Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review

Detalhes bibliográficos
Autor(a) principal: Rocha, Ana Carolina
Data de Publicação: 2022
Outros Autores: Sá, Maria Inês, Abrantes, Carlos, Sousa, Rita
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551
Resumo: Sebaceous carcinoma of the vulva is a rare malignancy of the sebaceous glands, with potentially aggressive behaviour, that is usually found in the peri-ocular area. Nonetheless, there are sebaceous glands in the vulva and this diagnosis is especially rare, with only ten cases described in the literature. We report a case of 78-year-old female patient who presented with vulvar pruritus, previously treated with topical steroid and antifungal treatments, without improvement. The vulvar examination showed a visible yellow papule, 12 x 10 mm on the right major labia, which was biopsied and the microscopic examination revealed an invasive sebaceous carcinoma of the vulva, with an in situ component. We performed an uneventful excisional biopsy, followed by a subsequent margin widening. Three months after the diagnosis, she presented with the first recurrence. Two and half years after the diagnosis, she recurred with a larger lesion (13 mm) in the upper half of small right lip, more than 10 mm away from the midline. In a multidisciplinary meeting it was decided that the patient should undergo partial right vulvectomy with homolateral inguino-femoral sentinel node biopsy (one negative node). There was no evidence of recurrence one-year post-surgery.
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spelling Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature ReviewCarcinoma Sebáceo da Vulva: Um Diagnóstico Inesperado e Revisão da LiteraturaSebaceous Gland NeoplasmsVulvaVulvar NeoplasmsNeoplasias das Glândulas SebáceasNeoplasias VulvaresVulvaSebaceous carcinoma of the vulva is a rare malignancy of the sebaceous glands, with potentially aggressive behaviour, that is usually found in the peri-ocular area. Nonetheless, there are sebaceous glands in the vulva and this diagnosis is especially rare, with only ten cases described in the literature. We report a case of 78-year-old female patient who presented with vulvar pruritus, previously treated with topical steroid and antifungal treatments, without improvement. The vulvar examination showed a visible yellow papule, 12 x 10 mm on the right major labia, which was biopsied and the microscopic examination revealed an invasive sebaceous carcinoma of the vulva, with an in situ component. We performed an uneventful excisional biopsy, followed by a subsequent margin widening. Three months after the diagnosis, she presented with the first recurrence. Two and half years after the diagnosis, she recurred with a larger lesion (13 mm) in the upper half of small right lip, more than 10 mm away from the midline. In a multidisciplinary meeting it was decided that the patient should undergo partial right vulvectomy with homolateral inguino-femoral sentinel node biopsy (one negative node). There was no evidence of recurrence one-year post-surgery.O carcinoma sebáceo da vulva é uma neoplasia maligna rara das glândulas sebáceas com comportamento potencialmente agressivo, mais comummente encontrada na área periocular. Este diagnóstico é particularmente raro nas glândulas sebáceas da vulva, com apenas dez casos previamente descritos na literatura. Relatamos o caso de uma paciente de 78 anos que se apresentou com prurido vulvar, anteriormente tratado com corticóides tópicos e antifúngicos, sem melhoria evidente. Ao exame objetivo apresentava uma pápula amarela visível, com cerca de 12 x 10 mm no grande lábio direito, que foi biopsada. O exame microscópico revelou um carcinoma sebáceo invasivo da vulva, com componente in situ. Posteriormente, foi realizada uma biópsia excisional, com alargamento de margens, sem intercorrências. Três meses após o diagnóstico, surgiu a primeira recidiva, pelo que foi realizada uma nova biópsia excisional. Dois anos e meio após o diagnóstico, a doente apresentava uma lesão com 13 mm, na metade superior do pequeno lábio direito, mais de 10 mm afastada da linha mediana. Em reunião multidisciplinar decidiu-se que deveria ser submetida a uma vulvectomia parcial direita, com pesquisa de gânglio sentinela inguino-femoral homolateral (um gânglio negativo). Um ano após a cirurgia, a doente encontra-se sem evidência de recorrência.Ordem dos Médicos2022-01-03info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfimage/jpegapplication/pdfimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegimage/jpegapplication/pdfapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551oai:ojs.www.actamedicaportuguesa.com:article/13551Acta Médica Portuguesa; Vol. 35 No. 1 (2022): January; 63-67Acta Médica Portuguesa; Vol. 35 N.