Search Results (36)

Background: Intestinal-type vulvar adenocarcinoma (VAIt) is an exceptionally rare form of primary vulvar cancer, characterized by histological features resembling mucinous colonic carcinomas, including villo-glandular structures composed of goblet and Paneth cells with intracytoplasmic mucin. Objective: To provide a comprehensive synthesis of the existing literature on VAIt and to also report a case from our institution in order to define its clinical, pathological, and immunohistochemical characteristics and its management and prognosis. Materials and Methods: A systematic review of the literature according to PRISMA guidelines was performed through searching five electronic databases (MEDLINE, EMBASE, Web of Science, SCOPUS and Cochrane Library), considering studies from 1998 to May 2025. In our research, we included all peer-reviewed studies which reported cases of VAIt. Data about VAIt were extracted by included studies and compared. Results: All in all, 32 studies with a total of 40 cases (including our case) of VAIt were assessed. The median age at diagnosis was 58 years. Most tumors arose in the labia or perineal structures, often mimicking benign lesions. Immunohistochemistry consistently showed CK20 and CDX2 positivity, with variable CK7 and p16 expression. FIGO stage IA was the most frequent stage at diagnosis. Surgical excision was the mainstay of treatment, while adjuvant therapy was less commonly reported. Lymph node metastases were present in about 31.5% of cases. Despite aggressive histology, most patients were disease-free at follow-up. Mortality due to disease occurred in 10% of cases. Conclusions: VAIt is a very rare histotype of vulvar cancer. Compared to vulvar squamous cell carcinomas, approximately 40% of early-stage clinical diseases reported in the literature presented positive inguinal lymph nodes with recurrence even after many years. The optimal treatment is not well defined and should be based on the individual clinical history of the patient, as there are no established guidelines. Further studies and longer follow-up periods are needed to clarify the best therapeutic management and its long-term prognosis. Full article

Background: Radioactive colloids are considered the standard of care for sentinel lymph node (SLN) detection. An alternative detection method using superparamagnetic iron oxide (SPIO) nanoparticles is well documented in breast cancer but poorly studied for gynecological tumors, including vulvar cancer (VC). Objective: Our aim was to evaluate the feasibility, accuracy, and safety of SPIO nanoparticles for SLN mapping in patients with VC as a stand-alone technique compared with the combination of two methods: the standard of care using a radioactive isotope (technetium-99; Tc-99) and SPIO as a new tracer. Methods: We conducted a prospective and observational study of SLN mapping in patients with stage IB VC and tumor size ≤ 4 cm. We calculated detection and malignancy rates per patient and per groin in both study groups. During the 36-month follow-up, the groin recurrence rate was estimated for positive and negative SLNs. Kaplan–Meyer curves were used to analyze the probability of survival, depending on disease-free survival. Results: A total of 110 groins assessed by SLN in 60 patients included in this study were analyzed (70 groins from 40 patients in the group with a single tracer and 40 groins from 20 patients in the group of combined tracers). At least one sentinel lymph node was detected in every patient while the bilateral detection rate was 92.3% for the SPIO group and 88.2% for the Tc-99 and SPIO group. The groin detection rate was 94.3% and 90%, respectively. SLN mapping failure was similar in both groups (2.8% and 2.5%, respectively). During a 3-year follow-up, the isolated groin recurrence rate was 2.1% for negative groins and for disease-free survival it was 28.9 months in the combined tracer group versus 32.8 months in the SPIO group. The Kaplan–Meyer curves showed the increased probability of survival for the SPIO group (87.5%); however, it was insignificant. Conclusions: SLN mapping using the SPIO technique in patients with VC is non-inferior to the combined SPIO and Tc-99 method. Full article

Introduction: Pelvic exenteration (PEx) was first described in the 1940s as a palliative procedure in managing cervical cancer. Since then, advancements in perioperative care have transformed the options available to patients. This highly morbid procedure now offers a “cure” in a select cohort of patients with locally advanced and recurrent pelvic cancers. The large volume of literature in this field has resulted in a heterogeneity of data reporting, making comparative analysis extremely difficult. As such, we set out to examine the current literature and identify currently reported outcomes to guide development of a core information set (CIS) for data reporting for PEx in non-rectal cancers. Methods: A systematic review was carried out. Studies reporting on outcomes following PEx for advanced and recurrent gynecological, urological, and other non-rectal malignancies were included. Standardized outcomes were extracted and mapped to pre-determined domains. Results: Forty-four studies were found to meet our inclusion criteria. A total of 1735 data elements (DEs) were extracted verbatim, and these were assimilated into 111 standard DEs across nine domains. A wide range of reporting frequencies was observed, with the pathological domain containing the highest overall frequencies of DE reporting. Conversely, patient-reported and functional outcomes were noted to be the domain with the lowest frequency. Conclusions: This review highlights recent trends of increased reporting in the field of PEx and how this had invariably resulted in heterogeneous data reporting. We aim to guide the development of a CIS for reporting in non-rectal pelvic malignancies to help standardize future reporting. Full article

