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Rhabdomyosarcoma of the genitourinary system in girls — the role of magnetic resonance imagining in diagnosis, treatment monitoring, and follow-up
- Department of Diagnostic Imaging, Institute of Mother and Child, Warsaw, Poland
- Department of Oncology and Surgical Oncology for Children and Youth, Institute of Mother and Child, Warsaw, Poland
- Clinic of Obstetrics and Gynaecology, Institute of Mother and Child, Warsaw, Poland
open access
Abstract
Objectives: Rhabdomyosarcoma of the genitourinary system in girls is a rare neoplasm, especially in non-dedicated centers. Our work aimed to sum up and present genitourinary rhabdomyosarcomas in girls from the radiological point of view. Material and methods: We retrospectively reviewed all girls with genitourinary RMS who underwent treatment at the Institute of Mother and Child in Warsaw between 2009 and 2022. We evaluated the demographic, clinical, and pathological patient data and imaging studies. Results: During the study period, ten patients presented with genitourinary RMS and underwent magnetic resonance imaging (MRI). The median age at the time of diagnosis was 2.8 years, six patients were younger than three years, and four patients were older than ten years. The most common clinical symptoms were tumor fragments protruding from the vagina/falling out of the vagina and vaginal bleeding or discharge, and the most common original location was the vagina. One hundred percent of patients had the embryonal subtype of RMS, and 100% of cases where molecular tests for PAX3/FOXO1 fusion gen status were performed had negative status. At presentation, the median tumor volume was 114 cm3. Eight patients (80%) were classified as clinical group III according to the IRS Group, and most patients (70%) were in a standard-risk group. All patients received multimodal treatment, including surgery and chemotherapy; 60% received radiotherapy. Neoadjuvant chemotherapy was the primary treatment for all our patients. In six patients (60%) with a measurable tumor mass after a biopsy, a gradual tumor volume reduction was observed after induction chemotherapy (approximately ten weeks of treatment) — all of which had a partial response (PR). All our patients (100%) responded completely to treatment. Conclusions: MRI was performed at every stage of diagnosis and treatment as well as during follow-up. It allowed for staging, monitoring of chemotherapy, and guided surgery.
Abstract
Objectives: Rhabdomyosarcoma of the genitourinary system in girls is a rare neoplasm, especially in non-dedicated centers. Our work aimed to sum up and present genitourinary rhabdomyosarcomas in girls from the radiological point of view. Material and methods: We retrospectively reviewed all girls with genitourinary RMS who underwent treatment at the Institute of Mother and Child in Warsaw between 2009 and 2022. We evaluated the demographic, clinical, and pathological patient data and imaging studies. Results: During the study period, ten patients presented with genitourinary RMS and underwent magnetic resonance imaging (MRI). The median age at the time of diagnosis was 2.8 years, six patients were younger than three years, and four patients were older than ten years. The most common clinical symptoms were tumor fragments protruding from the vagina/falling out of the vagina and vaginal bleeding or discharge, and the most common original location was the vagina. One hundred percent of patients had the embryonal subtype of RMS, and 100% of cases where molecular tests for PAX3/FOXO1 fusion gen status were performed had negative status. At presentation, the median tumor volume was 114 cm3. Eight patients (80%) were classified as clinical group III according to the IRS Group, and most patients (70%) were in a standard-risk group. All patients received multimodal treatment, including surgery and chemotherapy; 60% received radiotherapy. Neoadjuvant chemotherapy was the primary treatment for all our patients. In six patients (60%) with a measurable tumor mass after a biopsy, a gradual tumor volume reduction was observed after induction chemotherapy (approximately ten weeks of treatment) — all of which had a partial response (PR). All our patients (100%) responded completely to treatment. Conclusions: MRI was performed at every stage of diagnosis and treatment as well as during follow-up. It allowed for staging, monitoring of chemotherapy, and guided surgery.
Keywords
genitourinary system; rhabdomyosarcoma; magnetic resonance imaging (MRI)
Title
Rhabdomyosarcoma of the genitourinary system in girls — the role of magnetic resonance imagining in diagnosis, treatment monitoring, and follow-up
Journal
Issue
Article type
Research paper
Pages
32-39
Published online
2023-08-23
Page views
400
Article views/downloads
251
DOI
Pubmed
Bibliographic record
Ginekol Pol 2024;95(1):32-39.
Keywords
genitourinary system
rhabdomyosarcoma
magnetic resonance imaging (MRI)
Authors
Paulina Sobieraj
Zofia Malas
Tadeusz Issat
Anna Raciborska
Monika Bekiesinska-Figatowska
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