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Cancers Apr 2022Gynecological tract neuroendocrine neoplasms (NEN) are rare, aggressive tumors from endocrine cells derived from the neuroectoderm, neural crest, and endoderm. The... (Review)
Review
Gynecological tract neuroendocrine neoplasms (NEN) are rare, aggressive tumors from endocrine cells derived from the neuroectoderm, neural crest, and endoderm. The primary gynecologic NENs constitute 2% of gynecologic malignancies, and the cervix is the most common site of NEN in the gynecologic tract. The updated WHO classification of gynecologic NEN is based on the Ki-67 index, mitotic index, and tumor characteristics such as necrosis, and brings more uniformity in the terminology of NENs like other disease sites. Imaging plays a crucial role in the staging, triaging, restaging, and surveillance of NENs. The expression of the somatostatin receptors on the surface of neuroendocrine cells forms the basis of increasing evaluation with functional imaging modalities using traditional and new tracers, including Ga-DOTA-Somatostatin Analog-PET/CT. Management of NENs involves a multidisciplinary approach. New targeted therapies could improve the paradigm of care for these rare malignancies. This article focuses on the updated staging classifications, clinicopathological characteristics, imaging, and management of gynecologic NENs of the cervix, ovary, endometrium, vagina, and vulva, emphasizing the relatively common cervical neuroendocrine carcinomas among these entities.
PubMed: 35406607
DOI: 10.3390/cancers14071835 -
American Family Physician Nov 2020Common benign chronic vulvar conditions include genitourinary syndrome of menopause (formerly called vulvovaginal atrophy), lichen sclerosus, lichen planus, lichen...
Common benign chronic vulvar conditions include genitourinary syndrome of menopause (formerly called vulvovaginal atrophy), lichen sclerosus, lichen planus, lichen simplex chronicus, and vulvodynia. Genitourinary syndrome of menopause results from the hypoestrogenic state that leads to atrophy of normal vulvar and vaginal tissues. It is typically treated with lubricants, moisturizers, and intravaginal estrogen. Lichen sclerosus is an inflammatory condition characterized by intense vulvar itching. It is treated with topical steroids or, in some cases, topical calcineurin inhibitors. Patients with lichen sclerosus are at risk of vulvar squamous cell carcinoma and should be monitored closely for malignancy. Lichen planus is an inflammatory autoimmune disorder that can affect the vulva and vagina in addition to other skin and mucosal surfaces. The first-line treatment is topical steroids, and significant scarring can occur if left untreated. Lichen simplex chronicus manifests as persistent itching and scratching of the vulvar skin that leads to thickened epithelium. Breaking the itch-scratch cycle, often with topical steroids, is the key to treatment. Vulvodynia is a common vulvar pain disorder and is a diagnosis of exclusion. A multimodal treatment approach typically includes vulvar hygiene, physical therapy, psychosocial interventions, and antineuropathy medications.
Topics: Adult; Chronic Disease; Female; Humans; Lichen Planus; Lichen Sclerosus et Atrophicus; Middle Aged; Neurodermatitis; Vulva; Vulvar Diseases; Vulvodynia
PubMed: 33118795
DOI: No ID Found -
International Journal of Gynaecology... Oct 2021This review covers the significant new developments in the pathological classification of gynecological tumors. Many of these were included in the updated World Health... (Review)
Review
This review covers the significant new developments in the pathological classification of gynecological tumors. Many of these were included in the updated World Health Organization Classification of Female Genital Tract Tumours, published in 2020. Topics include the compelling evidence that a large majority of extrauterine high-grade serous carcinomas arise from the fallopian tube; the Cancer Genome Atlas (TCGA) Classification of endometrial carcinomas; the discovery that most so-called synchronous endometrial and ovarian endometrioid carcinomas represent metastasis from the endometrium to the ovary; and the division of cervical, vaginal, and vulval carcinomas into clinically meaningful HPV-associated and HPV-independent types. Newly described tumor types are covered, including endometrial and ovarian mesonephric-like adenocarcinoma, uterine sarcoma types associated with specific molecular abnormalities, and gastric (gastrointestinal)-type adenocarcinomas of the endometrium and vagina. Important molecular events in ovarian sex cord-stromal tumors are also discussed.