Background: Vulvar leiomyosarcoma (VLMS) is a rare and aggressive soft tissue malignancy arising from smooth muscle cells, comprising less than 3% of vulvar cancers. Its clinical resemblance to benign vulvar lesions often leads to delayed diagnosis. Despite surgical resection and adjuvant therapy, VLMS is associated with high recurrence rates and a poor prognosis, and due to its rarity, there is no standardized management or surveillance protocol. Case Report: We present a case of high-grade VLMS in a postmenopausal woman, initially diagnosed in 2020 and managed with surgical excision and adjuvant radiotherapy. The primary tumor was a 10 cm solid, lobulated mass involving the mons pubis, with histology confirming high-grade leiomyosarcoma based on marked cellular atypia, high mitotic activity, and smooth muscle differentiation. Immunohistochemistry was positive for SMA, vimentin, and CD34, and negative for S100 and MyoD1. Five years later, the patient developed a local recurrence with an enlarged inguinal lymph node. She underwent complete tumor resection and bilateral inguinal lymphadenectomy. Histology of the recurrent lesion mirrored the initial findings, with no lymph node metastases. This case highlights the aggressive nature and potential for late recurrence in vulvar leiomyosarcoma, underscoring the importance of long-term surveillance. Conclusions: High-grade VLMS is a rare malignancy with a high recurrence risk. This case highlights the importance of early diagnosis, radical surgical treatment, and long-term surveillance. Although recurrence occurred five years after the initial treatment, timely surgical intervention led to a favorable postoperative course. Multidisciplinary management and individualized follow-up strategies remain key to improving outcomes in these rare gynecologic sarcomas. Full article

Objectives: Despite the relatively high incidence of vulvar cancer, there is a noticeable lack of studies in Romania and other Eastern European countries focused on evaluating the long-term oncological outcomes and Quality of Life (QoL) for patients with this condition. Methods: A total of 91 patients were included in the study. The first objective was to evaluate the 5-year overall survival (OS) in patients with vulvar cancer at International Federation of Gynecology and Obstetrics (FIGO) stages IA-IVA who underwent surgery, ±adjuvant radiotherapy (RT). Additionally, the study aimed to identify prognostic factors that could either positively or negatively influence survival outcomes in these patients. The second objective was to assess the QoL, conducted using validated questionnaires issued by the European Organization for Research and Treatment of Cancer, specifically the QLQ-CX30 and QLQ-VU34. Results: The patients had an average age of 67.7 years (38–91). At the time of assessment, 51.6% of the patients were alive. Additionally, the 5-year OS was reported at 45%. The multivariate analysis indicated that age ≤ 50 years (p < 0.03), FIGO stage IB (p < 0.007), and tumor differentiation grade I (p < 0.01) were associated with improved survival rates. Conversely, age > 80 years (p < 0.05), FIGO stages IIIB (p < 0.01) and IIIC (p < 0.06), tumor size > 5 cm (p < 0.02), positive resection margins (p < 0.03), lymph node metastasis (p < 0.06), and pelvic exenteration (p < 0.002) were identified as independent negative prognostic factors. Of the 47 living patients, 32 completed the QoL questionnaires. The respondents reported a decent overall QoL score of 65.3. However, treatment-specific symptoms, such as vulvar scarring, vulvar swelling, groin lymphedema, and leg lymphedema, had a negative impact on QoL. Consequently, functional symptoms like fatigue, pain, and sleep disturbances persisted, leading to a body image perception score of 33.7 on a scale from 0 to 100. Conclusions: This study highlights decent OS and QoL outcomes. It is important to note that vulvar cancer primarily affects older women. In this study, 51.6% of patients were over 70 years old at the time of surgery. Consequently, the 5-year OS of 45% could not be attributed solely to oncological factors, as most of these patients did not die from recurrences but rather from associated comorbidities. The findings of this study provide a foundation for future randomized controlled trials aimed at further enhancing vulvar cancer patients’ care and outcomes. Full article

