Labia Majora Research Articles

Cavernous lymphangiomas involving bilateral labia major after caesarian section

Sir, Cavernous lymphangioma is an extremely rare benign mass in the vulva. In this study, we report a case of cavernous lymphangiomas involving the bilateral labia major after caesarian section. A 31-year-old woman with a history of previous caesarian section, G2 L2 that was delivered by caesarian section seven months before, referred to Shahid Beheshti Hospital in 2011 with symmetrical vulvar lesion that was onset form five months ago. The lesion was a small red papule similar in appearance to nevus or angium in the bilateral labia major [Figure 1]. It was removed by punched biopsy. Physical examination was normal. She did not have any particular disease in the past, and no history of radiation therapy or any other pathology. Laboratory data was normal; histological diagnoses showed cavernous lymphangioma [Figure 2]. The management of these lesions depends on type, size, and anatomical location. Two cases of cavernous lymphangioma involving the unilateral labium majora in a young woman[1] and involving the bilateral labia minor were reported.[2] In previous studies, it was discussed that increase in estrogen and progesterone levels may play a role in cavernous lymphangioma; however, in this case it was seen that after caesarian section, estrogen and progesterone levels were decreased. Hence, it can be concluded that there are other factors that might affect this lesion. Further studies with more cases are required to make a fair conclusion. Figure 1 Histological examination of cavernous lymphangioma of labia majora. This section reveals cavernous vessels in the dermis that contain only lymph without any red blood cells Figure 2 Gross picture of bilateral cavernous lymphangioma of thein labia majora

Angiomyofibroblastoma of the vulva

Angiomyofibroblastoma (AMFB) is an uncommon benign mesenchymal tumor. AMFB occurs almost in the vulvo-vaginal area of women. The gross features of AMFB are well-circumscribed so it clinically is often thought as Bartholin gland cyst or aggressive angiomyxoma. Usually, most tumors grow slowly, and patients do not feel pain. It also has low tendency for local recurrence. The histologic findings of the tumors are abundant thin-walled blood vessels with hypocellular and hypercellular areas. Almost all tumor cells have immunoreactivity for both desmin and vimentin. It also has estrogen and/or progesterone receptors, but staining for cytokeratin is negative. Here is a case of AMFB of the vulva occurring in a 40-year-old woman, involving the right labia majora. The patient described that her vulva mass grew in about few months. The maximum dimension of the tumor was measured as 2 cm, and we resected the tumor one month after as her second visit.

Water tubing injury: An overlooked boating injury statistic

A 23-year-old female sustained a large avulsion injury to the groin while water tubing on a local waterway. The laceration began at the mons pubis, extended along the labia majora with involvement of the external anal sphincter. The pubic bone and the pectineus muscle were exposed. Six weeks after surgery, she had resumed full activity without sequlae. Lack of data and the potential for significant injury caused by water tubing as described in this case report are a real concern. Water tubing injury should be classified as a primary accident type and analyzed by the U.S. Coast Guard.

Recurrent Proliferating Trichilemmal Tumor of the Vulva

Proliferating trichilemmal tumor (PTT) is a rare but morphologically distinct tumor that usually arises on the scalp of elderly women. It is composed of multiple cysts consisting of squamous epithelium with trichilemmal keratinization without granular layer interposition. Vulvar proliferating trichilemmal cyst is very rare, with, to the best of our knowledge, only 3 cases previously reported in the literature. We describe a 39-year-old woman with recurrent PTT on the left labium majus of the vulva, which had been excised from the same side 5 years before. She had a palpable nodule, approximately 2 cm in size, which was firm, mobile, and nontender; without erythema and ulceration; and covered by normal skin on the vulva. There was no inguinal lymphadenopathy. The lesion was removed by wide surgical excision; because of the tissue elasticity, primary closure was possible. The pathology result was reported as proliferating trichilemmal carcinoma with tumor-free margins. Although local recurrence after wide excision is rare, we recommend complete excision for treatment of PTT and long-term follow-up because of the possibility of recurrence.

