Targeted mutation analysis of endometrial clear cell carcinoma. Pathology of the endometriumpathology of the endometrium. Endometrioid tumors tend to have a favorable prognosis and typically present at an early stage with abnormal uterine bleeding. Pathology of the endometriumpathology of the endometrium thomas c. The purpose of this retrospective analysis was to elucidate the meaning of adjuvant radiotherapy rt for clinical outcome and to define prognostic factors in these patients pts. Mucinous adenocarcinoma refers to a subtype of endometrial adenocarcinoma in which more than 50% of the.
Gene expression profiles of serous, endometrioid, and clear cell subtypes of ovarian and endometrial cancer. Jun 18, 2014 type ii papillary serous and clear cell endometrial carcinoma ec is a rare subgroup and is considered to have an unfavorable prognosis. A variant containing eosinophilic cytoplasm has been termed the oxyphilic variant. From 20042012 fortytwo pts with type ii ec underwent surgery followed by. On gross pathological examination, the tumor is cystic and may be solid and some arise in cystic endometriosis. Papillary areas have cells with intermediate nuclear. Results the median follow up time was 28 months, and the mean age at diagnosis was 59. This is due to a number of factors such as the presence of clear cells in some serous carcinomas and serous. Dec 14, 2018 distinction of clear cell carcinoma and serous carcinoma, and mixed clear cell serous carcinoma. Endometrioid carcinomaendometrioid carcinoma serous carcinoma clear cell adenocarcinoma mucinous adenocarcinoma squamous cell carcinoma mixed carcinoma undifferentiated carcinoma. Should endometrial clear cell carcinoma be classified as. Histologically, it is characterized by polygonal to. Endometrial cancer rare endometrial cancersclear cell. Clear cell carcinoma of the endometrium radiology reference.
Diagnosis on hysterectomy was welldifferentiated figo grade i endometrioid adenocarcinoma. Uterine clear cell carcinoma of postmenopausal woman. It is increasingly recognized that ccc is a rare subtype in the endometrium, raising questions regarding its clinical connotations. Pathology of endometrial hyperplasia and carcinoma obgyn key. Pathogenesis of ovarian clear cell adenofibroma, atypical. Here, an expert outlines the management of earlystage disease, including surgical staging, and explores the role of adjuvant radiation therapy, lymphnode sampling, and laparoscopy. Mixed endometrioid and clear cell carcinoma of the. Currently, little is known regarding the genetic basis of endometrial ccc. Aggressive histopathologic variants of endometrial carcinoma and carcinosarcoma. Napsin a is an aspartic proteinase expressed in type ii pneumocytes, alveolar macrophages, and renal tubular epithelial cells. Endometrial cancer histopathology reporting guide version 3. Jul 28, 2017 clear cell carcinoma of mullerian origin is a rare and distinct histologic subtype, which occurs in the ovary, uterus, cervix, and vagina. An overview of endometrial carcinoma will be presented here.
Two broad guiding principles are worthy of consideration for tumors that display this pattern. There is intact expression of mismatch repair proteins pms2 and msh6 and wild type expression of p53, i. Endometrial cancer remains the most common malignancy of the female gynecologic tract. Immunohistochemical characterization of endometrial. For endometrial clear cell carcinoma, intraepithelial growth patterns, which may be seen in isolation, have long been recognized. The incidence of endometrial carcinoma is increasing, as the proportion of obese individuals is increasing. Aims endometrial clear cell carcinomas ccc constitute fewer than 5% of all carcinomas of the endometrium. The incidence of this type of carcinoma is less common 15% of all endometrial carcinomas, and occurs in older patients like serous adenocarcinoma. A case of mixed endometrioid and clear cell ccc endometrial carcinoma with ovarian and peritoneal metastasis of the ccc component is presented. They are part of the surface epithelial tumor group of ovarian neoplasms 1020% of which are the endometrioid type. Like uterine papillary serous carcinoma cc does not develop from endometrial hyperplasia and is not hormone sensitive, rather it arises from an atrophic endometrium. Type i, which encompasses approximately 80% to 85% of the cases, is estrogen dependent and consists of.
