Left Labia Majora Research Articles

Atypical Foot Swelling in Metastatic Endometrial Carcinoma: Role of FDG PET/CT.

An 83-year-old woman with a history of endometrial carcinoma (EMC), treated with surgery and chemotherapy in August 2020, presented with a 1-year history of left foot swelling, tenderness, and heat. Despite multiple consultations, no diagnosis was made, and she developed painful swelling with a limping gait. A biopsy was done on July 14, 2023, confirmed metastatic EMC. FDG PET/CT revealed widespread metastases, including in the lungs, pleura, mediastinal, hilar, inguinal, right external iliac nodes, left labia majora, left iliac bone, left tibia, and left foot. Excision of left inguinal and vulvar tumors also confirmed metastatic EMC.

Chemoradiation Therapy as the Treatment of the Adenoide Cystic Cancer of the Bartholin Gland : About One Case Report and Review of the Literature

Adenoid cystic carcinoma (ACC) of the Bartholin's gland is a rare yet clinically significant malignancy Originating from the Bartholin's gland, situated in the vulvar region, ACC exhibits unique histopathological features and clinical behaviors. In our article, we describe a clinical case involving a 28-year-old female patient who initially presented with a painful swelling on the left labia majora. Initially misdiagnosed as simple Bartholin's gland inflammation, biopsy revealed cystic adenoid carcinoma of the Bartholin gland upon histopathological examination. Radiological evaluation revealed a locally advanced left vulvar lesion with no evidence of distant metastasis. Given the impracticability of surgical intervention, concurrent chemoradiation therapy was administered, resulting in favorable outcomes. ACC has been a diagnostic therapeutic challenge, there is currently no established therapeutic standard. Despite its infrequent occurrence, ACC warrants attention due to its potential for aggressive local invasion, distant metastasis, and propensity for recurrence.

A CASE REPORT ON ANGIOMYXOMA OF VULVA - A RARE MESENCHYMAL TUMOR IN REPRODUCTIVE AGE GROUP WOMEN

Aggressive angiomyxoma (AAM) is a mesenchymal tumor that arises from perineal or lower pelvic connective tissue. This tumor is locally aggressive. It has mostly been found in reproductive age group females. The correct diagnosis and follow-up of this tumor are very important, as it has similar presentation to many other vulvar masses and it tends to recur due to its locally aggressive nature. We report here a case of 40-year-old female P2L2, who presented with a soft, non-tender swelling on the left labia majora.

Report of Incidental Vulvar Malignant Melanoma Upon Dermatologic Evaluation for Vaginal Cyst

BACKGROUND: Vulvar malignant melanoma is a rare, often aggressive and recurring disease with a poorly understood prognosis. Here, we describe a patient with a longstanding vaginal cyst who was diagnosed with vulvar malignant melanoma upon a dermatologic evaluation. THE CASE: Our patient, a 53-year-old female, with a family history of a father with malignant melanoma presented for evaluation of a vaginal cyst present for over 20 years on her right labia that had become painful and began draining over the past year. She was seen twice at urgent care and was treated with oral antibiotics (unspecified) and referred to dermatology. A comprehensive dermatologic evaluation of the epidermal inclusion cyst in the right labia majora incidentally revealed a neoplasm of uncertain behavior in the right labia minora suspected for vulvar malignant melanoma (Figure 1). Shave biopsy of her right labia minora revealed a Stage 1B malignant melanoma, superficial spreading type, with a 1.1 mm depth of invasion (Breslow thickness) and 11 per 1mm2 mitotic count. Right partial radical vulvectomy and bilateral inguinal sentinel lymph node biopsies were performed. Pathology showed malignant melanoma with negative margins and negative nodes. Treatment and excision for her vaginal cyst was not initiated as her symptoms were not bothersome at the time of visit. She presented with a post-operative infection after 2 weeks which was suggestive of bilateral inguinal vulval acute cellulitis with no definitive abscess upon computed tomography (CT) scan. She was initially treated with vancomycin with additional piperacillin/tazobactam for a total of three days. Trimethoprim/sulfamethoxazole was later initiated for 14 days. Medical oncology referral for evaluation of metastasis revealed normal positron emission tomography-computed tomography scan. Notably, the patient had extensive confluent/atypical lentiginous basal melanocytic hyperplasia at the periphery which could not be distinguished from melanoma in situ. Upon tumor board consultation, scouting biopsies of her left labia majora were performed. Pathology showed melanocytic hyperplasia instead of residual malignant melanoma at the periphery. The patient now follows up every few months with dermatology and medical oncology. CONCLUSION: Given the often fast, aggressive spread of vulvar malignant melanoma, timely dermatologic evaluations are necessary. Among those at higher risk due to a personal or family history of malignant melanoma, regular total body skin cancer screenings are indicated. Further, the sensitive and typically sun-protected location of a vulvar malignant melanoma might preclude a dermatologic evaluation in this area and highlights the need for more comprehensive vaginal screenings and better communication between dermatologic and gynecologic medical care teams.

