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Acta Chirurgica Belgica Jan 2012Aggressive angiomyxoma (AA) is a rare, slow-growing myxoid neoplasm of the pelvi-perineum. Because of its rarity, it is often initially misdiagnosed, frequently as a... (Review)
Review
Aggressive angiomyxoma (AA) is a rare, slow-growing myxoid neoplasm of the pelvi-perineum. Because of its rarity, it is often initially misdiagnosed, frequently as a gynaecological malignancy. Surgical resection is the main treatment modality of AA, but, local recurrence rates are high. In order to reduce recurrence, the scope of surgical resection should be as broad as possible. Intra-operative pathological diagnosis is useful in deciding the scope of surgery, and wide excision may reduce recurrence.
Topics: Adult; Female; Gynecologic Surgical Procedures; Humans; Immunohistochemistry; Intraoperative Period; Laparotomy; Middle Aged; Myxoma; Neoplasm Recurrence, Local; Vulvar Neoplasms
PubMed: 22442917
DOI: 10.1080/00015458.2012.11680802 -
International Journal of Gynecological... 2001A case of a 31-year-old woman with epithelioid sarcoma of the vulva which metastasized to the regional lymph node 8 years after onset of the disease is reported here.... (Review)
Review
A case of a 31-year-old woman with epithelioid sarcoma of the vulva which metastasized to the regional lymph node 8 years after onset of the disease is reported here. The patient first noticed a painless subcutaneous mass of 5 mm in diameter in the right labium majus at age of 21. This was excised locally at age 23, but recurred 17 months later. Although local excision was again performed, the tumor recurred and continued to enlarge very slowly. At this stage, based on the pathology of both the initial and second tumors, the diagnosis was of a benign inflammatory process. However, local recurrence and inguinal lymph node swelling occurred at age 29, and biopsy was taken. The pathology report indicated benign granulomatous changes. The slides were reconsidered and re-interpreted as epithelioid sarcoma, whereupon radical vulvectomy was performed at age 31. Vulvar epithelioid sarcoma with inguinal lymph node metastasis was first diagnosed at that time. Epithelioid sarcoma of the vulva is an exceedingly rare tumor, and only 15 cases have been reported thus far in the literature. Early diagnosis and curative treatment of this tumor may be problematic for gynecologists because of its rarity and therefore little-known characteristic clinical behavior and histology. Radical vulvectomy or extensive local excision with inguinal lymphadenectomy at the time of diagnosis is recommended as the treatment of choice.
Topics: Adult; Diagnosis, Differential; Female; Groin; Humans; Lymphatic Metastasis; Neoplasm Recurrence, Local; Sarcoma; Vulvar Neoplasms
PubMed: 11520372
DOI: 10.1046/j.1525-1438.2001.011004316.x -
BMJ Case Reports Nov 2013Vulvar Paget's disease is an extremely rare neoplasm that accounts for less than 1% of the vulvar malignancies. We present a case of a 75-year-old woman, who had an...
Vulvar Paget's disease is an extremely rare neoplasm that accounts for less than 1% of the vulvar malignancies. We present a case of a 75-year-old woman, who had an eczematoid lesion involving the labia majora and minora bilaterally, with infiltration to the clitoris. Enlarged non-fixed lymph nodes were palpable in the inguinal region bilaterally. A biopsy of the vulva showed Paget's disease. She underwent radical vulvectomy with bilateral inguinal lymph node dissection. The patient remained disease free at 6-month follow-up.
Topics: Aged; Diagnosis, Differential; Female; Humans; Paget Disease, Extramammary; Vulva; Vulvar Neoplasms
PubMed: 24265339
DOI: 10.1136/bcr-2013-200623 -
Pediatric Dermatology 2023Herein we describe the case of a Black adolescent who was found to have widely metastatic melanoma originating from a primary vulvar lesion. The lesion presented as a...
Herein we describe the case of a Black adolescent who was found to have widely metastatic melanoma originating from a primary vulvar lesion. The lesion presented as a pink, vegetative nodule of the clitoral hood which grew in size over several years and was confirmed to be melanoma on shave biopsy. This patient's amelanotic presentation in conjunction with the rare incidence of vulvar melanoma contributed to the delay in diagnosis. This case exemplifies the challenge of early recognition of potentially malignant vulvar lesions for primary care providers in adolescents.
Topics: Female; Adolescent; Humans; Melanoma, Amelanotic; Vulvar Neoplasms; Skin Neoplasms; Vulva; Vulvar Diseases
PubMed: 36949654
DOI: 10.1111/pde.15296 -
Pathology, Research and Practice 2000A new case of primary Merkel cell carcinoma (MCC) of the vulva is reported and the literature reviewed for noting its clinical presentation, microscopic,... (Review)
Review
A new case of primary Merkel cell carcinoma (MCC) of the vulva is reported and the literature reviewed for noting its clinical presentation, microscopic, immunohistochemical and ultrastructural features, as well as for establishing the role of immunohistochemistry in the ultimate diagnosis of this uncommon and aggressive tumor. The lesion occurred in a 79 year old patient. Histologically, the tumor was composed of intradermal small cells with high mitotic index and frequent apoptosis. The immunohistochemical study showed positivity for wide spectrum and low molecular weight cytokeratins, epithelial membrane antigen, neurofilaments, neuron specific enolase and chromogranin A. Electron microscopy revealed intermediate filaments in a typical globular paranuclear arrangement. The coexpression of cytokeratins (including cytokeratin 20) and neurofilaments, both in typical globular paranuclear arrangement, made possible the diagnosis of MCC, differentiating it from other malignant small cell tumors such as neuroendocrine metastatic carcinoma.
