Left Labium Research Articles

Primary Langerhans Cell Histiocytosis of the Vulva

Langerhans cell histiocytosis (LCH) of the female genital tract is very rare. A review of the literature revealed that only 12 cases of primary vulvar LCH have previously been published. We describe an additional case of LCH in which the disease was confined to the vulva. A 64-year-old woman was admitted to our hospital with a nodular mass on her left labium minus, and complete surgical excision was performed. On histological and immunohistochemical examination, the tumor fulfilled the criteria of LCH. A metastatic workup did not reveal any evidence of disease beyond the vulva. The patient received local radiotherapy, and 22 months later she is in excellent condition without local recurrence or metastatic disease.

A rare association of synchronous intraductal carcinoma of the breast and invasive carcinoma of ectopic breast tissue of the vulva: case report and literature review

Only 17 cases of breast carcinoma arising in vulvar ectopic mammary tissue have been reported. We present a unique case of synchronous pure intraductal carcinoma of the breast (DCIS) and invasive carcinoma of ectopic breast tissue of the vulva. A 53-year-old woman presented with a 2-cm nodule in left labium major of the vulva. A surgical biopsy revealed an invasive carcinoma of ectopic mammary tissue. The mammography showed irregular microcalcifications of the right breast. The patient underwent left hemivulvectomy, bilateral inguinal sentinel lymph node biopsy, and radioguided breast resection (radioguided occult lesion localization) of the microcalcifications. The definitive histology revealed negative inguinal sentinel nodes, no further residual tumor in the vulva, and a high-grade (grade 3) DCIS in the breast. The synchronous occurrence of primary breast carcinoma and ectopic breast tissue carcinoma in the vulva is an extremely rare finding, only once previously being reported and leading to unsolved problems of differential diagnosis. The presence of a pure DCIS of the breast makes this case really unique, definitively confirming the independent primary origin of both mammary tumors. The inguinal sentinel node biopsy avoided a bilateral inguinal dissection.

Acute leukaemia presenting as vulvar ulcers in an adolescent girl

The common causes of genital ulcers in young women are sexually transmitted infections. We report an unusual case of leukaemia in an adolescent who primarily presented with fever and genital ulcers. A 16-year-old unmarried girl presented with a 2-month history of painless ulcerative lesions over her external genitalia intermittent high-grade fever and swelling of the left knee joint. She had been investigated at other hospitals for rheumatic fever systemic lupus erythematosus typhoid fever and malaria since these are relatively common causes of prolonged fever in young patients in India. This work-up was negative after which she was referred to our hospital for review. Examination revealed a young girl in good general condition. She had a temperature of 101°F mild pallor and petechiae over her lower limbs but no lymphadenopathy. Respiratory cardiac and abdominal examinations were normal. Genital examination revealed a 3 × 2 cm superficial non-tender ulcer with a necrotic base and well-defined margins over the right labia majus. There were similar but smaller ulcerative lesions over the left labium majus and both groins. (excerpt)

Laser Ablation of Surgical Margins After Excisional Partial Vulvectomy for VIN

This paper presents the results of a retrospective review of all patients of a single gynecologist (n = 33) who underwent surgical treatment for vulvar intraepithelial neoplasia (VIN) 2 or 3 from 1994 through 2002. Four different surgical techniques were used. The size, location, and extent of the lesion influenced the choice of surgical technique. Sixteen patients (48.5%) underwent a laser and excisional partial vulvectomy in which all gross disease was excised, and the surgical margins were ablated an additional 4 mm laterally with a colposcopically directed CO2 laser. The excision was then closed with interrupted 2.0 polyglactin 910 sutures. Ten (30.3%) patients had a laser partial vulvectomy, which involved laser ablation of colposcopically visible disease to the basement membrane. An excisional partial vulvectomy used a traditional scalpel to excise all disease with 0.5- to 1.0-cm margins (n = 6). One additional patient was treated with a cavitational ultrasonic surgical aspirator. The median age of patients was 46 years (range, 31–80 years). Twenty-one women were smokers, 2 were using immunosuppressive medications, 12 had a history of infection with human papilloma virus, and 4 had previous nonvulvar intraepithelial neoplasia. None were positive for human immunodeficiency virus. Nine patients had undergone previous surgical treatment for VIN. The preoperative biopsy was VIN 3 in 30 of the 33 patients. Fourteen patients had multifocal lesions. The lesions were located on the posterior fourchette (n = 19), the right or left labium majus (n = 13 each), and the right or left labium minus (n = 11 and 9, respectively). With an average follow up of 20 months, 12 (36.4%) of the 33 patients had recurrent disease, including 7 of 10 women who had laser partial vulvectomy, 3 of 6 who had excisional partial vulvectomy, and the one patient treated with a cavitational ultrasonic surgical aspirator. Only one of the 16 patients who underwent laser/excisional partial vulvectomy had a recurrence of disease (P = .0016). Seven of the 14 patients with multifocal disease and 6 of the 21 who smoked recurred. Of the 6 patients who had excisional partial vulvectomy, 4 had positive surgical margins, one had negative, and one was unknown. The 3 patients in this group who recurred included one each with positive, negative, and unknown margins. Seven years 9 months after treatment, an invasive squamous cell carcinoma on the skin over the coccyx was diagnosed in a patient who was taking immunosuppressive medications after a combined kidney and liver transplant.

