Angiokeratoma of the vulva

Angiokeratoma of the vulva is relatively uncommon in the general population. We present two cases of angiokeratoma of the vulva and review the literature. The two patients presented with the complaint of vulvar pruritus. They underwent wide local excision of the lesions. Histopathology revealed angiokeratoma of the vulva. The women remain well with no evidence of recurrence 48 and 32 months after initial surgery. Although it is a rare disease, angiokeratoma of the vulva should be included in the differential diagnosis of a vulvar tumor.     

Hidradenoma papilliferum of the vulva: case report and review of literature

Hidradenoma papilliferum (HP) is a rare, small, benign, cystic, papillary, slow growing tumor occurring in anogenital region of adult women. The authors describe a case of large, rapidly enlarging HP of vulva in a 25-year-old woman and review the literature available on this rare condition.     

Angiokeratoma of the vulva: a rare benign vascular tumor mimicking malignancy--case reports

Angiokeratomas are rare benign dermal lesions of the external genital system and occur before the age of 50 years. Four cases of angiokeratoma of the vulva diagnosed at our institution in a ten-year-period are reported and issues of the differential diagnosis are discussed.     

Primary myoepithelial carcinoma of the vulva and review of the literature

Myoepithelial carcinoma of the vulva is extremely rare, with only five cases reported. Here, we describe a case of vulvar myoepithelial carcinoma along with a review of the literature. The patient, a 49-year-old woman, was referred for a tumor on the right labium majora. She underwent a wide local excision and bilateral inguinal lymph node dissection. Pathological examination revealed an unencapsulated, infiltrative pattern, with solid, nested and trabecular components and areas with myxoid or hyalinized stroma. The tumor consisted of oval to round epithelioid cells with moderate nuclear pleomorphism. By immunohistochemistry, the tumor cells were diffusely positive for cellular adhesion molecule (CAM) 5.2, epithelial membrane antigen (EMA), S-100 protein, and vimentin and focally positive for carcinoembryonic antigen (CEA) and p63, while negative for alpha- smooth muscle actin (SMA). The tumor was diagnosed as a myoepithelial carcinoma of the vulva, with metastases to the bilateral inguinal lymph nodes. Following completion of adjuvant radiotherapy, the patient remained alive without any evidence of recurrence at 56 months. A review of six cases of this tumor (including the present case), demonstrated variable morphology with some overlapping features. Therefore, immunohistochemistry using a panel of epithelial and myogenic markers is essential for definitive diagnosis. Two cases had inguinal lymph node metastases and received adjuvant radiotherapy or concurrent chemoradiotherapy, which resulted in good local control. One case had lung metastasis and was successfully treated by chemotherapy. Given the rarity of this disease and its uncertain prognosis, no clinical trials have been conducted regarding the necessity of adjuvant therapy. Myoepithelial carcinomas of the vulva are extremely rare making case series the most viable means of optimizing diagnosis and therapy.     

Cancer of the vulva and pregnancy: two cases and review of the literature

The cancer of vulva is predominantly a disease of postmenopausal women. But some cases have been reported in young women during pregnancy. Surgical management during pregnancy depends on several factors: gestational age at the diagnosis, lesion size, depth and location, lymph node status. We have conducted a national survey and report here the only two cases of squamous cell carcinoma diagnosed and treated during pregnancy at 26 and 15 weeks gestation. All vulva symptoms without signs of infectious should be examined with attention in order to detect vulvar lesions. When in doubt, a biopsy must be obtained. Management of vulva carcinoma is not modified during pregnancy, but the age of pregnancy must be considered.     

Primary breast carcinoma of the vulva: case report and review of literature

The occurrence of ectopic breast tissue within the vulva is uncommon and the development of breast carcinoma within vulvar ectopic breast tissue is very rare. To date, only 12 cases of primary vulvar breast carcinoma have previously been reported in the English literature. This paper presents the 13th reported case of primary breast carcinoma of the vulva. The patient presented with a vulvar ulcerated lump and the diagnosis was based on a morphologic pattern consistent with breast carcinoma and the presence of estrogen and progesterone receptors. Primary surgery consisted of radical vulvectomy and bilateral groin dissection. The groin lymph nodes were involved bilaterally. Adjuvant therapy consisted of systemic chemotherapy (4 cycles of adriamycin and cyclophosphamide followed by 4 cycles of paclitaxel) and pelvic radiotherapy. Oral tamoxifen 20 mg/day was started for the next five years. It is concluded that the management of primary breast carcinoma of the vulva should be modeled after that for primary carcinoma of the orthotopic breast with primary surgery followed by systemic chemotherapy and pelvic radiotherapy. Chemotherapy should be similar to that employed for breast carcinoma. Tamoxifen should be prescribed for patients whose tumors contain estrogen receptors.     