Endometrial carcinoma american journal of clinical. Mar 11, 2019 clear cell renal cell carcinoma ccrcc is a renal cortical tumor typically characterized by malignant epithelial cells with clear cytoplasm and a compactalveolar nested or acinar growth pattern interspersed with intricate, arborizing vasculature. These subtypes of primary ovarian tumors include benign, borderline, and malignant counterparts as opposed to other mullerian sites where these tumors usually present as malignant neoplasms carcinomas. The majority of these cancer cases are detected and diagnosed in women aged 50 years and older postmenopausal women. Histopathology uterusview 1 endometrial adenocarcinoma.
Microscopic pathology image showing clear cell carcinoma. In 40% of cases, endometrioid tumors are found bilaterally. Histologic and immunohistochemical analyses of endometrial. It presents mainly in postmenopausal women and shows poor response to platinumbased chemotherapy and radiation therapy typically, eccc is considered a prototypical type ii endometrial carcinoma. Endometrial cancer is the most common gynecologic malignancy in developed countries, and its incidence has more than doubled over the last 10 years in korea. Traditionally regarded as a type 2 aggressive histotype, there are current efforts to assess its true incidence and place within the clinical and molecular spectrum of endometrial carcinomas. 50% solid poorly differentiated high nuclear grade can increase the grade.
I highly suggest you follow up with doctors, and as needed, get medical treatment, as this clear cell carcinoma is highly aggresssive and known to be. Request pdf highresolution copy number analysis of clear cell endometrial carcinoma uterine cancer is the 6th leading cause of cancer death amongst american women. Endometrial clear cell carcinoma cc is an uncommon tumor and often carries a poor prognosis. Mismatch repair status and pdl1 expression in clear cell. It is clear that postmenopausal women treated with supplemental estrogens are at increased risk of endometrial hyperplasia and carcinoma if a progestin is not used to oppose the. There is an association with endometriosis and concurrent primary endometrial carcinoma endometrial cancer. Summary of prospective studies of postoperative radiation in endometrial cancer including clear cell histology. The 5year progression free survival pfs for the entire cohort was 61%, and was 88%, 75%, 22% and 28. Contemporary management of uterine clear cell carcinoma. Strategies for classifying endometrial carcinoma with. There is no right way in this journey, yet if u realize what is so about clear cell carcinoma and thru your amazing good luck are in remission, use compassion for others in your exact situation.
Gynecologic oncology 40, 207217 1991 clear cell carcinoma of the endometrium. At least one of the subtypes must be a type ii tumour and the second component. Primary squamous cell carcinoma of the endometrium. The focal nature of the clear cells on a background of normal endometrium in curettage material, an absence of invasion in hysterectomy specimens and a low proliferation index with ki67 favour a diagnosis of metaplasia. There is an association with endometriosis and concurrent primary endometrial carcinoma endometrial cancer on gross pathological examination, the tumor is cystic and may be solid and some arise in. Clear cell adenocarcinoma of the endometrium is a distinct histopathological subtype of endometrial carcinoma and is classified as type ii carcinoma of the endometrium. Clear cell carcinomas ccc is one of the rarest forms of endometrial carcinoma.
Endometrial carcinoma ec is the most common gynecologic malignancy in the united states, with 60,050 new cases and 10,470 deaths estimated in 2015. Clear cell histology is morphologically and genetically different from the more prevalent endometrioid. Ovarian endometrioid and clearcell tumors springerlink. Clear cell carcinoma of the endometrium mmr intact, p53 wild type see comment comment. Clear cell carcinoma and other rare forms of endometrial carcinoma comprise the few remaining cases. Renal clear cell cancer was included for clear cell analysis. In general, these rarer cancers are associated with later age, greater risk for metastases outside the uterus, and a poorer prognosis. Diagnosis of aggressive tumours such as serous carcinoma, clear cell carcinoma, carcinosarcoma, undifferentiated carcinoma and grade 3 endometrioid adenocarcinoma will usually result in full surgical staging including pelvic and paraaortic lymphadenectomy and omentectomy. Clear cell carcinoma of the uterus is an uncommon type of endometrial cancer and is considered a high risk group greater chance of relapse or recurrence than the more common type called adenocarcinoma. Endometrial carcinoma is generally divided into two settings, type i and the type ii, based primitively on whether or not it is estrogenic fig. It is considered as a reliable marker for primary pulmonary adenocarcinoma, being expressed in 76% to 91% of cases, with a sensitivity of 65% to 81% and specificity of 88% to 100%. Review endometrial hyperplasia wiley online library.
