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Open AccessEditor’s ChoiceReview

Aberrant SWI/SNF Complex Members Are Predominant in Rare Ovarian Malignancies—Therapeutic Vulnerabilities in Treatment-Resistant Subtypes

by Yue Ma, Natisha R. Field, Tao Xie, Sarina Briscas, Emily G. Kokinogoulis, Tali S. Skipper, Amani Alghalayini, Farhana A. Sarker, Nham Tran, Nikola A. Bowden, Kristie-Ann Dickson and Deborah J. Marsh

Cancers 2024, 16(17), 3068; https://doi.org/10.3390/cancers16173068 - 3 Sep 2024

Cited by 1 | Viewed by 1836

Abstract

SWI/SNF (SWItch/Sucrose Non-Fermentable) is the most frequently mutated chromatin-remodelling complex in human malignancy, with over 20% of tumours having a mutation in a SWI/SNF complex member. Mutations in specific SWI/SNF complex members are characteristic of rare chemoresistant ovarian cancer histopathological subtypes. Somatic mutations [...] Read more.

SWI/SNF (SWItch/Sucrose Non-Fermentable) is the most frequently mutated chromatin-remodelling complex in human malignancy, with over 20% of tumours having a mutation in a SWI/SNF complex member. Mutations in specific SWI/SNF complex members are characteristic of rare chemoresistant ovarian cancer histopathological subtypes. Somatic mutations in ARID1A, encoding one of the mutually exclusive DNA-binding subunits of SWI/SNF, occur in 42–67% of ovarian clear cell carcinomas (OCCC). The concomitant somatic or germline mutation and epigenetic silencing of the mutually exclusive ATPase subunits SMARCA4 and SMARCA2, respectively, occurs in Small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT), with SMARCA4 mutation reported in 69–100% of SCCOHT cases and SMARCA2 silencing seen 86–100% of the time. Somatic ARID1A mutations also occur in endometrioid ovarian cancer (EnOC), as well as in the chronic benign condition endometriosis, possibly as precursors to the development of the endometriosis-associated cancers OCCC and EnOC. Mutation of the ARID1A paralogue ARID1B can also occur in both OCCC and SCCOHT. Mutations in other SWI/SNF complex members, including SMARCA2, SMARCB1 and SMARCC1, occur rarely in either OCCC or SCCOHT. Abrogated SWI/SNF raises opportunities for pharmacological inhibition, including the use of DNA damage repair inhibitors, kinase and epigenetic inhibitors, as well as immune checkpoint blockade. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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19 pages, 1701 KiB

Open AccessReview

Novel Endocrine Therapeutic Opportunities for Estrogen Receptor-Positive Ovarian Cancer—What Can We Learn from Breast Cancer?

by Tine Ottenbourgs and Els Van Nieuwenhuysen

Cancers 2024, 16(10), 1862; https://doi.org/10.3390/cancers16101862 - 13 May 2024

Cited by 1 | Viewed by 1330

Abstract

Low-grade serous ovarian cancer (LGSOC) is a rare ovarian malignancy primarily affecting younger women and is characterized by an indolent growth pattern. It exhibits indolent growth and high estrogen/progesterone receptor expression, suggesting potential responsiveness to endocrine therapy. However, treatment efficacy remains limited due [...] Read more.

Low-grade serous ovarian cancer (LGSOC) is a rare ovarian malignancy primarily affecting younger women and is characterized by an indolent growth pattern. It exhibits indolent growth and high estrogen/progesterone receptor expression, suggesting potential responsiveness to endocrine therapy. However, treatment efficacy remains limited due to the development of endocrine resistance. The mechanisms of resistance, whether primary or acquired, are still largely unknown and present a significant hurdle in achieving favorable treatment outcomes with endocrine therapy in these patients. In estrogen receptor-positive breast cancer, mechanisms of endocrine resistance have been largely explored and novel treatment strategies to overcome resistance have emerged. Considering the shared estrogen receptor positivity in LGSOC and breast cancer, we wanted to explore whether there are any parallel mechanisms of resistance and whether we can extend endocrine breast cancer treatments to LGSOC. This review aims to highlight the underlying molecular mechanisms possibly driving endocrine resistance in ovarian cancer, while also exploring the available therapeutic opportunities to overcome this resistance. By unraveling the potential pathways involved and examining emerging strategies, this review explores valuable insights for advancing treatment options and improving patient outcomes in LGSOC, which has limited therapeutic options available. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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15 pages, 1210 KiB

