Bartholin's gland adenoid cystic carcinoma: Report of three cases and the review of literature.

The adenoid cystic carcinoma (ACC) of Bartholin's gland is a very rare tumor that usually grows aggressively due to perineural invasion. Although there is no guideline for the treatment due to the small number of patients, the primary treatment is considered as surgery. Adjuvant radiotherapy has also been shown to be beneficial. Three cases, which were histopathologically confirmed as ACC of Bartholin's gland and received adjuvant radiotherapy treatment, were presented in this study.

Metastatic Adenoid Cystic Carcinoma of the Bartholin Gland.

ABSTRACT: Adenoid cystic carcinoma (ACC) of the Bartholin gland is an exceedingly rare neoplasm. A 56-year-old woman with remote ACC resection (plus neoadjuvant chemoradiation and adjuvant chemotherapy) presented to an outside institution with shortness of breath. CT showed bilateral pulmonary nodules and mediastinal lymphadenopathy. With high clinical suspicion for metastatic disease, 18 F-FDG PET/CT was performed and showed scattered nodules with mild FDG uptake along with FDG-avid mediastinal, bilateral hilar, and bilateral cervical chain lymphadenopathy. Lung biopsy of a hypermetabolic nodule confirmed metastatic ACC.

Case series on the management and outcomes of Bartholin gland carcinoma.

OBJECTIVE: To evaluate the management and outcomes of Bartholin gland cancer at a single tertiary institution. STUDY DESIGN: A single institution retrospective review of 9 cases of BGC between 2004 and 2022 was conducted. Demographics, pathological characteristics, treatment, follow up and oncologic outcomes were extracted from clinical records. Data are summarised using descriptive statistics and survival probabilities are presented with Kaplan Meier graphs. RESULTS: Ten cases of BGC were identified at our institution over a period of 18 years. Nine out of ten clinical records were available for analysis. Eight patients presented with vulval swelling and four were treated initially for Bartholin cyst or abscess. One patient had a histological diagnosis of adenoid cystic carcinoma while the remaining were squamous cell carcinomas. With the exception of stage I disease chemoradiation was the primary mode of treatment. Adverse events included skin desquamation (4/9), venous thrombo-embolism (2/9), gastro-intestinal (1/9) and neurotoxicity (1/9). Median follow up was 60 months with a 5-year recurrence free and overall survival at 76 % and 64 % respectively. CONCLUSION: BGC may present after a long duration of symptoms and at advanced stages. Primary chemoradiation appears to be a feasible treatment option in advanced disease with the benefit of decreased morbidity.

Bilateral bartholin's gland abscesses in a 4-year-old girl with vitamin a deficiency: a case report.

BACKGROUND: A Bartholin's gland abscess is one of the most common infections in women of reproductive age. Although Bartholin's gland abscesses have been reported in prepubertal children, they are rarer in prepubertal children than in adults. Herein, we report a case of bilateral Bartholin's gland abscesses in a 4-year-old girl with vitamin A deficiency. CASE PRESENTATION: A 4-year-old girl diagnosed with autism spectrum disorder was admitted to the hospital for close examination and treatment because of persistent fever and malaise. The child was a marked fussy eater and was diagnosed with corneal ulceration and night blindness secondary to vitamin A deficiency. Both of the patient's labia were swollen, and a diagnosis of a bilateral Bartholin's gland abscess was made using computed tomography. Incisional drainage was performed under general anesthesia. The patient's postoperative course was uneventful, and she was discharged from the hospital on day 8 after the surgery. During hospitalization, attempts were made to correct the vitamin deficiency by adding nutritional supplements to the diet. Three months after the surgery, no recurrence of abscesses was noted. CONCLUSIONS: Decreased immunocompetence and mucosal barrier function due to vitamin A deficiency is thought to be the underlying cause of Bartholin's gland abscesses. Although prepubertal Bartholin's gland abscesses have been reported, they are rare. To the best of our knowledge, no reports of bilateral Bartholin's gland abscesses potentially caused by vitamin A deficiency have been reported. When prepubertal girls present with Bartholin's gland abscesses, the presence of immunodeficiency due to vitamin or trace element deficiency should also be considered.