Recurrent giant fibroepithelial stromal polyp of the vulva associated with congenital lymphedema

Background The relatively site-specific mesenchymal lesions of the vulvovaginal region can exhibit superficially overlapping histological features and can be diagnostically challenging. Fibroepithelial stromal polyp is generally an easily recognizable entity, but certain cases cause differential diagnostic problems. Case We present a case of a 16-year-old girl with a 10-cm polypoid lesion localized to the left labium. The patient has therapy resistant congenital lymphedema localized to the left arm and leg. The labial lesion was resected and recurred after 12 months and 6 years following the initial treatment. Histologically, it exhibited characteristics of a fibroepithelial stromal polyp with scattered bizarre multinucleated giant cells and ectatic tortuous lymphatic spaces. Conclusion This vulvar lesion represents a unique example of giant fibroepithelial stromal polyp developed in association with Nonne–Milroy–Meiges syndrome.

Adenocarcinoma of mammary-like glands in the vulva successfully treated by weekly paclitaxel

An 87-year-old was referred for gynecologic evaluation of a lesion involving the left labia majus noted 3 years earlier. Fine-needle aspiration cytology revealed clusters with an acinous structure or glandular formation. The tumor appeared as cell clusters with linear arrangements. Histologic examination showed the same morphologic findings as scirrhus type of primary breast carcinoma. Examinations of the breasts and axillary lymph nodes were normal. This disease was diagnosed as an adenocarcinoma arising in mammary-like glands of the vulva. Bone scan showed multiple foci in the sternum, costa, and vertebrae, consistent with metastatic disease. We administered five courses of weekly paclitaxel chemotherapy, which achieved a partial response. There were no severe adverse effects. In our case, the fine-needle aspiration cytology was a rapid and minimally invasive method of diagnosis, and the findings were extremely similar to those of the scirrhus type of primary breast carcinoma. Rapid and accurate diagnosis made with this technique might contribute to a good prognosis in the early-staged cases. Weekly paclitaxel chemotherapy may be one of the safe and effective treatments for this disease with distant metastases, even in extremely aged patients (over 80 years).

A rare female genital tract tumor: Benign granular cell tumor of vulva: Case report and review of the literature

Background The granular cell tumor (GCT) of vulva is a rare female genital tract tumor. Case A 59-year-old female was incidentally noted to have a 1-cm sized lump in her left labium majus that subsequently increased in size to approximately 4 cm. The mass was totally excised under general anesthesia. Her postoperative follow-up was uneventful. Histologic examination of the tumor showed sheets and clusters of infiltrating tumor cells with morphologic features consistent with granular cell tumor. Conclusion Although benign and slow growing, it has a tendency for recurrence and can cause morbidity and mortality when presenting with multicentric or multiple organ involvement due to the lack of effective systemic therapy. Therefore, clinicians and pathologists should be aware of its clinical and histopathologic features.