Case 2: A 14-year-old girl with oral and genital ulcers

A 14-year-old girl of Lebanese descent presented with a one-year history of intermittent, painful vulvar ulcers. The patient denied prodromal symptoms or vaginal discharge, and she had never been sexually active. The history was also remarkable for painless oral ulcers recurring every month, a three-month history of headaches and occasional upper abdominal pain. Before presentation, she was treated for conjunctivitis with antibiotic drops. She gave no history of visual disturbances, ocular pain, rashes or arthritis. The review of systems was otherwise negative, and her history was noncontributory. Initial examination revealed a well-appearing, afebrile, adolescent girl with two minor (<10 mm) oral ulcers on her tongue and two minor, healing, genital ulcers on her left labium majus. Her general physical examination was otherwise normal. She was found to have bilateral papillitis and panuveitis on a screening ophthalmological examination. Her complete blood counts were normal. Her inflammatory markers were elevated: her erythrocyte sedimentation rate was 54 mm/h (normal 1 mm/h to 10 mm/h) and her C-reactive protein was 190 nmol/L (normal 0 nmol/L to 76 nmol/L). Electrolyte levels, renal function and liver enzyme levels were normal. An infectious work-up was negative, which included testing for herpes simplex virus among other sexually transmitted infections. A screening rheumatological work-up was negative, including antinuclear antibody for systemic lupus erythematosus, antineutrophil cytoplasmic antibody for vasculitis, and serum calcium and angiotensin-converting enzyme for sarcoidosis. A urinalysis was positive for trace ketones and 1+ protein, and negative for blood, nitrites and leukocytes. Magnetic resonance imaging (MRI) of the head and orbits revealed bilateral, nonspecific, scattered small foci of abnormal signal in the bifrontal white matter but no abnormal enhancement of the optic nerves. A cerebral angiogram and lumbar puncture were normal.

A case report of vulvar lipoma

A lipoma is a benign tumor composed of adipose tissue and it is considered as the most common form of soft tissue tumor. Lipomas are soft on palpation, usually movable, and are generally painless. They are widely disseminated benign mesenchymal neoplasms commonly found over the nape of the neck and upper back, shoulders, abdomen, buttocks, and proximal portions of the extremities. Their occurrence in the vulva is said to be so rare that only a few cases have been reported. This case describes a patient; 47 years old who had admitted to our clinic with complaint of vulvar mass localized on labium majus approximately 1 year. Total excision of the mass was made and the pathological examination result was lipoma. Vulvar lipoma occurs in rare and should be kept in mind in the differential diagnosis of the other vulvar masses.

Late Recurrence of Aggressive Angiomyxoma of the Vulva

Aggressive angiomyxoma (AA) is found mainly in the pelvis and perineum, with a female-to-male ratio of approximately 6:1. Although it is a slow growing tumor, AA has a marked tendency to local recurrence with a low metastasis capacity. The study aimed to describe a case of vulvar angiomyxoma recurred almost 20 years after its initial surgery. We report the case of a 57-year-old gravida 5 para 4 woman with vulvar AA arising from the left labium majus, which recurred 20 years after initial surgery. There was a nontender, solid, mobile mass on the left vulva, which was 25 x 30 cm on physical examination. A pelvic computed tomographic scan showed a mass measuring 26 x 10 x 14 cm originating from left vulvar region, which has a fatty tissue density. Under general anesthesia, total excision of the tumor was performed. Macroscopically, the tumor weighed 723 g and measured 33 x 20 x 10 cm. The histopathological examination of the specimen revealed a myxoid tumor with sparse infiltrates of spindle-shaped to stellate cells and vessels of varying sizes. The final histopathological diagnosis was AA. Aggressive angiomyxoma may form extremely large tumors, and recurrence is not rare even many years after primary surgery.

Vulvar Fibrous Hamartoma of Infancy

Fibrous hamartoma of infancy is a benign mesenchymal tumor occurring infrequently in children, typically involving the axilla. An 18-month-old girl with a history of right labium majus enlargement, on examination, had a hard mass that was found strictly adherent to subcutaneous tissue and overlying skin. Postexcision histological examination showed arranged spindle cells, adipose tissue, and nests of immature small cells in a myxoid background, consistent with fibrous hamartoma of infancy. The main problem in the diagnosis is differentiating this lesion from soft tissue sarcomas, which require an aggressive therapeutic approach. Both surgeons and pathologists need to be aware of the existence of such benign condition in this unusual place to avoid unnecessary therapies.

