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

Vulvar intraepithelial neoplasia (VIN) is a precancerous lesion involving the squamous epithelium of the vulva. This retrospective descriptive study aims to determine the prevalence of VIN in a tertiary government hospital in a developing country. Medical records of outpatient consultations with the diagnosis of VIN from January 2000 to June 2012 were reviewed. The prevalence of VIN was 1.6/100,000 women over the 12 years. The diagnosis was based on biopsy results of an incidental finding of vulvar lesions on physical examination. The profile of a patient with VIN was a woman aged 40 years old and above, married, multigravid, nonsmoker, high school graduate, and unemployed. Vulvar lesions noted were multiple hyperpigmented papules located at the posterior labia majora. VIN was associated with abnormal colposcopy findings, and 40% were associated with concomitant cervical disease. Treatment was wide local excision. Prompt diagnosis and appropriate treatment of VIN aim to prevent its progression to vulvar carcinoma. Although vulvar carcinoma is a rare condition, there has been a notable rise in prevalence in recent years. Hence, gynecologists should be vigilant and have a high index of suspicion to detect the disease early in its course.
Vulvar Diseases

A girl, aged 22 months, attended the hospital due to recurrent vulvar rashes for more than half a year. Skin biopsy showed Langerhans cell histiocytosis, and evaluation of systemic conditions showed no systemic involvement. Therefore, the girl was diagnosed with Langerhans cell histiocytosis (skin type). In conclusion, for rashes on the vulva alone, if there are no specific clinical manifestations, the possibility of Langerhans cell histiocytosis should be considered after molluscum contagiosum, sexually transmitted diseases, and Fordyce disease are excluded.
Developmental Disabilities ; Exanthema/etiology* ; Female ; Histiocytosis, Langerhans-Cell ; Humans ; Infant ; Vulvar Diseases/diagnosis*

Isolated massive vulvar edema in pregnancy is rare. The causative mechanisms remain poorly understood but it is probably related to mechanical, osmotic and hormonal factors. The differential diagnoses of vulvar edema include infections, tumors, lymph birth defects, trauma, inflammatory and metabolic diseases. This is a case of a 24-year-old primigravid with twin pregnancy who was admitted at 24 weeks age of gestation for massive vulvar edema. Reported causes of vulvar edema were ruled out. The aim of this report is to discuss the clinical aspects, differential diagnosis, causes and evolution of vulvar edema in pregnancy.
Pregnancy ; Female ; Vulvar Diseases ; Edema

OBJECTIVE: To explore the changes of collagen fibrosis in the vulva skin of SD rats with lichen simplex chronicus (LSC) after focused ultrasound therapy and explore the mechanism by which focused ultrasound reduces the recurrence of vulvar LSC. METHODS: Fifty female SD rat models of vulvar LSC were established and randomly divided into the treatment group and the control group (=25) for treatment with focused ultrasound and sham treatment, respectively. Before and after the treatment, vulvar skin tissues were sampled to observe the pathological changes with HE staining and assess the density of collagen fibers using Masson staining. The ultrastructure of the collagen fibers in the superficial dermis was observed using transmission electron microscopy. The expressions of notch1 and c-fos in the vulvar tissue were detected by immunohistochemistry and Western blotting. RESULTS: After 4 weeks of focused ultrasound therapy, 16% (4/25) of the rats in the treatment group showed lesion progression to LSIL, 4% (1/25) still had LSC, and 80% (20/25) showed normal vulvar skin. In the control group, progression to LSIL occurred in 19 (76%) rats, 3 (12%) rats still showed LSC, and only 3 (12%) had normal vulvar skin. The difference in the cure rate differed significantly between the two groups ( < 0.05). The density of collagen fibers in the superficial dermis and the expressions of notch1 and c-fos in the vulvar skin were significantly lower in the treatment group than in the control group ( < 0.05). CONCLUSIONS Focused ultrasound therapy can inhibit superficial collagen fibrosis of the dermis by lowering the expressions of notch1 and c-fos in the vulvar skin to reduce the recurrence of vulvar LSC in rats.
Animals ; Female ; Neurodermatitis ; Rats ; Rats, Sprague-Dawley ; Recurrence ; Vulvar Diseases

Mucoepidermoid Carcinoma (MEC) is an epithelial malignant tumor that was first described as a salivary gland malignancy. Though common in salivary gland, it is extremely rare in the vulva with only 2 cases reported in the English language literature and none yet in the Philippines. Due to its low incidence, prognosis and definitive management is still unclear. This is a case of a 68-year-old woman with a history of vulvar pruritus and vulvar mass at the left labia majora. Punch biopsy and review of slides revealed Invasive Squamous Cell Carcinoma, Non-Keratinizing type. She underwent Radical Vulvectomy and Bilateral Lymph Groin Dissection; Wide Excision of Perineal Area; Protective Transverse Loop Colostomy; Gracilis Myocutaneous Flap with Identification of Right and Left Median Circumflex Artery with a final histopathology report of Primary Cutaneous MEC of the vulva with lymph node metastasis.
Vulva ; Vulvar Diseases ; Biopsy

