Vulvar Malignant Melanoma: A Narrative Review

Falcicchio, Giovanni; Vinci, Lorenzo; Cicinelli, Ettore; Loizzi, Vera; Arezzo, Francesca; Silvestris, Erica; Resta, Leonardo; Serio, Gabriella; Cazzato, Gerardo; Mastronardi, Marialuigia; Mongelli, Michele; Marinaccio, Marco; Cormio, Gennaro

doi:10.3390/cancers14215217

Cited by 5 publications

(10 citation statements)

References 97 publications

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“…Vulval melanoma is an uncommon condition with a poor prognosis that accounts for up to 10% of all primary vulval malignancies and for 1%-2% of all melanomas in females. 1,4,[19][20][21][22][23] Although rare, vulval melanoma is the second commonest form of vulval cancer after SCC of the vulva, which represents up to 90% of all vulval malignancies. 1,[19][20][21] The majority of vulval melanomas occur in postmenopausal Caucasian women with a median age at diagnosis between 56 and 68 years, but have also been reported in paediatric patients.…”

Section: Discussionmentioning

confidence: 99%

Primary vulval melanoma and genital lichen sclerosus

Mastoraki,

Kravvas,

Dear

et al. 2024

Skin Health and Disease

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BackgroundLichen sclerosus (LS) is a chronic, inflammatory skin disease with a predilection for the genitalia. Although, the association between squamous cell cancer and genital LS is well established, a link with genital melanoma has not been thoroughly explored. However, we have recently published a case series of penile melanoma where 9/11 (82%) of patients seen over a 10 year period with penile melanoma were retrospectively found to have histological and/or clinical evidence of genital LS on review.ObjectivesThe aim of this study was to illuminate further the relationship between vulval melanoma and genital LS by reviewing all the cases managed by our hospital and undertaking a literature review.MethodsWe identified all the cases with a diagnosis of vulval melanoma over a 16‐year period (2006–2022) where histology was available. The clinical notes were retrospectively reviewed, and the histological features of all cases were reassessed by two independent mutually ‘blinded’ histopathologists. We also performed a literature review of genital LS in patients with vulval melanoma.ResultsA total of 11 patients with vulval melanoma were identified for the review. Histopathological review found evidence of genital LS in seven of them (64%). Genital LS was not documented in any of the original histology reports. Clinical notes and letters were available in nine cases. The literature review identified 12 relevant studies with a total of 18 patients. Twelve cases concerned adult women, and six concerned female children.ConclusionThe presence of genital LS in as high as 64% of our vulval melanoma cases might indicate a causative relationship between genital LS and vulval melanoma. The pathogenesis of vulval melanoma remains largely unknown. Although ultraviolet radiation is an important pathogenic factor for cutaneous melanoma, it cannot be a factor in vulval melanoma. While possible mechanisms behind this association remain unclear, it is possible that chronic inflammation from genital LS leads to melanocytic distress and increased mutagenesis.

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Section: Discussionmentioning

confidence: 99%

Primary vulval melanoma and genital lichen sclerosus

Mastoraki,

Kravvas,

Dear

et al. 2024

Skin Health and Disease

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“…In the majority of GISTs, the tumor cells show diffuse and strong cytoplasmic expression of CD117. DOG‐1 is a sensitive and specific marker of GISTs and is particularly helpful in KIT‐negative or KIT‐variable GISTs 35 . It should be noted that variable expression of CD34 (~70%) and smooth muscle actin (30%) can be identified in GIST.…”

Section: Discussionmentioning

confidence: 99%

“…Desmin (2% in spindle cell, and 10% in epithelioid GIST) or keratin (1%) expression can also be seen. H‐caldesmon immunostaining cannot differentiate GISTs from smooth muscle neoplasms because 80% of GISTs are positive for this protein 35 . About 20% of duodenal GISTs can express S‐100 protein, albeit focally 36 .…”

Section: Discussionmentioning

confidence: 99%

“…DOG-1 is a sensitive and specific marker of GISTs and is particularly helpful in KIT-negative or KIT-variable GISTs. 35 It should be noted that variable expression of CD34 ($70%) and smooth muscle actin (30%) can be identified in GIST. Desmin (2% in spindle cell, and 10% in epithelioid GIST) or keratin (1%) expression can also be seen.…”

