Differential Diagnosis of a Unique Vulvar Mass in an Adolescent.

BACKGROUND: Vulvar masses in adolescents have a broad differential diagnosis, yet few reports exist detailing masses of mammary origin. CASE: A nulliparous, healthy 16-year-old adolescent presented with a longstanding, ulcerated, 17-cm vulvar mass of unknown origin and pronounced inguinal lymphadenopathy. The patient underwent a left radical partial vulvectomy, with pathology revealing terminal duct lobular units consistent with polymastia. CONCLUSION: Differential diagnosis of a vulvar mass in an adolescent should include polymastia.

Úlceras vulvares: un reto diagnóstico / Vulvar ulcers: A diagnostic challenge

Las úlceras vulvares son una entidad patológica relevante por su repercusión en la calidad de vida de la mujer y suponen un desafío diagnóstico dada la variedad etiológica y de presentación clínica. El diagnóstico se basa en la anamnesis, la exploración física detallada y pruebas complementarias.Presentamos el caso clínico de una paciente de 39 años con diagnóstico de úlceras vulvares graves, recidivantes, que requirió ingreso hospitalario prolongado en tres ocasiones. Se describe el proceso de identificación, los posibles diagnósticos diferenciales y el resultado del tratamiento aplicado.(AU)

Vulvar ulcers are an important pathological condition due to their impact on the quality of life of women. It is a diagnostic challenge given the aetiological variety and clinical presentation. The diagnosis is based on anamnesis, detailed physical examination, and complementary tests.The clinical case is presented of a 39-year-old patient diagnosed with severe, recurrent vulvar ulcers, and who required prolonged hospital admission on three occasions. The diagnostic process, possible differential diagnoses, and the outcome of the applied treatment are described.(AU)

Female congenital aphallia: a unique case of congenital absence of the clitoris with an ectopic labium majorum.

The spectrum of disorders of sexual development includes anatomical abnormalities of the external genitalia, the phenotypic variability of which and the underlying causes are numerous. However, female aphallia and ectopia of the labium majorum appear to be some of the rarest forms of external genitalia malformations. Aphallia is mostly described in males with a frequency of less than one per 40 000 male newborns. Although syndromic forms of aphallia in females have been reported, for example, in Robinow, CHARGE, and Prader-Willi syndrome, reports of isolated female aphallia are meager. Here, we describe the first case of isolated agenesis of the clitoris with an ectopic labium majorum and review the literature of this uncommon malformation and its potential dysmorphogenetic mechanism. We emphasize the need for a routine exhaustive physical examination to identify and characterize this unusual malformation correctly so that families can be appropriately counseled as to cause and potential complications.

A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues

Only 14 cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been documented in the literature. We report a case of a 63-year-old woman with EMC of the vulva confirmed by both EWSR1 and NR4A3 fluorescence in situ hybridization, the latter of which is a more specific probe for this entity. The unusual location of this tumor of prominent myxoid morphology gave rise to a wide differential diagnosis, which necessitated thorough histologic evaluation and confirmatory ancillary testing in the form of immunohistochemistry and cytogenetic studies. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva and various diagnostic clues to help differentiate it from its histologic mimics. This is the fifth case of vulvar EMC in the literature with confirmation of a NR4A3 gene rearrangement.

[Iatrogenic urethral coitus]. / Iatrogene urethrale coïtus.

This case discussion on urethral coitus in Mayer-Rokitansky-Küster-Hauser syndrome shows that this rare syndrome can have major physical and psychological consequences. Further consideration shows that a lack of common sense and a lack of action concerning sexual history-taking and careful vulvar examination is the reason that an iatrogenic sexual problem arose in this case. This was not the result of the initial denial of a rare diagnosis. Both attention to these aspects during training and supervision are necessary, since other disorders and medical treatments can also have a negative impact on sexuality.

Successful Surgical Correction of Congenital Colonic Duplication and Anogenital Cleft in a Cat.

