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Čelebić A, Harasani K, Miladinović M, Mandić A, Vasiljević T, Drusany Starič K, Radunović V, Tripac I, Starič Drusany AK. Neuroendocrine tumors of the gynecological tract: A narrative literature review. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2025; 51:109735. [PMID: 40057385 DOI: 10.1016/j.ejso.2025.109735] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2024] [Revised: 02/26/2025] [Accepted: 02/28/2025] [Indexed: 05/12/2025]
Abstract
Neuroendocrine neoplasms (NENs) of the gynecological tract are a rare, heterogenous and aggressive group of neoplasms, with high recurrence rates and poor prognosis. In this review we focus on NENs of the gynecological system emphasizing the classification, epidemiological and clinical characteristics of NENs across the gynecological tract (cervix, endometrium, ovary, vagina, and vulva), risk/prognostic factors, pathology and molecular biology (including actionable genomic mutations), imaging, staging and the most effective treatment modalities in the "standard of care" approach as well as the pipeline products. We also focused on metastatic spreading patterns of gynecological NENs. We searched for all available literature reviews, interventional studies, short series, case reports and meta-analyses published from 1990 to 2024. Deteriorated survival rate is essentially impacted by early development of lymph node, distant organ metastases and vascular propagation toward rapid extra-pelvic metastasis to the brain, liver, lung, bone marrow, lymph nodes and bones. Management of NENs needs to be customized on a case-based manner and comprises a multidisciplinary approach that involves gynecologists, surgeons, medical oncologists, radiologists, radiation oncologists, nuclear medicine specialists, pathologists, molecular biologists, qualified nurses etc. The treatment of extrapulmonary NENs arising in the female genital tract is basically extrapolated from that for small cell lung cancer. This includes, but is not limited to: surgery, chemotherapy, radiotherapy, peptide receptor radionuclide therapy, somatostatin analogs, and immunotherapy as well as investigational drugs in rare prospective clinical trials. Establishing modern therapeutic thesaurus is conditioned by the existence of well-designed clinical trials targeting a tumor's genomic profile and the incorporation of these data into the actual treatment landscape.
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Affiliation(s)
- Aleksandar Čelebić
- Medical School, University of Montenegro, Podgorica, Montenegro; Institute of Oncology, Clinical Center of Montenegro, Podgorica, Montenegro.
| | - Klejda Harasani
- Department of Pharmacy, Faculty of Medicine, University of Medicine of Tirana, Tirana, Albania
| | - Mirjana Miladinović
- Medical School, University of Montenegro, Podgorica, Montenegro; Institute of Pathology, Clinical Center of Montenegro, Podgorica, Montenegro
| | - Aljoša Mandić
- Oncology Institute of Vojvodina, Serbia; University of Novi Sad, Medical Faculty, Serbia
| | - Tijana Vasiljević
- Oncology Institute of Vojvodina, Serbia; University of Novi Sad, Medical Faculty, Serbia
| | - Kristina Drusany Starič
- Division of Gynecology and Obstetrics, Department of Gynecology, University Medical Centre Ljubljana, Ljubljana, Slovenia; Medical Faculty, University of Ljubljana, Ljubljana, Slovenia
| | | | - Irina Tripac
- Department of Oncology, Institute of Oncology, Chisinau, Moldavia
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Das S, Ahlawat S, Jain P, Panda AK, Sarangi J, Joshi R, Gogi R, Jain A. Neuroendocrine Tumors of the Endometrium: a Case Series with Clinico-Pathological Analysis and Review of Literature. Indian J Surg Oncol 2025; 16:211-220. [PMID: 40114864 PMCID: PMC11920455 DOI: 10.1007/s13193-024-02047-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/26/2024] [Accepted: 07/21/2024] [Indexed: 03/22/2025] Open
Abstract
Neuroendocrine carcinomas are rare aggressive tumors that are either mixed with endometrial carcinomas or pure neuroendocrine carcinoma. They show at least one or two neuroendocrine markers by immunohistochemistry. Here, we describe a case series of six cases that arise from endometrium. The mean age of presentation was 57 years with the most common type being mixed endometrioid and neuroendocrine carcinoma. Immunohistochemistry showed all cases positive for Pan-cytokeratin and INSM-1 while the majority were positive for synaptophysin and chromogranin. At least two neuroendocrine markers were positive in all cases in > 20% of tumor cells. The majority of the cases presented at FIGO (International Federation of Gynecology and Obstetrics) Stage III & IV. Three patients had a recurrence, one patient had bone metastasis, one patient died, and one patient was free of disease. Four patients with Stage IV disease were not alive for more than 18 months. A multidisciplinary approach is required for these aggressive tumors for better management of patients.