Objective: Vulvar cancer surgery is associated with high postoperative wound complication rates. Reconstructive surgery (RS) in vulvar cancer is generally reserved for surgery of extensive tumors or local recurrences. The primary aim of the study is to determine the incidence and risk factors for wound complications after vulvar cancer surgery. As a secondary aim, we compare the effects of primary closure (PC) versus reconstructive surgery on wound complications. Methods: In a retrospective cohort study in four gynecologic oncology centers in the Netherlands, patients undergoing surgical treatment (2018–2022) for vulvar cancer were included. Wound complications after PC and RS and risk factors associated with complications were analyzed by using logistic regression adjusting for confounds. Results: We included 394 women, 318 with PC and 76 with RS. The incidence of wound complications was 46.7%, with 42.4% of wound breakdowns comprising the majority of complications. The use of RS was associated with an increased risk of wound complications. Larger tumor size, proximity to the urethra, resection of the urethra during surgery, and perineal tumor location were additional risk factors for wound complications. However, after multivariate analyses, RS remained the only significant risk factor (OR 1.2; 95% CI 1.1–1.2). Conclusions: Risk factors for wound complications after vulvar cancer surgery include larger tumor size, proximity to the urethra, resection of the urethra during surgery, and perineal tumor location. RS is also associated with an increased risk of wound complications, probably related to case selection. Full article

Background/Objectives: Several histopathological risk factors have been examined in vulvar cancer (VC) so far. However, the prognostic relevance of morphological biomarkers such as tumor budding (TB) and poorly defined clusters (PDCs) remains to be determined. Material and Methods: We histologically analyzed the formation of peritumoral and intratumoral TB and PDCs in a cohort of 157 patients with VC. We assessed their association with clinico-pathological features and evaluated their prognostic impact in terms of the risk of local recurrence and occurrence of metastasis (Fisher’s exact test) as well as overall survival (Log-rank test). Results: We determined a distinct prognostic relevance of peritumoral TB with regard to occurrence of metastasis (Fisher’s exact test; p = 0.0415) as well as a significant reduced risk of local recurrence in the group with absent intratumoral TB (Fisher’s exact test; p = 0.0004). Furthermore, we showed that patients without peritumoral budding formation had a significant superior prognosis in terms of overall survival (p = 0.0366, x² = 4.370). Conclusions: This study shows that several new histomorphological biomarkers may serve useful in predicting the clinical course of patients with VC, identifying patients at a lower risk of developing metastases/local recurrence as well as improved overall survival. Full article

Objective: We aimed to analyze the current literature for IORT in gynecological cancers and summarized clinical outcomes regarding patient selection. Methods: A systematic search was conducted utilizing PUBMED, Embase, and CINAHL to identify studies following PRISMA-ScR guidelines. A PICOS structure was utilized: population: patients with epithelial gynecological cancers; intervention: IORT; C: a comparator was not required, as we aimed to analyze patient selection; outcome: clinical outcomes and overall survival; and S: experimental and quasi-experimental analytical observational studies and descriptive observational studies, excluding case series published in English and limited to the last 10 years. Data extraction was conducted for patient selection, IORT, oncological outcomes, and morbidity. Results: A total of 707 results were identified, and 509 studies were uploaded to Covidence for screening after removing duplications. Of the 21 eligible studies, 9 were included in the final review. The total number of patients included was 348. The studies were retrospective single-institution studies, except for one. There was significant heterogeneity in their design and protocols. IORT was exclusively used for recurrent and advanced stage gynecological cancers adjunct to pelvic exenteration or laterally extended endopelvic resections with variable indications across institutions. The mean number of IORT patients per study was 2.8 per year. Survival rates were variable and dependent on the surgical margin. Endometrial cancer had a favorable outcome compared to vulvar and cervical cancers. Conclusions: Current clinical practice, as demonstrated by the research, is consistent with NCCN guidelines that endorse the application of IORT in instances of recurrent cervical, vaginal, and vulvar malignancies; however, there are no established recommendations for primary tumors. The analysis shows that there are gaps in our knowledge, mainly regarding the status of the margins, the criteria used to choose patients, and the outcomes that are specific to each histology. The standardization of protocols and prospectively powered studies are needed to refine patient selection criteria. Full article