Proximal epithelioid sarcoma of the vulva. A rare neoplasm. A clinicopathological case

Epithelioid sarcoma is a rare aggressive soft tissue sarcoma, which can be distal or proximal types. The classic form (distal-type) of epithelioid sarcoma mainly occurs in teenagers and young adults. A rarer form, called large-cell (proximal-type) epithelioid sarcoma, tends to be more aggressive and mainly affects adults. The proximal subtype mostly arises from the proximal pelvis, limbs, and genital tract. We report a case of a 59 -year-old female, presented with a progressively growing mass in the left labia majora. Gynecologic examination revealed a 2 cm mobile and painless mass that was not attached to deep planes. The histological study showed a multinodular tumor was seen comprising sheets of oval to polygonal cells with moderate amount of cytoplasm. Interspersed were larger, rhabdoid cells with abundant eosinophilic cytoplasm and prominent nucleoli. On IHC, the tumor cells showed positivity for EMA and CKAE1/AE3 and do not expressed INI-1 in the nucleus. All tumor cells were negative for S-100 protein and CD34. The histopathological diagnosis was soft tissue of the vulvar region with proximal epithelioid sarcoma. The patient received adjuvant external pelvic radiotherapy and brachytherapy in the vulvar bed. Currently, 3 years after diagnosis, the patient does not present signs of tumor recurrence in her controls. Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. The prognosis is generally poor. A wide excision with clear margins is imperative with options of post-operative CT/RT in individual cases during a close follow-upbehavior, as seen in our case.

Treatment of Cutaneous Lesions in a Case of Blue Rubber Bleb Nevus Syndrome

Blue rubber bleb nevus syndrome (BRBNS), sometimes called Bean syndrome, is a rare sporadic congenital vascular disorder characterized by multiple venous malformations in various organ systems, most commonly the skin and gastrointestinal tract. The presence of characteristic skin lesions is often the first suggestion of this diagnosis, though chronic anemia and intestinal bleeding are also commonly present. We report a case of a patient with painful lesions on the plantar surfaces of the feet and a new cutaneous growth on the left labia majora. The plantar lesions were treated with Nd:YAG laser and the lesion on the labia majora was removed with a shave biopsy.

A CASE OF VUVLVAR ANGIOMYXOMA : REPORT AND LITERATUREREVIEW

Superficialangiomyxoma (SMA) is a rare benign skin tumorwith a peak incidence in middle age . The diseaseiscaused by mutations in the PRKAR1A gene. Which, codes for the type of I alpha regulatorysubunit of the enzyme protein kinase A. We report a rare case of vulvarangiomyxomain 19-year-old patient beneficied of large exicion of mass in the leftlabia majora. the diagnostic of SMA retained, afterhistologicalstudy of surgicalspecimen , The evolution has been good withoutrecurrence.

