Epithelioid sarcoma is an uncommon mesenchymal malignancy which represents less than 1% of all sarcomas. Rarer still are reports of this tumor initially presenting in the vulva. We report a case of vulvar proximal-type epithelioid sarcoma.

A 52-year-old had a 5-month history of slowly growing papule on the right labia majora. Excision of the mass revealed a tumor composed of large polygonal cells with abundant eosinophilic cytoplasm. An immunohistochemistry panel revealed cytokeratin AE1/AE3 positivity only. She underwent radical vulvectomy with bilateral groin node dissection. The specimen revealed a cream tan, firm, fairly defined mass at the right vulva. Microscopic examination showed a sheet-like growth pattern of large pleomorphic epithelioid cells with large vesicular nuclei and prominent nucleoli. The tumor showed loss of INI1 nuclear expression and absence of CD34 staining. EMA was positive. The case was signed out as proximal-type epithelioid sarcoma of the right vulva. Two months post-operatively, the patient was given concurrent chemotherapy with 5 cycles of cisplatin 40 mg/m2 and 6600 centigray vulvar intensity-modulated radiotherapy. She had no evidence of disease for five months until repeat workup showed tumor recurrence in the perineum. She was subsequently given 6 cycles of gemcitabine 900 mg/m2 and gemcitabine 900 mg/m2 with docetaxel 100 mg/m2. Two months after, repeat workup showed persistent progressive disease in the vulva. She was subsequently given 4 cycles of doxorubicin 60 mg/m2 and is for repeat workup.

The immunohistomorphologic features of this tumor, in addition to its unusual location, present a diagnostic challenge. Clues to the diagnosis include an initial presentation as a soft tissue mass and microscopic features showing the presence of epithelioid to spindle cytomorphology with an infiltrative growth pattern. Immunohistochemistry studies revealing the loss of INI1 nuclear expression and expression of epithelial markers would ultimately establish the diagnosis of this rare clinical entity.

: Epithelioid sarcoma is an uncommon mesenchymal malignancy which represents less than 1% of all sarcomas. Rarer still are reports of this tumor initially presenting in the vulva. We report a case of vulvar proximal-type epithelioid sarcoma. : A 52-year-old had a 5-month history of slowly growing papule on the right labia majora. Excision of the mass revealed a tumor composed of large polygonal cells with abundant eosinophilic cytoplasm. An immunohistochemistry panel revealed cytokeratin AE1/AE3 positivity only. She underwent radical vulvectomy with bilateral groin node dissection. The specimen revealed a cream tan, firm, fairly defined mass at the right vulva. Microscopic examination showed a sheet-like growth pattern of large pleomorphic epithelioid cells with large vesicular nuclei and prominent nucleoli. The tumor showed loss of INI1 nuclear expression and absence of CD34 staining. EMA was positive. The case was signed out as proximal-type epithelioid sarcoma of the right vulva. Two months post-operatively, the patient was given concurrent chemotherapy with 5 cycles of cisplatin 40 mg/m2 and 6600 centigray vulvar intensity-modulated radiotherapy. She had no evidence of disease for five months until repeat workup showed tumor recurrence in the perineum. She was subsequently given 6 cycles of gemcitabine 900 mg/m2 and gemcitabine 900 mg/m2 with docetaxel 100 mg/m2. Two months after, repeat workup showed persistent progressive disease in the vulva. She was subsequently given 4 cycles of doxorubicin 60 mg/m2 and is for repeat workup. The immunohistomorphologic features of this tumor, in addition to its unusual location, present a diagnostic challenge. Clues to the diagnosis include an initial presentation as a soft tissue mass and microscopic features showing the presence of epithelioid to spindle cytomorphology with an infiltrative growth pattern. Immunohistochemistry studies revealing the loss of INI1 nuclear expression and expression of epithelial markers would ultimately establish the diagnosis of this rare clinical entity.
epithelioid sarcoma ; vulvar neoplasms ; female urogenital diseases

Humans ; Female ; Carcinoma in Situ ; Vulvar Neoplasms ; Carcinoma, Squamous Cell