Pyoderma gangrenosum as a cause of spontaneous vulvovaginal ulceration

Pyoderma gangrenosum (PG) is a rare, destructive inflammatory disease in which a painful infiltration of the skin breaks down to a make an enlarging ulcer. The ulcers are usually characteristic with elevated and undermined borders and may be solitary or multiple. PG may present in association with systemic disease, such as inflammatory intestinal diseases, polyarthritis, gammopathy and others. The predilection sites are the lower extremities, buttocks and abdomen, but any area of the body may be involved. The female lower genital tract may also be affected (1–3). Therefore, this entity should be known by gynecologists. We report a case of vulvovaginal PG. A 55-year-old woman was admitted to the hospital with spontaneous ulcerations of the vulva, vagina and the right gluteal region that had developed during the preceding week. The ulcerations developed rapidly as painful redness and infiltrations of the skin, followed by necrotization. She stated that “pieces of the skin fell off,” resulting in deep and very painful ulcerations. Her general condition was otherwise unaffected, including no symptoms of bowel disorder. She did not use any drugs, including nonsteroid antiinflammatory drugs (NSAIDs) Examination revealed five deep ulcers located in the gluteal region, the vaginal introitus (Fig. 1), the vaginal wall (Fig. 2) and the cervix, with undermined borders, varying in size from 1 to 7 cm in diameter. Pyoderma gangrenosum ulcers of the vulva (the left labium minus and the posterior commissure) and the right gluteal region at the time of diagnosis. Note the undermined borders. Pyoderma gangrenosum ulcers of the anterior vaginal wall at the time of diagnosis. Biopsies from the ulcer margin showed nonspecific changes with ulceration and neutrophilic infiltration without certain signs of vasculitis. Immunohistochemical examination did not detect deposits of immunoglobulin complexes, fibrin or complement. Blood and urinalysis were normal except for an elevated sedimentation rate (81 mm), C-reactive protein (CRP) (114 mg/L) and P-potassium (4.4 mmol/L) and P-creatinine (99 µmol/L). Bacterial culture from the ulcers grew colonies of Escherichia coli, probably of no pathological significance. All other investigations, including P-antinuclear antibodies, P-ANCA immunofluorescence test, the tumor markers CEA and CA125, smear and chest X-ray were negative. A diagnosis of PG was made and treatment with cyclosporin A (Sandimmun Neoral®) 5 mg/kg body weight daily was started immediately as the only therapy. The therapeutic response was remarkable, resulting in a significant decline in inflammation within the first 24 h. After 1 week of treatment, the patient felt much better and the ulcers were healing. After 1 month of therapy the ulcers were partly healed, without signs of inflammation. After 2 months the ulcers were completely healed, just leaving some scars and a defect in the left labium minus that was caused by the biopsies. The cyclosporin A dosage was reduced stepwise and terminated 3.5 months after onset of the disease. At the 6-month follow-up there were no signs or symptoms of recurrence. We report this case to increase the attention regarding PG as a possible diagnosis when dealing with spontaneous ulcerations of the female genital area. The diagnosis may be easy, provided one is aware of this condition. The most important differential diagnoses are infectious ulcers, especially herpes simplex, syphilis and ulcus molle (chancroid), carcinomas and Bechet's disease. The clinical appearance of the ulcerations, the acute onset and the pain, combined with the absence of signs and symptoms of an infectious condition, will usually be a sufficient basis for the diagnosis. Infectious disease should be ruled out by means of adequate microbiological and serological tests. Histological examination is nonspecific as for ulcers of any cause and typically shows neutrophilic inflammation, edema, and thrombosis of small- and medium-sized vessels at the base of the ulcer (4). There are no specific laboratory markers for PG. Correct diagnosis is important for early therapeutic intervention, and also for avoiding unnecessary surgical procedures that may easily increase the ulcerations, especially in the acute phase of the disease. Cyclosporin A is usually very effective in the treatment of PG (5). The disease may relapse after termination of the immunosuppressive therapy, requiring sustained treatment, possibly for several years. As PG is associated with inflammatory bowel disease, these patients should be queried specifically with respect to these conditions.

