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Cancers
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Rare Gynecological Cancers
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Rare Gynecological Cancers

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Molecular Cancer Biology".

Deadline for manuscript submissions: 25 November 2024 | Viewed by 20170

Special Issue Editors

Prof. Dr. Gennaro Cormio

E-Mail Website
Guest Editor
2nd Unit of Obstetrics and Gynecology, Department of Biomedical and Human Oncological Science (DIMO), University of Bari, 70124 Bari, Italy
Interests: rare gynecological tumors
Special Issues, Collections and Topics in MDPI journals
Dr. Alice Bergamini

E-Mail Website
Guest Editor
Department of Obstetrics and Gynecology, Ospedale San Rafaele, 20132 Milan, Italy
Interests: gynecologic oncology; rare tumors; gestational trophoblastic disease; treatment
Special Issues, Collections and Topics in MDPI journals
Prof. Dr. Maria Gabriella Ferrandina

E-Mail Website
Guest Editor
1. IRCCS, UOC di Ginecologia Oncologica, Dipartimento per la Salute della Donna e del Bambino e della Salute Pubblica, Fondazione Policlinico Universitario A Gemelli, Rome, Italy
2. Istituto di Ginecologia e Ostetricia, Università Cattolica del Sacro Cuore, 00168 Rome, Italy
Interests: translational studies; uterine cervical cancer; clinical trials; chemotherapy

Special Issue Information

Dear Colleagues,  

The classification of gynecological cancers has changed during recent years, and a wide variety of “new” rare variants of the disease have been described. Pathologic criteria for diagnosis, clinical manifestations of the disease, molecular profiles and subsequent clinical treatment modalities have been widely developed in recent years for rare gynecological cancers. 

The aim of this Special Issue is to give readers (gynecologists, pathologists and radiotherapists) a clear picture of the new landscape of rare gynecologic tumors. It will focus on modern techniques for differential diagnosis as well as the appropriate clinical management of a number of diseases that, for their rarity, are almost “forgotten” in the medical literature. 

Systematic and narrative reviews, original articles, and all other types of scientific papers are accepted in this Special Issue. We are sure that your participation will contribute to an attractive and interesting Special Issue. 

Prof. Dr. Gennaro Cormio 
Dr. Alice Bergamini
Prof. Dr. Maria Gabriella Ferrandina
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • rare gynecological cancers
  • trophoblastic disease
  • uterine sarcomas
  • small-cell carcinoma of the genital tract
  • vaginal cancers
  • vulvar cancers

Published Papers (11 papers)

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15 pages, 1210 KiB  
Review
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://0-doi-org.brum.beds.ac.uk/10.3390/cancers16071375 - 31 Mar 2024
Viewed by 493
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  
Review
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://0-doi-org.brum.beds.ac.uk/10.3390/cancers16010222 - 03 Jan 2024
Viewed by 856
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  
Review
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://0-doi-org.brum.beds.ac.uk/10.3390/cancers15184614 - 18 Sep 2023
Cited by 2 | Viewed by 933
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  
Review
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://0-doi-org.brum.beds.ac.uk/10.3390/cancers15133403 - 29 Jun 2023
Cited by 1 | Viewed by 1189
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  
Review
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://0-doi-org.brum.beds.ac.uk/10.3390/cancers15112953 - 28 May 2023
Cited by 2 | Viewed by 1288
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  
Review
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://0-doi-org.brum.beds.ac.uk/10.3390/cancers15030890 - 31 Jan 2023
Cited by 2 | Viewed by 2081
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  
Review
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://0-doi-org.brum.beds.ac.uk/10.3390/cancers14215217 - 25 Oct 2022
Cited by 2 | Viewed by 4875
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  
Review
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://0-doi-org.brum.beds.ac.uk/10.3390/cancers14163852 - 09 Aug 2022
Cited by 2 | Viewed by 1913
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  
Systematic 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://0-doi-org.brum.beds.ac.uk/10.3390/cancers16061106 - 09 Mar 2024
Viewed by 794
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  
Systematic 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://0-doi-org.brum.beds.ac.uk/10.3390/cancers15061803 - 16 Mar 2023
Cited by 3 | Viewed by 2800
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  
Systematic 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://0-doi-org.brum.beds.ac.uk/10.3390/cancers14205171 - 21 Oct 2022
Cited by 3 | Viewed by 1803
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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