A 17 wk old sexually intact female domestic shorthair kitten presented for an anogenital cleft and enlarged colon. The cat had experienced bacterial cystitis and constipation since weaning. Contrast referral images revealed an enlarged colon with a patent anus. Clinical examination revealed an anogenital cleft with a common anovulvar orifice. The rectum was patent upon digital rectal palpation, and fecal contamination of the vulva was present. Abdominal radiographs revealed two distinct colons, both filled with a moderate amount of formed fecal material. Contrast-enhanced computed tomography revealed segmental duplication of the descending colon with a dominant right colon and a smaller accessary left colon. The two structures conjoined at the transverse colon proximally and at the pubic brim distally. A common anogenital orifice with anovulvar communication was also noted. The anogenital cleft malformation was successfully repaired surgically. A celiotomy was performed to remove the smaller accessory colon. An ovariectomy and partial hysterectomy were also performed. The patient recovered uneventfully and showed no gross evidence of recurrent cystitis or urinary or fecal incontinence postoperatively. This is believed to be the first report of a congenital anogenital cleft and complete communicating colonic duplication in a cat.

Self-Resolving Vulvar Breast Tissue Arising in the Post-Partum Setting: A Case Report and Review of Literature.

INTRODUCTION: During the postpartum period, breast engorgement in preparation for lactation may trigger the onset of vulvar labial nodules that present with pain and discomfort. These labial nodules may originate from ectopic breast tissue and can rarely present in women during the postpartum period in the labia majora. MAIN ISSUE: A 37-year-old African American female, gravida 1 para 1, presented to the Loma Linda University Medical Center with complaints of new onset labial swelling worsening 6 days following her full term spontaneous vaginal delivery. Additionally, our respondent complained of difficulty breastfeeding due to intermittent lack of milk production. She reported bilateral breast engorgement with tenderness, despite frequent attempts at breastfeeding. The respondent's presentation did not meet the criteria for other common differentials due to the physical characteristics of the nodules. The location of the nodules along the milk line led physicians to believe that the respondent was presenting with engorged extra-mammary breast tissue in the labia majora. MANAGEMENT: The participant was told to observe her course over the next few days as she began to have milk production and ejection. The respondent was seen in clinic for her 6-week postpartum visit, and was no longer complaining of difficulty with breastfeeding. The labial nodules had resolved spontaneously. CONCLUSION: A literature search yielded no case reports that described a case of an extra-mammary vulvar mass that self-resolved with resolution of breast engorgement. The infrequent presentation of extra-mammary vulvar tissue makes it difficult to conclude a guideline for diagnosis and management.

A Case of Vulvar Cavernous Lymphangioma.

Cystic (cavernous) lymphangioma of the vulva is a benign tumor of lymphatic vessels with only 10 cases previously reported in the medical literature worldwide. The following is a case of bilateral vulvar cavernous lymphangiomas in a 23-year-old woman with bilateral soft tissue masses of the labia majora. Prior unsuccessful management included aspiration, incision, and drainage. Surgical marsupialization of both masses led to resolution and the subsequent histologic diagnosis of cavernous lymphangioma. Although rare, cavernous lymphangioma should be included in the differential diagnosis of soft tissue masses of the distal labia. Cavernous lymphangioma can mimic other more common vulvar soft tissue masses such as Bartholin's cyst.

[Feminizing genitoplasty in virilization of the external genitalia].

The review focuses on the feminizing genitoplasty of the external genitalia in patients with disorders of sex development. The opinions of various surgeons and surgical schools on the timing, stages and methods of performing feminizing genitoplasty in girls with the virilization of the genitalia are presented. The development and improvement of surgical techniques for performing clitoroloplasty in patients with virilization of genitalia are described, as well as different types of labioplasty. The main methods of reconstruction of the urogenital sinus are given.