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Affiliation(s)
- Sumanta Das
- Department of Pathology, Agilus Diagnostics Ltd, Fortis Memorial Research Institute, Gurugram, India
| | - Sunita Ahlawat
- Department of Pathology, Agilus Diagnostics Ltd, Fortis Memorial Research Institute, Gurugram, India
| | - Priti Jain
- Department of Pathology, Agilus Diagnostics Ltd, Fortis Memorial Research Institute, Gurugram, India
| | - Arun Kumar Panda
- Department of Pathology, Agilus Diagnostics Ltd, Fortis Memorial Research Institute, Gurugram, India
| | - Jayati Sarangi
- Department of Pathology, Agilus Diagnostics Ltd, Fortis Memorial Research Institute, Gurugram, India
| | - Rama Joshi
- Department Obstetrics and Gynecology, Fortis Memorial Research Institute, Gurugram, India
| | - Ramana Gogi
- Department of Medical Oncology, Fortis Memorial Research Institute, Gurugram, India
| | - Ashutosh Jain
- Department of Medical Oncology, Fortis Memorial Research Institute, Gurugram, India
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Lyu YH, Liu HX, Han X, Yuan P, Wang MY, He YY, Ge JL, Zou W, Jing R, Xin CS, Yang H, Chen BL, Chen GW, Li J. Clinicopathologic characteristics and prognostic factors of patients with surgically treated high-grade neuroendocrine carcinoma of the cervix: A multicenter retrospective study. Int J Gynaecol Obstet 2024; 167:1055-1065. [PMID: 39031110 DOI: 10.1002/ijgo.15771] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2024] [Revised: 06/10/2024] [Accepted: 06/19/2024] [Indexed: 07/22/2024]
Abstract
OBJECTIVE To evaluate the prognostic factors and survival outcomes of patients with surgically treated high-grade neuroendocrine carcinoma of the cervix (NECC). METHODS This multicenter, retrospective study involved 98 cervical cancer patients with stage IA2-IIA2 and IIIC1/2p high-grade NECC. We divided the patients into two groups based on histology: the pure and mixed groups. All clinicopathologic variables were retrospectively evaluated. Cox regression and Kaplan-Meier methods were used for analysis. RESULTS In our study, 60 patients were in the pure group and 38 patients were in the mixed group. Cox multivariate analysis showed that mixed histology was a protective factor impacting overall survival (OS) (P = 0.026) and progression free survival (PFS) (P = 0.018) in surgically treated high-grade NECC. Conversely, survival outcomes were negatively impacted by ovarian preservation (OS: HR, 20.84; 95% CI: 5.02-86.57, P < 0.001), age >45 years (OS: HR, 4.50; 95% CI: 1.0-18.83, P = 0.039), tumor size >4 cm (OS: HR, 6.23; 95% CI: 2.34-16.61, P < 0.001), parity >3 (OS: HR, 4.50; 95% CI: 1.02-19.91, P = 0.048), and perineural invasion (OS: HR, 5.21; 95% CI: 1.20-22.53, P = 0.027). Kaplan-Meier survival curves revealed notable differences in histologic type (OS: P = 0.045; PFS: P = 0.024), chemotherapy (OS: P = 0.0056; PFS: P = 0.0041), ovarian preservation (OS: P = 0.00031; PFS: P = 0.0023), uterine invasion (OS: P < 0.0001; PFS: P < 0.0001), and depth of stromal invasion (OS: P = 0.043; PFS: P = 0.022). CONCLUSION Patients with mixed histologic types who undergo surgery for high-grade NECC have a better prognosis. Meanwhile, ovarian preservation, tumor size >4 cm, parity >3, age >45 years and perineural invasion were poor prognostic predictors. Therefore, patients with high-risk factors should be considered in clinical practice.
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Affiliation(s)
- Yan-Hong Lyu
- Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi Province, China
| | - Hai-Xia Liu
- Department of Obstetrics and Gynecology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China
- Department of Obstetrics and Gynecology, Liao Cheng People's Hospital, Liaocheng, Shandong, China
| | - Xue Han
- Department of Obstetrics and Gynecology, Shengjing Hospital of China Medical University, Shenyang, Liaoning Province, China
| | - Peng Yuan
- Department of Gynecology, Northwest Women's and Children's Hospital, Xi'an, Shaanxi Province, China
| | - Ming-Yi Wang
- Department of Gynecology and Obstetrics, General Hospital of Western Theater Command of PLA, Chengdu, Sichuan Province, China
| | - Yuan-Yuan He
- Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi Province, China
| | - Jun-Li Ge
- Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi Province, China
| | - Wei Zou
- Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi Province, China
| | - Ru Jing
- Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi Province, China
| | - Cai-Shi Xin
- Fourth Military Medical University, Xi'an, Shaanxi Province, China
| | - Hong Yang
- Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi Province, China
| | - Bi-Liang Chen
- Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi Province, China
| | - Gao-Wen Chen
- Obstetrics and Gynecology Center, Zhujiang Hospital, Southern Medical University, Guangzhou, China
| | - Jia Li
- Department of Obstetrics and Gynecology, Xijing Hospital, Fourth Military Medical University, Xi'an, Shaanxi Province, China
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Saraei P, Heshmati A, Hosseini S. Small-cell neuroendocrine carcinoma of the female genital tract: A comprehensive overview. J Neuroendocrinol 2024; 36:e13394. [PMID: 38626758 DOI: 10.1111/jne.13394] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/11/2023] [Revised: 02/15/2024] [Accepted: 04/03/2024] [Indexed: 04/18/2024]
Abstract
Small-cell neuroendocrine carcinomas (SCNECs) of the female genital tract are rare and aggressive tumors that are characterized by a high rate of recurrence and poor prognosis. They can arise from various sites within the female genital tract, including the cervix, endometrium, ovary, fallopian tube, vagina, and vulva. They are composed of cells with neuroendocrine features, such as the ability to produce and secrete hormones and peptides, and a high mitotic rate. Immunohistochemical staining for neuroendocrine markers, such as chromogranin A, synaptophysin, and CD56, can aid in the diagnosis of these tumors. This article provides an overview of the epidemiology, etiology, and risk factors associated with these tumors, as well as their clinical presentation, cellular characteristics, diagnosis, and finally the current treatment options for SCNECs, including surgery, chemotherapy, and radiation therapy, alone or in combination.
