget's cells stain periodic acid-Schiff (PAS) and carcinoembryonic antigen (CEA). Regarding immunohistochemistry, cytokeratin (CK) 7 was positive, CK 20 was negative and thrombomodulin was negative. This immunohistochemical profile showed that the condition was not urothelial pagetoid carcinoma in situ, but extramammary Paget's disease.2

image

Figure 3. The specimen is tiny, but diagnosable.

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image

Figure 4. Paget's cells were in and beneath the mucous membrane.

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We changed the treatment plan to chemotherapy and radiotherapy from surgery.

Discussion

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. Acknowledgments
  7. References

A surgical resection should be monitored by precise histological examinations to achieve an adequate excision without the unnecessary sacrifice of normal tissue. Complete excision and preservation of the largest possible area of normal tissue is the goal when treating extramammary Paget's disease. Therefore, an appropriate determination of the margin is very important and is linked to the correct staging decision.

Multiple perimeter biopsies 3 cm from the clinical disease site were recommended several days before surgery.1 This method is a good precept for surgery. The Japanese reference General Rules for Clinical and Pathological Studies on Malignant Neoplasms of the Skin presents the mapping biopsy.3 It is easy to perform the mapping biopsy for the skin, but very difficult to perform it for the mucous membrane. Therefore, one portion of the external urinary meatus, 3 cm from clinical margin, or the frozen sections during surgery, should be used as the margin of the mucous menbrane.4,5 The external urethral meatus is not an appropriate substitute. One portion is not sufficient because the urethra is a cylinder and at 3 cm is relatively long (the average length of the urethra of a woman is 4 cm), and the quality of frozen sections is low, sometimes producing false-negative results.1,6 In these respects our method is superior to the conventional method. More case experience with this method may contribute to the correct staging of primary extramammary Paget's disease of the vulva and the proper choice of treatment.

In summary, we experienced recurring extramammary Paget's disease on the urethra. In these cases, surgical treatment requires carefully planning because surgery related to the urinary tract is directly connected to quality of life. In the present case, intensive endoscopic biopsy of the urethra and the bladder mucosa was effective in diagnosing the subclinical progression of extramammary Paget's Disease of the vulva.

Acknowledgments

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. Acknowledgments
  7. References

We thank Kouichi Tanaka (Assistant Professor, Pathology, Nippon Medical School Hospital), Mikako Aoki (Assistant Professor, Dermatology, Nippon Medical School Hospital) and Mitsuhiro Sato (Lecturer, Urology, Nippon Medical School Hospital).

References

  1. Top of page
  2. Abstract
  3. Introduction
  4. Case report
  5. Discussion
  6. Acknowledgments
  7. References
  • 1
    Pitman GH, McCarthy JG, Perzin KH, Herter FP. Extramammary Paget's diease. Plast. Reconstr. Surg. 1982; 69: 238.
  • 2
    Lopez-Beltran A, Luque RJ, Moreno A, Bollito E, Carmona E, Montironi R. The pagetoid variant of bladder urothelial carcinoma in situ. Virchows Arch. 2002; 441: 14853.
  • 3
    The Japanese Skin Cancer Society. General Rules for Clinical and Pathological Studies on Malignant Neoplasms of the Skin, 1st edn. Kanehara, Tokyo, 2002; 58.
  • 4
    Ohara K, Onishi Y, Kawabata Y. The diagnosis and treatment of extramammary Paget's disease. Skin Cancer 1993; 8: 3960.
  • 5
    Orita T, Yamada H, Morikawa K, Maeda A, Tezuka T. Four cases of the extramammary Paget's disease in which surgical treatment was considered with mapping biopsy. Skin Cancer. 1994; 9: 106111.
  • 6
    Coldiron BM, Goldsmith BA, Robinson JK. Surgical treatment of extramammary Paget's disease. A report of six cases and a reexamination of Mohs micrographic surgry compared with conventional surgical excision. Cancer. 1991; 67: 9338.