HETEROZYGOTIC TWINNING IN A CASE OF FEMALE VESICOURETHRAL DUPLICATION

A 45-day-old female born at term after a normal pregnancy was referred for a urogenital malformation. Physical examination demonstrated normal vaginal introitus, urethra and labia minora. The upper part of the left labium presented as a 6 3 cm cylindrical mass and a clitoris-like structure with an accessory urethra located at the base. The anus was normally located (fig. 1). Blood, urine, renal function and hormone stimulation analyses were normal, and karyotype was 46,XX. Abdominal ultrasonography showed a normal right kidney and a hypoplastic ectopic left kidney with 2 bladders. The uterus and ovaries were normal. Retrograde cystourethrogram through the orthotopic urethra filled a bladder and induced grade II vesicoureteral reflux to the right kidney. That performed through the ectopic urethral meatus filled a second bladder located posterolaterally to the first one and induced vesicoureteral reflux to an ectopic left kidney. Cystoscopy confirmed 2 normal appearing bladders lined by normal mucosa, each receiving a corresponding ureter. Both urethras were also normal. At surgery 2 completely independent bladders were found together with an ectopic hypoplastic kidney, bicornuate uterus, 2 ovaries, a 36 cm long cecal prolongation resembling a mega-appendix and 1 vas deferens. The mega-appendix, left hypoplastic kidney, bladder, ectopic urethra and vas deferens were removed. No rudimentary gonadal tissue or testis was identified. Finally, the pubic mass was excised and vulvoplasty was performed. Pathological studies determined that the removed urethra was surrounded by prostatic tissue into which a blind ending vas deferens opened (fig. 2). Examination of the pubic mass revealed a chondrolipoma. Molecular studies for gender investigation were performed. Genomic DNA was extracted from paraffin blocks of kidney tissue, clitoris and vas deferens taken during surgery. DNA analysis was performed using polymerase chain reaction. Amplified products were electrophoresed on acrylamide gel and visualized using silver staining. Four of the loci tested on chromosomes 3, 9, 12 and 21 demonstrated different alleles, indicating the presence of 2 diploid genomes. Thus, haplotyping showed the heterozygotic origin of both duplicated elements. Fluorescent in situ hybridization was performed using an X centromere probe DX21 and Y cocktail probe but the results could not confirm 2 different genders. DISCUSSION

Extra-pelvic endometriosis presenting as a vulvar mass in a teenage girl

A 15-year-old girl had been experiencing a vulvar mass each month for 6 months. The mass was painful and swollen. She reported menstruating regularly since menarche 3 years earlier. Her medical history was unremarkable. On examination a solid eggsized mass involved the left labium major and extended to the clitoris without urethral or vaginal invasion. Ultrasonography showed a cystic mass measuring 5.6 x 4.1 cm/2 in diameter with hypoechoic and homogeneous content. Endometriosis was confirmed histopathologically from the endometriod focus endometrial glands and stroma after needle aspiration. Local excision and enucleation of the vulvar endometriosis was performed. Grossly the mass was cystic dark-yellowish with a capsule containing blood clots. Postoperative recovery was reported uneventful at a 4-month of follow-up visit. (excerpt)

Recurrent vulvar carcinoma in pregnancy

Background Vulvar carcinoma is rare in the premenopausal age group. The incidence during pregnancy is even more uncommon. To date, no cases of recurrent vulvar carcinoma in pregnancy have been reported. We describe the first case of recurrent vulvar carcinoma during the same pregnancy. Case A 36-year-old G5P4004 presented with vulvar pain at 18 weeks of pregnancy. She had a 2.2-cm tender mass anterior to the left labium majus, of which a biopsy revealed invasive squamous cell carcinoma. At 23 weeks' gestation, she underwent a modified radical vulvectomy and bilateral inguinofemoral lymph node dissection. Eleven weeks later, she complained of a newly tender area to the right of the clitoris. A biopsy at that time demonstrated predominantly severe vulvar intraepithelial neoplasia (VIN III) with a small focus of invasive squamous cell carcinoma. A radical local excision was performed at 9 weeks postpartum. Conclusion Careful surveillance is warranted as squamous cell carcinoma recurrence can occur quickly in the setting of pregnancy.