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MESH Headings
- Humans
- Female
- Carcinoma, Neuroendocrine/diagnosis
- Carcinoma, Neuroendocrine/therapy
- Carcinoma, Neuroendocrine/pathology
- Carcinoma, Small Cell/therapy
- Carcinoma, Small Cell/diagnosis
- Carcinoma, Small Cell/epidemiology
- Carcinoma, Small Cell/pathology
- Genital Neoplasms, Female/therapy
- Genital Neoplasms, Female/diagnosis
- Genital Neoplasms, Female/pathology
- Genital Neoplasms, Female/epidemiology
- Risk Factors
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Affiliation(s)
- Pouya Saraei
- Department of Medical Physics, Medicine School, Ahvaz Jondishapur University of Medical Sciences, Ahvaz, Iran
| | - Abbas Heshmati
- Cancer Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
| | - Sare Hosseini
- Cancer Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
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Liu X, Tian Y, Yan S, Fu H, Si L, Lai T, Mao M, Wang Q, Bai J, Li H, Guo R. Neuroendocrine carcinoma of the endometrium: a retrospective analysis of data from a single center. BMC Cancer 2024; 24:636. [PMID: 38789995 PMCID: PMC11127372 DOI: 10.1186/s12885-024-12393-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/06/2023] [Accepted: 05/16/2024] [Indexed: 05/26/2024] Open
Abstract
BACKGROUND Neuroendocrine carcinoma (NEC) originating from the endometrium is rare, and there is limited knowledge regarding its diagnosis and optimal management. In this study, we present our experience with 11 patients with endometrial NEC, aiming to provide guidance for clinical practice. METHODS We retrospectively collected the clinical, pathological, and treatment data of 11 patients with endometrial NEC who were treated at the First Affiliated Hospital of Zhengzhou University from January 2011 to July 2023. The clinicopathological characteristics, treatment and prognosis of these patients were analyzed. RESULTS The median age of the patients was 55.0 (39.0-64.0) years, and the median tumor size was 40.0 (33.0-60.0) mm. Irregular vaginal bleeding was the most common symptom observed in 10 out of 11 patients, while metabolic syndrome occurred in only 2 out of 11 patients. Six out of the 11 patients were diagnosed at an early stage. Among the patients, 6 were diagnosed with endometrial NECs, while the remaining patients had a combination of endometrial NEC and other non-NEC endometrial carcinomas. All patients underwent surgery, except for one who received only chemotherapy due to multiple metastases. After surgery, adjuvant chemotherapy was administered to 5 patients, chemotherapy combined with radiotherapy was given to 3 patients, and 2 patients did not receive any adjuvant therapy. A total of 10 patients completed the follow-up, with a median follow-up time of 51.0 (14.3-81.0) months. Unfortunately, 2 patients died from the disease. CONCLUSION NECs originating from the endometrium might not be affected by metabolic disorders. Preoperative diagnosis of these tumors was challenging. The primary approach for managing endometrial NEC can be multimodal treatment centered around surgery.
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Affiliation(s)
- Xueyan Liu
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China
| | - Yanpeng Tian
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China
| | - Shuping Yan
- Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, 450052, PR China
| | - Hanlin Fu
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China
| | - Lulu Si
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China
| | - Tianjiao Lai
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China
| | - Meng Mao
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China
| | - Qian Wang
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China
| | - Jing Bai
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China
| | - Heli Li
- Department of Ultrasonography, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, 450052, PR China
| | - Ruixia Guo
- Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, No. 1, Jianshe East Road, Erqi District, Zhengzhou, Henan, 450052, PR China.
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Ramchandrappa K, Shah R, Tyagi A. Metastasis to Thyroid Gland from Neuroendocrine Carcinoma of Endometrium: A Rare Entity. Indian J Otolaryngol Head Neck Surg 2023; 75:1049-1051. [PMID: 37274955 PMCID: PMC10235269 DOI: 10.1007/s12070-022-03335-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/15/2022] [Accepted: 11/29/2022] [Indexed: 12/14/2022] Open
Abstract
Neuroendocrine tumors of endometrium [NECE] are rare malignancy, representing 0.8% of endometrial cancers, having early lymphatic and hematogenous spread with distant metastasis to lungs and brain.
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Affiliation(s)
- Krishnappa Ramchandrappa
- M.C.H. Surgical Oncology, Department of Head and Neck Oncology, Kidwai Memorial Institiute of Oncolgy, Bengaluru, India
| | - Rahulkumar Shah
- M.C.H. Head and Neck Surgery Resident, Department of Head and Neck Oncology, Kidwai Memorial Institiute of Oncolgy, Bengaluru, India
| | - Amit Tyagi
- M.C.H. Head and Neck Surgery Resident, Department of Head and Neck Oncology, Kidwai Memorial Institiute of Oncolgy, Bengaluru, India
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Han Y, Zhang Y, Niu L, Sang C. Combined Carcinoid Carcinoma and Squamous Cell Carcinoma of the Endometrium: A Case Report and Survey of Related Literature. Case Rep Oncol 2023; 16:1402-1408. [PMID: 38028571 PMCID: PMC10651180 DOI: 10.1159/000534622] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2023] [Accepted: 10/09/2023] [Indexed: 12/01/2023] Open
Abstract
Introduction Histologically, endometrial neuroendocrine carcinoma is an extremely rare pathological type of endometrial cancer. In addition, this type is characterized by high invasiveness and poor clinical outcome, which was classified into carcinoid (low grade), and small-cell and large-cell neuroendocrine carcinoma (high grade). Globally, reports on endometrial carcinoid carcinoma are limited. Clinically, it is also rare to see primary squamous cell carcinoma of endometrium. Case Presentation Here, we report an interesting case of mixed carcinoma of endometrium with both carcinoid and squamous cell carcinoma, which presented with persistent vaginal bleeding and hyponatremia. Conclusion Careful pathologic review is necessary to diagnose this rare disease. More studies in the future are warranted to demonstrate the primary surgical treatments and the efficacy of adjuvant therapy of this disease.