Feasibility and Satisfaction With the Word Catheter in Treatment of Bartholin's Cyst and Abscess.

BACKGROUND/AIM: The Word catheter is a silicone device with a balloon system that may be inserted into a Bartholin's cyst or abscess in order to provide drainage and epithelization. The aim of this study was to evaluate the Word catheter as a therapy for Bartholin's cyst and abscess. Both patient and physician satisfaction, as well as the feasibility in an outpatient setting, were examined. PATIENTS AND METHODS: A total of 51 women with a Bartholin's cyst or abscess were given the option of Word catheter insertion in an outpatient setting between August 2013 and March 2018. Both the patients and the consulting physicians were asked to complete two questionnaires, before, during and after treatment, with a view to evaluating the overall pain level, any discomfort symptoms and sexual activity, as well as satisfaction levels. RESULTS: The insertion procedure seemed to constitute a short yet quite painful procedure. In most cases, the consulting physicians and the patients were content with the results. Nevertheless, dislodgement of the catheter or abscess recurrence were common. The removal of the Word catheter seemed to be short, painless, and uncomplicated. Most patients experienced pain and discomfort after catheter placement over the first days, with the symptoms fading over time. Sexual intercourse appeared to be negatively influenced. CONCLUSION: The Word catheter was frequently well tolerated for the treatment of Bartholin's cysts and abscesses, with few non-serious side-effects, however, it did interfere with sexual health. Nonetheless, it may not be possible to make general recommendations based on this exploratory study.

[Main Types of Cysts in Dermatopathology: Part 2]. / Principales tipos de quistes en dermatopatología: Parte 2.

This is the second article in a two-part series published in this journal, in which we examine the histopathological characteristics, as well as the differential diagnosis, of the main entities that present as cystic and pseudocystic structures in cutaneous biopsy. In this second article, we address ciliated cutaneous cysts, branchial cysts, Bartholin's cysts, omphalomesenteric cysts, thymic cysts, thyroglossal duct cysts, synovial cysts, and median raphe cysts, as well as mucocele, ganglion, and auricular and digital myxoid pseudocysts.

Adenoid cystic carcinoma of the Bartholin's gland is underpinned by MYB- and MYBL1- rearrangements.

OBJECTIVE: Adenoid cystic carcinoma (AdCC) of the Bartholin's gland (AdCC-BG) is a very rare gynecologic vulvar malignancy. AdCC-BGs are slow-growing but locally aggressive and are associated with high recurrence rates. Here we sought to characterize the molecular underpinning of AdCC-BGs. METHODS: AdCC-BGs (n = 6) were subjected to a combination of RNA-sequencing, targeted DNA-sequencing, reverse-transcription PCR, fluorescence in situ hybridization (FISH) and MYB immunohistochemistry (IHC). Clinicopathologic variables, somatic mutations, copy number alterations and chimeric transcripts were assessed. RESULTS: All six AdCC-BGs were biphasic, composed of ductal and myoepithelial cells. Akin to salivary gland and breast AdCCs, three AdCC-BGs had the MYB::NFIB fusion gene with varying breakpoints, all of which were associated with MYB overexpression by IHC. Two AdCC-BGs were underpinned by MYBL1 fusion genes with different gene partners, including MYBL1::RAD51B and MYBL1::EWSR1 gene fusions, and showed MYB protein expression. Although the final AdCC-BG studied had MYB protein overexpression, no gene fusion was identified. AdCC-BGs harbored few additional somatic genetic alterations, and only few mutations in cancer-related genes were identified, including GNAQ, GNAS, KDM6A, AKT1 and BCL2, none of which were recurrent. Two AdCC-BGs, both with a MYB::NFIB fusion gene, developed metastatic disease. CONCLUSIONS: AdCC-BGs constitute a convergent phenotype, whereby activation of MYB or MYBL1 can be driven by the MYB::NFIB fusion gene or MYBL1 rearrangements. Our observations further support the notion that AdCCs, irrespective of organ site, constitute a genotypic-phenotypic correlation. Assessment of MYB or MYBL1 rearrangements may be used as an ancillary marker for the diagnosis of AdCC-BGs.