Unlike papillary serous tumors, clear cell carcinoma does not seem to have a high propensity for abdominal failure. Prototypical examples of endometrial clear cell carcinoma are characterized by a distinctive set of histopathologic features described above that enables their distinction from other histotypes 6,7,10,11,34,3843,69100. The significance of l1cam expression in clear cell carcinoma of the endometrium. Endometrial polyp is a relatively common benign lesion. Diagnosis can be made using the same tests that are used in the diagnosis of other types. Clear cell endometrial intraepithelial carcinoma was identified in 41. Endometrial carcinomas with clear cells request pdf. Current management of earlystage endometrial cancer. The tumor cells are polygonal and frequently contain clear cytoplasm. Hyuk shim and wook youn kim, dedifferentiated carcinoma with clear cell carcinoma of the endometrium.
Demographics endometrial cancer is the most common. Clear cell carcinoma comprises a small percentage of endometrial cancers, frequently presents as a mixed histology, and has a poor overall outcome. Endometrial carcinoma, also endometrial adenocarcinoma, is a common gynecologic malingnancy that often arises from endometrial hyperplasia. Transitional cell carcinoma of the endometrium and.
Most authorities recommend additional therapy after surgery, though there is no consensus on whats best, radiation whole abdomen wart, or pelvis andor chemotherapy. Ep652 clinicopathological characterstics and survival. Uterine serous carcinoma esc, type ii, accounts for approximately 10% of cases, whereas emc, type i, encompasses about 80% to 85% of cases. Our results thus do not support the routine use of wholeabdominal rt in these. The significant interobserver variation that has historically been associated with the diagnosis of endometrial clear. Mixed endometrioid and clear cell carcinoma of the endometrium. Clear cell carcinoma of endometrium is a rare subtype of endometrial cancer, which begins in the lining of the uterus the endometrium. Endometrial and clear cell adenocarcinoma cancer survivors. Histopathological challenges in the diagnosis of endometrial.
Type ii papillary serous and clear cell endometrial carcinoma ec is a rare subgroup and is considered to have an unfavorable prognosis. Both mixed carcinomas and ccc fall into the category of tumors currently acknowledged to show ambiguous morphology, that is. Endometrial metaplasias and changes emcs are conditions frequently overlooked and misdiagnosed. Clear cell histology is diagnosed in less than 6% of all endometrial cancers and its incidence increases with age. A histopathological and clinical study of 97 cases vera m. Recent developments in surgical pathology of the uterine. Type i and type ii endometrial carcinomas are considered. Wright columbia university, new york, ny changes in the. Uterine serous carcinoma very high nuclearvery high nuclear grade tumor histology resembles that of ovarian ppp yapillary serous ca endometrial cancer histological grading. Histopathology uterus view 1 endometrial adenocarcinoma, view 2 fibromyoma leiomyoma. Clinicopathologic features of tumors with endometriosis or adenofibromatous components support two related pathways of tumor development chengquan zhao1,2. In the united states, there is a greater incidence among patients of european descent compared those of african american descent. A case report, pathology international, 67, 9, 472476, 2017. A variable proportion of cells with granular eosinophilic cytoplasm may be present.