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Affiliation(s)
- Ying Han
- Department of Obstetrics and Gynecology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - YanJun Zhang
- Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Lin Niu
- Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - CuiQin Sang
- Department of Obstetrics and Gynecology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
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Pang L, Chen J, Chang X. Large-cell neuroendocrine carcinoma of the gynecologic tract: Prevalence, survival outcomes, and associated factors. Front Oncol 2022; 12:970985. [PMID: 36457506 PMCID: PMC9707296 DOI: 10.3389/fonc.2022.970985] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2022] [Accepted: 10/27/2022] [Indexed: 07/26/2023] Open
Abstract
BACKGROUND We aimed to assess the clinical behavior of gynecologic large-cell neuroendocrine carcinoma (LCNEC) via a retrospective analysis of data from 469 patients. METHODS Patients diagnosed with gynecologic LCNEC from 1988 to 2015 were identified using the Surveillance, Epidemiology, and End Results database. Univariate and multivariate Cox hazard regression analyses were performed to assess independent predictors of overall survival (OS) and cancer-specific survival (CSS). OS and CSS were also evaluated using the Kaplan-Meier method, and the effects of different treatment regimens on prognosis were compared according to disease stage. RESULTS Cervical, ovarian, and endometrial LCNEC were observed in 169, 219, and 79 patients, respectively. The 5-year OS rates for patients with cervical, ovarian, and endometrial LCNEC were 35.98%, 17.84%, and 23.21%, respectively, and the median duration of overall survival was 26, 11, and 11 months in each group. The 5-year CSS rates for the three groups were 45.23%, 19.23%, and 31.39%, respectively, and the median duration of CSS was 41, 12, and 11 months in each group. Multivariate analysis revealed that American Joint Committee on Cancer stage, lymph node metastasis, and chemotherapy were independent prognostic factors for OS and CSS in patients with cervical LCNEC. Lymph node metastasis, surgery, and chemotherapy were independent prognostic factors for OS and CSS in the ovarian group and for OS in the endometrial group. Lymph node metastasis and surgery were also independent prognostic factors for CSS in the endometrial group. CONCLUSION Surgery alone may help to improve overall survival and CSS in patients with early-stage cervical LCNEC. In contrast, surgery+chemotherapy and surgery+radiotherapy may help to improve survival in those with early-stage ovarian and endometrial LCNEC, respectively. Regardless of subtype, comprehensive treatment involving surgery, CTX, and RT should be considered to improve prognosis in patients with advanced-stage gynecologic LCNEC.
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Affiliation(s)
- Li Pang
- Department of Obstetrics and Gynecology, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China
| | - Jie Chen
- Centre of Journals, China Medical University, Shenyang, Liaoning, China
| | - Xiaohan Chang
- Department of Obstetrics and Gynecology, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China
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Zhang Z, Wang J, Wu X, Liu Y, Xi X. Clinical characteristic and prognostic factors in high-grade endometrial neuroendocrine carcinoma. J Obstet Gynaecol Res 2022; 48:2180-2188. [PMID: 35778826 PMCID: PMC9544256 DOI: 10.1111/jog.15321] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2021] [Revised: 04/17/2022] [Accepted: 05/20/2022] [Indexed: 12/01/2022]
Abstract
Aim The aim of the present study was to summarize the clinical characteristics and analyze the independent prognostic factors in patients with high‐grade endometrial neuroendocrine carcinoma (ENC). Methods Patients diagnosed with ENC, endometrioid adenocarcinoma (EAC), endometrial clear‐cell carcinoma (ECC), endometrial serous carcinoma (ESC), endometrioid carcinoma with mucinous features (EMC) from 1987 to 2016 were screened from the National Cancer Institute database (surveillance, epidemiology, and end results [SEER]). Kaplan–Meier were used to assess survival. Univariate and multivariate Cox proportional hazards analysis were done to examine factors affecting survival. Results The median survival times of ENC were 11 months, shorter than that of EAC, ECC, ESC, and EMC (p < 0.01). There was no significant difference in ages, survival rate, and median survival time between large‐cell ENC (LCENC) and small‐cell ENC (SCENC), which were all belong to ENC. In a multivariable model, the hazard ratio (HR) of death for women with Federation International of Gynecology and Obstetrics (FIGO) stage I‐II of ENC was 0.37 compared to FIGO stage III‐IV (p < 0.01). The HR of patients who under the surgery was 0.39 compared to the patients who without surgery (p < 0.01), and the HR of patients who received chemotherapy was 0.51 compared to the patients who did not received chemotherapy (p < 0.01). Radiotherapy did not significantly reduce the mortality risk of patients. Conclusion ENC was a kind of devastating endometrial cancers with the poorest prognosis. Surgical treatment and chemotherapy were necessary for improving prognosis of ENC. Early diagnosis favored better prognosis. There was no prognostic difference between with and without radiotherapy.
