Z. A. Sidakova, A. A. Baranova, A. N. Gritsai, G. D. Efremov, L. N. Lyubchenko
{"title":"胃肠外肿瘤合并睾丸女性化综合征1例","authors":"Z. A. Sidakova, A. A. Baranova, A. N. Gritsai, G. D. Efremov, L. N. Lyubchenko","doi":"10.21294/1814-4861-2023-22-4-142-148","DOIUrl":null,"url":null,"abstract":"Background . According to literature data, errors in the diagnosis of gastrointestinal tumors (GIST) are uncommon, accounting for approximately 6% of cases that results in treatment failure. Case report . Here, we describe a rare case of a 58-year-old female patient with extragastrointestinal stromal tumor (EGIST) in the evidence of testicular feminisation syndrome (TFS) – Morris syndrome. This hereditary pathology is associated with complete insensitivity of target organs to androgens and was described in 1953 by the American gynaecologist John Morris. The patient was referred to the cancer clinic, where she was wrongly diagnosed with uterine leiomyoma. Interdisciplinary approach, cancer alarm, active surgical tactics, additional immunohistochemical (IHC) and molecular genetic studies (MGI) allowed verifcation of the true diagnosis. There are reported cases of EGIST of the bladder, prostate, retroperitoneum, mesentery, omentum, and posterior mediastinum. However, we were not able to fnd publications regarding the cases of EGIST originating from the vaginal wall. Combination of TFS and EGIST is a unique case in our clinical practice. Conclusion . The study of rare cases expands the understanding of the molecular pathogenesis of malignancies. Patients with rare types of malignant tumors should be obligatorily examined and treated in specialized cancer clinics with involvement of certifed oncologists, surgeons, chemotherapists, geneticists.","PeriodicalId":21881,"journal":{"name":"Siberian journal of oncology","volume":"39 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2023-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)\",\"authors\":\"Z. A. Sidakova, A. A. Baranova, A. N. Gritsai, G. D. Efremov, L. N. Lyubchenko\",\"doi\":\"10.21294/1814-4861-2023-22-4-142-148\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Background . According to literature data, errors in the diagnosis of gastrointestinal tumors (GIST) are uncommon, accounting for approximately 6% of cases that results in treatment failure. Case report . Here, we describe a rare case of a 58-year-old female patient with extragastrointestinal stromal tumor (EGIST) in the evidence of testicular feminisation syndrome (TFS) – Morris syndrome. This hereditary pathology is associated with complete insensitivity of target organs to androgens and was described in 1953 by the American gynaecologist John Morris. The patient was referred to the cancer clinic, where she was wrongly diagnosed with uterine leiomyoma. Interdisciplinary approach, cancer alarm, active surgical tactics, additional immunohistochemical (IHC) and molecular genetic studies (MGI) allowed verifcation of the true diagnosis. There are reported cases of EGIST of the bladder, prostate, retroperitoneum, mesentery, omentum, and posterior mediastinum. However, we were not able to fnd publications regarding the cases of EGIST originating from the vaginal wall. Combination of TFS and EGIST is a unique case in our clinical practice. Conclusion . The study of rare cases expands the understanding of the molecular pathogenesis of malignancies. Patients with rare types of malignant tumors should be obligatorily examined and treated in specialized cancer clinics with involvement of certifed oncologists, surgeons, chemotherapists, geneticists.\",\"PeriodicalId\":21881,\"journal\":{\"name\":\"Siberian journal of oncology\",\"volume\":\"39 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-09-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Siberian journal of oncology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.21294/1814-4861-2023-22-4-142-148\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Siberian journal of oncology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.21294/1814-4861-2023-22-4-142-148","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)
Background . According to literature data, errors in the diagnosis of gastrointestinal tumors (GIST) are uncommon, accounting for approximately 6% of cases that results in treatment failure. Case report . Here, we describe a rare case of a 58-year-old female patient with extragastrointestinal stromal tumor (EGIST) in the evidence of testicular feminisation syndrome (TFS) – Morris syndrome. This hereditary pathology is associated with complete insensitivity of target organs to androgens and was described in 1953 by the American gynaecologist John Morris. The patient was referred to the cancer clinic, where she was wrongly diagnosed with uterine leiomyoma. Interdisciplinary approach, cancer alarm, active surgical tactics, additional immunohistochemical (IHC) and molecular genetic studies (MGI) allowed verifcation of the true diagnosis. There are reported cases of EGIST of the bladder, prostate, retroperitoneum, mesentery, omentum, and posterior mediastinum. However, we were not able to fnd publications regarding the cases of EGIST originating from the vaginal wall. Combination of TFS and EGIST is a unique case in our clinical practice. Conclusion . The study of rare cases expands the understanding of the molecular pathogenesis of malignancies. Patients with rare types of malignant tumors should be obligatorily examined and treated in specialized cancer clinics with involvement of certifed oncologists, surgeons, chemotherapists, geneticists.
期刊介绍:
The main objectives of the journal are: -to promote the establishment of Russia’s leading worldwide positions in the field of experimental and clinical oncology- to create the international discussion platform intended to cover all aspects of basic and clinical cancer research, including carcinogenesis, molecular biology, epidemiology, cancer prevention, diagnosis and multimodality treatment (surgery, chemotherapy, radiation therapy, hormone therapy), anesthetic management, medical and social rehabilitation, palliative care as well as the improvement of life quality of cancer patients- to encourage promising young scientists to be actively involved in cancer research programs- to provide a platform for researches and doctors all over the world to promote, share, and discuss various new issues and developments in cancer related problems. (to create a communication platform for the expansion of cooperation between Russian and foreign professional associations).- to provide the information about the latest worldwide achievements in different fields of oncology The most important tasks of the journal are: -to encourage scientists to publish their research results- to offer a forum for active discussion on topics of major interest - to invite the most prominent Russian and foreign authors to share their latest research findings with cancer research community- to promote the exchange of research information, clinical experience, current trends and the recent developments in the field of oncology as well as to review interesting cases encountered by colleagues all over the world- to expand the editorial board and reviewers with the involvement of well-known Russian and foreign experts- to provide open access to full text articles- to include the journal into the international database- to increase the journal’s impact factor- to promote the journal to the International and Russian markets