Breast cancer metastatic to the vulva

Background Primary cancer of the vulva is not common, constituting only 3–5% of all gynecologic malignancies and <1% of all cancer in the female. Metastatic tumors of the vulva are even more unusual, constituting only 5–8% of all vulvar cancers. Case A 32-year-old female underwent total mastectomy with axillary lymph node dissection for left breast cancer. The patient was well with a postoperative follow-up period of 40 months till a 1.2-cm lump was noted in her left labium majus. Excisional biopsy was performed and histologic examination was done. Pathologic examination of the breast cancer revealed coexistence of intraductal carcinoma and invasive lobular carcinoma. The histologic feature of the invasive lobular carcinoma was consistent with that of the vulvar lesion. The vulvar cancer was consistent with a metastatic lobular carcinoma from the breast. Conclusion Only with careful gynecologic surveillance in women with breast cancer can the unusual sites of metastasis be detected earlier and appropriately treated.

Vulvar Paget's disease with underlying adenocarcinoma simulating breast carcinoma: case report and review of the literature

We report a case of extramammary Paget's disease with underlying adenocarcinoma simulating breast carcinoma of the vulva. An 82-year-old woman was found to have a 5 × 3-cm bulky tumor located in the left labium major, infiltrating to the clitoris, left labium minor, and left lateral tissue of the vulva. Small biopsy of the vulva showed intraepidermal proliferation of Paget cells. The patient underwent wide local excision of the vulvar tumor and dissection of left inguinal lymph nodes. Histopathological examination of the resected specimens revealed that Paget cells were distributed singly or tended to form small nests in the epidermis, and that association of these cells with the underlying carcinoma invading to the subcutis could be seen. The underlying carcinoma was composed of squamoid solid nests with central necrotic debris, mimicking ‘comedocarcinoma’ of the breast. In other areas, the tumor cells were present in tubular formations and solid cords reminiscent of invasive ductal carcinoma of the breast. Immunohistochemically, the Paget cells and the underlying carcinoma cells were positive for carcinoembryonic antigen, epithelial membrane antigen, estrogen receptors, and glandular keratins except for CK 20. We speculate that our case is vulvar Paget's disease presenting as a manifestation of underlying breast carcinoma of the vulva, which might have arisen from either the ectopic breast tissue or anogenital mammary-like glands.

Angiomyofibroblastoma of the Vulva

Angiomyofibroblastoma is a rare, recently described, distinctive benign mesenchymal tumor that occurs mainly in the vulvar region of premenopausal women. We report a case of angiomyofibroblastoma of the vulva. A 45-year-old woman reported a small, painless nodule on the left vulva which rapidly enlarged during the 6 months before she visited the hospital. Local examination showed a pedunculated, mobile mass on the superior aspect of the left labium majus (13 x 12 x 10 cm) with elastic texture. Left vulvectomy was performed and the excised specimen revealed a well-defined and multilobulated tumor with edematous changes on gross examination. Microscopically, the tumor had alternate hyper- and hypocellular areas characterized by benign-looking round- to spindle-shaped cells with eosinophilic cytoplasm. The tumor cells were admixed with scattered thin-walled vessels in an edematous stroma. Immunohistochemical analysis was diffusely positive for desmin, estrogen receptor and progesterone receptor; reactive in small focus for actin; and negative for S-100 and CD34. Angiomyofibroblastoma, a mesenchymal tumor, was diagnosed. The postoperative course was smooth with no recurrence during the following 8 months. Simple excision for angiomyofibroblastoma is recommended because local recurrence or metastasis has not been reported.

Vulval cytomegalovirus coexisting with herpes simplex virus in a patient with human immunodeficiency virus infection

.We describe the cutaneous manifestation, diagnosis,management and outcome of vulval CMV and herpessimplex virus (HSV) infection in a patient with humanimmunodeficiency virus (HIV) infection.Case reportA 34 year old patient was referred to our institution fora non-healing vulval ulcer. The ulcer had been present fora year and was being treated as a bacterial infection at aprimary health care centre for which there was no re-sponse. She had no other significant history and clinicalexamination revealed a patient in good general andsystemic condition. External genital examination revealedan 8 5 cm ulcer of the left labia majora extending tothe left buttock region. The ulcer had a raw base andslightly raised edges (Fig. 1). Genital examination wasotherwise normal. Biopsy of the ulcer revealed ulcera-tion with underlying granulation tissue, a mixed inflam-matory cell infiltrate and scattered cells with largeintranuclear inclusions consistent with CMV. In addition,the squamous epithelium shows foci of intranuclear inclu-sions with syncytia formation consistent with HSV in-fection. An HIV ELISA antibody test was positive and theCD

VIN 3: a clinicopathologic review.