Comparison of Word Catheter and Marsupialization in the Management of Bartholin's Glands: A Systematic Review and Meta-Analysis.

OBJECTIVE: There is no consensus on the best option for the management of Bartholin's gland abscesses. This systematic review and meta-analysis aimed to compare the Word catheter placement and marsupialization methods in the management of women with a cyst or abscess of the Bartholin's glands. DATA SOURCES: The PubMed, Scopus, Embase, Web of Science, and Cochrane Library databases, as well as Google Scholar, were searched to retrieve articles published between January 1990 and July 2023, comparing the Word catheter treatment with marsupialization for women with a cyst or abscess of the Bartholin's glands. STUDY SELECTION: Both observational studies and randomized controlled trials (RCTs) were included in this meta-analysis. DATA EXTRACTION AND SYNTHESIS: After the review, out of 9 relevant articles, only 4 (2 observational studies and 2 RCTs) were included in this meta-analysis, providing the data of 735 patients (396 in the Word catheter group vs. 339 in the marsupialization group). The data was extracted from the selected articles, using a data extraction form. Comparison of the Word catheter and marsupialization methods suggested that the risk of recurrence was approximately 7.6% in the Word catheter group and 9.4% in the marsupialization group. The findings indicated no significant difference in the recurrence of cysts or abscesses when comparing the Word catheter treatment with marsupialization (odds ratio = 0.99, 95% confidence interval: 0.29-3.43, P = 0.990, I2 = 77%). CONCLUSION: This meta-analysis found no significant difference in the rate of recurrence between the Word catheter and marsupialization methods. In general, marsupialization and Word catheter placement in the treatment of Bartholinitis seem to be equally effective.

Management of primary and recurrent Bartholin's gland carcinoma: A systematic review on behalf of MITO Rare Cancer Group.

Bartholin gland carcinoma is an extremely rare disease. Information regarding treatment is scarce and there is no strict consensus on best practice. All studies reporting cases of Bartholin's gland cancer were screened and evaluated for inclusion. Baseline characteristics of studies were extracted. A total number of 290 manuscripts collected were available for the review process. Studies included in a previous systematic review were not duplicated. In total, details of 367 patients were collected, as follows: histological features, clinical presentation, treatment, recurrent rate, treatment of recurrence and outcome. About 35% of Bartholin gland carcinoma were squamous cell carcinoma. Almost 50% of patients presented with advanced stage. The therapeutic approach was mainly surgery, and in 61% of those women lymph node assessment was performed. Recurrence occurred in 21% of cases. Bartholin gland cancer remains a challenge for gynecologic oncologists. Guidelines, centralization to referral centers and standardized therapy are needed.

A Metastasis of Ovarian Cancer in the Bartholin Gland: A Case Report with Systematic Literature Review.

The metastasis of a gynecological malignancy to the Bartholin gland is rare. We report the case of a 62-year-old patient who had undergone extensive treatment of metastatic ovarian cancer that involved the liver, spleen, and peritoneum. She presented with painful swelling of the left vulva. Clinical and sonographic examinations showed a solid tumor in loco typico of the Bartholin gland. Surgical excision was performed. The patient died 3 months after the diagnosis of this metastasis. We performed a systematic search of PubMed, which yielded 453 entries. We selected those with at least an abstract available in English that described metastatic lesions on the Bartholin gland (n = 5). The review showed that a variety of primary cancers (colorectal, medullary thyroid, breast cancer, and endometrial cancers) metastasize to this location. Some patients showed signs of visceral metastasis. Bartholin gland metastases appeared as initial and metachronous manifestations. Most patients were symptomatic, with painful swelling or abscess. Genetic alterations were mentioned in some cases. The main pathways of metastasis discussed were lymphatic, but the mechanism of such metastasis remains unclear. Surgical resection was the preferred treatment option. The literature review indicated that Bartholin gland metastasis of ovarian cancer is rare and associated with poor prognosis. Oncological reasons for vulvar pathologies should be taken into consideration in patients with metastases.

