Extramammary Paget's Disease in Perineo-inguinoscrotal Region

Authors

  • Gérson Junqueira Júnior Hospital Santa Rita (HSR). Porto Alegre (RS). Brasil.
  • Renato Luiz Amaral
  • Julio Walter Vedovato Fundação Faculdade Federal de Ciências Médicas de Porto Alegre (FFFCMPA). Porto Alegre (RS).
  • Olavo Machado Torres Fundação Faculdade Federal de Ciências Médicas de Porto Alegre (FFFCMPA). Porto Alegre (RS).
  • Wellington Gemelli dos Santos Hospital Santa Rita (HSR). Porto Alegre (RS). Brasil.
  • Adriano de Lima e Silva Hospital de Clinicas de Porto Alegre (HCPA). Porto Alegre (RS). Brasil.
  • André Cartell Universidade Federal do Rio Grande do Sul (UFRGS). Porto Alegre (RS). Brasil.

DOI:

https://doi.org/10.32635/2176-9745.RBC.1999v45n3.2784

Keywords:

Paget’s Disease, Extramammary, Skin, Malignant Neoplasms

Abstract

Extramammary Paget’s Disease is a rare disease. There is a dose correlation with anorrectal carcinoma when the disease is perianal. It is also associated with internal malignancies and bad prognosis. The authors describe a case of extramammary Paget’s Disease in perineo-inguinoscrotal region of a 59-year-old-white man, who had a hyperpigmented and pruritic lesion of approximately 6 cm of diameter which has increased and was presenting minimum bleeding. Staging tests were normal, without evidence of metastasis. The anatomopathological test of the lesion revealed na aspect compatible with extramammary Paget’s Disease. The patient was submmited to an enlarged excision of the lesion and thin skin graft. The aspects related to epidemiology, pathology, staging, prognosis and treatment of this neoplasia are discussed in this article.

Downloads

Download data is not yet available.

References

Brash D, Safai B- Cancers of the Skin. In.: DeVita VTJr, Hellman S, Rosember SA, eds. Câncer: Principies e Practice of Oncology. 5th ed. Philadelphia. Lippincott Company, 1879-1933, 1997.

Shutze WP, Gleysteen JJ. - Perianal Paget’s disease. Classification and review of man agement: Report of two cases. Dis Colon Rectum, 33(6): 502-507, 1990. DOI: https://doi.org/10.1007/BF02052147

Crum CP - Female Genital Tract. In.: Cotran RS, Kumar V, Robbins SL, eds. Pathologic Basis of Disease. 5th ed. Philadelphia. WB Saunders Company, 1033-1087, 1994.

Snow SN, Desouky S, Lo JS, Kurtycz D. Failure to detect human papillomavirus in extramammary Paget’s disease. Câncer, 69(1): 249-251, 1992. DOI: https://doi.org/10.1002/1097-0142(19920101)69:1<249::AID-CNCR2820690140>3.0.CO;2-O

Umemoto S, Inoue S, Amemiya T, et al. - A case of perianal Paget’s disease associated with a sigmoid colon carcinoma. Gastroenterol Jpn, 28(5): 719-724, 1993. DOI: https://doi.org/10.1007/BF02806354

Sleater JP, Ford MJ, Beers BB. - Extramammary Paget’s disease associated with prostate adenocarcinoma. Hum Pathol, 25(6): 615-617, 1994. DOI: https://doi.org/10.1016/0046-8177(94)90229-1

Smith DJ, Handy FC, Evans JW, Falzon M, Chapple CR. - Paget’s disease of the glans penis: an unusual urological malignancy. EurUrol, 25(4): 316-319, 1994. DOI: https://doi.org/10.1159/000475309

Ikoma A, Kubo H, Ohno T, Tanaka K, Yoshida A, Taira A. - Perianal extra mammary Paget’s disease associated with primary linits plastica of the rectum: report of a case. Surg Today, 26(9): 711-714,1996. DOI: https://doi.org/10.1007/BF00312090

Besa P, Rich TA, Delclos L, Edwards CL, Ota DM, Wharton JT. – Extramammary Paget’s disease of perineal skin: role of radioterapy. Int J Radiot Oncol Biol Phys, 24(1): 73-78, 1992. DOI: https://doi.org/10.1016/0360-3016(92)91024-H

Burrows NP, Jones Dh, Hudson PM, Pye RJ. - Treatment of extramammary Paget’s disease by radioterapy. Br J Dermatol, 132(6): 970-972, 1995. DOI: https://doi.org/10.1111/j.1365-2133.1995.tb16957.x

Voigt H, Bassermann R, Nathrath W. - Cytoreductive combination chemotherapy for regionally advanced unresectable extramammary Paget’s carcinoma. Câncer, 70(3): 704-708, 1992. DOI: https://doi.org/10.1002/1097-0142(19920801)70:3<704::AID-CNCR2820700327>3.0.CO;2-5

Yamazaki N, Yamamoto A, Wada T, Ishikawa M, Moriya Y, Nakanishi Y. A case of metastatic extramammary Paget disease that responded combination chemotherapy. J Dermatol, 26(5):311-6, 1999. DOI: https://doi.org/10.1111/j.1346-8138.1999.tb03477.x

Iwamura H, Horii Y, Tokuchi H, Arai E. A case of genital paget’s disease with severe dermal invasion and early dissemination. Hinnyokika Kiyo, 45(4):281-4, 1999.

Coldiron BM, Goldsmith BA, Robinson JK. - Surgical treatment of extramammary Paget’s disease. A report of six cases and a reexamination of Mohs Micrographic surgery compared with conventional surgical excision. Câncer, 67(4): 933-938, 1991. DOI: https://doi.org/10.1002/1097-0142(19910215)67:4<933::AID-CNCR2820670413>3.0.CO;2-3

Harris DW, Kist DA, Bloom K, Zachary CB. - Rapid staining with carcinoembryonic antign aids limited excision of extramammary Paget’s disease treated by Mohs surgery. J Dermatol Surg Oncol, 20(4): 260-264, 1994. DOI: https://doi.org/10.1111/j.1524-4725.1994.tb01622.x

Barlow RJ, Ramnarain N, Smith N, Mayou B, Markey AC, Walker NP. - Excision of selected skin tumours using Moh’s micrographic surgery with horizontal paraffin-embedded sections. Br J Dermatol, 135(6): 911-917, 1996. DOI: https://doi.org/10.1046/j.1365-2133.1996.d01-1094.x

Petrelli NJ, Cebollero JA, Rodriguez-Bigas M, Mang T. - Photodynamic therapy in the management of neoplasms of the perineal skin.ArchSurg, 127(12): 1436-1438,1992. DOI: https://doi.org/10.1001/archsurg.1992.01420120070013

Published

2022-09-21

How to Cite

1.
Junqueira Júnior G, Amaral RL, Vedovato JW, Torres OM, Santos WG dos, Silva A de L e, Cartell A. Extramammary Paget’s Disease in Perineo-inguinoscrotal Region. Rev. Bras. Cancerol. [Internet]. 2022 Sep. 21 [cited 2024 Jul. 3];45(3):51-5. Available from: https://rbc.inca.gov.br/index.php/revista/article/view/2784

Issue

Section

ORIGINAL ARTICLE