A retrospective review of the management of vulvar intraepithelial neoplasia 3 (VIN 3) over a 16-year period from 1981 to 1997 was conducted. Complete information was available for analysis on 101 patients. The mean age was 53.9 years (range 14-102 years). The mean duration of follow-up was 36 months (range 2-184 months). Fifty-eight percent of patients presented with pruritus. The disease was multifocal in 51% and unifocal in 49% of cases and the left labium majus was the most frequently affected site (27%). Co-existent or previous genital disease was identified in 39% of patients and 8% had a history of invasive gynecological cancer. Histologic evidence of human papillomavirus (HPV) infection was found in 31% of patients. Wide local excision was the most frequently used treatment modality (78%). Thirty-eight percent of patients required at least one further treatment for recurrent disease. Smoking, multifocality, HPV effect, and positive surgical margins were not found to be significant predictors of recurrence. There were three (3%) cases of progression to invasive squamous cell carcinoma of the vulva, one at 6, 7, and 7 years after initial treatment.

VIN 3: a clinicopathologic review

A retrospective review of the management of vulvar intraepithelial neoplasia 3 (VIN 3) over a 16-year period from 1981 to 1997 was conducted. Complete information was available for analysis on 101 patients. The mean age was 53.9 years (range 14–102 years). The mean duration of follow-up was 36 months (range 2–184 months). Fifty-eight percent of patients presented with pruritus. The disease was multifocal in 51% and unifocal in 49% of cases and the left labium majus was the most frequently affected site (27%). Co-existent or previous genital disease was identified in 39% of patients and 8% had a history of invasive gynecological cancer. Histologic evidence of human papillomavirus (HPV) infection was found in 31% of patients. Wide local excision was the most frequently used treatment modality (78%). Thirty-eight percent of patients required at least one further treatment for recurrent disease. Smoking, multifocality, HPV effect, and positive surgical margins were not found to be significant predictors of recurrence. There were three (3%) cases of progression to invasive squamous cell carcinoma of the vulva, one at 6, 7, and 7 years after initial treatment.

Sebaceous gland hyperplasia of the vulva

BACKGROUND: Sebaceous gland hyperplasia is a common condition of the face in elderly patients. We report a case of sebaceous gland hyperplasia on the vulva. CASE: A 19-year-old woman presented with a polypoid tumor on her left labium majus of which the greatest diameter was 2.5 cm, covered by normal appearing skin. Histologic examination revealed a sebaceous gland hyperplasia. Immunohistochemical techniques showed high expression of androgen receptors on sebaceous cells. CONCLUSION: Clinical and gross features of the two documented cases of sebaceous gland hyperplasia on the vulva are different from those of typical lesions on the face. Histologic study of a vulvar biopsy is required to reach a specific diagnosis of this hamartomatous benign condition.

陰毛の個人内における形態学的変動

This paper presents a study of the intra-individual variation of pubic hairs. Seventy pubic hairs were pulled from seven sites around the outer genital area of each of five Japanese males; sixty were obtained from six sites on each of four Japanese females, and fifty from six sites on one additional Japanese female. These hairs were examined with respect to twenty-two morphological characteristics. These morphological data were studied in relation to each site and analyzed by the statistical method of cluster analysis. It appeared that a pubic hair's length, luster, diameter, medullary index, disease and scale damage bore some relation to the site from which it was obtained. Dendrograms produced through the cluster analysis showed a hierarchy of similarities among all pairs of pubic hairs. Similar hairs were placed together into several large or small groups within the same person. Some groups contained pubic hairs from many sites, whereas others were almost exclusively from only one site. This means that some sites have different characteristics from other sites. Pubic hairs obtained from the penis area, scrotum and labium majus area showed greater dissimilarity than other sites within the same person. Therefore, in forensic hair comparison, known pubic hair samples should be obtained from many different sites. For males, at least thirty-five pubic hairs should be collected from seven sites (center, left and right abdomen; upper, left and right area surrounding the penis; and scrotum). For females, at least thirty pubic hairs should be collected from six sites (center, left and right abdomen; mons pubis; left and right labium majus).

Vulvar angiokeratoma following radical hysterectomy and radiotherapy

Vulvar angiokeratoma following radical hysterectomy and radiotherapy