[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.

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.

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.

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.

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 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.

Adenoid cystic carcinoma of the Bartholin's gland is easily misdiagnosed: A case report and literature review.

RATIONALE: Adenoid cystic carcinoma (ACC) often occurs in the major and minor salivary glands and other sites containing secretory glands, while ACC of the Bartholin's gland (BG-ACC) in the vulva is rare and easily misdiagnosed. PATIENT CONCERNS: A 58-year-old female was referred to our hospital for further valuation of a mass occurring on the left side of her vulva. In the other hospital, the beginning of the period, local ultrasound showed a vulva mass, which was suspected to be a Bartholin's gland cyst. Mixed neoplasms were considered in some biopsies. When transferred to our hospital, virtuous tumors were considered by ultrasound and magnetic resonance imaging. Pathology initially considered benign hyperplastic active tumor or borderline tumor. DIAGNOSES: Histological, immunochemical, and molecular tests confirmed a diagnosis of BG-ACC, negative surgical margin, without lymphatic metastasis. INTERVENTIONS: Extended excision of the mass at left labia majora plus left inguinal lymph node dissection was performed. OUTCOMES: The patient received surgery therapy, no recurrence was observed during a 18-month follow-up period. LESSONS: Due to its lack of specific characteristics in clinical, ultrasound and imaging, it is easy to be misdiagnosed, Due to its rarity and nonspecific clinical, radiologic and ultrasonographic manifestations, BG-ACC can be easily misdiagnosed. And its pathomorphological features overlap with other benign and malignant tumors occurring at vulva, BG-ACC can be easily misdiagnosed, and diagnosis by puncture biopsy is extremely difficult. Use of paraffin sections to identify tumor growth characteristics, combined with immunohistochemical findings, is the key to the diagnosis of ACC. In rare sites, MYB gene split are helpful in making a definite diagnosis.

Adenoid Cystic Carcinoma of Bartholin's Gland: A Case Report with Emphasis on Surgical Management.

BACKGROUND Adenoid cystic carcinomas of Bartholin's gland are rare among gynecological malignancies, accounting for 0.1% to 7% of vulvar carcinomas and 0.001% of all female genital tract malignancies. There are no specific guidelines regarding treatment recommendations; therefore, they are commonly treated like vulvar cancer. CASE REPORT We present the case of a 42-year-old premenopausal woman with an adenoid cystic carcinoma of Bartholin's gland diagnosed upon biopsy of a palpable, predominantly vaginally located mass causing foreign-body sensation, vaginal pain, and extreme dyspareunia. The adenoid cystic carcinoma of Bartholin's gland was treated by radical resection in an extensive interdisciplinary surgical approach including bilateral inguinal lymph node dissection, partial posterior colpectomy, amputation of the rectum, and creation of a descendostomy, as well as reconstruction of the vagina and defect coverage using flap plastic. CONCLUSIONS With the presentation of this case, we propose a possible therapeutic approach to adenoid cystic carcinomas of Bartholin's gland with emphasis on surgical management. Especially in young patients, we recommend primary radical surgery with the objective to obtain negative resection margins. However, additional data on the adenoid cystic carcinoma of Bartholin's gland is needed to better understand its biological behavior and thus optimize and standardize treatment. The role of systematic inguinal-femoral lymphadenectomy and adjuvant and neoadjuvant treatment modalities need further evaluation.

Leiomyoma of the Vulva: Case Report.

BACKGROUND: Leiomyoma of the vulva is rare, accounting for only 0.03% of all gynecological tumors, and it is seldom seen in teenagers. CASE: We describe a case of vulvar leiomyoma in a 14-year-old girl who presented a 10 × 10 cm solid tumor in the right hemivulva without other complaints. SUMMARY AND CONCLUSION: Differential diagnosis includes Bartholin cysts, abscesses, fibromas, and other solid lesions. Although rare, vaginal leiomyoma must be remembered and included as a differential diagnosis for solid lesions in the vagina.

Acinar Melanosis of the Bartholin Gland: A Report of 3 Cases.

Melanocytic pigmentation occurs in multiple sites in the lower female genital tract, but is rare within benign cysts of the vulva. We report 3 patients with multiloculated cystic lesions of the vulvar vestibule exhibiting prominent melanocytic pigmentation. The current cases differ from a previous report of melanosis in a Bartholin gland cyst in that the population of melanocytes occupies the acinar structures of the gland, rather than a squamous-lined surface. A similar cell population is demonstrated by immunoperoxidase methods in a fourth patient's nonpigmented gland, suggesting that melanin production may arise in a native, rather than metaplastic, cell population.

Pitfalls in Pathology-Nodular Hyperplasia of Bartholin's Gland.

Nodular hyperplasia of the Bartholin's gland is an underreported and extremely rare entity that presents as a solid lesion potentially raising concern for malignancy clinically, most solid lesions at this location are carcinomas. They may also be mistaken for a Bartholin cyst clinically. Nodular hyperplasia is rarer than carcinoma of the Bartholin gland, and hence pathologists may not be familiar with this entity, making it a pitfall in pathologic as well as a clinical diagnosis. Here we report a case originally considered a Bartholin cyst, but raising intraoperative concern due to solid elements. Histological examination of the lesion revealed nodular hyperplasia of Bartholin's gland. Recognition of this entity is important, as it is something that may be encountered by the pathologist.

Vulvar myoma: A case report and review of literature.