Vulvoscopy

Vulvoscopy with photography involves examination of external female genitalia using a binocular vulvoscope/colposcope with 4x, 6x, 10x, 16x and 25x magnification and light source. Indications for evaluation include vulvo/vestibulodynia and dermatologic lesions. The cotton swab test is performed to evaluate localized vestibular pain. The “vinegar test” can identify suspect lesions (condylomata/warts, lichen): application of diluted acetic acid (3–5%) to the skin using a cotton wad or gauze and evaluation of area for suspect whitening after > 1 minute. Photographs in figure 1 show: Hart's line on the inside of labia minora between vulva (keratinized squamous epithelium) and vestibule (non‐keratinized squamous epithelium); an epidermal cyst of the vulva/right labia majora; and fibroma pendulans (Acrochordon, skin tag) on the left inner thigh.

Fornah v Secretary of State for the Home Department, House of Lords [2006] UKHL 46, [2006] 3 FCR 381, [2007] 1 All ER 671

FEMALE GENITAL MUTILATION – VIOLENCE AGAINST GIRLS AND WOMEN AS A PARTICULAR SOCIAL GROUPZainab Esther Fornah was fifteen years old when on 15th March 2003, as an unaccompanied minor, she arrived at UK’s Gatwick airport claiming asylum. As she was a child she was taken into the care of West Sussex Social Services Child Asylum Team. She had fled Sierra Leone where she had been captured by rebels, who had killed her family, and was repeatedly raped. She did not want to return to her uncle’s village fearing that she would be forcibly genitally mutilated, as was customary practice. Whilst her father was alive he was able to protect her from this practice. Female genital mutilation, has variously been described as female circumcision and as clitoridectomy, as if to render benign this very specific inhumane habituated practice against females. It involves cutting away the clitoris, labia minora, labia majora and vulva of a female. The area remaining is then sewn together leaving a small aperture to allow for menstruation and urination. This maiming is often perpetrated without anaesthetic and some children do not survive. The World Health Organisation defines this particular form of torture, somewhat blandly, as “the partial or complete removal of the external female genitalia or other injury to the female genital organs whether for cultural or any other non-therapeutic reason.

Langerhans cell histiocytosis associated with lichen sclerosus of the vulva: case report and review of the literature

Langerhans cell histiocytosis (LCH) is characterized by a clonal proliferation of bone marrow-derived Langerhans cells. While cutaneous involvement is relatively common, LCH restricted to the vulvar area is a rare phenomenon and can occur in different clinical settings. Occasionally, vulvar LCH heralds subsequent multi-organ involvement with an aggressive clinical course. Even cases of LCH isolated to the vulvar area can present with local recurrences despite excision and radiation. We present a case of a 68-year-old female with a 1-month history of pruritic lesions on her vulva. Physical examination showed whitish plaques with scattered nodular areas on the labia majora. A vulvar biopsy showed a background of lichen sclerosus (LS) with foci of oval to polygonal cells with moderately abundant eosinophilic cytoplasm and folded nuclei showing frequent nuclear grooves. Immunohistochemical staining showed that the cells were positive for CD1a and S-100, confirming the diagnosis of LCH. On further workup, there was no evidence of disseminated disease involving other organs. While vulvar LCH is uncommonly seen, and with only one previous case report in the literature associated in the setting of lichen sclerosus, this case illustrates the importance of recognizing this condition and ensuring proper clinical follow-up to rule out a systemic involvement.

Giant condyloma acuminatum of vulva treated by surgical excision and reconstruction of defect