Context: Vulvar cancers are rare gynecologic tumors. Ultrasound can characterize primary tumors and guide the biopsy of suspicious nodes. Currently, there are no studies on the sonologic features of different vulvar cancer types. Aims: The aim is to determine the sonologic features of different vulvar malignancies. To compare the accuracy of ultrasound in identifying lymph node status. Settings and Design: This is a retrospective descriptive study. Subjects and Methods: Patient records, ultrasound images, and reports of vulvar cancer with long‑ and short‑axis (L/S) ratio of inguinal nodes were reviewed from the year 2010 to 2019. Statistical Analysis Used: The accuracy of ultrasound to detect lymph nodes and the correlation of L/S ratio to histopathology were done. Results: The study included 49 patients. The most common histopathologies were squamous cell carcinoma (SCCA), vulvar melanoma (VM), and adenocarcinoma (ADCA). Most tumors were > 4 cm, unifocal, and lateral in location. SCCA, verrucous carcinoma, VM, ADCA, adenosquamous carcinoma (ASCA), eccrine carcinoma (ECCA), and carcinosarcoma (CS) were irregular heterogeneous masses. Leiomyosarcoma (LMS) and proximal‑type epithelioid sarcoma (PES) were regular, heterogeneous masses. Paget’s disease of the vulva (PDV) was hypoechogenic with regular borders. Vascularities were absent in PDV and LMS, minimal in ECCA, moderate in ASCA and PES, moderate to abundant in CS, and variable in VM. The accuracy of ultrasound in detecting lymph nodes was 78%; the accuracy of the L/S ratio was 75%. Tumor border and FIGO stage showed significant association with histopathologic type. Conclusions Sonologic features and accuracy of ultrasound in predicting lymph node status help prognostication in vulvar cancer. Nodal morphometric studies are recommended for future researches.
Vulvar Neoplasms

Objective: We aim to evaluate the morbidity and mortality of cancer attributable to human papillomavirus (HPV) infection in China in 2016. Methods: Based on the cancer incidence and mortality rates, national population data, and population attributable fraction (PAF) in China, we calculated the number of incidence and death cases attributed to HPV infection in different areas, age groups, and gender in China in 2016. The standardized incidence and mortality rates for cancer attributed to HPV infection were calculated by using Segi's population. Results: In 2016, a total of 124 772 new cancer cases (6.32 per 100 000) were attributed to HPV infection in China, including 117 118 cases in women and 7 654 cases in men. Of these cancers, cervical cancer was the most common one, followed by anal cancer, oropharyngeal cancer, penile cancer, vaginal cancer, laryngeal cancer, oral cancer, and vulvar cancer. A total of 41 282 (2.03 per 100 000) deaths were attributed to HPV infection, of which 37 417 occurred in women and 3 865 in men. Most deaths were caused by cervical cancer, followed by anal cancer, oropharyngeal cancer, penile cancer, laryngeal cancer, vaginal cancer, oral cancer, and vulvar cancer. The incidence and mortality rates of cervical cancer increased rapidly with age, peaked in age group 50-54 years, then decreased obviously. The morbidity and mortality rates of non-cervical cancer increased with age. The cancer case and death numbers in rural areas (57 089 cases and 19 485 deaths) were lower than those in urban areas (67 683 cases and 21 797 deaths). However, the age-standardized incidence rate (ASIR) and age-standardized mortality rate (ASMR) of cervical cancer were higher in rural areas than in urban areas. There were no significant differences in ASIR and ASMR of non-cervical cancers between urban areas and rural areas. Conclusions: The incidence of cancers attributed to HPV infection in China was lower than the global average, but the number of incidences accounted largely, furthermore there is an increasing trend of morbidity and mortality. The preventions and controls of cervical cancer and male anal cancer are essential to contain the increases in cancer cases and deaths attributed to HPV infection.
China/epidemiology* ; Female ; Humans ; Incidence ; Laryngeal Neoplasms ; Male ; Middle Aged ; Mouth Neoplasms ; Oropharyngeal Neoplasms/epidemiology* ; Papillomavirus Infections/epidemiology* ; Penile Neoplasms/epidemiology* ; Registries ; Uterine Cervical Neoplasms/epidemiology* ; Vaginal Neoplasms ; Vulvar Neoplasms