Section: Referencesmentioning

confidence: 99%

“…H-caldesmon immunostaining cannot differentiate GISTs from smooth muscle neoplasms because 80% of GISTs are positive for this protein. 35 About 20% of duodenal GISTs can express S-100 protein, albeit focally. 36 Succinate dehydrogenase (SDH) subunit B immunostains are used to identify SDH-deficient GISTs, which show loss of cytoplasmic (mitochondrial) staining, regardless of the underlying mutation (SDHA or SDHB).…”

Section: Agementioning

confidence: 99%

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Primary vulvar extragastrointestinal stromal tumor in a 77‐year‐old woman

Mohamed¹,

Hosler

Tumminello

2023

J Cutan Pathol

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Extragastrointestinal stromal tumors (EGISTs) carry the same morphological, immunohistochemical and molecular features as gastrointestinal stromal tumors (GISTs) and involve extragastrointestinal tract soft tissue. The majority of reported EGIST cases arise from intraabdominal, retroperitoneal, or pelvic soft tissue. A significant subset of such tumors originates from the gastrointestinal muscle layer, grows in an exophytic manner, then loses attachment to the gastrointestinal tract. Consequently, true EGISTs are exceedingly rare. Herein, we are reporting a case of a vulvar EGIST. A 77-year-old woman presented with a painless subcutaneous nodule on the right perineum. An excisional biopsy showed a fairly circumscribed bland spindle cell lesion in the dermis. The tumor cells were positive for CD117 and ANO1/DOG-1 and negative for smooth muscle myosin, smooth muscle actin, STAT6, low-and high-molecular-weight cytokeratins, SOX10, MART-1, CD10, S-100 protein, and estrogen and progesterone receptors. A diagnosis of EGIST was made and complete excision was recommended. Superficial/subcutaneous EGISTs are extremely rare, and it is important for dermatopathologists to be aware of this entity as it can be misdiagnosed as more common spindle cell neoplasms, both benign and malignant, including but not limited to smooth muscle neoplasms (leiomyoma/leiomyosarcoma), spindle cell melanoma, and sarcomatoid squamous cell carcinoma.

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Role of immunotherapy for lymph node positive vulvar melanoma: utilization and outcomes

Nasioudis,

Labban,

Latif

et al. 2023

Int J Gynecol Cancer

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ObjectiveTo investigate the utilization and outcomes of adjuvant immunotherapy for patients with vulvar melanoma and inguinal lymph node metastases.MethodsThe National Cancer Database was accessed and patients with vulvar melanoma diagnosed between 2004 and 2015 who did not have distant metastases, underwent inguinal lymphadenectomy, had positive lymph nodes, and at least 1 month of follow-up were identified. Administration of immunotherapy was evaluated and clinicopathological characteristics were compared. Median overall survival was compared with the log-rank test. Stratified analysis based on clinical status of lymph nodes was performed. A Cox model was constructed to evaluate survival after controlling for confounders.ResultsA total of 300 patients were identified; the rate of immunotherapy use was 25% (75 patients). Patients who received immunotherapy were younger (median 58 vs 70 years, p<0.001); however, the two groups were comparable in terms of clinical lymph node status, rate of positive tumor margins, presence of tumor ulceration, tumor size, Breslow thickness, and performance of comprehensive lymphadenectomy. There was no overall survival difference between patients who did (median 31.08 months) and did not (median 22.77 months) receive immunotherapy (p=0.18). Following stratification by clinical lymph node status, immunotherapy did not improve overall survival of patients with clinically negative (median 35.35 vs 33.22, p=0.75) or positive lymph nodes (median 23.33 vs 16.99, p=0.64). After controlling for confounders, administration of immunotherapy was not associated with better overall survival (HR 0.81, 95% CI 0.57 to 1.14).ConclusionsIn this study approximately one in four patients received adjuvant immunotherapy. Immunotherapy was not associated with improved overall survival.

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Vulvar Malignant Melanoma: A Narrative Review

Cited by 5 publications

References 97 publications

Primary vulval melanoma and genital lichen sclerosus

Primary vulval melanoma and genital lichen sclerosus

Primary vulvar extragastrointestinal stromal tumor in a 77‐year‐old woman

Role of immunotherapy for lymph node positive vulvar melanoma: utilization and outcomes

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