Topics: Aged; Biomarkers, Tumor; Carcinoma, Merkel Cell; Fatal Outcome; Female; Humans; Immunoenzyme Techniques; Inclusion Bodies; Neoplasm Proteins; Neoplasm Recurrence, Local; Neurofilament Proteins; Radiotherapy, Adjuvant; Vulvar Neoplasms
PubMed: 10926328
DOI: 10.1016/S0344-0338(00)80052-7 -
Current Opinion in Obstetrics &... Feb 2002The incidence of human papilloma virus-related vulvar intraepithelial neoplasia is increasing worldwide. This is associated with an increasing incidence of invasive... (Review)
Review
The incidence of human papilloma virus-related vulvar intraepithelial neoplasia is increasing worldwide. This is associated with an increasing incidence of invasive vulvar cancer in young women. Undifferentiated vulvar intraepithelial neoplasia has an invasive potential; a subset of very young patients with pigmented lesions and spontaneous regression has been described. Differentiated vulvar intraepithelial neoplasia is human papilloma virus negative and affects older women, who are at risk of invasive cancer. Chromosomal changes and angiogenesis may play a role in carcinogenesis. Immunocompromised women bear a substantial risk of vulvar intraepithelial neoplasia. These facts demand the awareness of both women and physicians, because there is evidence of diagnostic delays in patients with vulvar cancer. The standard treatment is surgical excision, which may be combined with laser treatment in extensive disease. Preliminary results of topical antiviral agents and photodynamic therapy are available, but remain to be confirmed by prospective, placebo-controlled studies.
Topics: Biomarkers, Tumor; Carcinoma in Situ; Female; Humans; Incidence; Neoplasm Invasiveness; Photochemotherapy; Vaccination; Vulva; Vulvar Neoplasms
PubMed: 11801875
DOI: 10.1097/00001703-200202000-00007 -
Cancer Nov 1994
Topics: Carcinoma, Squamous Cell; Female; Humans; Lymphatic Metastasis; Neoplasm Staging; Survival Rate; Vulvar Neoplasms
PubMed: 7922992
DOI: 10.1002/1097-0142(19941101)74:9<2399::aid-cncr2820740903>3.0.co;2-v -
International Journal of Gynecological... Oct 2011Vulval squamous cell carcinoma appears to arise via 2 distinct pathways. A significant minority are associated with oncogenic human papillomavirus (HPV) infection and... (Review)
Review
Vulval squamous cell carcinoma appears to arise via 2 distinct pathways. A significant minority are associated with oncogenic human papillomavirus (HPV) infection and undifferentiated vulval intraepithelial neoplasia (VIN). However, the majority arises in the absence of HPV, on a background of chronic inflammation. Until recently, it was assumed that lichen sclerosus was the underlying inflammatory condition in the majority of HPV-negative cancers. This pathway of carcinogenesis has been less well studied than the HPV pathway. Emerging evidence implicates differentiated VIN (DVIN), rather than lichen sclerosus, as the most likely precursor lesion in HPV-negative vulval squamous cell carcinoma. Here we discuss the clinical and molecular evidence that implicates DVIN as a lesion with a high malignant potential. This lesion is probably underdiagnosed and may be undertreated. Better recognition of DVIN by gynecologists and pathologists may therefore offer an opportunity to prevent some vulval cancers.
Topics: Carcinoma in Situ; Carcinoma, Squamous Cell; Female; Humans; Neoplasm Recurrence, Local; Vulva; Vulvar Neoplasms
PubMed: 21946295
DOI: 10.1097/IGC.0b013e31822dbe26 -
European Journal of Gynaecological... 2014Angiomyofibroblastoma (AMF) is a rare benign mesenchymal neoplasm that arises in the pelviperial region.
BACKGROUND
Angiomyofibroblastoma (AMF) is a rare benign mesenchymal neoplasm that arises in the pelviperial region.
CASE
A patient presented with a painless mass in the right vulva. Under the preoperative diagnosis of Bartholin cyst, she underwent a simple tumor excision. Pathological examination revealed an AMF. Immunohistochemical examination showed that tumor cells were positive for estrogen receptor, progesterone receptor, vimentin, and CD34. She has been with no evidence of local recurrence for ten months after surgery.
CONCLUSION
AMF of the vulva is a distinctive mesenchymal tumor that is curable with a simple excision.
Topics: Adult; Angiomyoma; Female; Humans; Neoplasms, Muscle Tissue; Vulvar Neoplasms
PubMed: 24654468
DOI: No ID Found -
La Clinica Terapeutica Sep 2021A sarcomatoid squamous cell carcinoma of the vul-va is a very rare malignancy of the female genital tract . It has been characterized as an aggressive disease typically...
BACKGROUND
A sarcomatoid squamous cell carcinoma of the vul-va is a very rare malignancy of the female genital tract . It has been characterized as an aggressive disease typically associated with early development of local recurrences as well as distant metastases.
CASE
A 77-year old woman presented to our hospital with an aggravated vulvar mass, complaining about pruritus and pain. The patient underwent a radical vulvectomy with bilateral inguinal lymph node dissection and bilateral pelvic lymph node sampling. The histo-pathologic examination was consistent with a sarcomatoid squamous cell carcinoma of the vulva with an identical microscopic and im-munohistochemical appearance. The FIGO stage was IB and further management was decided to be radiotherapy.
CONCLUSION
A sarcomatoid squamous cell carcinoma is a rare entity with an aggressive biological behavior. To date, there have been only a few cases of the disease reported in the literature. Therefore a more systematic collection and closer study of such cases would be helpful for achieving an early diagnosis and more effective therapeutic strategies in the future.
Topics: Aged; Carcinoma, Squamous Cell; Female; Humans; Lymphatic Metastasis; Neoplasm Recurrence, Local; Vulvar Neoplasms
PubMed: 34625766
DOI: 10.7417/CT.2021.2345