Vaginal Swelling After Intercourse: A Case Report

Introduction: A vulvar hematoma is a hemorrhagic fluid collection in the female external genitalia. The majority occur as an obstetrical complication, especially during labor. Non-obstetrical vulvar hematomas are usually the result of trauma, with coitus being the most common etiology. Case Report: We present the case of a 25-year-old woman with significant vaginal pain and swelling after vigorous sexual intercourse. She exhibited tenderness and swelling of the left labia majora and minora. The differential diagnosis included bleeding, abscess, and deep venous thrombosis. Laboratory studies were normal and computed tomography of the pelvis indicated the swelling was most likely due to blood. The patient was taken to the operating room, and approximately 150 cubic centimeters of clot was evacuated. The patient had an uneventful recovery and was discharged home the next day. Conclusion: This case illustrates the unique presentation and challenges in making the diagnosis of vulvar hematoma.

Treatment of a Chronic Vulvar Wound by Hyperbaric Oxygen Therapy (HBOT)

We hereby report the case of a 66-year-old obese patient (BMI 30) with type 2 diabetes, who presented a chronic vulvar lesion on the left labia majora following surgical drainage of an abscess. After multiple unsuccessful treatments by antibiotics and local wound care, we proposed a trial of hyperbaric oxygen therapy (HBOT). The patient fully recovered after 54 sessions at 2.5 ATA, 95 minutes each. HBOT has been studied for perineal lesion such as skin atrophy or necrosis caused by irradiation but not for vulvar nonhealing chronic lesions in the case of impaired vascularization caused by diabetes. This case is, to our knowledge, one of the first publications about the healing boost of HBOT in chronic vulvar wounds due to vascular impairment.

Aggressive Angiomyxoma of Vulva

Aggressive angiomyxoma is a rare, slowly growing, and benign tumor of mesenchymal origin that usually occurs in the pelvis and perineum of young females. Steeper and Rosai in 1983, first described angiomyxoma. The term “aggressive” underlines the tumour’s locally infiltrative nature and high risk for local recurrence. The rate of relapse varies from 35% to 72%, however, it rarely metastasizes so it has an overall good prognosis. Diagnosis is mainly made on histopathology after surgical resection. The optimal treatment for aggressive angiomyxoma is wide local excision with tumor free margin. We here report a case of aggressive angiomyxoma in 22 years female who presented with mass on left labia majora.

28581 Vulvar mammary-like adenocarcinoma with overlying extramammary Paget disease

Observations: An 88-year old Asian female with a history of thyroid cancer presented with an itchy 5 cm erythematous inflammatory plaque on the right labia majora extending to the clitoris and left labia majora. Biopsy revealed mammary-like adenocarcinoma beneath extramammary paget disease of the vulva. Breast mammograms revealed no malignancy. The invasive dermal tumor resembled invasive ductal adenocarcinoma of the breast, being positive for cytokeratin 7 and negative for cytokeratin 20. Malignant pagetoid cells were in the epidermis. Immunohistochemical reactions found PR negative, weakly ER positive, 3+ positive for HER2, and S100 negative. Abdominal and pelvic PET/CT images revealed a hypermetabolic area only in the vulvar region. Advanced age advised against chemotherapy. The patient was treated with 4500 cGy intensity-modulated radiation therapy followed by 2160 cGy boost to the vulva and pelvic lymph nodes. A month after completion, there were no complications. She died 8 months later, cause not known.

Vulvar pyogenic granuloma: A rare location

Pyogenic granuloma is an acquired vascular tumor. It most commonly presents on head, neck, trunk, distal extremities and face. It is relatively rare on female genitalia. We report two cases of solitary vulvar pyogenic granuloma. Case 1: A 23 years old female presented with single lesion on her left labia majora since 20 days. Case 2: A 48-year-old woman also presented with a single lesion on her left labia majora since 3months. Excisional biopsies were done in both the cases which showed features suggestive of pyogenic granuloma. On follow-up, no recurrences were found. Our emphasis is to make dermatologists aware of the rare location of pyogenic granuloma on vulva. Keywords: Pyogenic granuloma, Vascular tumor, Vulva.

Giant primary lymphedema of the vulva in a young female

A 21-year-old single female with a large swelling of the vulva’s left labia majora was diagnosed with a case of primary lymphedema of the left lower limb and external genitalia. The giant labial swelling was excised, and the patient experiences no complications or recurrence for 7 months postoperatively. Following the surgical excision of the swelling, the left labia majora and minora were reconstructed successfully. The excised mass weighed 6.3 kg and measured 40 cm x 35 cm x 30 cm. There were no complications postoperatively, and the patient has not experienced recurrence during the 7-month follow-up period. The patient returned to her normal life. Level of evidence: Level V, therapeutic study.