Giant condyloma acuminatum (GCA) is a rare sexually transmitted disease caused by Human papilloma virus (HPV) and characterised by invasive growth. Very few cases of GCA of vulva have been reported till now in the English literature [1–3]. We report a case of 34-year-old lady with GCA of vulva who was successfully treated by simple vulvectomy and reconstruction of the defect by ‘‘four-flap technique’’. A 34-year-old, married woman presented with complaints of growth on her vulva for the last 8 months. The patient was 6 months pregnant when she first noted the lesion and did not seek any medical help. Two months after her caesarean delivery, she underwent cryosurgery, but with no improvement. She denied any history of sexual promiscuity. On examination there was a 14 cm 9 10 cm 9 6 cm friable, foul smelling growth on her vulva involving both labia majora, extending anteriorly to mons pubis and posteriorly to perianal region (Fig. 1a). There were a few satellite lesions on labia minora and perianal region. Clitoris, urethra and external anal sphincter were not involved. Colposcopic, proctoscopic and oropharyngeal examinations were normal. The patient and her partner were screened negative for sexually transmitted diseases and high-risk HPV DNA. Incisional biopsy showed squamous cell papilloma with koilocytic changes and it was positive for HPV DNA type 6. A simple vulvectomy, achieving wide margins, was performed. Small lesions in perianal area and labia minora were also excised. Reconstruction of vulva was performed using ‘four flap technique’ (personal communication by Dr. Prakash V, Department of plastic surgery, Safdarjung Hospital, New Delhi). In this technique, four random pattern flaps in the dimension of 1:1 or 1:2 were raised adjacent to the defect and advanced medially to resurface the defect. This resulted in defect being transferred laterally, but this defect was smaller than the original defect and could be closed primarily (Fig. 1b). Final histopathology report confirmed the diagnosis of condyloma acuminatum with mild degree of dysplasia. Functional outcome was good with no sexual complaints. She remained free of recurrence at 12 months of follow-up (Fig. 1c). Many treatment strategies have been documented in the literature for management of GCA, but mainly in the form of case reports. Complete surgical excision with histologically clear margins, with or without adjuvants, is the mainstay of the treatment for GCA, including that of vulva [1, 3]. Other modalities include combined radiotherapy and chemotherapy, topical agents (e.g. podophyllin, 5-FU, imiquimod, bleomycin) and intralesional injections of interferons [4–6]. The skin defects created after excision of GCA in the peri-anal region can be managed with mesh skin grafting, flaps and even healing by secondary intention [1, 7–9]. Flaps have been shown to give better results for reconstruction of vulva [8, 9]. The additional advantage of our ‘four flap technique is that the scars do not extend over the perineum or thighs. In our patient, the postoperative period P. Mittal (&) R. Gupta R. Dewan S. Singhal J. Suri Department of Obstetrics and Gynaecology, VMMC and Safdarjung Hospital, New Delhi 110029, India e-mail: drpratima@hotmail.com

Should a Psychiatrist Give Legal Advice on Cosmetic Genital Surgery?

The UK Female Genital Mutilation (FGM) Act 2003 states that‘‘A person is guilty of an offence if he excises, infibulates or otherwise mutilates the whole or any part of a girl’s [or woman’s] labia majora, labia minora or clitoris.’’ In a case report about a woman undergoing clitoridectomy following a previous unsatisfactory cosmetic labiaplasty, Veale and Daniels (2012) noted that ‘‘Anatomically, there is little to distinguish FGM from some of the procedures involved in carrying out legitimate surgery.’’This is correct, but begs the question as to who decides the legitimacy of such surgery. TheAct states that‘‘no offence iscommittedbyanapproved person who performs a surgical operation on a girl which is necessary for her physical or mental health’’but also that‘‘...it is immaterial whether she or any other person believes that the operation is required as a matter of custom or ritual.’’ Is cosmetic surgery as performed in clinics such as Restore UK (http://www.gynecosmetics.com/) really necessary for mental health or are we witnessing the emergence of new culturally determined customs and rituals? More pertinently, will patient consent protect surgeons from criticism? Veale and Daniels (2012) stated that‘‘The role of the mental health specialist is to assess and advise the surgeon on the wisdom and legitimacy of carrying out the proposed procedure.’’ Veale remained ‘‘very uncomfortable’’ about advising a go-ahead (presumably thinking it unwise). He admits that perhaps he should have spoken to the husband or obtained an ethical opinion (Veale, 2012). He does not state whether he had any conflict of interest with respect to payment for the consultation or whether he took legal advice. Nevertheless, ‘‘The advice [he gave] to her surgeon was that a clitoridectomy could be exempted from the Female Genital Mutilation Act for cosmetic reasons. The rationale was that, if labiaplasty can be done for cosmetic reasons for perceived abnormality, then there was no reason that clitoridectomy could not be done on the same grounds’’ (Veale & Daniels, 2012, p. 728). This is simply wrong. Doctors do not decide legitimacy. It is a matter for the Courts. Did the authors, reviewers or Editor consider contacting a Medical Defence Organization, the General Medical Council or the police? It seems rash to advise patients or publish articles regarding a potential serious criminal offence without properly investigating the legality.