Elefantiasis vulvar por filarias / Vulvar Elephantiasis by Filarias

En el año 2000, había más de 120 millones de personas infectadas por filarias en el mundo; unos 40 millones están desfiguradas e incapacitadas por la enfermedad. La infestación de los ganglios linfáticos por el parásito Wuchereira bancrofti, es la causa más frecuente del linfedema secundario. Se presenta un caso inusual de elefantiasis vulvar por filariasis. Se analizan las alternativas terapéuticas que incluyen el tratamiento farmacológico y quirúrgico, con el fin de considerar esta última como un tratamiento paliativo más que curativo tanto para el linfedema como para la cirugía reconstructiva(AU)

In 2000, there were more than 120 million people infected by filarias in the world; some 40 million are disfigured and disabled by the disease. Infestation of the lymph nodes by Wuchereira bancrofti parasite is the most frequent cause of secondary lymphoedema. An unusual case of vulvar elephantiasis due to filariasis is reprted in this paper. The therapeutic alternatives that include pharmacological and surgical treatment are analyzed, in order to consider the latter as a palliative rather than a curative treatment both for lymphedema and for reconstructive surgery(AU)

Developmental and family history-based analysis of congenital fused labia phenotype in the captive common marmoset (Callithrix jacchus).

BACKGROUND: Congenital fused labia (CFL) is defined as a failure or significant delay in the opening of the juvenile sealed labia majora. This phenotype is known to be variably common in adult captive female marmosets but has never been investigated in detail before. MATERIALS AND METHODS: Here, we define, describe and quantify the variations in the degree of closure of the vulva in 122 captive marmosets (Callithrix jacchus) from 1.2 to 42 months old and include colony analysis. RESULTS: There was a negative correlation between the degree of labial fusion and animal age after prepubertal period (P < 0.05). CFL females had higher number CFL relatives (4.3 ± 0.6 vs 2.4 ± 0.5 for non-CFL, P < 0.05) and more external ancestors compared to non-CFL (P < 0.05). CONCLUSIONS: Our results therefore suggest that CFL phenotype is most likely associated with epigenetic effects induced by the captive environment and colony management strategy of extensive crossing of family lines to promote heterozygosity.

AP endonuclease EXO-3 deficiency causes developmental delay and abnormal vulval organogenesis, Pvl, through DNA glycosylase-initiated checkpoint activation in Caenorhabditis elegans.

AP endonuclease deficiency causes cell death and embryonic lethality in mammals. However, the physiological roles of AP endonucleases in multicellular organisms remain unclear, especially after embryogenesis. Here, we report novel physiological roles of the AP endonuclease EXO-3 from larval to adult stages in Caenorhabditis elegans, and elucidated the mechanism of the observed phenotypes due to EXO-3 deficiency. The exo-3 mutants exhibited developmental delay, whereas the apn-1 mutants did not. The delay depended on the DNA glycosylase NTH-1 and checkpoint kinase CHK-2. The exo-3 mutants had further developmental delay when treated with AP site-generating agents such as methyl methane sulfonate and sodium bisulfite. The further delay due to sodium bisulfite was dependent on the DNA glycosylase UNG-1. The exo-3 mutants also demonstrated an increase in dut-1 (RNAi)-induced abnormal vulval organogenesis protruding vulva (Pvl), whereas the apn-1 mutants did not. The increase in Pvl was dependent on UNG-1 and CHK-2. Methyl viologen, ndx-1 (RNAi) and ndx-2 (RNAi) enhanced the incidence of Pvl among exo-3 mutants only when combined with dut-1 (RNAi). This further increase in Pvl incidence was independent of NTH-1. These results indicate that EXO-3 prevents developmental delay and Pvl in C. elegans, which are induced via DNA glycosylase-initiated checkpoint activation.

Unilateral hypertrophy of the labia minora: A case series.

Asymmetric hypertrophy of the labia minora is a variant of normal anatomy that has not been described in the pediatric dermatology literature. Although often asymptomatic, in some cases, it can cause functional, emotional, and psychological problems. We report the clinical characteristics and outcomes of four children who presented with unilateral labium minus hypertrophy. This case series aims to establish awareness of this condition among pediatric dermatologists and provide recommendations regarding management.

Facets of Clinical Appearance and Aetiology in an Unusual Bovine Amorphus Globosus.

