Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour

We report a case of a 51-year-old woman with neurofibromatosis who presented in 2012 with postmenopausal bleeding. Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvec...

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Veröffentlicht in:BMJ case reports 2019-01, Vol.12 (1), p.e224744
Hauptverfasser: Linehan, Anna, Harrold, Emily, Pilson, Keith, McCaffrey, John
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Harrold, Emily
Pilson, Keith
McCaffrey, John
description We report a case of a 51-year-old woman with neurofibromatosis who presented in 2012 with postmenopausal bleeding. Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvectomy and adjuvant interferon. Local recurrence in January 2017 was further resected. Positron emission tomography (PET)-CT in May 2017 identified an FDG avid omental deposit; consistent histologically with MM when resected. Postoperative PET-CT in August 2017 demonstrated local recurrence. In the setting of resected stage IV disease and a third local recurrence, the decision was made to instigate immunotherapy. Vulvar melanoma is rare accounting for 0.2% of all melanoma. Presentation is typically a decade later than cutaneous melanoma with a tendency to late metastases and poorer prognosis. Given their rarity the treatment paradigm is less clearly defined and largely extrapolated from that of cutaneous melanomas.
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Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvectomy and adjuvant interferon. Local recurrence in January 2017 was further resected. Positron emission tomography (PET)-CT in May 2017 identified an FDG avid omental deposit; consistent histologically with MM when resected. Postoperative PET-CT in August 2017 demonstrated local recurrence. In the setting of resected stage IV disease and a third local recurrence, the decision was made to instigate immunotherapy. Vulvar melanoma is rare accounting for 0.2% of all melanoma. Presentation is typically a decade later than cutaneous melanoma with a tendency to late metastases and poorer prognosis. 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source MEDLINE; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; PubMed Central
subjects Biopsy
Case reports
Colorectal cancer
Dermatology
Female
Gastrointestinal cancer
Gastrointestinal Stromal Tumors - diagnostic imaging
Gastrointestinal Stromal Tumors - drug therapy
Genetic disorders
Humans
Immunotherapy
Medical imaging
Melanoma
Melanoma - diagnostic imaging
Melanoma - drug therapy
Middle Aged
Mutation
Neoplasm Recurrence, Local - diagnostic imaging
Neoplasm Recurrence, Local - drug therapy
Neurofibromatoses - diagnostic imaging
Neurofibromatoses - drug therapy
Neurological disorders
Positron Emission Tomography Computed Tomography
Rare Disease
Skin cancer
Treatment Outcome
Tumors
Vulvar Neoplasms - diagnostic imaging
Vulvar Neoplasms - drug therapy
title Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour
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