Open AccessReview

Aggressive Angiomyxoma of the Lower Female Genital Tract: A Review of the MITO Rare Tumors Group

by Miriam Dellino, Francescapaola Magazzino, Lavinia Domenici, Stefania Cicogna, Salvatora Tindara Miano, Sandro Pignata, Giorgia Mangili and Gennaro Cormio

Cancers 2024, 16(7), 1375; https://doi.org/10.3390/cancers16071375 - 31 Mar 2024

Cited by 2 | Viewed by 1458

Abstract

Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors [...] Read more.

Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors and absence of characteristic signs and symptoms. The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Other non- surgical modalities have been employed, such as radiotherapy, embolization, GnRH analogues or other anti-estrogenic agents. Local relapses occur in 30–40% of the cases, and often appear many years (sometimes decades) after the first excision. Occasional distant metastasis has also been reported. A limited number of cases have been reported in the literature, mostly in the form of small case series or isolated case reports. Therefore, the aim of this paper by a team of experts from the MITO rare tumors group is to review clinical findings, pathologic characteristics and outcome of patients affected by this rare condition in order to be able to offer up-to-date guidance on the management of these cases. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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15 pages, 1708 KiB

Open AccessReview

Dermatofibrosarcoma Protuberans of the Vulva: A Review of the MITO Rare Cancer Group

by Rosanna Mancari, Raffaella Cioffi, Francescapaola Magazzino, Laura Attademo, Miriam Sant’angelo, Gianluca Taccagni, Giorgia Mangili, Sandro Pignata and Alice Bergamini

Cancers 2024, 16(1), 222; https://doi.org/10.3390/cancers16010222 - 3 Jan 2024

Cited by 1 | Viewed by 1558

Abstract

Background: Vulvar dermatofibrosarcoma protuberans is an extremely rare disease. Its rarity can hamper the quality of treatment; deeper knowledge is necessary to plan appropriate management. The purpose of this review is to analyse the data reported in the literature to obtain evidence regarding [...] Read more.

Background: Vulvar dermatofibrosarcoma protuberans is an extremely rare disease. Its rarity can hamper the quality of treatment; deeper knowledge is necessary to plan appropriate management. The purpose of this review is to analyse the data reported in the literature to obtain evidence regarding appropriate disease management. Methods: We made a systematic search of the literature, including the terms “dermatofibrosarcoma protuberans”, “vulva”, and “vulvar”, alone or in combination. We selected articles published in English from two electronic databases, PubMed and MEDLINE, and we analysed their reference lists to include other potentially relevant studies. Results: We selected 39 articles, with a total of 68 cases reported; they were retrospective case reports and case series. Dermatofibrosarcoma protuberans of the vulva tends towards local recurrence; an early and timely pathological diagnosis, together with an appropriate surgical approach, are of utmost importance to ensure free margins and maximise the curative potential. Conclusions: Even if this is an indolent disease and it generally shows a good prognosis, appropriate management may help in reducing the rate of local recurrences that may hamper patients’ quality of life. Management by a multidisciplinary team is highly recommended. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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19 pages, 931 KiB

Open AccessReview

Primary Retroperitoneal Carcinomas: New Insights into Pathogenesis and Clinical Management in Comparison with Ovarian Carcinomas and Carcinoma of Unknown Primary

by Isao Otsuka

Cancers 2023, 15(18), 4614; https://doi.org/10.3390/cancers15184614 - 18 Sep 2023

Cited by 2 | Viewed by 1576

Abstract

Primary retroperitoneal carcinomas are very rare tumors. Their pathogenesis remains unknown but may be associated with that of ovarian carcinomas, considering the similarity in morphology and gender preference. Although metaplasia of coelomic epithelium is the most widely accepted theory, the pathogenesis of retroperitoneal [...] Read more.

