Infantile hepatic hemangioendothelioma: Clinical presentation and treatment
Yıl: 2007 Cilt: 18 Sayı: 3 Sayfa Aralığı: 182 - 187 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022
Infantile hepatic hemangioendothelioma: Clinical presentation and treatment
Öz: Amaç: Karaciğer hemanjioendotelyoması çocukluk çağının nadir tümörlerindendir.Bu çalışmada üçüncü basamak sağlık hizmeti veren merkezimizde 10 yıllık deneyimimiz sunuldu. Yöntem: Çalışmada infantil karaciğer endotelyoması olan sekiz hastanın retrospektif analizi yapıldı. Bulgular: Tanıda median yaş 24 gün (yaş sınırları: 1-70gün) ve kız/erkek oranı 5/3 bulundu. Ana yakınma karın şişliği ve solunum sıkıntısı idi. Cilt hemanjiomları dört hastada mevcuttu. Üç olguda Kasabach- Merritt sendromu saptandı. Dört hastada tek, diğerlerinde birden fazla karaciğer lezyonu vardı. Tümor çapı 2 cm ile 10 cm arasında değişiyordu. Lezyonlar sağ ve sol karaciğer lobunda eşit dağılmış olup, üç bebekte her iki lobda tutulum vardı. Multifokal karaciğer tümörlü olguların çoğunda cilt hemanjiomları saptandı. Tedavi seçenekleri bireysel olarak değerlendirildi. Sistemik prednizolon (2 mg/kg/gün) altı hastaya verildi. Beş hastada kortikosteroidlere yanıt alındı. Kasabach-Merritt sendromlu bir erkek bebekte tedaviye yanıt yoktu. ‹nterferon– alfa (1 milyon ünite/m2/gün) başlanıp klinik düzelme olana kadar doz arttırımı ile 10 MU/ m2 /gün , haftada 3 kez uygulandı. ‹yi yanıt alındı, önemli yan etki gözlenmedi. ‹ki hasta ameliyat oldu. Biri ameliyatta kanama ile öldü. Diğer hastalar 11-66 aylık izlem süresinde iyi olup, serimizde sağkalım oranı % 87 bulundu. Sonuç: Tedavi yaklaşımı merkezlerin deneyimine bağlıdır. En iyi tedavi seçeneği multidispliner yaklaşımla belirlenmelidir
Anahtar Kelime: Konular:
İnfantil karaciğer hemanjioendotelyoması: Klinik ve tedavi
Öz: Background/aims: Hepatic hemangioendotheliomas are rare tumors in childhood. We report our 10-years’ experience in a tertiary health center. Methods: This retrospective analysis included eight patients with infantile hepatic hemangioendothelioma. Results: The median age at diagnosis was 24 days (age range: 1 to 70 days) and the female/male ratio was 5/3. The main symptoms were abdominal distention and respiratory distress. Cutaneous hemangiomas were present in four cases. Three infants had Kasabach-Merritt syndrome. Four cases had single hepatic tumors while the others had multiple. The tumor size ranged from 2 cm to 10 cm in diameter. These lesions were located equally in the right and left hepatic lobes, and three babies had bilobar involvement. Most of the multifocal hepatic tumors were associated with skin hemangiomas. Treatment options were assessed individually. Systemic prednisolone therapy (2 mg/kg/d) was commenced in six patients. Five patients responded to corticosteroids. One boy with Kasabach-Merritt syndrome did not respond to this therapy. Interferon–alpha (1 million units (MU)/m2/day) was started, and the daily dose of the drug was increased up to 10 MU/m2, administered 3 times per week, until clinical improvement was achieved. The response was very good and we observed only constitutional adverse symptoms. Two cases were operated; one died from intraoperative bleeding. Other patients were alive and well for 11 to 66 months. Overall survival was 87% in our series. Conclusions: The treatment approaches depend on the center’s experience. A multidisciplinary approach is required for the best treatment option.
Anahtar Kelime: Konular:
Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
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APA | SEVİNİR N, ÖZKAN T (2007). Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. , 182 - 187. |
Chicago | SEVİNİR Netül,ÖZKAN Tanju B. Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. (2007): 182 - 187. |
MLA | SEVİNİR Netül,ÖZKAN Tanju B. Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. , 2007, ss.182 - 187. |
AMA | SEVİNİR N,ÖZKAN T Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. . 2007; 182 - 187. |
Vancouver | SEVİNİR N,ÖZKAN T Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. . 2007; 182 - 187. |
IEEE | SEVİNİR N,ÖZKAN T "Infantile hepatic hemangioendothelioma: Clinical presentation and treatment." , ss.182 - 187, 2007. |
ISNAD | SEVİNİR, Netül - ÖZKAN, Tanju B.. "Infantile hepatic hemangioendothelioma: Clinical presentation and treatment". (2007), 182-187. |
APA | SEVİNİR N, ÖZKAN T (2007). Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. Turkish Journal of Gastroenterology, 18(3), 182 - 187. |
Chicago | SEVİNİR Netül,ÖZKAN Tanju B. Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. Turkish Journal of Gastroenterology 18, no.3 (2007): 182 - 187. |
MLA | SEVİNİR Netül,ÖZKAN Tanju B. Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. Turkish Journal of Gastroenterology, vol.18, no.3, 2007, ss.182 - 187. |
AMA | SEVİNİR N,ÖZKAN T Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. Turkish Journal of Gastroenterology. 2007; 18(3): 182 - 187. |
Vancouver | SEVİNİR N,ÖZKAN T Infantile hepatic hemangioendothelioma: Clinical presentation and treatment. Turkish Journal of Gastroenterology. 2007; 18(3): 182 - 187. |
IEEE | SEVİNİR N,ÖZKAN T "Infantile hepatic hemangioendothelioma: Clinical presentation and treatment." Turkish Journal of Gastroenterology, 18, ss.182 - 187, 2007. |
ISNAD | SEVİNİR, Netül - ÖZKAN, Tanju B.. "Infantile hepatic hemangioendothelioma: Clinical presentation and treatment". Turkish Journal of Gastroenterology 18/3 (2007), 182-187. |