Öz
Botryoid rabdomyosarkom çocukluk çağı ve infantil dönemde en sık görülen malign mesenkimal tümördür. Bu tümör vajina, mesane, biliyer system gibi içi boş organları örten rhabdomyoblast denilen immatür iskelet sistemi hücrelerinden orijin alır. Botryoid sarkom; erken çocukluk çağı ve infantlarda vajinada, reprodüktif çağda serviksde, postmenaposal yıllarda uterus korpusta bulunur. Tedavi cerrahinin adjuvant kemoterapi ve radyoterapi ile kombinasyonudur. Biz vajinal kanama ve vajinadan prolabe olan kitle ile başvuran 2 aylık kız çocuğu olgusu bildirdik. Difüzyon Manyetik Rezonans Görüntüleme (MRG) ‘de rektovajinal septumda yaklaşık 6 cm çapında kitle görüldü. Vajinadan alınan eksizyonel biyopsi botryoid rabdomyosarkom tanısını doğruladı. Biyopsi sonrası hasta çoklu kemoterapi tedavisi aldı. Hasta şuan hala hastalıksız şekilde remisyonda takip edilmektedir.
Anahtar Kelimeler
Kaynakça
- Lanzkowsky P. Manual of Pediatric Hematology and Oncology Fifth Edition. Elsevier; 2013. p. 718-732.
- Chrysoula Margioula-Siarkou, Stamatios Petousis, Aristarchos Almperis, Georgia Margioula-Siarkou, Antonio Simone Laganà, Maria Kourti, et al. Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children. Diagnostics (Basel). 2023;13(5): 924.
- van Sambeeck SJ, Mavinkurve-Groothuis AM, Flucke U, Dors N. Sarcoma botryoides in an infant. BMJ Case Rep. 2014 Dec 17;2014:bcr2013202080.
- Hettmer S, Wagers AJ. Muscling in: Uncovering the Origins of Rhabdomyosarcoma. Nat. Med. 2010;16:171–173. doi: 10.1038/nm0210-171.
- Li RF, Gupta M, McCluggage WG, Ronnett BM. Embryonal Rhabdomyosarcoma (Botryoid Type) of the Uterine Corpus and Cervix in Adult Women: Report of a Case Series and Review of the Literature. Am. J. Surg. Pathol. 2013;37:344–355. doi: 10.1097/PAS.0b013e31826e0271.
- Maharaj NR, Nimako D, Hadley GP. Multimodal therapy for the initial management of genital embryonal rhabdomyosarcoma in childhood. Int J Gynecol Cancer. 2008 Jan-Feb;18(1):190-192.
Öz
Botyroid rhabdomyosarcoma is the most common malignant mesencymal tumor of the vagina in children and infants. It is a malignant tumor that arises from immature skeletal muscle cells (i.e., rhabdomyoblasts) lining the walls of hollow organs, including the vagina, bladder, and biliary tract. This cancer most commonly affects children under the age of 4, although it can sometimes present in older individuals. Botyroid sarcoma is usually found in the vagina during infancy and early childhood, in the cervix during reproductive years, and in the corpus uteri during postmenapausal years. Treatment is combination of surgery with adjuvants chemotherapy and radiotherapy. We report a case of two-month-old girl presented with a history of vaginal bleeding and a mass protruding from vagina. Diffusion Magnetic Resonance Imaging (MRI) showed an approximately 6 cm mass in rectovaginal septum. Excisional biopsy from the vagina confirmed the diagnosis of botyroid rhabdomyosarcoma. After biopsy the patient received multiple cycles of chemotherapy. The patient is disease-free on remission at this time.
Anahtar Kelimeler
Kaynakça
- Lanzkowsky P. Manual of Pediatric Hematology and Oncology Fifth Edition. Elsevier; 2013. p. 718-732.
- Chrysoula Margioula-Siarkou, Stamatios Petousis, Aristarchos Almperis, Georgia Margioula-Siarkou, Antonio Simone Laganà, Maria Kourti, et al. Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children. Diagnostics (Basel). 2023;13(5): 924.
- van Sambeeck SJ, Mavinkurve-Groothuis AM, Flucke U, Dors N. Sarcoma botryoides in an infant. BMJ Case Rep. 2014 Dec 17;2014:bcr2013202080.
- Hettmer S, Wagers AJ. Muscling in: Uncovering the Origins of Rhabdomyosarcoma. Nat. Med. 2010;16:171–173. doi: 10.1038/nm0210-171.
- Li RF, Gupta M, McCluggage WG, Ronnett BM. Embryonal Rhabdomyosarcoma (Botryoid Type) of the Uterine Corpus and Cervix in Adult Women: Report of a Case Series and Review of the Literature. Am. J. Surg. Pathol. 2013;37:344–355. doi: 10.1097/PAS.0b013e31826e0271.
- Maharaj NR, Nimako D, Hadley GP. Multimodal therapy for the initial management of genital embryonal rhabdomyosarcoma in childhood. Int J Gynecol Cancer. 2008 Jan-Feb;18(1):190-192.
Ayrıntılar
| Birincil Dil | İngilizce |
|---|---|
| Konular | Sağlık Kurumları Yönetimi |
| Bölüm | Olgu Sunumları |
| Yazarlar | |
| Erken Görünüm Tarihi | 28 Mart 2024 |
| Yayımlanma Tarihi | 28 Mart 2024 |
| Gönderilme Tarihi | 16 Aralık 2022 |
| Kabul Tarihi | 8 Eylül 2023 |
| Yayımlandığı Sayı | Yıl 2024 Cilt: 19 Sayı: 1 |
