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Dermatologic Surgery : Official... Jun 2017Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous neoplasm with a low propensity for metastasis and a high rate of local recurrence. It... (Review)
Review
BACKGROUND
Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous neoplasm with a low propensity for metastasis and a high rate of local recurrence. It typically presents as a dermal plaque or nodule on the trunk, limbs, or head and neck region. Vulvar DFSP has also been described, although it is less common.
OBJECTIVE
To review the available literature and discuss the clinical course of DFSP affecting the vulva.
MATERIALS AND METHODS
We reviewed the existing English-language literature on DFSP of the vulva with respect to clinical presentation, diagnosis, treatment, and outcome.
RESULTS
Thirty three case reports and series were included (n = 54 patients). Vulvar DFSP most commonly presents as a slowly enlarging tender or asymptomatic mass on the labia majora, with histological findings of classic DFSP. Most patients were treated with wide local excision. Three patients were treated with Mohs micrographic surgery, which may decrease local recurrence and seems well suited for use in vulvar DFSP.
CONCLUSION
This literature review comprehensively reviews and describes the clinical presentation of vulvar DFSP and the treatment options for this rare vulvar neoplasm.
Topics: Dermatofibrosarcoma; Female; Humans; Skin Neoplasms; Vulvar Neoplasms
PubMed: 28323651
DOI: 10.1097/DSS.0000000000001113 -
Journal of the Egyptian National Cancer... Jun 2017
Topics: Female; Humans; Incidence; Neoplasm Grading; Neoplasm Staging; Rare Diseases; Vulvar Neoplasms
PubMed: 28462851
DOI: 10.1016/j.jnci.2017.04.002 -
Revista Brasileira de Ginecologia E... Jun 2018Hemangioma is a benign neoplasm that may affect the vulva, and it can cause functional or emotional disability. This article reports the case of a 52-year-old female...
Hemangioma is a benign neoplasm that may affect the vulva, and it can cause functional or emotional disability. This article reports the case of a 52-year-old female patient with a history of a genital ulcer for the past 3 years and who had undergone various treatments with creams and ointments. The patient was biopsied and diagnosed with vulvar hemangioma and was subsequently submitted to surgical excision of the lesion. We emphasize the importance of following the steps of the differential diagnosis and proceeding with a surgical approach only if necessary.
Topics: Female; Hemangioma; Humans; Middle Aged; Vulvar Neoplasms
PubMed: 29980161
DOI: 10.1055/s-0038-1657786 -
Journal of Translational Medicine Apr 2023A causal link between microbiota composition (dysbiosis) and oncogenesis has been demonstrated for several types of cancer. Neutrophils play a role in both immune...
BACKGROUND
A causal link between microbiota composition (dysbiosis) and oncogenesis has been demonstrated for several types of cancer. Neutrophils play a role in both immune protection against bacterial threats and carcinogenesis. This study aimed to characterise intratumoral bacteria in vulvar squamous cell carcinoma (VSCC) and their putative effect on neutrophil recruitment and cancer progression.
METHODS
Clinical material was obtained from 89 patients with VSCC. Next-generation sequencing (NGS) of 16S rRNA and quantitative polymerase chain reaction (qPCR) were used to detect bacterial species in VSCC. To verify neutrophil activation, CD66b expression in tumour specimens was analysed by immunohistochemistry (IHC). Subsequently, IHC was applied to detect the main neutrophil serine proteases (NSPs), cathepsin G (CTSG), neutrophil elastase (ELANE), and proteinase 3 (PRTN3) in VSCC.
RESULTS
Fusobacterium nucleatum and Pseudomonas aeruginosa were identified as tumour-promoting bacteria, and their presence was found to be associated with a shorter time to progression in VSCC patients. Furthermore, high abundance of CD66b, the neutrophil activation marker, in VSCC samples, was found to relate to poor survival of patients with VSCC. The selected NSPs were shown to be expressed in vulvar tumours, also within microabscess. The increased numbers of microabscesess were correlated with poor survival in VSCC patients.
CONCLUSIONS
Our results show that neutrophilic inflammation seem to be permissive for tumour-promoting bacteria growth in VSCC. The findings provide new therapeutic opportunities, such as based on shifting the balance of neutrophil populations to those with antitumorigenic activity and on targeting NSPs produced by activated neutrophils at the inflammation sites.
Topics: Female; Humans; Vulvar Neoplasms; RNA, Ribosomal, 16S; Carcinoma, Squamous Cell; Inflammation; Epithelial Cells; Tumor Microenvironment
PubMed: 37118737
DOI: 10.1186/s12967-023-04113-7 -
Journal of Cutaneous Pathology Oct 2020Malignant tumor of the vulva is the fourth gynecological malignancy in frequency. Close to 70% of all vulvar malignancies are related to high-risk human papillomavirus... (Review)
Review
BACKGROUND
Malignant tumor of the vulva is the fourth gynecological malignancy in frequency. Close to 70% of all vulvar malignancies are related to high-risk human papillomavirus (HPV) infection.
METHODS
A search for non-HPV-related malignant tumors of the vulva was performed in the last 20 years (2000-2020) in the pathology database of a single tertiary institution. We aim to estimate the prevalence of non-HPV-related malignancies in our population, describe clinicopathological features of these tumors and investigate the expression of some potential therapeutic targets.