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Affiliation(s)
- Zhifang Zhang
- Department of Obstetrics and Gynecology, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Jing Wang
- Department of Pathology, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Xiaomei Wu
- Department of Obstetrics and Gynecology, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Yuan Liu
- Department of Obstetrics and Gynecology, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai, China
| | - Xiaowei Xi
- Department of Obstetrics and Gynecology, Shanghai General Hospital, Shanghai Jiao Tong University, Shanghai, China
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10
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Zhang J, Pang L. Primary Neuroendocrine Tumors of the Endometrium: Management and Outcomes. Front Oncol 2022; 12:921615. [PMID: 35814474 PMCID: PMC9260008 DOI: 10.3389/fonc.2022.921615] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2022] [Accepted: 05/25/2022] [Indexed: 11/24/2022] Open
Abstract
Objective To analyze clinical behavior of, optimal treatment regimens for, outcomes, and prognosis of 170 patients with neuroendocrine tumors (NETs) of the endometrium. Methods The Surveillance, Epidemiology, and End Results database was used to identify patients with endometrial NETs diagnosed between 2004 and 2015. Clinical features and treatment regimens were analyzed, and 5-year overall survival (OS) and cancer-specific survival (CSS) were compared among different stages and treatment regimens. Univariate and multivariate analyses were performed to identify independent prognostic factors associated with endometrial NETs. Finally, prognosis was compared between small- and large-cell neuroendocrine carcinoma (SCNEC and LCNEC, respectively) of the endometrium. Results There were 20, 8, 47, and 95 patients with stage I, II, III, and IV NET, respectively. The 5-year OS rates of patients in each stage were 59.86%, 42.86%, 32.75%, and 6.04%, respectively. The 5-year CSS survival rates were 59.86%, 50.0%, 38.33%, and 6.39%, respectively. In the multivariate analysis, American Joint Committee on Cancer (AJCC) stage and treatment were associated with poor OS, while AJCC stage, nodal metastasis, and treatment were associated with poor CSS. Neither pathological type nor distant metastasis was associated with prognosis. The rate of distant metastasis was significantly higher for LCNEC than for SCNEC, while 5-year OS and CSS rates were significantly lower. Conclusion Complete surgical treatment should be selected regardless of staging for patients with endometrial NETs. For early-stage disease, individualized postoperative treatment with single chemotherapy or radiotherapy may improve OS and CSS. For advanced-stage disease, comprehensive postoperative adjuvant therapy may improve OS and CSS.
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Affiliation(s)
- Jingjing Zhang
- Department of Nursing, Shengjing Hospital of China Medical University, Shenyang, China
| | - Li Pang
- Department of Obstetrics and Gynecology, Shengjing Hospital of China Medical University, Shenyang, China
- *Correspondence: Li Pang,
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Pang L, Wang S. Prevalence, Associated Factors, and Survival Outcomes of Small-Cell Neuroendocrine Carcinoma of the Gynecologic Tract: A Large Population-Based Analysis. Front Med (Lausanne) 2022; 9:836910. [PMID: 35479939 PMCID: PMC9035633 DOI: 10.3389/fmed.2022.836910] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2021] [Accepted: 03/17/2022] [Indexed: 11/17/2022] Open
Abstract
Small-cell carcinomas are highly malignant tumors with neuroendocrine function and which often occur in the lungs. Primary small-cell neuroendocrine carcinomas of the gynecologic tract are extremely rare. This study aimed to evaluate the prevalence of independent predictors related to the prognosis and overall survival of patients with small-cell neuroendocrine carcinomas of the gynecologic tract. Patients with gynecologic small-cell neuroendocrine carcinomas diagnosed between 1973 and 2015 were identified from the Surveillance, Epidemiology, and End Results database. Univariate and multivariate Cox risk regression analyses were performed to determine the independent predictors of overall survival. Overall survival was calculated using the Kaplan–Meier method, and the log-rank test was used for comparison. We included 5,15,393 eligible carcinomas in the present study; the prevalence of gynecologic small-cell neuroendocrine carcinomas was 0.121% (N = 622). Multivariate analysis indicated that advanced age, stage III and IV cancer, and no chemotherapy treatment may be predictors of poor small-cell neuroendocrine cervical carcinoma prognosis. Stage III and IV cancer and lack of surgery, radiotherapy, or chemotherapy were identified as potential predictors of poor prognosis in patients with ovarian small-cell neuroendocrine carcinoma. Kaplan–Meier analysis suggested that the median survival was 19, 11, and 12 months for cervical, ovarian, and endometrial small-cell neuroendocrine carcinomas, respectively. The 1-, 3-, and 5-year overall survival rates were as follows: 58.8, 31.4, and 26.1%, respectively, for small-cell neuroendocrine cervical carcinoma; 46.3, 23.5, and 22.0%, respectively, for ovarian small-cell neuroendocrine carcinoma; and 49.4, 29.4, and 25.9%, respectively, for endometrial small-cell neuroendocrine carcinoma. Our findings indicate that comprehensive and individualized treatment of small-cell neuroendocrine carcinomas of the gynecologic tract may prolong patient survival, although further studies are required.
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Zhou N, Reese N, Giashuddin S, Kanis MJ. A rare case of neuroendocrine carcinoma of the endometrium metastatic to the thyroid. Gynecol Oncol Rep 2021; 37:100816. [PMID: 34258356 PMCID: PMC8254128 DOI: 10.1016/j.gore.2021.100816] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2021] [Revised: 06/07/2021] [Accepted: 06/16/2021] [Indexed: 10/28/2022] Open
Affiliation(s)
- Nancy Zhou
- Division of Gynecologic Oncology, SUNY Downstate Medical Center, Brooklyn, NY, United States
| | - Nicolette Reese
- Division of Gynecologic Oncology, New York Presbyterian - Brooklyn Methodist Hospital, Brooklyn, NY, United States
| | - Shah Giashuddin
- Department of Pathology and Laboratory Medicine, New York Presbyterian - Brooklyn Methodist Hospital, Brooklyn, NY, United States
| | - Margaux J Kanis
- Division of Gynecologic Oncology, New York Presbyterian - Brooklyn Methodist Hospital, Brooklyn, NY, United States
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13
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Du R, Jiang F, Wang ZY, Kang YQ, Wang XY, Du Y. Pure large cell neuroendocrine carcinoma originating from the endometrium: A case report. World J Clin Cases 2021; 9:3449-3457. [PMID: 34002157 PMCID: PMC8107915 DOI: 10.12998/wjcc.v9.i14.3449] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/08/2020] [Revised: 02/10/2021] [Accepted: 03/04/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Large cell neuroendocrine carcinoma (LCNEC) of the endometrium is an uncommon and highly aggressive tumor that has not been comprehensively characterized. We report a case of pure endometrial LCNEC and review the current literature of similar cases to raise awareness of the histological features, treatment, and prognosis of this tumor.