Background: Vulvar cancer is a rare gynecologic malignancy with increasing incidence. Lymph node status is the most critical prognostic factor, traditionally assessed through inguinofemoral lymphadenectomy, a procedure associated with significant morbidity. Sentinel lymph node biopsy (SLNB), in selected cases, has emerged as a less invasive alternative with favorable oncologic outcomes. Objective: This review summarizes current evidence on the indications, technique, safety, and oncologic outcomes of SLNB in vulvar cancer, with a focus on controversial scenarios such as recurrent and larger tumors. Methods: A narrative review of PubMed-indexed studies published in English over the last 35 years was conducted. Eligible studies included original research, systematic reviews, meta-analyses, randomized controlled trials, and case-control studies. Results: SLNB is recommended for unifocal vulvar tumors < 4 cm with stromal invasion > 1 mm and clinically negative nodes. Landmark trials, including GROINSS-V-I and GOG-173, confirmed its accuracy and lower morbidity compared to lymphadenectomy. SLNB utilization has increased since its inclusion in guidelines, with a concurrent decline in lymphadenectomy rates. Combined detection techniques are mandatory, while indocyanine green (ICG) is an emerging option. Future studies should focus on refining patient selection criteria, improving detection techniques, and clarifying the implications of low-volume nodal disease to further optimize outcomes for patients with vulvar cancer. Conclusion: SLNB is a validated, minimally invasive staging approach in early-stage vulvar cancer. Further research is needed to refine its role in high-risk cases and optimize detection methods. Full article

Angiomyofibroblastoma (AMFB) is an exceedingly rare mesenchymal tumor of the lower genital tract. AMFB primarily affects the pelviperineal region, especially the vulvar in premenopausal women. Typically, AMFB is a benign disease and does not have the potential for metastasis or recurrence, requiring complete surgical excision. Its accurate differentiation from aggressive angiomyxoma is critical due to varying prognoses. A 51-year-old woman, diagnosed with mucinous carcinoma of the breast, presented with a 12 cm abdominopelvic mass identified during breast cancer staging. Imaging suggested an ovarian origin; however, surgical exploration revealed a stalk-attached cystic mass in the anterior body of the uterus. Histopathology confirmed AMFB. Immunohistochemical analysis showed positivity for estrogen and progesterone receptors and smooth muscle actin. The patient continued breast cancer treatment postoperatively without pelvic mass recurrence or complications for a postoperative follow-up period of one year. This case highlights AMFB’s potential uterine body origin, expending known tumor sites and complicating diagnosis due to overlapping features with other mesenchymal tumors. Accurate diagnosis using immunohistochemical markers and pathological features is essential to avoid unnecessary aggressive treatments. The uterine location in this case suggests a possible shared pathogenesis with uterine myomas, warranting further research into their connection. Reporting the first case of AMFB originating in the uterine body enhances understanding of this rare condition and underscores the importance of clinical awareness and precise diagnostic strategies to guide management and improve outcomes. Full article

Background: Vulvar stenosis is a debilitating condition that compromises sexual function, urination, and the ability to undergo gynecological examinations. The purpose of this study is to describe the technique of capillary perforator perineal flaps (CPPF) for the correction of vulvar stenosis. Methods: We retrospectively examined patients with vulvar stenosis treated through surgical separation and reconstruction with CPPF. The procedure involved vulvar separation with the creation of a subsequent defect, repaired using a flap, harvested laterally to the labia majora including a capillary perforator and transferred through a subcutaneous tunnel to repair the vulvar defect. The functional outcome was evaluated with the Bradford scale, comparing the preoperative and postoperative scores using the Student’s t-test. Results: thirteen patients were included, three with stenosis following treatment for vulvar cancer and ten due to lichen sclerosus. In total, we analyzed 29 flaps, with an average size of 15.6 cm². We always included just one perforator in the flap and no postoperative complications. Stenosis was resolved in all patients, with no recurrences one year after the surgery. The preoperative average severity of the stenosis was 2.3 + 0.6, reducing to 0.3 + 0.4 post-intervention, indicating a significant improvement (p < 0.01). Conclusions: CPPF has proven to be a quick and safe method for the reconstruction of vulvar stenosis. Full article

