We report the case of a 67-year-old female with clear cell sarcoma (CCS) of the vulva. Grossly, the tumor was a partly exophytical vulvar mass, measuring 20 x 15 cm. At the time of presentation, the patient showed metastases to the lung, inguinal and pelvic lymph nodes. Histologically, the tumor consisted of oval or spindle cells with only mild nuclear pleomorphism and rare mitoses (up to 2/10 HPF). The cytoplasm was pale eosinophilic or clear. The tumor cells were arranged in confluent sheets. There were large areas of necrosis and surface ulceration. Immunohistochemically, the tumor cells showed expression of S-100 protein and focal melan A and HMB45 expression. Fluorescent in situ hybridization analysis revealed rearrangement of the EWSR1 gene. To the best of our knowledge, this is the first report of CCS arising in the vulva.

• Klíčová slova
• clear cell sarcoma - EWSR1 gene - melanoma of soft parts - vulva.,
• MeSH
• diferenciální diagnóza MeSH
• hybridizace in situ fluorescenční MeSH
• lidé MeSH
• nádory vulvy diagnóza   MeSH
• proteiny S100 MeSH
• sarkom z jasných buněk diagnóza   MeSH
• senioři MeSH
• vulva MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• práce podpořená grantem MeSH
• Názvy látek
• proteiny S100 MeSH

Extramammary Paget disease (EMPD) is a rare neoplasm with uncertain histogenesis, usually presenting in the anogenital area, most commonly in the vulva. The disease is characterized by slow grow and high recurrence rates. This article reviews the epidemiological, clinical, morphological, genetic and treatment features of EMPD of the vulva reported in recent years.

• Klíčová slova
• Extramammary Paget disease, Pathology, Treatment, Vulva,
• MeSH
• diferenciální diagnóza MeSH
• extramamární Pagetova nemoc diagnóza   epidemiologie   patologie   MeSH
• lidé MeSH
• nádory vulvy diagnóza   epidemiologie   patologie   MeSH
• prognóza MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

For preinvasive lesions of vulva, a common term VIN - vulval intraepithelial neoplasia is used. VIN is a histological dia-gnosis based on abnormal squamous epithelial proliferation. There are two types of VIN apart from their association with human papillomavirus (HPV). Undifferentiated (usual) vulval intraepithelial neoplasia is commonly associated with carcinogenic genotypes of HVP, whereas differentiated vulval intraepithelial neoplasia is not associated with high-risk genotypes of HPV. The article presents an overview of VIN occurence and epidemiology, its classification system and dia-gnostics. In conclusion, VIN therapeutical possibilities are presented. It can be treated with surgical therapy (local excision, partial vulvectomy, vulvectomy, laser vaporization) or medical therapy (imiquimod).

• Klíčová slova
• vulval intraepithelial neoplasia -  VIN -  epidemiology -  therapy,
• MeSH
• infekce papilomavirem klasifikace   diagnóza   epidemiologie   terapie   MeSH
• karcinom in situ klasifikace   diagnóza   epidemiologie   terapie   MeSH
• lidé MeSH
• nádory vulvy klasifikace   diagnóza   epidemiologie   terapie   MeSH
• prekancerózy MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH

BACKGROUND: Sentinel node biopsy in vulvar cancer is associated with much less morbidity than inguinofemoral node dissection. Our study focused on describing the morphology of superficial lymphatic drainage of the vulva and its relationship to regional nodes, which may facilitate orientation during surgery. MATERIALS AND METHODS: In 24 female cadavers, injections of patent blue (at various localisations medially, unilaterally and bilaterally) were used to visualise the lymphatic drainage of the vulva. After dissection of lymphatic vessels and nodes, their course was documented by photograph and then analysed. Subsequently, a map of vulvar superficial lymphatics was created. RESULTS: The cutaneous and subcutaneous tissue of the vulva primarily drained to superficial inguinal nodes. There was no evidence of a solitary lymph node that drained the unilateral vulva. Each area of the vulva drained to its own lymph node, which was variably localised in the subcutaneous groin around the great saphenous vein. Anastomoses between individual inguinal superficial lymph nodes are likely. Right-left symmetry in the course of lymphatic collectors was not detected. Natural drainage of the medial and paramedial areas to contralateral inguinal nodes was also not detected. The drainage pattern to ipsilateral inguinal nodes was consistent in cadavers without evidence of vulvar disease and may be applicable in the early stages of vulvar cancer. CONCLUSIONS: There was no evidence of a solitary node that drained the unilateral vulva. Each part of the vulva may drain to a corresponding lymph node in a different localisation of the groin. The surgeon should take this variability into account.