Vulval angiomyofibroblastoma

A 25-year old woman presented to the Gynaecology Clinic at Base Hospital, Diyatalawa with a history of a lump in the right labium majus for 3 month duration. The lump was surgically excised. The histopathological diagnosis was an angiomyofibroblastoma (AMFB) a rare benign mesenchymal tumour arising in the genital region predominantly of the pre-menopausal women. DOI: <a href="http://dx.doi.org/10.4038/sljog.v33i4.4807">http://dx.doi.org/10.4038/sljog.v33i4.4807</a> <em>Sri Lanka Journal of Obstetrics and Gynaecology </em>2011; <strong>33</strong>: 168-169

A rare case of inguinolabial lipoblastoma in a 13-month-old female

Lipoblastoma is a rare, rapidly growing, benign mesenchymal tumor composed of various stages of maturing adipocytes that most often occurs in children under the age of 3. The common locations are the extremities and the trunk. Presentation in the genitoinguinal area is rare. We report a case of a 13-month-old female infant with a 4-month history of a progressively enlarging left labial mass that encompassed her left labium majora and inguinal region. Pelvic MRI confirmed growth from previous ultrasound size of 3 × 2 × 1 cm to 7 × 2 × 2 cm. Composition was suggestive of adipose tissue. The mass was excised through a left inguinal incision. The final pathology results described a lipoblastoma. Six year follow-up has not revealed any signs or symptoms of recurrence.Circumscribed lipoblastomas should be distinguished from their infiltrative counterpart, diffuse lipoblastoma or lipoblastomatosis, which can be more difficult to excise and thus, more likely to recur. Lipoblastoma should also be distinguished from myxoid liposarcoma, which has malignant features, carries a high risk of recurrence, and requires a more aggressive management protocol. Although rare, lipoblastoma should be considered as part of the differential diagnosis of a rapidly growing vulvar mass in prepubertal children.

Mammary gland in vulva (rare clinical case)

A rare clinical case of a patient with fibroadenoma of the vulva’s right labium majus with a typical hystological structure of mammary grand is described. The patient noticed a tumor of the right labium majus approximately 10 years before admission. After the childbirth while breastfeeding patient noticed a whitish discharge form the tumor which looked like breast milk. At the oncologic dispensary biopsy was performed after the tumor punction, atypical basal epithelium was found, and the basal cell carcinoma was suspected. Histological examination revealed fibroadenoma with a typical hystological structure of mammary grand.

Vulvar Metastasis From Bilateral Lobular Breast Carcinoma

INTRODUCTION: The invasive lobular carcinoma is the second most frequent pathological type of breast cancer, accounting for about 5-10% of breast carcinomas. Recent studies suggest an increase in incidence in relation to invasive ductal carcinoma, especially in women over age 50. The CLI has characteristic patterns of metastasis, showing the most commonly bilateral and metastases to peritoneum, gastrointestinal tract, gynecological organs, bone marrow and leptomeninges. The case reported below refers to a patient treated at the Hospital Aristides Maltez - BA, in 2002. REPORT: C. L. S. , 77 years old woman, sent to Mastology department by the Gynecology’s sector from the Hospital Aristides Maltez on 03/09/2010, whose main complaint was a lump in the vulva for 03 years. The patient had noted a small lump in the vulva for about 03 years, with faster growth in the last 06 months. Vulvar examination showed bulging of the right labia majora with almost complete erasure of the small ipsilateral lip. Hard and irregular mass of about 2.5 x2, 0cm in the right labium, which bulged the vaginal opening. We performed an incisional biopsy of that lesion which was consistent with invasive carcinoma with standard immunohistochemistry resembling ductal carcinoma of the breast. Under evaluation by mastologist there were no founds to inspection, there was a palpable dense area of about 02cm in upper outer quadrant (UOQ) right breast as well as in the union of the upper quadrants (UUQ) in the left breast, another nodule in the left breast measuring 01 cm fibroelastic consistency in UOQ. In the left axilla there was a lymph node measuring 01 cm with fibroelastic consistency. There was no nipple discharge. Mammography showed focal asymmetry in right breast union of lower quadrants (ULQ), Bi-Rads 0. Left breast presenting benign vascular calcifications, Bi-Rads 2. Ultrasound showed a heterogeneous hypoechoic poorly delimited area, measuring about 13. 3 x 6, 7mm, which may correspond to solid nodule in the right breast. The left breast showed a hypoechoic heterogeneous area in UUQ, measuring 10. 3 x7, 5mm. We performed core needle biopsy of palpable areas, the result was compatible with invasive lobular carcinoma in both breasts. The staging tests were normal, except for the chest x-ray showed diffuse interstitial infiltrate predominating in lung bases. The patient died before starting treatment due to respiratory complications. DISCUSSION: This case is relevant to show an unusual site for metastasis from infiltrating lobular carcinoma. It also shows the importance of inclusion of mammary carcinoma in the differential diagnosis of vulvar diseases.