Topics: Adenocarcinoma; Carcinoma, Endometrioid; Endometrial Neoplasms; Female; Humans; Ovarian Neoplasms; Uterine Neoplasms
PubMed: 34669206
DOI: 10.1002/ijgo.13871 -
Histopathology May 2021Mucin 4 (MUC4) is a transmembrane glycoprotein normally expressed by several human epithelial surfaces, including those of the colon, vagina, and respiratory tract....
AIMS
Mucin 4 (MUC4) is a transmembrane glycoprotein normally expressed by several human epithelial surfaces, including those of the colon, vagina, and respiratory tract. Although MUC4 overexpression is seen in various carcinomas, its expression among mesenchymal neoplasms is fairly specific to low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma. Having observed unanticipated anti-MUC4 immunoreactivity in rhabdomyosarcoma, we aimed to further characterise its expression.
METHODS AND RESULTS
Expression of MUC4 was assessed by immunohistochemistry in a total of 97 rhabdomyosarcomas using formalin-fixed paraffin-embedded tissue sections. MUC4 was expressed by 21 of 26 PAX3/7-FOXO1 fusion-positive cases, wherein immunoreactivity, varying from weak to strong, was present in 20-100% of neoplastic cells. With the exception of one sclerosing rhabdomyosarcoma showing immunoreactivity in 20% of cells, MUC4 was not expressed by embryonal (n = 28), sclerosing (n = 20), or pleomorphic (n = 23) rhabdomyosarcomas. Analysing published gene expression microarray data from a separate cohort of 33 fusion-positive and 25 fusion-negative rhabdomyosarcomas, we found on average a 11.4-fold increased expression in fusion-positive tumours (P = 0.0004).
CONCLUSIONS
MUC4 is expressed to a variable extent in the majority of PAX3/7-FOXO1 fusion-positive (alveolar) rhabdomyosarcomas, while expression in other rhabdomyosarcoma subtypes is rare.
Topics: Adult; Female; Head and Neck Neoplasms; Humans; Mucin-4; Rhabdomyosarcoma, Alveolar; Soft Tissue Neoplasms
PubMed: 33368602
DOI: 10.1111/his.14321 -
Gynecologic Oncology Reports Feb 2022Hidradenitis suppurativa (HS) is a debilitating disorder characterized by chronic inflammation in intertriginous areas. Malignant transformation to squamous cell...
BACKGROUND
Hidradenitis suppurativa (HS) is a debilitating disorder characterized by chronic inflammation in intertriginous areas. Malignant transformation to squamous cell carcinoma (SCC) is rare and is mostly diagnosed in the perianal area in men. The clinical behavior of SCC in HS can be aggressive, with local invasion and distant metastases.Case descriptions.We describe two cases of vulvar SCC in HS. The first demonstrates a 75 year old woman with a severe undertreated HS for over 30 years, who presented with a widespread vulvar cancer with lymphangitis carcinomatosa and inguinal and pelvic lymphadenopathy within several weeks after first suspicion of a malignancy. She died shortly after diagnosis. The second case describes a 61 year old woman diagnosed with HS 7 years ago, who presented with a rapidly progressive vulvar cancer with suspicion for ingrowth in the anal sphincter, vagina and levator ani muscle with inguinal and pelvic lymphadenopathy. She received radical chemoradiation with a complete response on imaging, but had a local recurrence within 2 months after finishing treatment. A posterior exenteration was performed but 5 months after surgery she had a second recurrence in the vulvar scar and pelvic floor muscles with possible bone metastases. She received palliative chemotherapy.
CONCLUSION
Vulvar SCC in an area of HS is a rare condition which is difficult to diagnose. It can have an aggressive course with rapid progression and a high frequency of metastases at presentation. Early surgical excision of HS to diagnose occult malignant transformation, appropriate imaging to establish the extent of the disease and an aggressive treatment plan without any delays are recommended.