CASE SUMMARY We report the case of a 73-year-old woman who presented with irregular postmenopausal vaginal bleeding. Ultrasonography showed an enlarged uterus and a 5.1 cm × 3.3 cm area of medium and low echogenicity in the uterine cavity. Biopsy by dilatation and curettage suggested poorly differentiated carcinoma. Magnetic resonance imaging revealed a heterogeneously enhanced uterine tumor with diffuse infiltration of the posterior wall of the uterine myometrium and enlarged pelvic lymph nodes. The patient underwent a hysterectomy and bilateral adnexal resection. Gross observation revealed an ill-defined white solid mass of the posterior wall of the uterus infiltrating into the serosa with multiple solid nodules on the serous surface. Microscopically, the tumor cells showed neuroendocrine morphology (organoid nesting). Immunohistochemistry revealed the tumor cells were diffusely positive for the neuroendocrine markers CD56, chromogranin A, and synaptophysin. Thus, the tumor was diagnosed as stage IIIC endometrial LCNEC.
CONCLUSION Pathologic findings and immunohistochemistry are essential in making a diagnosis of endometrial LCNEC.
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Affiliation(s)
- Ran Du
- Department of Pathology, Liaocheng People’s Hospital, Liaocheng 252000, Shandong Province, China
| | - Feng Jiang
- Department of Thoracic Surgery, Liaocheng Tumor Hospital, Liaocheng 252000, Shandong Province, China
| | - Zheng-Yan Wang
- Department of Pathology, Liaocheng People’s Hospital, Liaocheng 252000, Shandong Province, China
| | - Yan-Qing Kang
- Department of Radiology, Liaocheng People’s Hospital, Liaocheng 252000, Shandong Province, China
| | - Xiu-Yu Wang
- Department of Pathology, Liaocheng People’s Hospital, Liaocheng 252000, Shandong Province, China
| | - Ying Du
- Department of Pathology, Liaocheng People’s Hospital, Liaocheng 252000, Shandong Province, China
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Matsumoto H, Nasu K, Shikama A, Shiozaki T, Futagami M, Kai K, Mori T, Yano M, Yamada T, Teramoto N. Pathological processes and pretreatment cytologic diagnosis of neuroendocrine carcinoma of the endometrium: addendum report of the Kansai Clinical Oncology Group/Intergroup study in Japan. Med Mol Morphol 2021; 54:237-244. [PMID: 33934220 DOI: 10.1007/s00795-021-00288-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2021] [Accepted: 04/09/2021] [Indexed: 11/28/2022]
Abstract
A previous retrospective study of a neuroendocrine carcinoma of the endometrium including 42 cases employed a central pathologic review to ensure the reliability of the findings. However, the pathological processes were not described in detail. In this study, we further analyzed these processes and the results of pretreatment endometrial cytology of neuroendocrine carcinoma. Of the 65 patients from 18 institutions registered in the study, 42 (64.6%) were diagnosed with neuroendocrine carcinoma of the endometrium based on the central pathological review. Thirteen of the 23 excluded cases conflicted from their original diagnoses: 5 (38.5%) were diagnosed with endometrioid adenocarcinoma, 5 (38.5%) with undifferentiated carcinoma, and 3 (23.1%) with carcinosarcoma. Immunohistochemical staining led to a change in diagnosis for 8 (61.5%) of the 13 cases. Pretreatment endometrial cytology was examined in 38 (90.5%) cases; 34 (89.5%) of these 38 cases were found, or suspected, to be positive. To ensure the selection of appropriate therapy and keeping patients correctly informed, it is important to distinguish neuroendocrine carcinoma from other similar histologic types. Endometrial cytology may help in the early detection of this disease.
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Affiliation(s)
- Harunobu Matsumoto
- Department of Obstetrics and Gynecology, Faculty of Medicine, Oita University, Hasama-machi, Yufu-shi, Oita, 8795593, Japan.
| | - Kaei Nasu
- Department of Obstetrics and Gynecology, Faculty of Medicine, Oita University, Hasama-machi, Yufu-shi, Oita, 8795593, Japan
| | - Ayumi Shikama
- Department of Obstetrics and Gynecology, University of Tsukuba, Tsukuba-shi, Ibaraki, Japan
| | - Takaya Shiozaki
- Division of Gynecology, Hyogo Cancer Center, Akashi-shi, Hyogo, Japan
| | - Masayuki Futagami
- Department of Obstetrics and Gynecology, Hirosaki University, Hirosaki-shi, Aomori, Japan
| | - Kentaro Kai
- Department of Obstetrics and Gynecology, Faculty of Medicine, Oita University, Hasama-machi, Yufu-shi, Oita, 8795593, Japan
| | - Taisuke Mori
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Kyoto-shi, Kyoto, Japan
| | - Mitsutake Yano
- Division of Pathology, Saitama Medical University International Medical Center, Hidaka-shi, Saitama, Japan
| | - Takashi Yamada
- Department of Pathology, Osaka Medical College, Takatsuki-shi, Osaka, Japan
| | - Norihiro Teramoto
- Division of Pathology, Shikoku Cancer Center, Matsuyama-shi, Ehime, Japan
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15
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Inoue K, Kai K, Sato S, Nishida H, Hirakawa K, Nasu K, Narahara H. Mixed large and small cell neuroendocrine carcinoma and endometrioid carcinoma of the endometrium with high microsatellite instability: A case report and literature review. SAGE Open Med Case Rep 2021; 9:2050313X21999200. [PMID: 33738100 PMCID: PMC7934024 DOI: 10.1177/2050313x21999200] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/04/2021] [Accepted: 02/04/2021] [Indexed: 11/16/2022] Open
Abstract
A 65-year-old, gravida 3, para 2 Japanese woman was referred to our hospital for symptomatic thickening of the endometrial lining. Endocervical and endometrial cytology revealed an adenocarcinoma. The endometrial biopsy specimen was mixed, with a glandular part diagnosed as endometrioid carcinoma and a solid part diagnosed as high-grade mixed large and small cell neuroendocrine carcinoma (L/SCNEC). She underwent extra-fascial hysterectomy with bilateral salpingo-oophorectomy, complete pelvic and para-aortic lymphadenectomy, and omentectomy (FIGO IIIB, pT3b pN0 M0). She currently has no deleterious germline mutation, but high tumor mutation burden and high microsatellite instability (MSI) were identified. She underwent six cycles of platinum-based frontline chemotherapy and achieved complete remission. Immune checkpoint blockade therapy is a promising second-line therapy for MSI-high solid tumors. However, the MSI or mismatch repair (MMR) status of endometrial L/SCNEC remains unclear in the literature. Universal screening for MSI/MMR status is needed, particularly for a rare and aggressive disease.
