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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 -
Anais Brasileiros de Dermatologia 2015Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer... (Review)
Review
Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.
Topics: Breast Neoplasms; Carcinoma; Diagnosis, Differential; Female; Genital Neoplasms, Male; Humans; Male; Nipples; Paget Disease, Extramammary; Paget's Disease, Mammary; Skin Neoplasms; Vulvar Neoplasms
PubMed: 25830993
DOI: 10.1590/abd1806-4841.20153189 -
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 -
Human papillomavirus and p16 in squamous cell carcinoma and intraepithelial neoplasia of the vagina.International Journal of Cancer Jul 2019We estimated the overall and type-specific prevalence of human papillomavirus (HPV) and p16 overexpression in vaginal cancer and vaginal intraepithelial neoplasia... (Meta-Analysis)
Meta-Analysis
We estimated the overall and type-specific prevalence of human papillomavirus (HPV) and p16 overexpression in vaginal cancer and vaginal intraepithelial neoplasia (VaIN). We conducted a systematic search of PubMed, Embase and Cochrane Library to identify studies published between 1986 and 2017 using PCR-based or Hybrid Capture 2 tests to evaluate the presence of HPV DNA and/or using any method to detect p16 overexpression in VaIN, vaginal squamous cell carcinoma (VaSCC), or other types of vaginal cancer. Applying a random effects model, we estimated the pooled prevalence of HPV and p16 overexpression along with 95% confidence intervals (CIs). The I statistic was used to assess heterogeneity. We included 26 studies, reporting HPV prevalence and six studies evaluating p16 overexpression. The pooled HPV prevalences in VaSCC (n = 593) and VaIN (n = 1,374) were 66.7% (95% CI = 54.7-77.8) and 85.2% (95% CI = 78.2-91.0), respectively. Substantial inter-study heterogeneity was observed, and analyses stratified on geographic region, type of tissue, HPV detection method or PCR primer type did not fully explain the observed heterogeneity. The most predominant HPV type among the HPV positive VaSCC and VaIN cases was HPV16, followed by HPV33, and HPV45 (in VaIN) and HPV18, and HPV33 (in VaSCC). In pooled analyses, 89.9% (95% CI = 81.7-94.6) of HPV positive and 38.9% (95% CI = 0.9-90.0) of HPV negative vaginal cancers were positive for p16 overexpression. Our findings suggest that vaccination against HPV might prevent a substantial proportion of vaginal neoplasia and highlight the need for further studies of the possible clinical value of p16 testing in these patients.
Topics: Carcinoma in Situ; Carcinoma, Squamous Cell; Cyclin-Dependent Kinase Inhibitor p16; DNA, Viral; Female; Humans; Neoplasm Grading; Papillomaviridae; Papillomavirus Infections; Prevalence; Vaginal Neoplasms
PubMed: 30561092
DOI: 10.1002/ijc.32078 -
International Journal of Applied &... 2016Human papillomavirus (HPV) infection is linked with several cancers such as cancer cervix, vagina, vulva, head and neck, anal, and penile carcinomas. Although there is a... (Review)
Review
Human papillomavirus (HPV) infection is linked with several cancers such as cancer cervix, vagina, vulva, head and neck, anal, and penile carcinomas. Although there is a proven association of HPV with these cancers, questions regarding HPV testing, vaccination, and treatment of HPV-related cancers continue to remain unanswered. The present article provides an overview of the HPV-associated cancers.
PubMed: 27127735
DOI: 10.4103/2229-516X.179027 -
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 -
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 -
CytoJournal Aug 2013Rare entities in the Pap test, which include neoplastic and non-neoplastic conditions, pose challenges due to the infrequent occurrence of many of these entities in the... (Review)
Review
Rare entities in the Pap test, which include neoplastic and non-neoplastic conditions, pose challenges due to the infrequent occurrence of many of these entities in the daily practice of cytology. Furthermore, these conditions give rise to important diagnostic pitfalls to be aware of in the Pap test. For example, cases with adenoma malignum (AM) have been called benign. Recognition of these conditions can help correctly interpret Pap tests as abnormal and thereby ensure that patients get appropriately diagnosed. In this paper, we illustrate and discuss selected uncommon primary neoplastic lesions of the cervix and the vagina that may be seen in Pap test, with a focus on cytomorphology, differential diagnosis and the role of possible ancillary studies. These cases include high-grade squamous intraepithelial lesion cells with small cell morphology; small cell carcinoma; large neuroendocrine carcinoma; glassy cell carcinoma; AM; malignant mixed Müllerian tumor; clear cell carcinoma and primary malignant melanoma. Recognition of these rare variants/neoplasms is important so that involved Pap tests are not diagnosed as benign and that patients with these conditions get additional follow-up.
PubMed: 24082913
DOI: 10.4103/1742-6413.117356 -
BJR Case Reports Feb 2023Primary vaginal cancer is rare, accounting for only 2% of all gynecological malignant tumors. Primary vaginal cell carcinoma is mainly squamous cell carcinoma,...
Primary vaginal cancer is rare, accounting for only 2% of all gynecological malignant tumors. Primary vaginal cell carcinoma is mainly squamous cell carcinoma, accounting for about 90%, and adenocarcinoma only accounts for 8-10%. Primary signet ring cell carcinoma of vagina is rare and has not been reported in the literature. This paper reports a case of signet ring cell carcinoma in vagina.
PubMed: 36873240
DOI: 10.1259/bjrcr.20220050