Aggressive angiomyxoma of the vulva: a case report and review of literature

Background Aggressive angiomyxoma (AA) is a rare vulvovaginal mesenchymal neoplasm with a marked tendency to local recurrence but which usually does not metastasize. Case report We describe a case of AA in the left labium majus pudendi in a 47-year-old woman who underwent incomplete surgical excision. Follow-up 2 years later revealed no recurrence. Conclusion In the past, most authors advocated wide excision even if genitourinary and digestive tract resection were necessary. These days, a less radical surgery is recommended, but the significance of hormonal treatment and/or radiation therapy is not clear yet. Further investigation is necessary.     

Primary breast carcinoma of the vulva: a case report and literature review

BACKGROUND: In 1872, Hartung was the first to describe the case of a fully formed mammary gland arising in the left labium majora of a 30-year-old woman. Since Hartung's initial report, 38 additional cases of ectopic vulvar breast tissue have been described. This case report describes the rare occurrence of primary mammary adenocarcinoma arising within the vulva. CASE: A 64-year-old G4P4 white female presented with a 4-year history of a 2 x 1 cm firm, indurated, raised lesion of the left lateral mons. A wide local excision with ipsilateral inguinofemoral lymphadenectomy was performed. Given histological findings characteristic of both invasive ductal carcinoma and invasive lobular carcinoma, in conjunction with the presence of estrogen and progesterone receptors within the tumor, a diagnosis of infiltrating adenocarcinoma arising within ectopic breast tissue was made. CONCLUSIONS: Thirty-nine reported cases of ectopic breast tissue arising within the vulva have been reported in the world literature. Though the diagnosis of primary breast carcinoma arising within the vulva is based primarily upon histologic pattern, estrogen and progesterone receptor positivity provide supporting evidence. Given the rarity of this condition, guidelines for therapy are unavailable; we therefore suggest looking to the current management of breast cancer in order to establish a sensible approach.     

Cystic papillary hidradenoma of the vulva: case report and review of the literature

Papillary hidradenoma of the vulva is a rare, benign neoplasm arising from apocrine sweat glands of the skin. Frequently, this lesion has been mistaken for carcinoma. The treatment of choice is local excision. The prognosis for patients with this tumor is excellent. We present a patient who is unique because she had a lesion which was the largest ever recorded, and which existed over twice as long as any previously described. This case is also presented to remind the clinician that, despite the gross appearance of the tumor which resembles carcinoma on sectioning, biopsy and histological diagnosis should guide the ultimate management of patients with such lesions. The findings in our patient support the view that no matter how large or how long in existence, hidradenoma remains benign.     

Dermatofibrosarcoma protuberans of the vulva: a case report and review of the literature

OBJECTIVE: The purpose of this study is to describe the management of a patient with dermatofibrosarcoma protuberans (DFSP) of the vulva and to review the literature. METHODS: A 39-year-old was referred by a district hospital for incomplete excision of a vulval mass. The lesion involved the left labium major and measured 8 x 12 cm. The lesion was reexcised with a 3-cm margin of normal skin. RESULTS: The patient made an uneventful postoperative recovery. Histology confirmed a diagnosis of DFSP with clear margins. Immunohistochemistry was positive for CD34 glycoprotein. CONCLUSIONS: DFSP of the vulva is a rare fibrous tumor of intermediate grade malignancy, with a tendency for local recurrence. However, it rarely metastasizes. Management should be multidisciplinary and Mohs' micrographic surgery is generally advocated to ensure precise margin control. Survival rates range from 91 to 100%, and local recurrence rates of 20 to 49% have been reported. Therefore close follow-up is recommended.     

Metastatic lung cancer involving the vulva: two new cases and literature review

Two new cases of metastatic lung cancer involving the vulva are presented. Metastasis from primary lung cancer is a rare cause of a vulval tumour and may require special immunohistochemical stains to determine whether the vulval tumour is primary or secondary. Awareness of the possibility of metastatic disease involving the vulva is of importance as the patient's prognosis is dependent on an accurate diagnosis and palliative treatment where appropriate.     

Malignant eccrine spiradenoma of the vulva: a case report and review of the literature

Malignant eccrine spiradenoma is a rare skin tumor of sweat gland origin. We present the first reported case of this tumor in the female genitalia. Due to the rarity of this tumor, there has yet to be an established standard of care. The present case is that of a 41-year-old woman with malignant eccrine spiradenoma of the periclitoral region. She had an 18-month history of a recurrent, painful mass adjacent to the clitoris. Her diagnosis was made after excision of the cystic tumor. The patient then underwent a partial radical vulvectomy with bilateral sentinel lymph node sampling. As malignant eccrine spiradenoma is a rare tumor, no standard care exists for treatment and postoperative management. Based on our review of the literature, wide local excision appears to be the preferred initial treatment. Furthermore, adjuvant chemotherapy and/or radiation does not seem to improve survival in patients with advanced or recurrent cancer. Although lymph node sampling and/or lymphadenectomy is frequently reported in the treatment of this tumor, hematogenous metastasis can also occur. Therefore, these patients require close postoperative follow-up for recurrent disease.     