OBJECTIVE: To present a case of vulvar myoma and the factors differentiating this tumor from Bartholin's cyst. CASE REPORT: A 50-year-old woman presented with a nodule over the left labia majora. Pelvic examination showed swelling and redness of the left labia majora. A 2-cm nodule with firm consistency was found near the vaginal opening. There was no inguinal lymphadenopathy. Bartholin's cyst was suggested, and oral cephalexin was prescribed for 1 week, but no improvement was seen. Therefore, she underwent excision of the nodule. Pathology revealed it to be a benign vulvar myoma. The patient recovered well, and no recurrence was noted after 2 months of follow-up. CONCLUSION: Vulvar myoma is rare. Sexual history, nodule consistency, and imaging are helpful, but the final diagnosis of vulvar myoma is usually made following surgical excision and histopathological analysis.

Intestinal-type mucinous adenocarcinoma of the Bartholin gland in a perimenopausal woman. A case report and review of the literature.

We report the case of a 46-year-old woman who presented with a tumor on the left labium majus in the region of the Bartholin gland. Surgical excision revealed a mucinous adenocarcinoma of intestinal-type (CK20+, CDX-2+). Magnetic resonance imaging, computed tomography of the chest and abdomen and colonoscopy ruled out the presence of other tumors. A second immunohistochemical study showed negative results for GATA-3, mammaglobin and GCDFP-15. Molecular analysis revealed a mutation in exon 2 of the KRAS gene. We discuss its differential diagnosis and the importance of being aware of this unusual variant of a mucinous adenocarcinoma the Bartholin gland.

Bartholin gland carcinoma in a young female: a rare disease in an unusual age group.

Primary Bartholin gland carcinoma (BGC) is an extremely rare disease. It typically presents in elderly women. It can be confused with Bartholin gland cyst, which is a benign condition leading to a delay in diagnosis and treatment. We are presenting a case report of BGC in a 35-year-old woman, which has created a diagnostic as well as therapeutic dilemma.

Primary Bartholin Adenocarcinoma: a Rare Case and Radiotherapy as Definitive Treatment.

Primary vulvar adenocarcinoma is a very rare neoplasm, accounting for only 1% of all gynecologic malignancies. Most of the glandular carcinomas originated from the Bartholin's gland. Because of the rare incidence, the pathogenesis and radiotherapy response are not fully understood. A 47-year-old female from our hospital was diagnosed with primary Bartholin adenocarcinoma and received radiotherapy as definitive treatment. We evaluated the presence of high-risk and low-risk human papillomavirus (HPV) DNA to associate the role of HPV infection, and evaluated its molecular features by the expression of vimentin, p16, estrogen receptor, progesterone receptor, S-100, and Ki 67.

Enteric Type Bartholin Gland Adenocarcinoma: An Unusual Variant of a Rare Neoplasm.

Vulval cancer is rare. With Bartholin gland carcinomas representing <5% of all vulval carcinomas they present both diagnostic and management challenges. There are a small number of cases in the literature describing Bartholin gland carcinomas with unusual histology which necessitates the need to explore the possibility of metastases from elsewhere. We present a case of a 55-yr-old woman presenting with a vulval lesion within the Bartholin gland. Morphology demonstrated enteric type adenocarcinoma and the immunohistochemistry profile was positive for CK7, CK20, CDX2, CEA, and CA19-9. There was no evidence of an alternative primary cancer and the tumor was excised with negative regional sentinel node assessment. Genotyping showed no detectable mutations in KRAS, BRAF or NRAS suggesting a possible future role for anti-EGFR therapy.

Primary adenocarcinomas of the vulva and related structures: An enigmatic and diverse group of tumors✰.

Approximately half of adenocarcinomas that involve the vulva are secondary, either through direct extension or metastases from elsewhere. Primary vulvar adenocarcinomas are rare and encompass a diverse array of neoplasms that are nominally classified based on the presumed tissue or organ of origin, the tumoral phenotype, or both. In this review, we summarize the clinicopathologic features of adenocarcinomas that originate from the vulva and related structures, including the terminal urethra. Adenocarcinomas of this region encompass lesions that are defined by their primary site (such as adenocarcinomas of the Bartholin gland, which by definition must be in the region of the Bartholin gland), histomorphology and immunophenotype (such as clear cell carcinoma and adenocarcinoma of intestinal [cloacogenic] type), or both (such as adenocarcinoma of skene gland origin, which is associated with that specific organ but which also displays a distinctive phenotype that is similar to the phenotype of high grade prostatic adenocarcinoma). Other types, such as mammary-type adenocarcinomas, are presumed to originate from the putative mammary-like glands of the vulva and display a spectrum of pathologic features that are similar to their mammary counterparts. Similarly, vulvar carcinomas of sweat gland origin are pathologically similar to their counterparts in the non-vulvar skin and include a variety of cutaneous adnexal-type malignancies such as apocrine adenocarcinoma and eccrine adenocarcinoma. Some tumors, such as adenoid cystic carcinoma, may represent a Bartholin gland adenocarcinoma, a carcinoma of sweat gland origin, or a carcinoma arising from extramammary Paget disease (EMPD), depending on the context. Invasive carcinomas of various types have been reported in 7-12.7% of EMPD, and these are likely the most common primary glandular malignancy of the vulva. Occasional vulvar adenocarcinomas have been reported to be HPV-associated, although this association has not been established for the broader group of vulvar adenocarcinomas. Rare adenocarcinomas are not classifiable by the aforementioned nosologic scheme, and are designated as vulvar adenocarcinoma NOS.