Interstitial magnetic resonance lymphography is an effective diagnostic tool for the detection of lymph node metastases in patients with cervical cancer

BackgroundThe aim of the present study was to determine the feasibility of detecting sentinel lymph node (SLN) metastases using interstitial magnetic resonance (MR) lymphography in patients with cervical cancer. MR data were compared to pathological results from the lymph nodes excised during surgery.MethodsTwenty-eight patients with cervical cancer were enrolled and studied from January 2006 to December 2010. All patients underwent interstitial MR lymphography to determine the presence of sentinel lymph nodes and visualize lymphatic vessel drainage in the pelvis. Radical hysterectomy and excision of pelvic lymph nodes was performed according to their lesion grade. Gadodiamide was injected either intradermally into the bipedal toe web, into the labia majora or into the cervical tissue. MR results were compared with pathological reports.ResultsIn 28 patients, lymphatic vessel drainage and lymph node groups were clearly visualized. Of these, 5 were MR lymphography positive and 23 were MR lymphography negative. Six had pathologically proven metastasis, five had true positives and 1 had a false negative in the obturator lymph node.ConclusionsInterstitial MR lymphography can be used to determine the extent and shape of pelvic lymphatic vessel drainage and lymph node metastases in patients with cervical cancer.

Hybrid Schwannoma/Perineurioma

Hybrid schwannoma/perineurioma is a recently described benign nerve sheath tumor that typically manifests as a dermal tumor on the extremities and trunk. Occurrence outside the skin is uncommon. This article describes 10 cases of hybrid schwannoma/perineurioma arising in diverse anatomic locations. They all occurred in adult patients (age range 27-81 years, median 35 years) with a marked female predominance (2 males and 8 females). Of the 10 tumors, 7 were situated in the subcutis of trunk (n = 3), extremities (n = 2), neck (n = 1), and labium majus (n = 1) and 3 in the submucosa of nasal cavity, sigmoid colon, and rectum, respectively. Histologically, they were composed of intimately admixed plump spindle cells and elongated slender spindle cells forming storiform, lamellar or fascicular architecture. By immunohistochemistry, the tumor showed dual differentiation of schwannian cells (plump-spindled) and perineurial cells (slender-spindled), characterized by strong S100 protein expression in the former component and variable immunoreactivity of epithelial membrane antigen, claudin-1, and CD34 in the latter. Awareness of its morphological characteristics and potential occurrence in diverse sites may aid in the recognition of this rare tumor type.

Primary Vulvar Ewing Sarcoma/Primitive Neuroectodermal Tumor: A Report of One Case and Review of the Literature

BackgroundEwing sarcoma/primitive neuroectodermal tumors (ES/PNET) are aggressive malignancies characterized by translocation involving the EWS gene. Primary extraskeletal ES/PNET rarely affects the female genital tract. CaseWe report the case of a 20-year-old female, who presented with a rapidly growing painful mass arising from the left labium majus for six months. Multiple lesions in bones and nodules in lungs were considered metastasis. The diagnosis was established by immunohistochemical stain and reverse transcriptase polymerase chain reaction analysis. The patient ceased treatment and died of respiratory failure one month after discharge. ConclusionVulvar ES/PNET mainly affects adolescent females. ES/PNET should be considered in the differential diagnosis of tumors involving the lower gynecologic tract in adolescent female.