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...

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Main Authors: Z. A. Sidakova, A. A. Baranova, A. N. Gritsai, G. D. Efremov, L. N. Lyubchenko
Format: Article
Language:Russian
Published: Russian Academy of Sciences, Tomsk National Research Medical Center 2023-09-01
Series:Сибирский онкологический журнал
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Online Access:https://www.siboncoj.ru/jour/article/view/2690
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author Z. A. Sidakova
A. A. Baranova
A. N. Gritsai
G. D. Efremov
L. N. Lyubchenko
author_facet Z. A. Sidakova
A. A. Baranova
A. N. Gritsai
G. D. Efremov
L. N. Lyubchenko
author_sort Z. A. Sidakova
collection DOAJ
description 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.
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spelling doaj-art-a3c380b4e9ef45fc862f60aefcbc0d712025-08-20T03:43:40ZrusRussian Academy of Sciences, Tomsk National Research Medical CenterСибирский онкологический журнал1814-48612312-31682023-09-0122414214810.21294/1814-4861-2023-22-4-142-1481152A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)Z. A. Sidakova0A. A. Baranova1A. N. Gritsai2G. D. Efremov3L. N. Lyubchenko4N.A. Lopatkin Institute of Urology and Interventional Radiology – branch of the National Medical Research Radiological Centre of the Ministry of Health of the RussiaN.A. Lopatkin Institute of Urology and Interventional Radiology – branch of the National Medical Research Radiological Centre of the Ministry of Health of the RussiaN.A. Lopatkin Institute of Urology and Interventional Radiology – branch of the National Medical Research Radiological Centre of the Ministry of Health of the RussiaN.A. Lopatkin Institute of Urology and Interventional Radiology – branch of the National Medical Research Radiological Centre of the Ministry of Health of the RussiaN.A. Lopatkin Institute of Urology and Interventional Radiology – branch of the National Medical Research Radiological Centre of the Ministry of Health of the Russia; National Medical Research Radiological Centre of the Ministry of Health of the RussiaBackground. 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.https://www.siboncoj.ru/jour/article/view/2690gastrointestinal stromal tumorextragastrointestinal tumortesticular feminization syndromeimmunohistochemical study
spellingShingle Z. A. Sidakova
A. A. Baranova
A. N. Gritsai
G. D. Efremov
L. N. Lyubchenko
A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)
Сибирский онкологический журнал
gastrointestinal stromal tumor
extragastrointestinal tumor
testicular feminization syndrome
immunohistochemical study
title A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)
title_full A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)
title_fullStr A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)
title_full_unstemmed A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)
title_short A clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome (Morris syndrome)
title_sort clinical case of extragastrointestinal tumor in combination with testicular feminization syndrome morris syndrome
topic gastrointestinal stromal tumor
extragastrointestinal tumor
testicular feminization syndrome
immunohistochemical study
url https://www.siboncoj.ru/jour/article/view/2690
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