Extensive Squamous Metaplasia in Hidradenoma Papilliferum of Vulva: Illusion of Squamous Cell Carcinoma

Hidradenoma papilliferum is a rare, slow-growing, benign, cystic, papillary tumour occurring in middle-aged females, in the anogenital region commonly. It involves the anogenital mammary-like glands and is usually asymptomatic. Simple excision is curative. We present a case of hidradenoma papilliferum of long-standing duration with extensive squamous metaplasia. A post-menopausal lady presented with an exophytic growth on the left labia majora and part of mons pubis. It was initially misdiagnosed on the basis of vulvar biopsy and managed as squamous cell carcinoma of the vulva. The final histopathology revealed an epithelial tumour involving the vulva with extensive squamous metaplasia. The tumour involved mainly the dermis and the subcutaneous tissue. This is the first case in the literature where the diagnosis of hidradenoma papilliferum was confused with squamous cell carcinoma of the vulva due to its clinical presentation and extensive squamous metaplasia. Diagnosis of hidradenoma papilliferum should be kept in mind if a woman presents with vulval papillary or nodular lesions. An expert clinical and histopathological review should be done to avoid misdiagnosis.

29: Ovotesticular disorder of sexual development: a case report

To present a rare case of ovotesticular disorder of sexual development (OT-DSD). We present a 21-year-old patient with gynandromorphic genitalia and breast development diagnosed with 46,XX OT-DSD. He was raised as a male and desired correction of his feminized anatomic features. A computer tomography of the abdomen and pelvis demonstrated normal kidneys, bilateral ureters, a uterine horn, an intra-abdominal gonad on the left, a descended gonad on the right and a structure consistent with a vagina. On physical examination, the patient demonstrated the presence of bilateral, symmetrical breasts and the external genitalia showed the presence of a 7 cm long penile-like structure that was immobile and adherent to the ventral aspect of a clitoral hood. A small, presumed testis within the scrotum was noted only on the right side, while the testis and scrotum were absent on the left. A perineal opening appearing to be the vaginal introitus was ultimately identified as the urethra on further investigation. Intra-operative findings included a small, boggy uterine horn and a left gonad which appeared to be a normal ovary with a dilated left fallopian tube. Bilateral ureters with efflux were identified in the normal anatomical position leading to a normal appearing bladder. In accordance with the patient’s wishes, an abdominal hysterectomy, left salpingo-oophorectomy, right trans-scrotal testicular biopsies and bilateral simple mastectomy were performed. Histopathologic examination of the excised gonads was performed at our institution. Examination of the right trans-scrotal biopsy confirmed testicular tissue. Examination of the left-sided gonad was consistent with an ovarian tissue. Traditional cytogenetic analysis revealed 46, XX. Six months after the initial surgery, the patient underwent the second stage of gender confirmation surgery. Penile reconstructive surgery and metoidioplasty were performed by dividing the skin of the phallus/clitoris from the underlying cavernous body. The reconstruction of the penis was completed by advancing another skin flap from the left labia majora. The patient’s 6-month follow-up demonstrated an uncomplicated post-operative course, living as a male. For many children with ambiguous genitalia in the developing world, it is not until puberty before problems associated with feminization become apparent. Very often, as in our case, multiple surgical procedures are necessary to fulfill the gender choice of the patient. Most patients would prefer to live as a male because of cultural bias. However, it is rare that a patient in the developing world who has been feminized or masculinized during their post-pubescent years actually find surgeons to perform gender assignment surgery. This is usually due to the absence of knowledgeable medical resources and the prohibitive cost.View Large Image Figure ViewerDownload Hi-res image Download (PPT)View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Invasive vulvar squamous cell carcinoma and chronic psoriasis

