High grade sarcoma, with predominant neuroectodermal and minor embryonal rhabdomyosarcomatous tumor of the uterus : A case report
BACKGROUND: There have been few documented cases of combined primitive neuroectodermal and embryonal rhabdomyosarcomas (ERMS) in the uterus. Due to their rarity, there is no consensus on the optimal treatment for patients with primitive neuroectodermal tumor (PNET) and ERMS of the uterus. Studies on treatment and outcome are limited.
CASE PRESENTATION: A 32 year-old female presented with heavy vaginal bleeding. Ultrasound revealed an 18 cm uterus with thickened endometrium. Histopathology revealed embryonal rhabdomyosarcoma. She underwent a total abdominal hysterectomy, bilateral salpingectomy, lymph node dissection, and omentectomy. Pathologic review confirmed a tumor with mainly central-type PNET and focally ERMS within the uterus and cervix. She was treated with adjuvant chemoradiation.
CONCLUSION: Treatment of the predominant tumor, PNET, should be the primary goal of therapy. Vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide with tumor directed radiation may be efficacious for the treatment of this specific high grade uterine sarcoma.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2019 |
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| Erschienen: |
2019 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:28 |
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| Enthalten in: |
Gynecologic oncology reports - 28(2019) vom: 20. Mai, Seite 128-132 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Chang, Leona [VerfasserIn] |
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| Links: |
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| Themen: |
Case Reports |
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| Anmerkungen: |
Date Revised 08.04.2022 published: Electronic-eCollection Citation Status PubMed-not-MEDLINE |
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| doi: |
10.1016/j.gore.2019.04.001 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM296523100 |
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| 245 | 1 | 0 | |a High grade sarcoma, with predominant neuroectodermal and minor embryonal rhabdomyosarcomatous tumor of the uterus |b A case report |
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| 500 | |a Date Revised 08.04.2022 | ||
| 500 | |a published: Electronic-eCollection | ||
| 500 | |a Citation Status PubMed-not-MEDLINE | ||
| 520 | |a BACKGROUND: There have been few documented cases of combined primitive neuroectodermal and embryonal rhabdomyosarcomas (ERMS) in the uterus. Due to their rarity, there is no consensus on the optimal treatment for patients with primitive neuroectodermal tumor (PNET) and ERMS of the uterus. Studies on treatment and outcome are limited | ||
| 520 | |a CASE PRESENTATION: A 32 year-old female presented with heavy vaginal bleeding. Ultrasound revealed an 18 cm uterus with thickened endometrium. Histopathology revealed embryonal rhabdomyosarcoma. She underwent a total abdominal hysterectomy, bilateral salpingectomy, lymph node dissection, and omentectomy. Pathologic review confirmed a tumor with mainly central-type PNET and focally ERMS within the uterus and cervix. She was treated with adjuvant chemoradiation | ||
| 520 | |a CONCLUSION: Treatment of the predominant tumor, PNET, should be the primary goal of therapy. Vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide with tumor directed radiation may be efficacious for the treatment of this specific high grade uterine sarcoma | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Embryonal rhabdomyosarcoma | |
| 650 | 4 | |a PNET | |
| 650 | 4 | |a Primitive neuroectodermal tumor | |
| 650 | 4 | |a Uterine sarcoma | |
| 650 | 4 | |a VAC/IE | |
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| 700 | 1 | |a Lerman, Nati |e verfasserin |4 aut | |
| 700 | 1 | |a Wilson-Smith, Robin |e verfasserin |4 aut | |
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