• Klíčová slova
• anatomy, cancer, lymphatic mapping, sentinel node, vulva,
• MeSH
• biopsie sentinelové lymfatické uzliny MeSH
• lidé MeSH
• lymfatické cévy * patologie   MeSH
• lymfatické uzliny patologie   MeSH
• nádory vulvy * patologie   MeSH
• třísla patologie   MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

The structure of vulva of four Trichocephalus species has been studied using scanning electron microscopy (SEM). The shape of vulva and cuticular elements on vulval appendages is described and documented. The cuticular structures on the vulva have a characteristic shape specific for each species. It is suppossed that SEM can be successfully employed in a detailed study of vulval structure, which is an important taxonomic character for the differentiation of females of Trichocephalus species.

• MeSH
• kozy parazitologie   MeSH
• mikroskopie elektronová rastrovací MeSH
• ovce parazitologie   MeSH
• Trichuris ultrastruktura   MeSH
• vulva ultrastruktura   MeSH
• zvířata MeSH
• Check Tag
• ženské pohlaví MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH

The occurrence of basal cell carcinoma (BCC) of the vulva is rare. We report the case of a 79-year-old woman with a medical history of intravaginal condyloma acuminatum and vaginal intraepithelial neoplasia 3 (VaIN 3) who presented with a solitary whitish lesion sized 8x5 mm with a central desquamation located on the right labium majus. Histopathologic examination revealed a typical superficial and nodular BCC. Additionally, there were multiple remarkable foci of epidermolytic hyperkeratosis (EH). These foci both merged with superficial BCC or were sharply demarcated from the tumor. Retrospective molecular-biological examination of all the available material revealed HPV type 42 in both condyloma acuminatum and VaIN 3 specimen but not in the BCC associated with EH. To our best knowledge, involvement of the lower female genitalia by EH is a rare finding with six cases published to date. Awareness of EH in this location and its distinction is important because it may be potentially misinterpreted as a viral condyloma.

• MeSH
• bazocelulární karcinom komplikace   patologie   MeSH
• epidermolytická ichtyóza komplikace   patologie   MeSH
• karcinom in situ patologie   MeSH
• kondylomata akuminata komplikace   patologie   MeSH
• lidé MeSH
• nádory kůže MeSH
• nádory vaginy komplikace   patologie   MeSH
• nádory vulvy komplikace   patologie   MeSH
• Papillomaviridae MeSH
• retrospektivní studie MeSH
• senioři MeSH
• vulva MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

• MeSH
• lichen sclerosus vulvy patologie   terapie   MeSH
• lidé MeSH
• menopauza MeSH
• vulva patologie   MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

OBJECTIVE: The authors demonstrate the case of a rare vulvar angiomyxoma in a 57-year-old female. DESIGN: Case report. SETTINGS: Department of Obstetrics and Gynecology, Medical Faculty Charles Uni Prague and Faculty Hospital, Hradec Králové. SUBJECT AND METHOD: A rare case of pedunculated angiomyxoma involving the vulva of a 57-year-old female is reported. Complete excision of the tumor was performed. CONCLUSION: Angiomyxoma is a rare locally infiltrative neoplasm of the vulvovaginal region, perineum and pelvis. Notorious recurring after surgical resection is due to its infiltrative border that makes it difficult to attain a cleared margin at the time of resection.