Vulvar Leiomyosarcomas: A Case Series with Clinical Comparison to Uterine Leiomyosarcomas and Review of the Literature.

BACKGROUND Leiomyosarcomas of the vulva (VLMS) are very rare among gynecological malignancies, with a lack of knowledge on clinical presentation, prognosis, and therapeutic management. CASE REPORT The database of the German Clinical Center of Competence for Genital Sarcomas and Mixed Tumors in Greifswald (DKSM) was reviewed between the years 2010 and 2020. A total of 8 cases of VLMS were retrieved and analyzed retrospectively. One exemplary case of VLMS was outlined in detail: A 45-year-old premenopausal woman presented with increasing vulvar swelling and discomfort. Given the suspicion of a Bartholin's gland abscess, the mass was excised. Final pathology revealed a solid tumor consistent with a moderately differentiated leiomyosarcoma of the vulva. A wide local excision was subsequently performed followed by adjuvant external beam radiation. The clinical features of these 8 cases of VLMS were compared to 26 cases of VLMS found in a review of the literature and to a total of 276 cases of uterine leiomyosarcoma (ULMS) from the same database (DKSM). CONCLUSIONS In addition to rapid growth, observed in both tumor entities, VLMS most commonly presented as Bartholin's gland abscess or cyst and ULMS as leiomyoma. In this cohort, the prognosis of VLMS was much better than that of ULMS, most probably due to the significantly smaller tumor size of VLMS at diagnosis. Further data and larger studies on VLMS are needed to calculate recurrence and survival rates more accurately and define the role of adjuvant radiotherapy.

Bartholin's gland cyst caused by Sneathia amnii: a case report.

BACKGROUND: Sneathia amnii is a conditional pathogen of the female genital tract that is involved in bacterial vaginosis and poor reproductive and perinatal outcomes. Few studies have reported subcutaneous cysts following invasive infection caused by S amnii. CASE PRESENTATION: Here we report the case of a 27-year-old woman who presented with Bartholin's gland cyst due to S amnii infection, and was successfully treated with surgical neostomy and antibiotic agents. The isolate was gram-negative, bacillary, anaerobic, and was identified by polymerase chain reaction (PCR) amplification of the 16 S rRNA. CONCLUSIONS: S amni is an important but underappreciated pathogen that needs further investigation. This report describes the microbial and pathogenic characteristics of S amnii and is expected to provide a valuable reference in obstetric and gynecologic clinical practice.

Synchronous ovarian and Bartholin gland carcinoma: Case report and review of literature.

To our knowledge, this is the first reported case of synchronous ovarian and vulva (Bartholin gland) cancer. A postmenopausal woman presented with a complex multiloculated left adnexal mass and 2-cm right Bartholin gland mass. CA 125 was 59 IU/mL. Computed tomography of chest, abdomen, and pelvis showed a very large (32 × 13.5 × 22.5 cm) complex mass arising from the pelvis and extending to the level of the T12/L1 disk space. A right Bartholin mass with suspicious right inguinal nodes was seen. Midline laparotomy, total abdominal hysterectomy, bilateral salpingo-oophrectomy, infracolic omentectomy, pelvic peritoneal biopsies, and peritoneal washings were carried out. Wide local excision of the right Bartholin gland mass was carried out in the same setting. Histopathology came back as Stage 2B left ovarian clear-cell carcinoma and synchronous right Bartholin gland adenoid cystic carcinoma with lymphovascular invasion, incompletely excised, staged at least FIGO Stage 1B. Following local multidisciplinary team discussion and positron emission tomography scan review, the local committee agreed to start three cycles of adjuvant chemotherapy then proceed with Bartholin gland scar re-excision and bilateral groin lymph node dissection. After the three cycles, the groin lymph nodes came back as metastatic adenocarcinoma with overall morphologic and immunohistochemical features consistent with metastatic ovarian clear-cell carcinoma. Postoperative adjuvant chemotherapy was given. Initial follow-up period over 9 months was uneventful.

Adenoid Cystic Carcinoma (ACC) of the Bartholin's gland misdiagnosed for three times: a case report.