RESULTS
A total of 71 patients were recovered; 26 patients (36%) had the diagnosis of extramammary Paget disease, 17 patients (24%) had basal cell carcinomas, 17 patients (24%) had primary melanomas, 10 patients (14%) had metastatic disease to the vulva and one patient (1%) had a primary dermatofibrosarcoma protuberans. Fifty-four percent of patients with extramammary Paget disease had a secondary malignancy and 12.5% had invasive disease. Programmed death-ligand 1 (PDL-1) was positive in seven out of nine primary melanomas and Her2/neu was overexpressed in six out of seven extramammary Paget disease.
CONCLUSION
Non-HPV-related malignancies are important differential diagnoses in patient with vulvar lesions. Additional research is necessary to further understand these complex malignancies and potential new therapeutic targets.
Topics: Adult; Aged; Aged, 80 and over; B7-H1 Antigen; Carcinoma, Basal Cell; Databases, Factual; Dermatofibrosarcoma; Diagnosis, Differential; Female; Humans; Melanoma; Middle Aged; Neoplasm Invasiveness; Neoplasm Metastasis; Neoplasms; Neoplasms, Second Primary; Paget Disease, Extramammary; Papillomavirus Infections; Prevalence; Receptor, ErbB-2; Retrospective Studies; Skin Neoplasms; Vulvar Neoplasms
PubMed: 32511773
DOI: 10.1111/cup.13768 -
Ugeskrift For Laeger Aug 2019This review summarises the current guidelines for vulva cancer in Denmark. Vulva cancer is a rare disease. The diagnosis is often delayed, which results in large tumours... (Review)
Review
This review summarises the current guidelines for vulva cancer in Denmark. Vulva cancer is a rare disease. The diagnosis is often delayed, which results in large tumours and regional spread. The most important prognostic factor is inguinal lymph node metastases. Staging and treatment is centralised to two hospitals. Primary treatment is wide local excision combined with removal of either inguinal sentinel nodes or lymphadenectomy. Treatment is associated with considerable morbidity, and supportive care is often necessary. Local curable recurrences are common. Relapses in the groin are associated with a poor prognosis. Thus, long term follow-up is essential. *) On behalf of Dansk Gynækologisk Cancer Gruppe for vulvacancer.
Topics: Denmark; Female; Follow-Up Studies; Humans; Lymph Node Excision; Lymph Nodes; Lymphatic Metastasis; Neoplasm Recurrence, Local; Neoplasm Staging; Prognosis; Sentinel Lymph Node Biopsy; Vulvar Neoplasms
PubMed: 31495360
DOI: No ID Found -
Archives of Pathology & Laboratory... Nov 2014Phyllodes tumors of the vulva are rare proliferations that share morphologic similarities with breast neoplasms. Their histogenetic origin is elusive and may be... (Review)
Review
Phyllodes tumors of the vulva are rare proliferations that share morphologic similarities with breast neoplasms. Their histogenetic origin is elusive and may be associated with specialized mammary-like glands of the vulva. Because of their rarity, the clinical and pathologic features, classification, and therapy are not well defined, and their biologic behavior is difficult to predict by histology alone. Immunohistochemical expression of estrogen and progesterone receptors and breast markers provide further support for a common origin. Surgical resection is the current mainstay of therapy and is definitive in most cases.
Topics: Diagnosis, Differential; Female; Humans; Immunohistochemistry; Phyllodes Tumor; Prognosis; Vulvar Neoplasms
PubMed: 25357118
DOI: 10.5858/arpa.2013-0581-RS -
Ethiopian Journal of Health Sciences Jan 2022Lymphangioma circumscriptum is a rare benign disorder of lymphatic channels in deep dermal and subcutaneous layers. It can occur either as a congenital abnormality or as...
Lymphangioma circumscriptum is a rare benign disorder of lymphatic channels in deep dermal and subcutaneous layers. It can occur either as a congenital abnormality or as acquired damage to previously normal lymphatic channels. It occurs in different parts of the body, and the vulva is one of the parts which is commonly affected. Here, we presented a 39 years old para 2 who presented with vulvar swelling. She was diagnosed with acquired lymphangioma circumscriptum of the vulva and superficial vulvectomy was done.
Topics: Adult; Edema; Female; Humans; Lymphangioma; Vulva; Vulvar Neoplasms
PubMed: 35250235
DOI: 10.4314/ejhs.v32i1.25 -
Journal of Pediatric and Adolescent... Jun 2022Leiomyoma of the vulva is rare, accounting for only 0.03% of all gynecological tumors, and it is seldom seen in teenagers.
BACKGROUND
Leiomyoma of the vulva is rare, accounting for only 0.03% of all gynecological tumors, and it is seldom seen in teenagers.
CASE
We describe a case of vulvar leiomyoma in a 14-year-old girl who presented a 10 × 10 cm solid tumor in the right hemivulva without other complaints.
SUMMARY AND CONCLUSION
Differential diagnosis includes Bartholin cysts, abscesses, fibromas, and other solid lesions. Although rare, vaginal leiomyoma must be remembered and included as a differential diagnosis for solid lesions in the vagina.
Topics: Adolescent; Bartholin's Glands; Diagnosis, Differential; Female; Humans; Leiomyoma; Vaginal Neoplasms; Vulva; Vulvar Neoplasms
PubMed: 34767968
DOI: 10.1016/j.jpag.2021.10.020 -
Pediatric Dermatology Sep 2022
Topics: Female; Humans; Infant; Vulva; Vulvar Neoplasms
PubMed: 36206215
DOI: 10.1111/pde.15010