CLINICAL IMAGE
Pigmented perianal macules Efstathios Rallis1 & Panagiotis Tsibouris2 1
Department of Dermatology, Veterans Administration Hospital, Athens, Greece Department of Gastroenterology, Veterans Administration Hospital, Athens, Greece
2
Correspondence Efstathios Rallis, Department of Dermatology, Veterans Administration Hospital, 11 Pafsaniou Street, Athens, Greece, 11635 Athens, Greece. Tel/Fax: +30 210 7244008; E-mail: [email protected] Funding Information No sources of funding were declared for this study. Received: 26 April 2015; Revised: 5 August 2015; Accepted: 10 September 2015
Key Clinical Message Primary mucosal melanoma occurs in under 2% of melanomas. Anorectal melanoma is a rare disorder, approximately accounting for 1% of all anorectal carcinomas. Primary anorectal melanoma presents predominantly in women, in the 4th–6th decade of life. Typical clinical manifestations include rectal bleeding and tenesmus. The prognosis remains poor. Keywords Anorectal, melanoma, metastasis.
Clinical Case Reports 2016; 4(1): 95–96 doi: 10.1002/ccr3.413
Photo Quiz A 64-year-old woman presented with 6–8 sharply demarcated, darkly pigmented macules around the anus (Fig. 1) and a 3-month history of intermittent rectal bleeding. The patient underwent a colonoscopy and an ulcerated 3.6 cm in diameter, polypoid, pigmented tumor of the anorectal verge was found (Fig. 2).
Question Based on the patient’s history and physical examination findings, which one of the following is the most likely diagnosis? A Peutz-Jeghers syndrome. B Genital warts. C Neurofibromatosis type 1. D Acrodermatitis enteropathica. E Metastatic anorectal melanoma.
ceeded to an abdominoperineal resection. Histopathology of the operative specimen showed a primary melanoma (HMB45 and S-100 stains positive) with a Breslow thickness of 1.4 mm. Regional lymph nodes were free from any signs of the disease. A 3 mm-punch biopsy from the hyperpigmented macules confirmed the perianal metastases of melanoma. Abdominal ultrasonography and contrast CT-scan of chest, abdomen, and pelvis showed no evidence of metastases. Primary mucosal melanoma occurs in under 2% of melanomas [1]. Anorectal malignancies are commonly adenocarcinomas. Anorectal melanoma is a rare disorder, approximately accounting for 1% of all anorectal carcino-
Discussion The correct answer is E: Metastatic anorectal melanoma. Numerous biopsies were taken from the tumor at the time of colonoscopy and pathologic results were consistent with the diagnosis of melanoma. The patient pro-
Figure 1. Dark pigmented macules around the anus.
ª 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
95
Pigmented perianal macules
E. Rallis and P. Tsibouris
noma. Primary rectal melanoma presents in the fourth decade with an increase of incidence in the fifth or sixth decade of life. It appears predominantly in women.
Conflict of Interest None declared. References
Figure 2. An ulcerated, polypoid tumor of the anorectal verge was seen at colonoscopy.
mas [2]. The anorectum is the third most common location for melanoma following cutaneous and ocular mela-
96
1. Carcoforo, P., M. T. Raiji, G. M. Palini, M. Pedriali, U. Maestroni, G. Soliani, et al. 2012. Primary anorectal melanoma: an update. J. Cancer 3:449–453. 2. van Schaik, P. M., M. F. Ernst, H. A. Meijer, and K. Bosscha. 2008. Melanoma of the rectum: a rare entity. World J. Gastroenterol. 14:1633–1635.
ª 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.
Pigmented perianal macules Efstathios Rallis1 & Panagiotis Tsibouris2 1
Department of Dermatology, Veterans Administration Hospital, Athens, Greece Department of Gastroenterology, Veterans Administration Hospital, Athens, Greece
2
Correspondence Efstathios Rallis, Department of Dermatology, Veterans Administration Hospital, 11 Pafsaniou Street, Athens, Greece, 11635 Athens, Greece. Tel/Fax: +30 210 7244008; E-mail: [email protected] Funding Information No sources of funding were declared for this study. Received: 26 April 2015; Revised: 5 August 2015; Accepted: 10 September 2015
Key Clinical Message Primary mucosal melanoma occurs in under 2% of melanomas. Anorectal melanoma is a rare disorder, approximately accounting for 1% of all anorectal carcinomas. Primary anorectal melanoma presents predominantly in women, in the 4th–6th decade of life. Typical clinical manifestations include rectal bleeding and tenesmus. The prognosis remains poor. Keywords Anorectal, melanoma, metastasis.
Clinical Case Reports 2016; 4(1): 95–96 doi: 10.1002/ccr3.413
Photo Quiz A 64-year-old woman presented with 6–8 sharply demarcated, darkly pigmented macules around the anus (Fig. 1) and a 3-month history of intermittent rectal bleeding. The patient underwent a colonoscopy and an ulcerated 3.6 cm in diameter, polypoid, pigmented tumor of the anorectal verge was found (Fig. 2).
Question Based on the patient’s history and physical examination findings, which one of the following is the most likely diagnosis? A Peutz-Jeghers syndrome. B Genital warts. C Neurofibromatosis type 1. D Acrodermatitis enteropathica. E Metastatic anorectal melanoma.
ceeded to an abdominoperineal resection. Histopathology of the operative specimen showed a primary melanoma (HMB45 and S-100 stains positive) with a Breslow thickness of 1.4 mm. Regional lymph nodes were free from any signs of the disease. A 3 mm-punch biopsy from the hyperpigmented macules confirmed the perianal metastases of melanoma. Abdominal ultrasonography and contrast CT-scan of chest, abdomen, and pelvis showed no evidence of metastases. Primary mucosal melanoma occurs in under 2% of melanomas [1]. Anorectal malignancies are commonly adenocarcinomas. Anorectal melanoma is a rare disorder, approximately accounting for 1% of all anorectal carcino-
Discussion The correct answer is E: Metastatic anorectal melanoma. Numerous biopsies were taken from the tumor at the time of colonoscopy and pathologic results were consistent with the diagnosis of melanoma. The patient pro-
Figure 1. Dark pigmented macules around the anus.
ª 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
95
Pigmented perianal macules
E. Rallis and P. Tsibouris
noma. Primary rectal melanoma presents in the fourth decade with an increase of incidence in the fifth or sixth decade of life. It appears predominantly in women.
Conflict of Interest None declared. References
Figure 2. An ulcerated, polypoid tumor of the anorectal verge was seen at colonoscopy.
mas [2]. The anorectum is the third most common location for melanoma following cutaneous and ocular mela-
96
1. Carcoforo, P., M. T. Raiji, G. M. Palini, M. Pedriali, U. Maestroni, G. Soliani, et al. 2012. Primary anorectal melanoma: an update. J. Cancer 3:449–453. 2. van Schaik, P. M., M. F. Ernst, H. A. Meijer, and K. Bosscha. 2008. Melanoma of the rectum: a rare entity. World J. Gastroenterol. 14:1633–1635.
ª 2015 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.