BACKGROUND: Adenoid cystic carcinoma (ACC) in Bartholin's gland is an uncommon malignant tumor. These tumors have a vague clinical feature, so they are diagnosed late and discovered at a high-level stage. Our case presented Three Recurrences and Three times Misdiagnosis of Adenoid Cystic Carcinoma (ACC). CASE PRESENTATION: We report a case of adenoid cystic carcinoma arising in Bartholin's gland of a 64-year-old female patient that appeared after three previous vulvar tumors were excised. The patient underwent bilateral radiotherapy which was performed on the perineum. CONCLUSION: ACC of the vulvar sweat glands is prone to misdiagnosis and delay in both diagnosis and treatment. As seen in our case, it was misdiagnosed three times as Chondroid Syringoma. Further studies need to be conducted to better understand the tumor prognosis, and its optimal treatment options.

Adenoid cystic carcinoma of Bartholin's gland, a case report with genomic data and literature review.

Adenoid cystic carcinoma of the Bartholin's gland (ACCBG) is a rare, slowly but aggressive malignancy. We reported the case of a 31-year-old woman who was treated by local excision and then hemi-vulvectomy, with positive margins and perineural invasion. Radiation therapy (RT) was then performed delivering 45Gy in 25 fractions in bilateral inguinal lymph nodes and 64.8Gy in 36 fractions on the vulvar area. After 30 months, there was no local relapse (LR) but the patient presented a histologically documented lung recurrence. Genomic profiling of the tumor showed a MYB-NFIB fusion transcript and a somatic mutation of PLCG1. A treatment by Lenvatinib was started. We conducted a literature review of 100 published cases. Patients were mainly treated by radical vulvectomy (30%), hemi-vulvectomy (17%), wide or local excision (21% and 24%, respectively) or other. Forty-four percent of patients received postoperative RT, more frequently in case of positive margin (71.9% versus 29.5%). RT may reduce the risk of LR regardless of margin status, with 15.4% vs. 41.9% of LR with or without RT, respectively, in patients with negative margins, and 13% vs. 33.3% of LR with or without RT, respectively, in patients with positive margins. The risk of relapse of any type was 40.9% in patients who received adjuvant RT vs. 48.2% in patients who did not. Median time to relapse was 24 months (range 6-156 months). The most frequent metastatic sites were lung (76.7%) and bone (26.7%). Optimal treatment for ACCBG is still not clearly defined but pooling the data from published case report help us better understand this rare disease and help in the therapeutic decision.

Vulvar leiomyoma in an adolescent girl: a case report and review of the literature.

BACKGROUND: Vulvar leiomyoma is a rare soft tissue tumor, with only around 300 cases described in the literature. Owing to its low incidence of just 0.03% of all gynecological tumors, it often poses a great diagnostic challenge, especially in teenagers. We report this rare occurrence of vulvar leiomyoma in a teenage girl who was primarily left untreated due to cultural taboos and fear of loss of virginity. The main aim in presenting such rare case studies is to raise awareness and expand the diagnostic horizon of the surgeon for appropriate management. CASE PRESENTATION: We describe a case of a 15-year-old North Indian, sexually inactive unmarried girl, who presented with a history of painless swelling in the left labia majora for the last 1 year, which gradually increased in size. There was no associated pain or any other difficulty. Local examination revealed a 14 × 10 cm solid, unilateral nonpedunculated mass on the left labia majora with superficial vascularity. Differential diagnoses of sarcoma, lipoma, Bartholin cyst, and fibroid were kept in mind. Ultrasonography revealed a solid mass with superficial vascularity with normal internal genitalia. The mass was enucleated with an intact capsule under anesthesia. Histopathology confirmed it to be benign vulvar leiomyoma. The patient was discharged after 3 days in a satisfactory condition. CONCLUSION: Leiomyoma of the vulva is an exceptionally rare tumor and is seldom seen in teenagers. It is often misinterpreted as a Bartholin cyst and should be kept as one of the differential diagnosis in teenage girls presenting with unilateral vulvar swelling. Vulvar leiomyoma can be completely cured by surgical removal if diagnosed timely without compromising virginity, so should never be missed in adolescents.