Angiokeratoma of vulva with coexisting human papilloma virus infection: a case report

We report a 25-year-old unmarried girl who developed multiple papular lesions on both labia majora with the past history of documented HPV-6 viral infection in the vulva. A wide local excision was performed and histopathological report confirmed a case of angiokeratoma. To the best of our knowledge this is the first case of angiokeratoma of vulva following chronic HPV infection.     

Dermal lymphangiomata of the vulva and laser therapy: a case report and literature review

Dermal lymphangioma of the vulva is a rare disorder of the lymphatic system. The mainstay of therapy has been vulvectomy. A 65-year-old woman with a remote history of cervical cancer who successfully underwent CO2 laser vaporization for extensive vulva lymphangiomata, and a literature review inclusive of all 28 previously reported cases are described. Ten (35.7%) of these patients had previously received pelvic radiation for cervical cancer, 8 of whom (80%) had also undergone radical hysterectomy. Including the present case, 5 patients, 3 of whom had previously received radiation therapy, have been treated successfully with laser therapy. These data support laser vaporization with retreatment of persistent or recurrent focal disease as the treatment of choice for dermal lymphangiomata. Surgical excision should be reserved for treatment failures. Radical hysterectomy in association with postoperative pelvic radiation therapy appears to be an important, previously unrecognized risk factor for its development.     

Malignant melanoma of the vulva: report of six cases and review of the literature.

Six patients with vulvar malignant melanoma are reported. They accounted for 5.2% of all females with vulvar malignancies diagnosed in the south of Israel between 1961 and 1997. Age ranged from 25 to 66 years. Presenting symptoms were pruritus, bleeding and ulcer. Lesion originated in the labia minora in four patients and the labia majora in two, and lesion size ranged from I to 8 cm. Five patients had nodular melanoma, and one had superficial spreading melanoma. Breslow depth ranged from 2.5 to 8 mm, Clark level was IV in four patients and III in two, and Chung level was IV in all patients. Two patients had radical vulvectomy and bilateral groin lymphadenectomy, one had wide local excision, and one refused surgery. The two patients who had radical hemivulvectomy and bilateral groin lymphadenectomy were given adjuvant active specific immunotherapy with allogeneic vaccine and have survived disease-free, whereas the remaining four patients died of disease. It is concluded that vulvar malignant melanoma is a rare and aggressive tumor. For patients who present with deep lesions (Breslow depth > 0.76 mm, Clark level > II, Chung level > II) the recommended treatment is wide radical local excision (or at the most, radical hemivulvectomy) and bilateral groin lymphadenectomy.     

Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature

Isolated Langerhans cell histiocytosis (LCH) of the female genital tract is very rare. A review of the literature revealed that only 15 cases of primary vulvar LCH have previously been published in the English literature. We describe an additional case of confined vulvar LCH. A 49-year-old woman presented with an ulcerous lesion that turned out to be LCH confined to the vulva only. After surgical excision, four recurrences followed, which were treated again by surgery. After the fourth recurrence, adjuvant radiotherapy was applied. When the fifth recurrence occurred, only surgical excision was performed, and the patient has now been disease-free for 51 months. There are no standard treatment options for this rare disease. The most effective treatment options still remain elusive. In our case in the end surgery proved to be effective.     

Paget''s disease of the vulva and urogenital malignancies: A case report and review of the literature

The vulva is one of the extramammary sites which has a potential for developing Paget's Disease with which an underlying sweat gland carcinoma or breast carcinoma is frequently associated. Urogenital malignancy may be a third contender. Such a case managed by the authors herein reported and a review of the literature about such lesions support this assumption.     

Alveolar soft part sarcoma of the vulva: Report of first case and review of literature

A case of primary alveolar soft part sarcoma of the vulva is reported, with review of literature on this tumor arising from other parts of the human body. The patient is a 62-year-old caucasian secundigravida, with a 4-cm asymptomatic tumor in the right labium minus. Histology shows polygonal cancer cells with granular cytoplasm, forming alveolar patterns. Radical vulvectomy with complete dissection of the right groin and superficial dissection of the left groin lymph nodes was carried out. The surgical margins and lymph nodes were free of cancer. This tumor is rare, and the histogenesis is uncertain. It occurs most frequently in the limbs of young females, and runs an indolent course but usually results in a lethal outcome. It is recommended that wide local excision and dissection of regional lymph nodes be carried out. Adjuvant systemic chemotherapy and/or immunotherapy for a finite period of time is logical, but appropriate agents have yet to be defined. Local radiation may be indicated for the tumor not amenable to excision with wide free margin. Lifelong follow-up is mandatory based on a propensity for late recurrence.