Diffuse leiomyomatosis in a child

Khashchenko E.P., Silenok Е.R., Uvarova E.V., Chuprynin V.D., Kyurdzidi S.O., Kulabukhova E.A., Uchevatkina P.V., Mamedova F.Sh., Asaturova A.V., Tregubova A.V., Badlaeva A.S.

1) Academician V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology, and Perinatology, Ministry of Health of Russia, Moscow, Russia; 2) N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, Moscow, Russia; 3) I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia, Moscow, Russia; 4) Russian Medical Academy of Continuing Professional Education, Ministry of Health of Russia, Moscow, Russia

Background: Uterine leiomyomas are not uncommon in adolescence; however, an unusual growth model, such as diffuse uterine leiomyomatosis (DUL), has been registered in single cases. The differential diagnosis of DUL is made with common multiple uterine leiomyomas and nodular adenomyosis. In view of the fact that the early diagnosis is difficult and there is no universal approach to the tactics of patient management, uterine masses frequently reach large sizes and motivate organ-removing operations.
Case report: The paper presents a review of literature data and an extremely rare clinical case of DUL in a 14-year-old girl. The patient visited a gynecologist with complaints of severe lower abdominal pain increasing in the last days of menstruation, which has been bothering her for the past year. She was suspected of diffuse nodular adenomyosis or multiple uterine myoma and was referred to be admitted to Gynecology Department Two, V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology, and Perinatology, Ministry of Health of the Russian Federation. The Department made an instrumental and laboratory additional study, diagnosed uterine leiomyomatosis and external genital endometriosis of the pelvic peritoneum, ruled out other sites of possible leiomyomatosis; oncomarkers were obtained within the reference values. The girl received surgical treatment (laparoscopy, adhesiolysis, removal of a subserous leiomyomatous node, corpus uteri biopsy, coagulation of the foci of external genital endometriosis, and diagnostic hysteroscopy. Morphological examination confirmed uterine leiomyomatosis with hyalinosis. Immunohistochemical study of a myometrial biopsy sample revealed that the expression of estrogen receptors was 3 scores and that of progesterone receptors was 4 scores (according to the Allred scale; that of the proliferation marker Ki-67 was 20%. The patient was discharged home; after 3 months during therapy with gestagens (norethisterone), pelvic organ ultrasound indicated no substantial changes in the uterine sizes. Therapy and a follow-up were continued.
Conclusion: DUL is extremely rare in pediatric practice and is frequently detected late, which determines the relevance of timely preventive examinations and early diagnosis in order to maximize the organ-sparing tactics of management.

Authors' contributions: Khashchenko Е.P., Silenok E.R. – the conception of the investigation, search and analysis of literature sources, setting up the tasks and the design of the investigation, writing and editing the text; Khashchenko E.P., Uvarova E.V., Kyurdzidi S.O., Chuprynin V.D. – analysis and interpretation of the data of the patients and surgical intervention;
Mamedova F.Sh. – conduction and analysis of the results of pelvic organ study; Kulabukhova Е.А., Uchevatkina P.V. – conduction, description, and analysis of pelvic magnetic resonance imaging, identification of significant factors; Asaturova A.V., Tregubova A.V., Badlaeva A.S. – conduction, description, and analysis of morphopathological and immunohistochemical study; Khashcheenko E.P., Uvarova E.V. – final editing of the text.
Conflict of interest: The authors declare that there are no conflicts of interest.
Funding: The investigation has been conducted with financial support of State Assignment 18-A21 of the Ministry of Health “Role of impairments in energy metabolism and immune defense in the development of different forms of endometriosis, the development of personified therapy and prognosis of its efficiency in the early reproductive period (from menarche
to 18 years of age”.
Ethical Approval: The investigation has been approved by the Commission on Ethics of Biomedical Research, Academician V.I. Kulakov National Medical Research Center of Obstetrics, Gynecology, and Perinatology, Ministry of Health of the Russian Federation.
Patient Consent for Publication: An informed consent has been obtained from the female patient and her legal representative for the publication of her data and associated images.
For citation: Khashchenko E.P., Silenok Е.R., Uvarova E.V., Chuprynin V.D., Kyurdzidi S.O., Kulabukhova E.A., Uchevatkina P.V., Mamedova F.Sh., Asaturova A.V., Tregubova A.V., Badlaeva A.S. Diffuse leiomyomatosis in a child.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2023; (7): 162-170 (in Russian)
https://dx.doi.org/10.18565/aig.2023.75