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Affiliation(s)
- Kotaro Inoue
- Department of Obstetrics and Gynecology, Faculty of Medicine, Oita University, Yufu, Japan
| | - Kentaro Kai
- Department of Obstetrics and Gynecology, Faculty of Medicine, Oita University, Yufu, Japan
| | - Shimpei Sato
- Department of Obstetrics and Gynecology, Faculty of Medicine, Oita University, Yufu, Japan
| | - Haruto Nishida
- Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Japan
| | - Koji Hirakawa
- Department of Diagnostic Pathology, Faculty of Medicine, Oita University, Yufu, Japan
| | - Kaei Nasu
- Division of Obstetrics and Gynecology, Support System for Community Medicine, Faculty of Medicine, Oita University, Yufu, Japan
| | - Hisashi Narahara
- Department of Obstetrics and Gynecology, Faculty of Medicine, Oita University, Yufu, Japan
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16
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Zou Q, Zhang L, Cheng Z, Guo X, Cao D. INSM1 Is Less Sensitive But More Specific Than Synaptophysin in Gynecologic High-grade Neuroendocrine Carcinomas: An Immunohistochemical Study of 75 Cases With Specificity Test and Literature Review. Am J Surg Pathol 2021; 45:147-159. [PMID: 33264139 DOI: 10.1097/pas.0000000000001641] [Citation(s) in RCA: 19] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/05/2023]
Abstract
Insulinoma-associated protein 1 (INSM1) has emerged as a promising diagnostic marker for high-grade neuroendocrine carcinomas (HGNECs); however, it is controversial whether INSM1 is more sensitive than conventional markers chromogranin, synaptophysin, and CD56. Here, we investigated immunohistochemical expression of INSM1 in 75 gynecologic HGNECs using full tissue sections (30 small-cell carcinomas [SmCCs], 34 large-cell neuroendocrine carcinomas [LCNECs], and 11 mixed SmCC and LCNEC), with specificity analysis in 422 gynecologic non-neuroendocrine tumors (410 in tissue microarrays and 12 full sections) and comparison with conventional neuroendocrine markers for their sensitivity and specificity. Positive INSM1 staining was seen in 69 (92%) HGNECs, whereas chromogranin, synaptophysin, and CD56 staining was seen in 61 (81%), 72 (96%), and 44 (69%) tumors, respectively (INSM1 vs. chromogranin, P=0.09; INSM1 vs. synaptophysin, P=0.4942; and INSM1 vs. CD56, P<0.001). The mean percentage of INSM1-positive tumor cells was 54% (median: 60%, range: 0% to 100%), similar to chromogranin (58%, P=0.2903) and higher than CD56 (30%, P=0.00001) but significantly lower than synaptophysin (89%, P<0.00001). INSM1 showed no staining difference among SmCCs, LCNECs, and mixed SmCC-LCNECs. Among the 422 non-neuroendocrine tumors, positive staining was seen in 5% tumors for INSM1, 18% for chromogranin, 19% for synaptophysin, and 25% for CD56. Our study indicates that INSM1 is a highly specific marker (95% specificity) for gynecologic HGNECs with high sensitivity (92%), but it is less sensitive than synaptophysin (96% sensitivity). INSM1 is more specific than chromogranin, synaptophysin, and CD56 for gynecologic HGNECs. Our literature review reveals that INSM1 has consistently (the same antibody clone A8 used for all reported studies) shown higher or similar sensitivity to chromogranin (for all 3 chromogranin antibody clones LK2H10, DAK-A3, DAKO polyclonal); however, whether INSM1 is more or less sensitive than synaptophysin or CD56 for HGNECs is highly dependent on the antibody clones used for synaptophysin (clones MRQ-40 and SNP88 showing higher sensitivity than clones 27G12 and DAK-SYNAP) or CD56 (clones CD564, MRQ-42, and MRQ-54 showing higher sensitivity than clones 123C3D5, 1B6, and Leu243).