Primary retroperitoneal carcinomas are very rare tumors. Their pathogenesis remains unknown but may be associated with that of ovarian carcinomas, considering the similarity in morphology and gender preference. Although metaplasia of coelomic epithelium is the most widely accepted theory, the pathogenesis of retroperitoneal carcinomas may differ by histologic subtype, like ovarian carcinomas. Mucinous carcinoma, which develops in both women and men, may originate in both primordial germ cells and Walthard cell nests that may be derived from the fallopian tube. Serous carcinomas may be associated with endosalpingiosis, the presence of fallopian tube-like epithelium outside the fallopian tube, and a remnant Müllerian tract. Endometrioid and clear cell carcinomas appear to be associated with extraovarian endometriosis. Additionally, both carcinomas in the retroperitoneal lymph nodes may be metastatic diseases from endometrial and/or renal cell cancer that regress spontaneously (carcinoma of unknown primary). Retroperitoneal carcinomas are difficult to diagnose, as they have no characteristic symptoms and signs. Surgery is the cornerstone of treatment, but the necessity of chemotherapy may depend on histological subtype. Further studies are necessary, in particular studies on endosalpingiosis, as endosalpingiosis is a poorly understood condition, although it is associated with the development of both serous and mucinous carcinomas. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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11 pages, 1114 KiB

Open AccessReview

Aggressive Angiomyxoma of the Lower Female Genital Tract in Pregnancy: A Review of the MITO Rare Tumors Group

by Stefania Cicogna, Miriam Dellino, Salvatora Tindara Miano, Francescapaola Magazzino, Lavinia Domenici, Sandro Pignata, Giorgia Mangili and Gennaro Cormio

Cancers 2023, 15(13), 3403; https://doi.org/10.3390/cancers15133403 - 29 Jun 2023

Cited by 1 | Viewed by 1857

Abstract

Deep (aggressive) angiomyxoma of the lower genital tract is a rare malignancy affecting women of reproductive age. Being a hormone-sensitive tumor, its growth is particularly benefitted during pregnancy. Surgical excision with complete resection is indicated, even if a wait-and-see approach can be considered [...] Read more.

Deep (aggressive) angiomyxoma of the lower genital tract is a rare malignancy affecting women of reproductive age. Being a hormone-sensitive tumor, its growth is particularly benefitted during pregnancy. Surgical excision with complete resection is indicated, even if a wait-and-see approach can be considered until delivery, to avoid destructive surgeries. The mode of delivery is to be evaluated based on the location and size of the neoplasm; vaginal delivery is not contraindicated, as long as the tumor does not obstruct the birth canal. Positive surgical margins are the most important prognostic factor for recurrence. Adjuvant therapy with gonadotropin-releasing hormone analogues may be proposed after pregnancy, in the case of non-radical surgery. Despite the high local relapse rate, the outcomes for mother and child are favorable. Since recurrences can occur after many years, the patient should be included in long-term follow-up. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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30 pages, 9288 KiB

Open AccessReview

Primary Ovarian Leiomyosarcoma Is a Very Rare Entity: A Narrative Review of the Literature

by Vincenzo Dario Mandato, Federica Torricelli, Valentina Mastrofilippo, Andrea Palicelli, Luigi Costagliola and Lorenzo Aguzzoli

Cancers 2023, 15(11), 2953; https://doi.org/10.3390/cancers15112953 - 28 May 2023

Cited by 4 | Viewed by 1791

Abstract

Background: Primary ovarian leiomyosarcoma is a very rare malignancy characterized by unclear management and poor survival. We reviewed all the cases of primary ovarian leiomyosarcoma to identify prognostic factors and the best treatment. Methods: We collected and analyzed the articles published in the [...] Read more.