Vulvar carcinoma is the fourth most common gynecologic malignancy[1]. A 50 year old patient presented to us for evaluation of an infiltrated, ulcerated vulvar tumour mass, with irregular borders, localized on the left labia majora. Also, palms, soles and elbows presented typical psoriasis plaques .
 The biopsy of the palmar tegument was concludent for vulgar psoriasis. The incisional vulvar biopsy revealed a squamous cell carcinoma. HPV testing was positive for HPV 16 infection with high-oncogenic risk. The CT scan showed the tumour located on the left labia majora, measuring 3 cm, with irregular borders and inguinal left node metastasis.
 The surgical treatment was partial vulvectomy with bilateral inguinal lymphadenectomy. The postoperatory course was good, with no local complications. Postoperative histological examination result was invasive squamous cell carcinoma, poorly differentiated(G3), basaloid, infiltrative(>1 mm, pT1b), with lymph node, ganglionar metastasis and capsular invasion(N2a). The surgical treatment was recommended to be followed by chemotherapy and radiotherapy.
 Our case highlights the aggresive, rapidly evolution of a squamocelular vulvar carcinoma in a patient with concomitantly chronic psoriasis. Psoriasis is a disease that alters the immune system and increases overall inflammation, which can expand the risk of developing cancers.. There is a high risk for malignancy in psoriatic patients [2]. A study found a higher frequency of all malignity among the patients with psoriasis; also, the risk of nonmelanoma skin cancer was higher [3].

146 Standardization of Vulvoscopy: A Step-Wise Approach

In the medical evaluation of a woman with sexual dysfunction, there is no standard methodology for physical examination. The purpose of this was to develop a standardized technique of magnified examination of the vulvar, vestibular and vaginal region by vulvoscopy. The technique of medical healthcare providers in our facility who perform vulvoscopy using regularly, was reviewed. Options for examination are a standard colposcope with an attached camera to project the image onto a flat screen for viewing, or a tripod mounted smart phone with blue tooth remote, connected to the flat screen wirelessly. Photographs are taken at each step of the examination. Maximal diagnostic information was gained using a step-wise approach to vulvoscopy, beginning from lateral to medial, external to internal, conducted in a standardized manner. The vulvoscopy protocol begins with examination of right and left labia majora, followed by sulci, and then labia minora, assessing for pathology, and followed by cotton swab (Q-tip) palpation used to assess for pain. Labia minora width (cm) is then measured and degree of resorption from posterior fourchette assessed. The glans clitoris is examined via cephalad retraction of the prepucial hood so the corona can be visualized to assess for presence of clitoral adhesions. Glans size is compared to size of the cotton swab. Lateral retraction of labia minora enables visualization of Hart's line and close inspection of the vestibule and urethral meatus. The ostia of minor vestibular glands are observed for erythema and Q-tip testing performed at 1:00, 3:00, 5:00, 6:00, 7:00, 9:00, 11:00 to evaluate for pain on a scale of 0-10. Speculum exam is then performed by rotating the speculum 90 degrees to evaluate presence of vaginal rugae, vaginal pathology, as well as quality of the anterior vaginal wall periurethral tissue.

Labia Majora Share.

Defects involving specialised areas with characteristic anatomical features, such as the nipple, upper eyelid, and lip, benefit greatly from the use of sharing procedures. The vulva, a complex 3-dimensional structure, can also be reconstructed through a sharing procedure drawing upon the contralateral vulva. In this report, we present the interesting case of a patient with chronic, massive, localised lymphedema of her left labia majora that was resected in 2011. Five years later, she presented with squamous cell carcinoma over the left vulva region, which is rarely associated with chronic lymphedema. To the best of our knowledge, our management of the radical vulvectomy defect with a labia majora sharing procedure is novel and has not been previously described. The labia major flap presented in this report is a shared flap; that is, a transposition flap based on the dorsal clitoral artery, which has consistent vascular anatomy, making this flap durable and reliable. This procedure epitomises the principle of replacing like with like, does not interfere with leg movement or patient positioning, has minimal donor site morbidity, and preserves other locoregional flap options for future reconstruction. One limitation is the need for a lax contralateral vulva. This labia majora sharing procedure is a viable option in carefully selected patients.