Survival of women with clear cell and papillary serous. Heterogeneity of molecular pathogenesis histopathology 2015. Clear cell carcinoma mixed cell adenocarcinoma undifferentiated carcinoma. The other 10% of endometrial cancers are caused by rare cell types papillary serous carcinoma, clear cell carcinoma, papillary endometrial carcinoma, mucinous carcinoma. They show papillary, glandular, tubulocystic, andor solid architecture. Clear cell carcinomas extended to the uterine corpus significantly more often than squamous cell and non clear cell adenocarcarcinomas p carcinoma, clear cell carcinoma, carcinosarcoma, undifferentiated carcinoma and grade 3 endometrioid adenocarcinoma will usually result in full surgical staging including pelvic and paraaortic lymphadenectomy and omentectomy. Often associated with an advanced stage of disease and thus, poor prognosis. Benign and borderline variants are rare, as the majority are malignant. In one series of 852 consecutive endometrial carcinomas managed at a single academic center, only 3 0. Clear cell carcinoma of the uterine corpus ucc, a rare subtype accounting for 16% of uterine cancers, is characterized histologically by the clearing of tumor cell cytoplasm 25. This raised several diagnostic issues, beginning with the morphological recognition of ccc. Endometrial clear cell carcinoma eccc is a rare subtype of endometrial carcinoma accounting for only 2% to 5. Pathogenesis of ovarian clear cell adenofibroma, atypical proliferative borderline tumor, and carcinoma.
Rare endometrial cancersuterine papillary serous carcinoma upsc, usc and clear cell carcinoma cc of the endometrium 1. Napsin a expression has also been reported in extrapulmonary. Therefore, questions regarding their designation as the type ii carcinomas have been raised. Other histologic types of endometrial carcinoma eg, serous, clear cell as well as other types of uterine cancer are associated with a poor prognosis. Tb of the endometrium is uncommon in theuncommon in the u. Diagnosis can be made using the same tests that are used in the diagnosis of other types of endometrial cancer.
Author, year surgical staging stage arms rad pfs 3 or 5years p os 3 or 5years p value creutzberg et al. We performed immunohistochemical staining for hepatocyte nuclear factor1. Gene expression profiles of serous, endometrioid, and clear cell subtypes of ovarian. Clearcell and endometrioid tumors are subtypes of mullerian epithelial tumors that can arise in the uterus as well as the ovary. Strategies for classifying endometrial carcinoma with clear cells. It presents mainly in postmenopausal women and shows poor response to platinumbased chemotherapy and radiation therapy. An introduction to the endometrium is in the article endometrium. There were 18 72% patients with pure clear cell carcinoma and 728% with mixed histology. Aside from ovarian tcc, small numbers of cases of tcc have been reported in the cervix, 1416 with isolated cases reported in the fallopian tube, 17, 18 the broad ligament, 19 a paratubal cyst, 20 and the endometrium.
Napsin a is frequently expressed in clear cell carcinoma. Endometrial carcinoma is the most common gynecological malignancy, with peak incidence at around the 6 th decade, though 12% of cases present in premenopausal women. Uterine clear cell carcinoma cc is a rare form of endometrial cancer with distinct morphological features on pathology. It occurs in cells lining the deepest part of the skins outer layer. Although prototypical examples of serous and clear cell carcinoma are readily distinguished, some cases present diagnostic problems 1,2,4. Clear cell adenocarcinoma of endometrium is, by definition, considered a highgrade tumour and is not further graded.
The prognosis of clear cell carcinoma of endometrium is worse than that for endometrioid adenocarcinoma but better than that for serous carcinoma. Some clear cell carcinomas are admixed with serous adenocarcinoma. Twelve of the fifteen clear cell carcinomas 80% were endophytic and tended toward deep cervical infiltration. Distinction of clear cell carcinoma and serous carcinoma, and mixed clear cellserous carcinoma. It has histologic features that overlap with other endometrial carcinomas and is frequently. Clear cell carcinoma ccc of the endometrium is an uncommon histological subtype of endometrial cancer. Patients with ucc are more likely to present with higherstage disease than those with endometrioid histology, and clear cell histology has been considered a. Clear cell adenocarcinoma present exclusively within. Hormonal or irritative stimuli are the main inducing factors of emcs, although some metaplasias have a mutational origin.
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