The aim of this study was to explore the outcomes of pelvic reconstruction with a rectus abdominis myocutaneous (RAM) or rectus abdominis myoperitoneal (RAMP) flap following radical surgery for gynecologic malignancy. This is a retrospective case series of all pelvic reconstructions with RAM or RAMP flap performed in a gynecologic oncology service between 1998 and 2023. Reconstructions with other flaps were excluded. A total of 28 patients were included. Most patients had vulvar cancer (n = 15, 53.6%) and the majority had disease recurrence (n = 20, 71.4%). Exenteration was the most common procedure, being carried out in 20 (71.4%) patients. Pelvic reconstruction was carried out with a RAM flap in 24 (85.7%) cases and a RAMP flap in 4 (14.3%) cases. Flap-specific complications included cellulitis (14.3%), partial breakdown (17.9%), and necrosis (17.9%). Donor site complications included surgical site infection and necrosis occurring in seven (25.0%) and three (10.7%) patients, respectively. Neovaginal reconstruction was performed in 14 patients. Out of those, two (14.3%) had neovaginal stenosis and three (21.4%) had rectovaginal fistula. In total, 50% of patients were disease-free at the time of the last follow up. In conclusion, pelvic reconstruction with RAM/RAMP flaps, at the time of radical surgery for gynecologic cancer, is an uncommon procedure. In our case series, we had a significant complication rate with the most common being infection and necrosis. The development of a team approach, with input from services including Gynecologic Oncology and Plastic Surgery should be developed to decrease post-operative complications and improve patient outcomes. Full article

Pelvic exenteration (PE) is one of the most radical surgical approaches. In earlier times, PE was associated with high morbidity and mortality. Nowadays, due to improved selection of suitable patients, perioperative settings, and postoperative care, patients’ outcomes have been optimized. To investigate patients’ outcomes and identify possible influencing clinical and histopathological factors, we analysed 17 patients with recurrent vulvar cancer who underwent PE in our department between 2007 and 2022. The median age was 64.9 years, with a difference of 40 years between the youngest and the oldest patient (41 vs. 81 years). The mean overall survival time was 55.7 months; the longest survival time reached up to 164 months. The achievement of complete cytoreduction (p = 0.02), the indication for surgery (curative vs. palliative), and the presence of distant metastases (both p = 0.01) showed a significant impact on overall survival. The presence of lymphatic metastases (p = 0.11) seems to have an influence on overall survival (OS) time. Major complications appeared in 35% of the patients. Our results support the existing data for PE in cases of recurrent vulvar cancer; for a group of selected patients, PE is a treatment option with good overall survival times and acceptable morbidity. Full article

Vulvar cancer is a relatively rare neoplasm. The essential treatment is surgery for the primary tumour. However, postoperative recurrence rates are high, even in early-stage disease when tumour-free surgical margins are achieved or in the absence of associated risk factors (lymph node metastases, deep stromal invasion or invasion of the lymphatic vascular space). Radiotherapy plays an important role in the treatment of vulvar cancer. Adjuvant treatment after surgery as well as primary treatment of locally advanced vulvar cancer (LAVC) is composed of two key radiotherapy treatment scenarios, external beam radiation therapy (EBRT) either combined or not combined with brachytherapy (BT). In a recurrence setting, where surgery is not an option, BT alone or in combination with EBRT can be used. Compared to EBRT, BT has the radiobiological potential to improve dose to the target volume, minimise the dose to organs at risk, and facilitate hypofractionated-accelerated treatment. This narrative review presents recent data on the role of BT in the treatment of primary and/or recurrent vulvar cancer, including radiobiological, clinical, and therapeutic aspects. Full article

Background: The aim of this study was to evaluate the impact of pathological tumor-free margin distance on survival in SCC patients treated with surgery alone. Methods: This retrospective study included 128 patients with node-negative disease that received no adjuvant treatment. Disease-free and overall survival were analyzed according to pathological tumor-free margin distance. Results: The patients were subclassified into three resection margin category groups: “1 to 3 mm” (n = 42), “>3 to 8 mm” (n = 47) or “>8 mm” (n = 39). Thirty-nine of the 128 patients (30.5%) developed recurrent disease. Median follow-up for disease-free survival (DFS) was 6.49 years (95% CI 5.16 years; 7.62 years), and median follow-up for overall survival (OS) was 6.29 years (95% CI 5.45 years; 7.33 years). The 5-year DFS rate was 0.70 (95% CI: 0.62–0.79), and the 5-year OS rate was 0.79 (95% CI: 0.71–0.87). Regarding the survival outcome, there were no independent significant differences in either disease-free survival (DFS) (p = 0.300) or overall survival (p = 1.000) among patients within the three tumor-free resection margin categories. Multivariate analyses did not show any statistically significant association between tumor-free resection margin distance and recurrent disease or death, either when analyzed as a categorical variable or when analyzed as a continuous variable. Conclusion: The present study did not show a significant impact of pathological tumor-free resection margin distance following surgery in patients with node-negative SCC of the vulva (that did not receive adjuvant treatment) on disease-free and overall survival. Full article