PubMed: 35106355
DOI: 10.1016/j.gore.2022.100929 -
Medicina (Kaunas, Lithuania) Dec 2021Neuroendocrine neoplasms (NENs) are particularly rare in all sites of the gynecological tract and include a variety of neoplasms with variable prognosis, dependent on... (Review)
Review
Neuroendocrine neoplasms (NENs) are particularly rare in all sites of the gynecological tract and include a variety of neoplasms with variable prognosis, dependent on histologic subtype and site of origin. Following the expert consensus proposal of the International Agency for Research on Cancer (IARC), the approach in the latest World Health Organization (WHO) Classification System of the Female Genital Tumours is to use the same terminology for NENs at all body sites. The main concept of this novel classification framework is to align it to all other body sites and make a clear distinction between well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The previous WHO Classification System of the Female Genital Tumours featured more or less the same principle, but used the terms 'low-grade neuroendocrine tumor' and 'high-grade neuroendocrine carcinoma'. Regardless of the terminology used, each of these two main categories include two distinct morphological subtypes: NETs are represented by typical and atypical carcinoid and NEC are represented by small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC). High-grade NECs, especially small cell neuroendocrine carcinoma tends to be more frequent in the uterine cervix, followed by the endometrium, while low-grade NETs usually occur in the ovary. NENs of the vulva, vagina and fallopian tube are exceptionally rare, with scattered case reports in the scientific literature.
Topics: Carcinoma, Neuroendocrine; Female; Genital Neoplasms, Female; Humans; Neuroendocrine Tumors; Prognosis; World Health Organization
PubMed: 34946283
DOI: 10.3390/medicina57121338 -
Journal of Lower Genital Tract Disease Jan 2023Small cell carcinoma of the vagina (SmCCV) is an extremely rare disease. Evidence-based data and specific guidelines are lacking. We conducted the first systematic...
OBJECTIVES
Small cell carcinoma of the vagina (SmCCV) is an extremely rare disease. Evidence-based data and specific guidelines are lacking. We conducted the first systematic review of case reports to provide the most overall picture of SmCCV.
MATERIALS AND METHODS
Literature search in PubMed and Scopus was performed using the terms "small cell carcinoma" and "vagina." English-language case reports of primary SmCCV up to January 2022 were included.
RESULTS
Twenty-nine articles describing 44 cases met our inclusion criteria. We report a new case of our hospital. The global median overall survival (mOS) was 12.00 months (95% CI = 9.31-14.69). The mOS was not reached for stage I, and it was 12.00, 12.00, 9.00, and 8.00 months for stages II, III, IVA, and IVB, respectively (statistically significant differences between stage I and stages II, III, or IVA [log rank p = .003-.017]). Thirty-five cases received local treatments (77.8%). The mOS of patients treated with surgery ± complementary chemotherapy, radiotherapy ± complementary chemotherapy, chemoradiation ± complementary chemotherapy, and surgery + radiotherapy ± complementary chemotherapy were 11.00, 12.00, 17.00, and 29.00 months, respectively. The use of adjuvant or neoadjuvant chemotherapy (64.5%, mostly platinum + etoposide) showed longer mOS (77.00 vs 15.00 months). Four of 5 tested cases presented human papillomavirus infection, 3 of them presenting type 18.
CONCLUSIONS
Small cell carcinoma of the vagina shows dismal prognosis. Multimodal local management plus complementary chemotherapy seems to achieve better outcomes. Human papillomavirus could be related to the development of SmCCV. A diagnostic-therapeutic algorithm is proposed.