Breast Neoplasms ; Female ; Humans ; Paget Disease, Extramammary/surgery* ; Sweat Gland Neoplasms ; Vulvar Neoplasms/surgery*

Extramammary Paget’s disease (EMPD) of the vulva is a rare vulvar neoplasm but commonly arises during the postmenopausal period. Intraepithelial Paget’s disease may persist for prolonged periods without demonstrating invasion but with high rates of recurrence. Appearance of Paget’s disease in a split-thickness skin graft, is associated with an occurrence outside the grafted area. It demonstrates retrodissemination as the pathologic process hypothesized in the spread of the disease within the skin via lymphatics and vessels creating tissue bridges between sites of involvement. We present a case of an 81-year-old female, the patient came in for complaints of vulvar pruritus beginning at the left inguinal area three years prior to her diagnosis. She consulted with a dermatologist and was initially treated with steroids and emollients. Persistence of symptoms and enlargement of the lesion prompted a vulvar punch biopsy which showed Paget’s disease and referral to the Gynecologic Oncology service. Wide local excision with split-thickness skin grafting was performed. However, one year after her surgery, patient noted vulvar pain and palpable vulvar lesions. Biopsy was done which showed Extramammary Paget’s Disease recurrence. Patient underwent repeat wide local excision with frozen section, and split-thickness skin grafting. With the aid of frozen section, the intraepithelial involvement was noted to spread beyond the grossly apparent lesion. After 6 months post re-excision, patient noted vulvar pruritus and palpable vulvar lesions. Biopsy was done which showed Extramammary Paget’s Disease recurrence. Due to the proximity of the lesion to the sphincter and need for a colostomy, the patient did not consent for re-excision. Imiquimod 5% was chosen as the mode of treatment. The challenges of interventions are to remove or treat disease that may not be visible, without overtreatment and to minimize morbidity from radical surgery. Surgery remains the primary management for EMPD of the vulva. Imiquimod 5% can be used in recurrences. Despite the advances in the knowledge and management of vulvar Paget’s disease the high rate of recurrent disease remains a challenge for optimal management and would require frequent and long-term follow-up.
Paget Disease, Extramammary ; Female ; vulvar neoplasms ;

OBJECTIVE: To evaluate the impact of age-adjusted Charlson comorbidity index (ACCI) in predicting disease-free survival (DFS), overall survival (OS), and cancer-specific survival (CSS) among surgically treated patients with vulvar carcinoma. The secondary aim is to evaluate its impact as a predictor of the pattern of recurrence. METHODS: We retrospectively evaluated data of patients that underwent surgical treatment for vulvar cancer from 1998 to 2016. ACCI at the time of primary surgery was evaluated and patients were classified as low (ACCI 0–1), intermediate (ACCI 2–3), and high risk (>3). DFS, OS and CSS were analyzed using the Kaplan-Meir and the Cox proportional hazard models. Logistic regression model was used to assess predictors of distant and local recurrence. RESULTS: Seventy-eight patients were included in the study. Twelve were classified as low, 36 as intermediate, and 30 as high risk according to their ACCI. Using multivariate analysis, ACCI class was an independent predictor of worse DFS (hazard ratio [HR]=3.04; 95% confidence interval [CI]=1.54–5.99; p < 0.001), OS (HR=5.25; 95% CI=1.63–16.89; p=0.005) and CSS (HR=3.79; 95% CI=1.13–12.78; p=0.03). Positive nodal status (odds ratio=8.46; 95% CI=2.13–33.58; p=0.002) was the only parameter correlated with distant recurrence at logistic regression. CONCLUSION: ACCI could be a useful tool in predicting prognosis in surgically treated vulvar cancer patients. Prospective multicenter trials assessing the role of ACCI in vulvar cancer patients are warranted.
Aged ; Comorbidity* ; Disease-Free Survival ; Humans ; Logistic Models ; Multicenter Studies as Topic ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models ; Prospective Studies ; Recurrence ; Retrospective Studies ; Vulvar Neoplasms*