Intravascular papillary endothelial hyperplasia of the vulva: report of a patient with Masson tumor of the vulva and literature review

Intravascular papillary endothelial hyperplasia -- also known as Masson tumor -- is a rare, benign vascular condition that manifests on the skin as a firm, blue-black colored nodule or papule. Lesions range in size from 0.25 to 5 centimeters in diameter and may be tender or painless. In some individuals, nodules appear red colored, mimicking hemangioma or pyogenic granuloma. Histologically, intravascular papillary endothelial hyperplasia is characterized by the presence of an organizing thrombus in the vascular lumen with accompanying hyperplastic endothelial cell proliferation. Common sites of presentation include the head, neck, and extremities. However, albeit rarely, lesions may also appear in the genital region. We describe the clinical and pathologic findings of a woman who developed intravascular papillary endothelial hyperplasia of the vulva. We also review the characteristics of other patients with intravascular papillary endothelial hyperplasia of the vulva and summarize the differential diagnosis and treatment options for this condition. The features of a woman with intravascular papillary endothelial hyperplasia of the vulva are presented. Using PubMed, the following terms were searched and relevant citations assessed: intravascular papillary endothelial hyperplasia, IPEH, labia majora, Masson hemangioma, Masson pseudoangiosarcoma, Masson tumor, and vulva. In addition, the literature on intravascular papillary endothelial hyperplasia is reviewed. A 32-year-old woman presented with a 3 x 2 millimeter painless, black colored submucosal papule on her left labia majora. The lesion was removed by excisional biopsy. Microscopic examination revealed a re-canalizing thrombus and a proliferation of erythrocytes within a dilated vascular structure. Based on correlation of the clinical presentation and histopathologic findings, a diagnosis of intravascular papillary endothelial hyperplasia was established. The patient applied mupirocin 2% ointment to the biopsy site, which subsequently healed without complication or recurrence. Intravascular papillary endothelial hyperplasia -- also known as Masson tumor -- is a rare, benign vascular eruption. Cutaneous lesions typically present as red colored or blue-black colored nodules ranging in size from 0.25 to 5 centimeters in diameter. The most common sites of presentation include the head, neck, and extremities. However, albeit rarely, lesions may also appear in the genital region. The histologic hallmark of intravascular papillary endothelial hyperplasia is an organizing thrombus in the vascular lumen with associated hyperplastic endothelial cell proliferation. Lesions often appears similar to other neoplastic and non-neoplastic cutaneous tumors, including hemangiomas, pyogenic granulomas, and cutaneous angiosarcomas. Therefore, pathologic examination is required to confirm the suspected diagnosis and exclude malignancy. The condition can usually be treated with simple local excision.

Recurrent Aggressive Angiomyxoma: A Case Report

Aggressive angiomyxoma (AA), a rare mesenchymal tumor with high recurrence rate and infiltrative nature, most generally arises in the vulvovaginal region, perineum, and pelvis of women in reproductive age peaking from age 31 to 35 but uncommonly found on men. It appears as a slow-growing gelatinous mass, which commonly asymptomatic. Imaging studies including USG, CT scan, and MR imaging are of great importance for the diagnosis of AA on identifying the mass characteristics. This paper reports a case of a 37 year old P3A0 woman with a lump on the left side of vaginal lip for 2 weeks before admission. The lump was soft to touch and was not painful, without other accompanying symptoms. Past medical history showed the lump has recurred after being surgically removed. On physical examination, a lump was found on the left vulva with soft consistency. CT scan showed a well-circumscribed hypodense mass on left labia majora and showed post contrast wall enhancement. Fine needle aspiration biopsy was done and showed hypocellular smear with myxoid matrix and bleeding. The mass was then removed by vulvectomy procedure. Histopathological analysis of the mass shows infiltration of myxoid stroma with blood vessels consistent with aggressive angiomyxoma. In conclusion, AA is usually asymptomatic and has high tendency of recurrence. Imaging procedure is useful on identifying mass characteristics and biopsy can be done to establish AA diagnosis. Surgery is the main modality for the treatment of AA, and administration of gonadotropin-releasing hormone analogue can prevent further recurrence.Keywords: Aggressive angiomyxoma, gonadotropin-releasing hormone agonist, recurrent