Background: Primary ovarian leiomyosarcoma is a very rare malignancy characterized by unclear management and poor survival. We reviewed all the cases of primary ovarian leiomyosarcoma to identify prognostic factors and the best treatment. Methods: We collected and analyzed the articles published in the English literature regarding primary ovarian leiomyosarcoma from January 1951 to September 2022, using PubMed research. Clinical and pathological characteristics, different treatments and outcomes were analyzed. Results: 113 cases of primary ovarian leiomyosarcoma were included. Most patients received surgical resection, associated with lymphadenectomy in 12.5% of cases. About 40% of patients received chemotherapy. Follow-up information was available for 100/113 (88.5%) patients. Stage and mitotic count were confirmed to affect survival, and lymphadenectomy and chemotherapy were associated with a better survival rate. A total of 43.4% of patients relapsed, and their mean disease-free survival was 12.5 months. Conclusions: Primary ovarian leiomyosarcomas are more common in women in their 50s (mean age 53 years). Most of them are at an early stage at presentation. Advanced stage and mitotic count showed a detrimental effect on survival. Surgical excision associated with lymphadenectomy and chemotherapy are associated with increased survival. An international registry could help collect clear and reliable data to standardize the diagnosis and treatment. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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11 pages, 286 KiB

Open AccessReview

Lymph Node Involvement in Recurrent Serous Borderline Ovarian Tumors: Current Evidence, Controversies, and a Review of the Literature

by Laureline Wetterwald, Apostolos Sarivalasis, Aikaterini Liapi, Patrice Mathevet and Chahin Achtari

Cancers 2023, 15(3), 890; https://doi.org/10.3390/cancers15030890 - 31 Jan 2023

Cited by 5 | Viewed by 2718

Abstract

Borderline ovarian tumors (BOTs) account for 10–20% of epithelial ovarian neoplasms. They are characterized by their lack of destructive stromal invasion. In comparison to invasive ovarian cancers, BOTs occur in younger patients and have better outcome. Serous borderline ovarian tumor (SBOT) represents the [...] Read more.

Borderline ovarian tumors (BOTs) account for 10–20% of epithelial ovarian neoplasms. They are characterized by their lack of destructive stromal invasion. In comparison to invasive ovarian cancers, BOTs occur in younger patients and have better outcome. Serous borderline ovarian tumor (SBOT) represents the most common subtype of BOT. Complete surgical staging is the current standard management but fertility-sparing surgery is an option for SBOT patients who are at reproductive age. While most cases of SBOTs have an indolent course with favorable prognosis, late recurrence and malignant transformation can occur, usually in the form of low-grade serous carcinoma (LGSC). Thus, assessment of the recurrence risk is essential for the management of those patients. SBOTs can be associated with lymph node involvement (LNI) in up to 30% of patients who undergo lymph node dissection at diagnosis, and whether LNI affects prognosis is controversial. The present review suggests that recurrent SBOTs with LNI have poorer oncological outcomes and highlights the biases due to the scarcity of reports in the literature. Preventing SBOTs from recurring and becoming invasive overtime and a more profound understanding of the underlying mechanisms at play are necessary. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

11 pages, 1677 KiB

Open AccessReview

Vulvar Malignant Melanoma: A Narrative Review

by Giovanni Falcicchio, Lorenzo Vinci, Ettore Cicinelli, Vera Loizzi, Francesca Arezzo, Erica Silvestris, Leonardo Resta, Gabriella Serio, Gerardo Cazzato, Marialuigia Mastronardi, Michele Mongelli, Marco Marinaccio and Gennaro Cormio

Cancers 2022, 14(21), 5217; https://doi.org/10.3390/cancers14215217 - 25 Oct 2022

Cited by 9 | Viewed by 6658

Abstract

Malignant melanoma is a fatal disease that affects all skin sites. Among these, vulvar melanoma (VM) is a rare gynecological condition that accounts for 5% of all vulvar neoplasms. VM primarily affects older Caucasian women and its relationship to sun exposure is undefined. [...] Read more.

Malignant melanoma is a fatal disease that affects all skin sites. Among these, vulvar melanoma (VM) is a rare gynecological condition that accounts for 5% of all vulvar neoplasms. VM primarily affects older Caucasian women and its relationship to sun exposure is undefined. Diagnosis is defined by biopsy but many clinical, dermatoscopic, and confocal microscopic features can guide doctors. The molecular profile is characterized by the KIT mutation, revealed by all of the technologies that are used (classical sequencing, next-generation sequencing, and immunohistochemical staining). BRAF and NRAS mutations are also common in VM. All of these mutations are possible therapeutic targets. Today, surgery remains the first treatment choice for primary VM. The role of neoadjuvant and adjuvant therapy is scarce and the treatment of relapses is widely debated. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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14 pages, 12402 KiB