º 1 (2022): Janeiro; 63-671646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/6499https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12293https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12324https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12380https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12381https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12382https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12383https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12384https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12385https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12386https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12387https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12388https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12389https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12390https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12391https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12392https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12393https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12394https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12395https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12396https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12397https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12398https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12399https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12400https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12401https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12402https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/12403https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/13047https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/13551/14142Direitos de Autor (c) 2022 Acta Médica Portuguesainfo:eu-repo/semantics/openAccessRocha, Ana CarolinaSá, Maria InêsAbrantes, CarlosSousa, Rita2022-12-20T11:06:55Zoai:ojs.www.actamedicaportuguesa.com:article/13551Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:20:22.029121Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review
Carcinoma Sebáceo da Vulva: Um Diagnóstico Inesperado e Revisão da Literatura
title Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review
spellingShingle Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review
Rocha, Ana Carolina
Sebaceous Gland Neoplasms
Vulva
Vulvar Neoplasms
Neoplasias das Glândulas Sebáceas
Neoplasias Vulvares
Vulva
title_short Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review
title_full Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review
title_fullStr Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review
title_full_unstemmed Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review
title_sort Sebaceous Carcinoma of the Vulva: An Unexpected Diagnosis and Literature Review
author Rocha, Ana Carolina
author_facet Rocha, Ana Carolina
Sá, Maria Inês
Abrantes, Carlos
Sousa, Rita
author_role author
author2 Sá, Maria Inês
Abrantes, Carlos
Sousa, Rita
author2_role author
author
author
dc.contributor.author.fl_str_mv Rocha, Ana Carolina
Sá, Maria Inês
Abrantes, Carlos
Sousa, Rita
dc.subject.por.fl_str_mv Sebaceous Gland Neoplasms
Vulva
Vulvar Neoplasms
Neoplasias das Glândulas Sebáceas
Neoplasias Vulvares
Vulva
topic Sebaceous Gland Neoplasms
Vulva
Vulvar Neoplasms
Neoplasias das Glândulas Sebáceas
Neoplasias Vulvares
Vulva
description Sebaceous carcinoma of the vulva is a rare malignancy of the sebaceous glands, with potentially aggressive behaviour, that is usually found in the peri-ocular area. Nonetheless, there are sebaceous glands in the vulva and this diagnosis is especially rare, with only ten cases described in the literature. We report a case of 78-year-old female patient who presented with vulvar pruritus, previously treated with topical steroid and antifungal treatments, without improvement. The vulvar examination showed a visible yellow papule, 12 x 10 mm on the right major labia, which was biopsied and the microscopic examination revealed an invasive sebaceous carcinoma of the vulva, with an in situ component. We performed an uneventful excisional biopsy, followed by a subsequent margin widening. Three months after the diagnosis, she presented with the first recurrence. Two and half years after the diagnosis, she recurred with a larger lesion (13 mm) in the upper half of small right lip, more than 10 mm away from the midline. In a multidisciplinary meeting it was decided that the patient should undergo partial right vulvectomy with homolateral inguino-femoral sentinel node biopsy (one negative node). There was no evidence of recurrence one-year post-surgery.
publishDate 2022
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dc.publisher.none.fl_str_mv Ordem dos Médicos
publisher.none.fl_str_mv Ordem dos Médicos
dc.source.none.fl_str_mv Acta Médica Portuguesa; Vol. 35 No. 1 (2022): January; 63-67
Acta Médica Portuguesa; Vol. 35 N.º 1 (2022): Janeiro; 63-67
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