OBJECTIVE: We aimed to identify the minimum tumor-free margin distance conferring long-term oncological safety in patients diagnosed with International Federation of Gynecology and Obstetrics (FIGO) stage IB/II vulvar squamous cell carcinoma (VSCC). METHODS: This was a retrospective cohort study in patients with stage IB/II VSCC treated at a single institution in Turin, Italy. The main aim was to identify the minimum tumor-free margin distance that confers oncological safety in early-stage VSCC. Patients were divided in groups according to tumor-free histological margin distance to compare survival outcomes. Overall survival (OS), disease-specific survival (DSS), and recurrence rate (RR) were estimated by the Kaplan-Meier method for the newly proposed and the currently recommended 8 mm margin cut-off. Log-rank test was used to compare survival between groups. RESULTS: One hundred and fourteen patients met the study criteria. Median age was 68 years and median follow-up was 80 months. The minimum margin distance that conferred long-term oncological safety was 5 mm. OS, DSS were significantly lower in the < 5 mm group when compared with the ≥ 5 mm group (p = 0.002 and p = 0.033, respectively) although no difference in RR was observed between groups. Analysis at the 8-mm cut-off indicated there is no difference in OS, DSS, or RR between groups. CONCLUSION: FIGO stage IB/II VSCC patients' prognosis is affected by margin distance. Long-term survival is significantly reduced in patients with tumor-free margins < 5 mm, even in the absence of lymph node metastasis. Thus, these patients should be offered further surgical or adjuvant treatment.
Carcinoma, Squamous Cell* ; Cohort Studies ; Epithelial Cells* ; Follow-Up Studies ; Gynecology ; Humans ; Italy ; Lymph Nodes ; Methods ; Neoplasm Metastasis ; Obstetrics ; Prognosis ; Recurrence ; Retrospective Studies ; Vulvar Neoplasms

OBJECTIVE: To establish a SD rat model of vulvar squamous intraepithelial lesions. METHODS: Seventy female SD rats were randomized into 4 groups, namely the blank control group (=10), mechanical irritation group (=10), acetone solution group (=10), and mechanical irritation with DMBA acetone solution group (=40, model group), and the corresponding treatments were administered 3 times a week for 14 weeks. The changes of the vulvar skin of the rats were observed regularly until the 18th week. The expression of mutant p53 (mtp53) and vascular endothelial growth factor (VEGF) proteins were detected using immunohistochemistry and Western blotting, and the expressions of mtp53 and VEGF mRNA were detected with qRT- PCR in the blank control group and model group. RESULTS: No significant differences were found in the morphological or histopathological changes of the skin among the blank control group, mechanical irritation group and acetone solution group. In the model group, low-grade squamous intraepithelial lesions (LSIL) occurred in 28 rats (70%) and high-grade squamous intraepithelial lesions (HSIL) in 11 rats (27.5%) at 14 weeks, with a success rate of 97.5% in inducing vulvar squamous intraepithelial lesions. Compared with the blank control group, the rats in the model group showed significantly increased expressions of mtp53 and VEGF at both the protein level ( < 0.05) and the mRNA level ( < 0.05). CONCLUSIONS DMBA in acetone solution combined with mechanical irritation can induce vulvar squamous intraepithelial lesions in female SD rats.
9,10-Dimethyl-1,2-benzanthracene ; Acetone ; Animals ; Blotting, Western ; Carcinogens ; Disease Models, Animal ; Female ; Friction ; Immunohistochemistry ; Precancerous Conditions ; chemically induced ; metabolism ; pathology ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Skin ; pathology ; Solvents ; Tumor Suppressor Protein p53 ; metabolism ; Vascular Endothelial Growth Factor A ; metabolism ; Vulvar Neoplasms ; chemically induced ; metabolism ; pathology