Open AccessReview

MRI in the Evaluation of Locally Advanced Vulvar Cancer Treated with Chemoradiotherapy and Vulvar Cancer Recurrence: The 2021 Revision of FIGO Classification and the Need for Multidisciplinary Management

by Maura Miccò, Luca Russo, Salvatore Persiani, Miriam Dolciami, Lucia Manganaro, Teresa Margarida Cunha, Catarina Janicas, Stefania Rizzo, Olivera Nicolic, Giorgia Garganese, Luca Tagliaferri, Valentina Lancellotta, Giovanni Scambia, Riccardo Manfredi and Benedetta Gui

Cancers 2022, 14(16), 3852; https://doi.org/10.3390/cancers14163852 - 9 Aug 2022

Cited by 4 | Viewed by 2425

Abstract

Magnetic resonance imaging (MRI) plays an essential role in the management of patients with locally advanced vulvar cancer (LAVC), who frequently benefit from a multidisciplinary approach. Accordingly, chemoradiotherapy (CRT) with radical or neoadjuvant intent seems to provide a better quality of life and [...] Read more.

Magnetic resonance imaging (MRI) plays an essential role in the management of patients with locally advanced vulvar cancer (LAVC), who frequently benefit from a multidisciplinary approach. Accordingly, chemoradiotherapy (CRT) with radical or neoadjuvant intent seems to provide a better quality of life and less morbidity than extensive surgery alone. In this overview, we discuss the role of MRI in the post-CRT assessment of LAVC, emphasizing the evaluation of primary tumor response. In order to assess treatment response and select candidates for post-CRT local excision, the MRI findings are described according to signal intensity, restricted diffusion, enhancement, and invasion of adjacent organs. We also focus on the role of MRI in detecting vulvar cancer recurrence. It occurs in 30–50% of patients within two years after initial treatment, the majority appearing near the original resection margins or in ipsilateral inguinal or pelvic lymph nodes. Finally, we describe early and delayed complications of CRT, such as cellulitis, urethritis, vulvar edema, bone changes, myositis, and fistulization. By describing the role of MRI in assessing LAVC response to CRT and detecting recurrence, we hope to provide suitable indications for a personalized approach. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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24 pages, 1552 KiB

Open AccessSystematic Review

Malignant Brenner Tumor of the Ovary: A Systematic Review of the Literature

by Nektarios I. Koufopoulos, Abraham Pouliakis, Menelaos G. Samaras, Christakis Kotanidis, Ioannis Boutas, Adamantia Kontogeorgi, Dionysios Dimas, Kyparissia Sitara, Andriani Zacharatou, Argyro-Ioanna Ieronimaki, Aris Spathis, Danai Leventakou, Magda Zanelli, Ioannis S. Pateras, Ioannis G. Panayiotides, Andrea Palicelli and John Syrios

Cancers 2024, 16(6), 1106; https://doi.org/10.3390/cancers16061106 - 9 Mar 2024

Cited by 1 | Viewed by 2413

Abstract

Background: Malignant Brenner tumors are rare ovarian tumors, accounting for less than 1% of malignant ovarian neoplasms. The aim of this manuscript is to systematically review the current literature concerning malignant Brenner tumors. Methods: We searched three medical databases (PubMed, Scopus, and Web [...] Read more.

Background: Malignant Brenner tumors are rare ovarian tumors, accounting for less than 1% of malignant ovarian neoplasms. The aim of this manuscript is to systematically review the current literature concerning malignant Brenner tumors. Methods: We searched three medical databases (PubMed, Scopus, and Web of Science) for relevant articles published until 15 September 2023. Results: After applying inclusion and exclusion criteria, 48 manuscripts describing 115 cases were included in this study from the English literature. Conclusions: We analyzed the demographic, clinical, pathological, and oncological characteristics of 115 patients with malignant Brenner tumors. The statistical analysis showed that recurrence was marginally statistically significantly related to tumor stage and was more common in patients with ascites and in women with abnormal CA-125 levels; patients that were treated with lymphadenectomy had better disease-specific survival. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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47 pages, 856 KiB

