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Fertility-sparing strategy in a rare case of highly malignant Dicer-1-associated sarcoma of the cervix

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Titel: Fertility-sparing strategy in a rare case of highly malignant Dicer-1-associated sarcoma of the cervix
Autoren: J. Altmann, K. Kubiak, J. Sehouli, E. Roser
Quelle: Arch Gynecol Obstet
Verlagsinformationen: Springer Science and Business Media LLC, 2024.
Publikationsjahr: 2024
Schlagwörter: Ribonuclease III, Cryopreservation, Adolescent, Uterine Cervical Neoplasms, Fertility Preservation, Sarcoma, Cervix Uteri, Gynecologic Oncology, DEAD-box RNA Helicases, 03 medical and health sciences, 0302 clinical medicine, Doxorubicin, Mutation, Ifosfamide/administration, Adolescent [MeSH], Ribonuclease III/genetics [MeSH], Mutation [MeSH], Sarcoma/diagnostic imaging [MeSH], Uterine Cervical Neoplasms/diagnostic imaging [MeSH], Humans [MeSH], Treatment Outcome [MeSH], Dicer-1 sarcoma, Fertility [MeSH], Uterine Cervical Neoplasms/drug therapy [MeSH], Antineoplastic Combined Chemotherapy Protocols/therapeutic use [MeSH], Tomography, X-Ray Computed [MeSH], Magnetic Resonance Imaging [MeSH], Cervical sarcoma, Doxorubicin/administration, Sarcoma/drug therapy [MeSH], Fertility preservation, Fertility sparing, Humans, Female, Ifosfamide
Beschreibung: Introduction We present the rare case of an 18-year-old patient with a Dicer-1 mutation-associated sarcoma of the cervix uteri. Case The patient presented with irregular vaginal bleeding in July 2022. The clinical examination showed an exophytic tumor of the cervix, uterus and ovaries were normal in sonogram. The tumor of the cervix was resected, followed by a diagnostic hysteroscopy and abrasion of the uterine cervix and cavity. Hysteroscopy showed normal findings of the cervix and uterus. After diagnosis of a highly malignant Dicer-1 mutation-associated sarcoma of the cervix, cryopreservation of oocytes was realized. Based on the principle of obtaining maximum oncological safety while preserving fertility in this 18-year-old patient, we recommended chemotherapy rather than radiation with its far severe implications on the patient´s reproductive organs. 4 cycles of chemotherapy consisting of doxorubicin and ifosfamide were applied until December 2022. After re-staging in December 2022 via CT scan and MRI, the abdomen and pelvis as well as control hysteroscopy and abrasion were unremarkable. Until now, the patient is tumor free. Discussion Primary sarcomas of the cervix are very rare. Recent literature hints towards a distinct DICER-1 sarcoma entity characterized by specific mutational clusters. Limited follow-up data suggested that DICER1-mutant tumors might exhibit a less aggressive clinical course than DICER1-wild-type tumors. Conclusion Decision-making in case of rare histological entities with sparse recommendations in the literature poses a challenge to the treating physician. Treatment strategies should consider oncological safety as well as options of preserving fertility. Gonadotoxic potential of different strategies should be taken into consideration and discussed in detail with the affected patient.
Publikationsart: Article
Other literature type
Sprache: English
ISSN: 1432-0711
DOI: 10.1007/s00404-024-07588-x
Zugangs-URL: https://pubmed.ncbi.nlm.nih.gov/38976022
https://repository.publisso.de/resource/frl:6491538
Rights: CC BY
Dokumentencode: edsair.doi.dedup.....d9dc89015b5de25082024435d37a092c
Datenbank: OpenAIRE
Beschreibung
Abstract:Introduction We present the rare case of an 18-year-old patient with a Dicer-1 mutation-associated sarcoma of the cervix uteri. Case The patient presented with irregular vaginal bleeding in July 2022. The clinical examination showed an exophytic tumor of the cervix, uterus and ovaries were normal in sonogram. The tumor of the cervix was resected, followed by a diagnostic hysteroscopy and abrasion of the uterine cervix and cavity. Hysteroscopy showed normal findings of the cervix and uterus. After diagnosis of a highly malignant Dicer-1 mutation-associated sarcoma of the cervix, cryopreservation of oocytes was realized. Based on the principle of obtaining maximum oncological safety while preserving fertility in this 18-year-old patient, we recommended chemotherapy rather than radiation with its far severe implications on the patient´s reproductive organs. 4 cycles of chemotherapy consisting of doxorubicin and ifosfamide were applied until December 2022. After re-staging in December 2022 via CT scan and MRI, the abdomen and pelvis as well as control hysteroscopy and abrasion were unremarkable. Until now, the patient is tumor free. Discussion Primary sarcomas of the cervix are very rare. Recent literature hints towards a distinct DICER-1 sarcoma entity characterized by specific mutational clusters. Limited follow-up data suggested that DICER1-mutant tumors might exhibit a less aggressive clinical course than DICER1-wild-type tumors. Conclusion Decision-making in case of rare histological entities with sparse recommendations in the literature poses a challenge to the treating physician. Treatment strategies should consider oncological safety as well as options of preserving fertility. Gonadotoxic potential of different strategies should be taken into consideration and discussed in detail with the affected patient.
ISSN:14320711
DOI:10.1007/s00404-024-07588-x