Fibroepithelial stromal polyp, more commonly known as acrochordon, skin tag, or soft fibroma is a type of mesenchymal tumor occurring among women of reproductive age. Fibroepithelial polyp, although the most common cutaneous tumor, is rare in the vulvovaginal region and there is currently no established protocol in approaching these kinds of lesions. Presented here is a series of cases of gradually enlarging labial masses among reproductive age women. These giant vulvar masses presented as solitary, flesh-colored, polypoid masses, initially non-tender but later becoming associated with local pain. Diagnosis is mainly through history, clinical examination aided by ultrasonography, and histopathologic examination, which would show a central fibrovascular core covered with squamous epithelium. Surgical excision serves as both diagnostic and therapeutic modality for these lesions. Vulvar fibroepithelial polyp do not seem to be as rare as literature says, they are relatively easy to diagnose and presents with benign clinical course.
Vulvar Neoplasms ; Vulvar Diseases

Vaginal atrophy is a common condition among peri- and post-menopausal women. Symptoms of vaginal dryness, pruritus, irritation, loss of subcutaneous fat, sparse pubic hair and dyspareunia occur due to decreased estrogen level. Estrogen-based treatments are effective. But many patients are reluctant to be treated due to health concerns. As alternatives, we explored the efficacy of platelet-rich plasma (PRP) and lipofilling. A 67-year-old female patient with vaginal atrophy was referred to our department. Treatment using estrogen cream had failed to improve patient's symptoms. Diminished volume and aged look of genitalia were also major concerns. We treated her using lipofilling mixed with PRP. A total of 40 cc of autologous fat mixed with PRP was transferred to labia majora. Lipofilling with PRP relieved the clinical symptoms. Missing fullness and tone was corrected and the augmented volume was well maintained. White patchy lesions of lichen sclerosus on labia minora also improved. Lipofilling with PRP relieved symptoms, restored contour of the labia majora and achieved remission of lichen sclerosus on labia minora. As vulvar lesions were repaired and the aged appearance of genitalia was rejuvenated, both functional and cosmetic outcomes were satisfactory. Lipofilling with PRP can be effective for vaginal atrophy and lichen sclerosus.
Aged ; Atrophy* ; Dyspareunia ; Estrogens ; Female ; Genitalia ; Hair ; Humans ; Lichen Sclerosus et Atrophicus* ; Lichens* ; Platelet-Rich Plasma* ; Pruritus ; Rejuvenation* ; Subcutaneous Fat ; Vaginal Diseases ; Vulvar Lichen Sclerosus

OBJECTIVE: To evaluate the prevalence and risk factors for unrecognized invasive carcinoma in a series of patients undergoing surgical excision after an office biopsy of vulvar high-grade squamous intraepithelial lesion (VHSIL). METHODS: Two hundred and sixteen consecutive patients treated in a tertiary-level referral center for vulvar disease in north-western Italy were recruited. Patients' records were reviewed by trained personnel. Factors showing a statistically significant (p<0.05) association with detection of stromal invasion at excisional surgery in univariate analysis were further examined in a backward stepwise multiple logistic regression model. RESULTS: The median patient age was 50 years (range, 19–88). More than 25% patients with VHSIL at biopsy had associated cervical/vaginal intraepithelial neoplasia, and more than 35% had a multifocal lesion. Invasive carcinoma was detected in surgical specimens from 24 patients (11%). The depth of stromal invasion varied between 0.1 mm and 3.0 mm with a median of 0.5 mm. In multivariate analysis, the risk of invasive carcinoma detection was greater for patients in the highest tertile of age (p=0.008), for patients with a lesion ≥20 mm in size (p=0.013) and with clitoral involvement (p<0.001), and for patients presenting with a nodular lesion (p=0.078). CONCLUSION: Our study suggests that patient age, lesion size, clitoral involvement and nodular appearance in patients with VHSIL at vulvoscopy-directed biopsy are independently associated with the risk of unrecognized invasive carcinoma.
Biopsy* ; Humans ; Italy ; Logistic Models ; Multivariate Analysis ; Prevalence ; Referral and Consultation ; Risk Factors* ; Squamous Intraepithelial Lesions of the Cervix* ; Vulvar Diseases ; Vulvar Neoplasms