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About the Authors

Elena P. Khashchenko, PhD, obstetrician-gynecologist, Senior Researcher at the 2nd Gynecological (Children and Adolescents) Department, Academician V.I. Kulakov
NMRС for OG&P, Ministry of Health of Russia, +7(495)438-85-09, khashchenko_elena@mail.ru, https://orcid.org/0000-0002-3195-307X,
117997, Russia, Moscow, Academician Oparin str., 4.
Elena R. Silenok, 4th year student, Faculty of General Medicine, N.I. Pirogov RNIMU, Ministry of Health of Russia, +7(495)434-36-90,
117321, Russia, Moscow, Ostrovityanova str., 1.
Elena V. Uvarova, Dr. Med. Sci., Professor, Corresponding Members of the RAS, Head of the 2nd Gynecological (Children and Adolescents) Department,
Academician V.I. Kulakov NMRС for OG&P, Ministry of Health of Russia, 117997, Russia, Moscow, Academician Oparin str., 4; Professor of the Department of Obstetrics, Gynecology, Perinatology and Reproductology of the Institute of Professional Education, I.M. Sechenov First MSMU, Ministry of Health of Russia (Sechenov University), 119991, Russia, Moscow, Trubetskaya str., 8-2, +7(495)438-85-09, elena-uvarova@yandex.ru, https://orcid.org/0000-0002-3105-5640
Vladimir D. Chuprynin, PhD, Head of the Surgery Department, Academician V.I. Kulakov NMRС for OG&P, Ministry of Health of Russia, +7(495)438-85-42,
v_chuprynin@oparina4.ru, 117997, Russia, Moscow, Academician Oparin str., 4.
Stanislav O. Kyurdzidi, PhD student at the Department of Obstetrics, Gynaecology and Perinatology, Institute of Professional Education, I.M. Sechenov First MSMU,
Ministry of Health of Russia (Sechenov University), +7(495)438-24-00, dr.kyurdzidis@gmail.com, https://orcid.org/0000-0002-6316-1325,
119991, Russia, Moscow, Trubetskaya str., 8-2.
Elena A. Kulabukhova, PhD, Radiologist at the Department of Radiation Diagnostics, Academician V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia,
+7(495)438-76-47, e_kulabukhova@oparina4.ru, 117997, Russia, Moscow, Ac. Oparina str., 4.
Polina V. Uchevatkina, Radiologist at the Department of Radiation Diagnostics, Academician V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia, +7(495)438-76-47, p_uchevatkina@oparina4.ru, 117997, Russia, Moscow, Ac. Oparina str., 4.
Fatima Sh. Mamedova, PhD, Physician at the Department of Ultrasound Diagnostics in Neonatology and Pediatrics, Academician V.I. Kulakov NMRC for OG&P,
Ministry of Health of Russia; assistant at the Department of Radiation Diagnostics of Pediatric Age, RMACPE, Ministry of Health of Russia, f_mamedova@oparina4.ru,
https://orcid.org/0000-0003-1136-7222, 4, Oparina str., Moscow, 117997, Russia.
Aleksandra V. Asaturova, PhD, Head of the 1th Pathology Department, Academician V.I. Kulakov NMRС for OG&P, Ministry of Health of Russia, a_asaturova@oparina4.ru, https://orcid.org/0000-0001-8739-5209, 117997, Russia, Moscow, Academician Oparin str., 4.
Anna V. Tregubova, Junior Researcher, 1st Pathology Department, Academician V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia, a_tregubova@oparina4.ru,
https://orcid.org/0000-0003-4601-1330, 117997, Russia, Moscow, Oparina str., 4.
Alina S. Badlaeva, Junior Researcher at the 1st Pathology and Anatomical Department, Academician V.I. Kulakov NMRC for OG&P, Ministry of Health of Russia,
https://orcid.org/0000-0001-5223-9767, 117997, Russia, Moscow, Oparina str., 4.

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