Amorphus globosus is a rare entity, more common in the cow but also reported in mares, buffaloes and goats. In respect of both development and clinical presentation, this abnormity can be evolved very variably. Previously, it has been discussed whether it is a form of twin pregnancy or placental teratoma. This case report deals with morphology and genetic observations in an unusual bovine amorphus globosus exhibiting a rudimentary clitoris and vulva, gut-like structures and rudimentary bones. The amorphus globosus was shown to be dizygotic to the normal male twin using the BovineSNP50 v2 BeadChip and had a genetical female sex. Aspects of aetiology and pathogenesis as well as the possible impact of amorphus globosus in the emergence of freemartinism are discussed.

Rare Giant Angiokeratoma of the Vulva: A Case Report.

BACKGROUND: Angiokeratoma of fordyce occurring over on the vulva is a rare condition. Fordyce angiokeratoma is observed more frequently among men than women. In women, it is generally observed in later life, and appears as multiple dark purple papules, measuring 2-4 mm, on the vulva. CASE REPORT: We present the case of a 17-year-old white teenage girl with giant Fordyce angiokeratoma on the right vulva. The angiokeratoma was removed and a V-Y advancement flap was made. CONCLUSION: In the literature, this is the first childhood case reported in which a reconstruction of the vulva was performed.

Ninfoplastía: caso problema / Ninfoplasty: case problem

La hipertrofia de labios menores en una patología frecuente con 6 de cada 10 mujeres afectadas entre los 20 y 40 años. Se trata de una variante anatómica cuya etiología aún es incierta, que se describe como el tejido protuberante localizado tras los labios mayores, ya sean por alternaciones con tamaño o volumen. Se trata de una entidad de carácter benigno cuya síntomatología es muy variada, abarcando desde síntomas leves tales como irritación local o problema con la higiene local, hasta infecciones crónicas, dolor al caminar, disparcunia o problemas psicológicos y estéticos

The hypertrophy of the vulva lower lips is a frecuently pathology: six of every ten women afected, between 20 to 40 years old. it¨s about an anatomical variation whose etiology is still unknown. It´s described as the The hypertrophy of the lower lips is a frecuently pathology: six of every ten women afected, between 20 to 40 years old. It¨s about an anatomical variation whose etiology is still unknown. It´s described as the bulging tissue behind vulva mayor lips.

[Postoperative complications of labia minora reduction. Comparative study between wedge and edge resection]. / Complications postopératoires des nymphoplasties de réduction. Étude comparative rétrospective entre résections longitudinale et cunéiforme.

BACKGROUND: Labia minora reduction interventions rise in Europe and in North America. Several techniques are described. The objective of this study was to compare postoperative complications of the two most practiced interventions: wedge resection and edge resection. METHODS: Primary labia minora reductions realized in our unit between October 2009 and July 2016 have been retrospectively identified. Two techniques were used by two surgeons: edge resection technique and wedge resection technique. The main evaluation criterion was the occurrence and the quantity of wound dehiscence: superior to 50% (total or subtotal) and inferior to 50% (partial). Patients were systematically examined at 1 week, 1 month and 6 months postoperatively. Data analysis between both groups was made with an exact Fisher test. RESULTS: Mean follow-up was 5.3 months after intervention. Sixty-four patients have been included, 42 wedge resections (group C) and 22 edge resections (group L). Global complication rate at 1 month was 13% (n=8). Among wedge resections 14% (n=6) developed complication and 2% (n=9) among edge resection. Seven surgical revisions were necessary: 5 for wound dehiscence (4 in the group C and 1 in the group L) and 2 for hematoma, one in each group. Three (5%) partial wound dehiscence (inferior to 50%) have been identified and let in secondary intention healing: 2 (19%) in the group C and 1 (27%) in the group L. Complication rates between both techniques were not significantly different. CONCLUSIONS: Postoperative wound dehiscence is the main labia minora reduction complication. Our global complication rate, 13%, matches with the current literature. A tendency can be shown where wedge resection is more likely to develop wound dehiscence than edge resection.