Topics: Female; Humans; Algorithms; Carcinoma; Neoadjuvant Therapy; Neoplasm Staging; Prognosis; Vagina
PubMed: 36282979
DOI: 10.1097/LGT.0000000000000712 -
Reproduction & Fertility Jul 2022The phenomenal extracellular matrix (ECM) remodelling of the cervix that precedes the myometrial contraction of labour at term or preterm appears to share some common... (Review)
Review
ABSTRACT
The phenomenal extracellular matrix (ECM) remodelling of the cervix that precedes the myometrial contraction of labour at term or preterm appears to share some common mechanisms with the occurrence, growth, invasion and metastasis of cervical carcinoma. Matrix metalloproteinases (MMPs) are zinc-dependent endopeptidases that are pivotal to the complex extracellular tissue modulation that includes degradation, remodelling and exchange of ECM components, which contribute to homeostasis under normal physiological conditions such as cervical remodelling during pregnancy and puerperium. However, in cancer such as that of the uterine cervix, this extensive network of extracellular tissue modulation is altered leading to disrupted cell-cell and cell-basement membrane adhesion, abnormal tissue growth, neovascularization and metastasis that disrupt homeostasis. Cervical ECM remodelling during pregnancy and puerperium could be a physiological albeit benign neoplasm. In this review, we examined the pathophysiologic differences and similarities in the role of MMPs in cervical remodelling and cervical carcinoma.
LAY SUMMARY
During pregnancy and childbirth, the cervix, which is the barrel-shaped lower portion of the womb that connects to the vagina, gradually softens, shortens and opens to allow birth of the baby. This process requires structural and biochemical changes in the cervix that are stimulated by enzymes known as matrix metalloproteinases. Interestingly, these enzymes also affect the structural and biochemical framework of the cervix during cervical cancer, although cervical cancers usually occur after infection by human papillomavirus. This review is intended to identify and explain the similarities and differences between the structural and chemical changes in the cervix during pregnancy and childbirth and the changes seen in cervical cancer.
Topics: Pregnancy; Female; Humans; Animals; Uterine Cervical Neoplasms; Cervix Uteri; Matrix Metalloproteinases; Extracellular Matrix; Carcinoma
PubMed: 37931406
DOI: 10.1530/RAF-22-0015 -
Gynecologic Oncology Reports Dec 2023To perform a retrospective review of patients diagnosed with vaginal malignant neoplasms (VMN) in the Republic of Belarus.
OBJECTIVE
To perform a retrospective review of patients diagnosed with vaginal malignant neoplasms (VMN) in the Republic of Belarus.
METHODS
The Belarusian Cancer Registry was reviewed for patients diagnosed with VMN from 1990 to 2019. The data collected included age at diagnosis, demographic information, histology, stage, treatment modalities, and outcomes. Data were compared across decades (1990-1999, 2000-2009, and 2010-2019).
RESULTS
A total of 868 patients were diagnosed with VMN in Belarus between 1990 and 2019. The estimated age-standardized incidence rate of VMN per 100,000 female population increased from 0.1 in 1999 to 0.4 in 2019 ( < 0.05). The mortality rate for this period was 0.0-0.2 per 100,000 female population. Of all newly diagnosed cases of VMN, 70.9% ( = 615) lived in urban centers and 29.1% (n = 253) lived in rural areas. The most common histological type was squamous cell carcinoma, accounting for 78.4% of cases. The median age at diagnosis was 63.4 years (range, 15.0-87.0 years). When compared across the three decades, the number of stage I cases increased slightly more than twofold (from 19.1% to 38.5% for 1990-1999 to 2010-2019). Furthermore, the number of stage III cases decreased from 30.3% to 13.0% from 1990 to 1999 to 2010-2019. There were no significant changes in the number of patients diagnosed with stage II or IV disease over time. The overall 5-year survival rate for the entire group was 68.7 ± 5.1%, with no statistically significant difference between women living in urban centers vs. rural areas (67.8 ± 5.1% vs. 65.8 ± 10.4%; = 0.99). However, there was a 26.2% increase in the 5-year survival rate for the entire group over the study period. This increase was higher for women living in rural areas (+61.7%) than for those in urban centers (+51.3%); > 0.05. Furthermore, a comparison of 5-year adjusted survival rates between 2000 and 2015 showed increased survival for stages I, II, and III, with the most significant increase noted for stage III disease (2.4-fold increase).
CONCLUSIONS
This retrospective study found that the survival rates of women with VMN in Belarus have improved over the past 30 years. This is likely due to improvements in early detection as well as improved approaches to treatment, particularly for those living in rural areas. Additional study is needed to further understand and improve the outcomes of women diagnosed with VMN in Belarus.
PubMed: 38088958
DOI: 10.1016/j.gore.2023.101309