• MeSH
• lidé středního věku MeSH
• lidé MeSH
• myxom * patologie   MeSH
• nádory vulvy * patologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

Lipoblastoma-like tumor of the vulva was first described as a benign mesenchymal neoplasm of adipocytic differentiation having features of lipoblastoma, myxoid liposarcoma, and spindle cell lipoma. Prior studies of lipoblastoma-like tumor have evaluated PLAG1, HMGA2, and RB1 immunohistochemistry and DDIT3 rearrangement status, with results supporting its distinction from lipoblastoma and myxoid liposarcoma. However, absent RB1 expression was reported in a majority of tested cases, suggesting that lipoblastoma-like tumor may have underlying 13q alterations and be related to RB1-deleted soft tissue tumors. To further understand the molecular genetics of lipoblastoma-like tumor, we examined 7 cases by RB1 immunohistochemistry, DDIT3 and PLAG1 break apart FISH probes, RB1 enumeration FISH probe, and genomic copy number analysis by microarray. Patient age ranged from 21 to 56 years (median 35 years). Clinical follow up was available for 5 patients (71%) ranging 3-264 months (median 74 months). Microscopically, lipoblastoma-like tumor formed large lobules separated by thin and/or thick bands of fibrous tissue and had a prominent network of thin-walled vessels. Each tumor was predominantly composed of spindle cells and lipoblasts with variable quantities of mature adipocytes. RB1 immunohistochemistry exhibited a heterogeneous or "mosaic" pattern of weak and negative nuclear expression in all seven cases. DDIT3 and PLAG1 FISH were negative in each case. No evidence of RB1 regional gain or loss was identified by FISH. Genomic copy number analysis by chromosomal microarray showed a normal diploid profile in six tumors (86%). One tumor had copy number abnormalities consisting of an 11.9 megabase deletion from 1p13.3 to 1p11.2 and monosomy 14. Although lipoblastoma-like tumor has features of lipoblastoma, myxoid liposarcoma, and spindle cell lipoma, it is genetically different from these tumors. Furthermore, lipoblastoma-like tumor does not appear to have structural abnormalities of 13q resulting in deletion of RB1.

• MeSH
• dospělí MeSH
• genová dávka MeSH
• hybridizace in situ fluorescenční MeSH
• imunohistochemie MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• nádory vulvy genetika   patologie   MeSH
• nádory z pojivové a měkké tkáně genetika   patologie   MeSH
• stanovení celkové genové exprese MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Review of revised staging system for vulva, explaining the changes of staging and their impact on the prognosis of disease is presented. AIM: The main objectives of a reliable staging system include an assessment of prognosis, planning treatment, and the evaluation of their outcomes. A good staging system must meet three basic characteristics: validity, reliability and practicality. Since medical research and practice in the field of oncology have shown explosive growth, the staging of vulvar cancer and some other cancers did not give a good spread of prognostic groupings. Changes based on new findings were proposed in 2008 by the FIGO Committee on Gynecologic Oncology, approved, and published a year later the changes in the staging system for carcinoma of the vulva. Stage 0 was deleted, since it represents preinvasive lesion. Stage IA remained unchanged and stage I and II were combined. The number and morphology of the involved nodes were taken into account, and the bilaterality of positive nodes has been discounted. CONCLUSION: The purpose of a good staging system is to offer a classification of the extent of gynecological cancer, in order to provide a method of conveying ones clinical experience to others for the comparison of different treatment methods. As a result of the explosion of medical research in the field of oncology, the staging of some of the gynecological cancers became outdated and did not give a good spread of prognostic groupings. According to the revised staging for carcimona of the vulva, patients are divided to groups with similar prognosis. Therefore, exchange of relevant information between oncological centers is facilitated, thus disseminating knowledge and stimulating research in other parts of the world.

• Klíčová slova
• cancer staging - cancer of the vulva - TNM staging - FIGO staging - gynecological cancer,
• MeSH
• karcinom klasifikace   patologie   MeSH
• lidé MeSH
• nádory vulvy klasifikace   patologie   MeSH
• prognóza MeSH
• progrese nemoci MeSH
• směrnice pro lékařskou praxi jako téma MeSH
• staging nádorů metody   normy   MeSH
• zdraví žen MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• přehledy MeSH