Open AccessSystematic Review

Vulvar Paget’s Disease: A Systematic Review of the MITO Rare Cancer Group

by Giuseppe Caruso, Amelia Barcellini, Roberta Mazzeo, Roberta Gallo, Maria Giuseppa Vitale, Anna Passarelli, Giorgia Mangili, Sandro Pignata and Innocenza Palaia

Cancers 2023, 15(6), 1803; https://doi.org/10.3390/cancers15061803 - 16 Mar 2023

Cited by 7 | Viewed by 3845

Abstract

Vulvar Paget’s disease (VPD) is a rare form of cutaneous adenocarcinoma of the vulva, which accounts for about 1–2% of all vulvar neoplasms and mainly affects post-menopausal women. The clinical presentation is usually non-specific and mimics chronic erythematous skin lesions; therefore, the diagnosis [...] Read more.

Vulvar Paget’s disease (VPD) is a rare form of cutaneous adenocarcinoma of the vulva, which accounts for about 1–2% of all vulvar neoplasms and mainly affects post-menopausal women. The clinical presentation is usually non-specific and mimics chronic erythematous skin lesions; therefore, the diagnosis is often difficult and delayed. Although VPD is typically diagnosed at a locally advanced stage and has a high recurrence rate, the prognosis is overall favorable with a 5-year survival of nearly 90%. Due to the limited and poor-quality evidence, there is no global consensus on optimal management. Therefore, we performed a systematic review of the literature through the main electronic databases to deepen the current knowledge of this rare disease and discuss the available treatment strategies. Wide surgical excision is recommended as the standard-of-care treatment and should be tailored to the tumor position/extension and the patient’s performance status. The goal is to completely remove the tumor and achieve clear margins, thus reducing the rate of local recurrences. Non-surgical treatments, such as radiotherapy, chemotherapy, and topical approaches, can be considered, especially in the case of unresectable and recurrent disease. In the absence of clear recommendations, the decision-making process should be individualized, also considering the new emerging molecular targets, such as HER2 and PD-L1, which might pave the way for future targeted therapies. The current review aims to raise awareness of this rare disease and encourage international collaboration to collect larger-scale, high-quality evidence and standardize treatment. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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11 pages, 547 KiB

Open AccessSystematic Review

“Intestinal-Type” Vulvar Adenocarcinoma: A Review of the MITO Rare Tumors Group

by Miriam Dellino, Stefania Cicogna, Francesca Falcone, Marco Mitidieri, Roberta Mazzeo, Sandro Pignata, Giorgia Mangili and Gennaro Cormio

Cancers 2022, 14(20), 5171; https://doi.org/10.3390/cancers14205171 - 21 Oct 2022

Cited by 6 | Viewed by 2311

Abstract

Intestinal-type adenocarcinoma (VAIt) represents a sporadic variant of vulvar carcinoma. It appears frequently localized to epithelial glands in the vulvar region, and it probably derives from cloacal remnants persisting in the adult. We performed a systematic review of the limited cases reported in [...] Read more.

Intestinal-type adenocarcinoma (VAIt) represents a sporadic variant of vulvar carcinoma. It appears frequently localized to epithelial glands in the vulvar region, and it probably derives from cloacal remnants persisting in the adult. We performed a systematic review of the limited cases reported in the literature, with the intent to assess the specific peculiarities of this rare neoplasia and to state consistent management recommendations. The principal histological VAIt characteristic is that it resembles mucinous colonic carcinomas. Therefore, immunohistochemical workup, with different tumor markers including CK20, CDX2, and CK7 staining, is needed. To confirm vulvar origin, a thorough diagnostic, and radiological examination is required to rule out other primary malignancies. The gold standard of treatment for VAIt is surgery, with local excision with tumor-free margins. Lymph node staging is an option advised if the tumor size is >2 cm or if lymph node metastases are suspected on imaging. On the other hand, the role of neoadjuvant therapy is still in doubt, but a good response to adjuvant chemotherapy treatments has been described in both advanced and recurrent diseases. Sometimes, VAIt behavior can be unpredictable, with relapses even after many years, so more experiences and longer follow-up periods are needed to elucidate the best therapeutic management and its long-term prognosis. Full article

(This article belongs to the Special Issue Rare Gynecological Cancers)

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