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Affiliation(s)
- Qiong Zou
- Department of Pathology, The Third Xiangya Hospital, Central South University, Changsha, Hunan Province
| | - Lily Zhang
- Department of Pathology and Immunology, Washington University School of Medicine, Saint Louis, MO
| | - Zhiqiang Cheng
- Department of Pathology, Shenzhen People's Hospital, Shenzhen, Guangdong Province, China
| | - Xiaojing Guo
- Department of Pathology, Shenzhen People's Hospital, Shenzhen, Guangdong Province, China
| | - Dengfeng Cao
- Department of Pathology and Immunology, Washington University School of Medicine, Saint Louis, MO
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17
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Molecular Characterization of Neuroendocrine Carcinomas of the Endometrium: Representation in All 4 TCGA Groups. Am J Surg Pathol 2020; 44:1541-1548. [PMID: 32773531 DOI: 10.1097/pas.0000000000001560] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
High-grade neuroendocrine carcinomas (NEC) of the endometrium are rare and account for <1% of all endometrial carcinomas. Both small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC) morphologies have been reported. Little is known regarding the molecular features of endometrial NEC including how they compare to pulmonary NEC (the most common site for these neoplasms) and the more common endometrial carcinoma histotypes. In this study, we investigated the molecular alterations in a series of endometrial NEC using a targeted next generation sequencing panel (Oncopanel). Fourteen NEC were sequenced; pure NEC (n=4) and mixed (n=10) with endometrioid adenocarcinoma (n=9) or carcinosarcoma (n=1). The NEC components of mixed tumors comprised LCNEC (n=6) and SCNEC (n=4). The 4 pure NEC comprised LCNEC (n=2) and SCNEC (n=2). Molecular analysis classified tumors into the 4 The Cancer Genome Atlas groups: (1) POLE-mutated/ultramutated (1/14; 7%), (2) microsatellite instability/hypermutated (6/14; 43%), (3) TP53 mutated/copy number high (2/14; 14%), or (4) no specific molecular profile (5/14; 36%). Overall, 50% of cases were ultramutated or hypermutated. In 8 cases of mixed carcinomas, the different histologic components were macrodissected and separately sequenced; molecular alterations were nearly identical among the 2 components, with the non-NEC component harboring slightly increased tumor mutational burden. Only 2 carcinomas (both with pure SCNEC morphology) had a molecular profile that would be expected in typical pulmonary SCNEC (RB1 deletion and TP53 mutations). Our findings, similar to data from NECs of other anatomic sites, suggest that the molecular context may be important when selecting therapies for women with endometrial NEC. Immune checkpoint inhibition may be a reasonable approach to treatment of microsatellite instability-NEC and we thus recommend that all endometrial NEC be tested for mismatch repair abnormalities, either molecularly or by mismatch repair protein immunohistochemistry.
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18
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Akgor U, Kuru O, Sakinci M, Boyraz G, Sen S, Cakır I, Turan T, Gokcu M, Gultekin M, Sayhan S, Salman C, Ozgul N. Neuroendocrine carcinoma of the endometrium: A very rare gynecologic malignancy. J Gynecol Obstet Hum Reprod 2020; 50:101897. [PMID: 32827837 DOI: 10.1016/j.jogoh.2020.101897] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2020] [Revised: 08/16/2020] [Accepted: 08/17/2020] [Indexed: 12/17/2022]
Abstract
OBJECTIVE To investigate the clinicopathologic characteristics, prognostic factors, outcome, and treatment of the neuroendocrine carcinoma (NEC) of the endometrium. MATERIALS AND METHODS We retrospectively reviewed the clinicopathologic and survival data of 10 patients who underwent surgery for NEC. The patients were collected between 1999 and 2017 from four referral centers in Turkey. RESULTS The median age of patients was 67 years (range: 34-75 years). The NEC of endometrium consist of 9 cases with small cell carcinoma (SC) NEC (two with mixed histotypes), and one with a large cell (LC) NEC. According to FIGO 2009 criteria, 70 % (7/10) of patients had advanced stage (III and IV) disease. All patients except one underwent surgical staging, eight patients received platinum-based chemotherapy (CTX) and of 6 those were additionally treated with radiotherapy (RT). Four patients died of disease ranging from 2 to 10 months and six were alive 12-72 months with no evidence of disease. In addition, 4 SC NEC cases raised in polypoid features had no evidence of disease from 24 to 72 months. DISCUSSION NEC of the endometrium is a rare disease with poor prognosis, which frequently diagnosed in advanced stages. The main treatment modality was the administration of platinum-based CTX as an adjuvant to surgery or surgery and RT. Our result suggests that the polypoid feature of the tumor might be one of the best predictors for the prognosis of SC NEC.
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Affiliation(s)
- Utku Akgor
- Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
| | - Oguzhan Kuru
- Department of Gynecologic Oncology, Tepecik Education and Research Hospital, Izmir, Turkey
| | - Mehmet Sakinci
- Department of Obstetrics and Gynecology, Akdeniz University, Faculty of Medicine, Antalya, Turkey
| | - Gokhan Boyraz
- Division of Gynecologic Oncology, Etlik Zubeyde Hanim Women's Health Teaching and Research Hospital, Ankara, Turkey
| | - Serhat Sen
- Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Izmir Katip Celebi University, Izmir, Turkey
| | - Ilker Cakır
- Department of Gynecologic Oncology, Tepecik Education and Research Hospital, Izmir, Turkey
| | - Taner Turan
- Division of Gynecologic Oncology, Etlik Zubeyde Hanim Women's Health Teaching and Research Hospital, Ankara, Turkey
| | - Mehmet Gokcu
- Department of Gynecologic Oncology, Tepecik Education and Research Hospital, Izmir, Turkey
| | - Murat Gultekin
- Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
| | - Sevil Sayhan
- Department of Pathology, Tepecik Education and Research Hospital, Izmir, Turkey
| | - Coskun Salman
- Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
| | - Nejat Ozgul
- Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
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19
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Long-term survival in a stage IV small cell carcinoma of the endometrium. Gynecol Oncol Rep 2020; 32:100580. [PMID: 32420439 PMCID: PMC7218260 DOI: 10.1016/j.gore.2020.100580] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/03/2020] [Revised: 04/25/2020] [Accepted: 04/29/2020] [Indexed: 11/23/2022] Open
Abstract
Small cell carcinoma of the endometrium is a rare malignancy with poor survival. A patient was diagnosed with stage IV small cell carcinoma of the endometrium. She was treated with surgery, chemotherapy (cisplatin/etoposide) and radiotherapy. She remains disease free 5 years after completion of her treatments.
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