Update on Pediatric Vascular Tumors

Transcription

1 Update on Pediatric Vascular Tumors Jim Treat, MD Associate Professor of Clinical Pediatrics and Dermatology No Relevant Conflicts of Interest

2 PHOTOGRAPHY & VIDEOTAPING ARE STRICTLY PROHIBITED IN ALL EDUCATIONAL SESSIONS CELL PHONES MUST BE PLACED ON VIBRATE OR TURNED OFF Violations of this policy will result in removal from the session and possible revocation of meeting registration. Session directors will be closely monitoring such occurrences. FOTOGRAFIA E FILMANDO SÃO ESTRITAMENTE PROIBIDOS EM TODAS AS SESSÕES EDUCACIONAIS TELEFONES CELULARES DEVEM SER COLOCADOS EM VIBRAR OU DESLIGADOS Violações desta política resultará na remoção de sessão e possível revogação do registo da reunião. Diretores de sessão irão acompanhar de perto tais ocorrências.

3 DISCLOSURE OF RELATIONSHIPS WITH INDUSTRY JAMES TREAT, MD UPDATE ON VASCULAR TUMORS DISCLOSURES NONE

4 ISSVA classification for vascular anomalies (Approved at the 20th ISSVA Workshop, Melbourne, April 2014) Overview table Vascular anomalies Vascular tumors Vascular malformations Simple Combined of major named vessels associated with other anomalies Benign Capillary malformations CVM, CLM See details See list Locally aggressive or borderline Malignant Lymphatic malformations Venous malformations Arteriovenous malformations* Arteriovenous fistula* LVM, CLVM CAVM* CLAVM* others

5 ISSVA classification of vascular tumors Benign vascular tumors Infantile hemangioma / Hemangioma of infancy see details Congenital hemangioma Rapidly involuting (RICH) * Non-involuting (NICH) Tufted angioma * Partially involuting (PICH) Spindle-cell hemangioma Epithelioid hemangioma Pyogenic granuloma (aka lobular capillary hemangioma) Others Locally aggressive or borderline vascular tumors Kaposiform hemangioendothelioma * Retiform hemangioendothelioma Papillary intralymphatic angioendothelioma (PILA), Dabska tumor Composite hemangioendothelioma Kaposi sarcoma Others Malignant vascular tumors Angiosarcoma Epithelioid hemangioendothelioma Others es/file/classifications 2014 Final.pdf

6 Present at birth but already with significant involution Atrophy, halo, often dilated veins Further regression after birth No known therapy

7 Infantile Fibrosarcoma Soft tissue malignant tumor Mimics other vascular tumors Clinically: FIRM Growth does not respect tissue planes

8 Appearance Typically not present at birth 4.5% of all children have a hemangioma Usually begin as a feint red patch or bruise at age 1 4 weeks Rapid growth phase Grow rapidly (faster than the child) between months 1 4 More Specifically weeks Most done growing by 4 months Slow growth phase Grow can continue until 12 months Involution Can start after 1 year of age but can take up to 9 years (age 10)

9 EKG Prior to Propranolol? MacArthur et al: 180 patients 43% with abnormal EKG ALL were allowed to be treated with Propranolol Castelo Soccio et al: 202 patients initiated on propranolol 4 with PHACE excluded 49 had abnormal EKG ALL were allowed to be treated with Propranolol Breuer et al: 109 patients 6.5% with abnormal EKG ALL were allowed to be treated with Propranolol ( J Am Acad Dermatol 2015;72: ) (Pediatric Dermatology Vol. 33 No , 2016)

10 Conclusions: Drops Gel Forming Solution are closer to the correct dose although more variably doses The Gel Forming Solution is used by most practitioners Conclusions: 38% had detectable blood levels and Those with higher doses had higher levels BUT no difference in response rate Blood levels found in 44% of scalp hemangiomas but 0% of face hemangiomas 33% on limbs, 22% trunk British Journal of Dermatology (2018) 178, ppe51 e52

11 Liver Hemangiomas Clinically 3 categories based on Fishman Isolated congenital lesion: (RICH) Multifocal lesions: Typically infantile hemangiomas GLUT 1 positive Diffuse Lesions Response to Propranolol has been excellent as expected Christison Lagay ER, Burrows PE, Alomari A, Dubois J, Kozakewich HP, Lane TS, Paltiel HJ, Klement G, Mulliken JB, Fishman SJ. Hepatic hemangiomas: subtype classification and development of a clinical practice algorithm and registry. Journal of pediatric surgery Jan 1;42(1):62 8.

12 Liver Hemangiomas: What s New? Hepatic hemangiomas risk factors for mortality (123 patients) Diffuse Pattern CHF Cardiomegaly was not statistically Associated Journal of pediatric surgery May 1;50(5):

13 Lesions Biopsied and proven GLUT 1 pos hemangiomas Regressed with propranolol Then resurged due to KRAS mutation Clinical point: Consider following hepatic Lesions should be followed to resolution

14 What to do about infants not able to eat? Buccal administration can lead to therapeutic serum levels Clark AB, Chandrasekar I, Nickleson J. Novel Route of Sirolimus Administration in a Neonate. Journal of pharmacy practice. 2017

15 Conclusions Many different pediatric vascular tumors can simulate infantile hemangioma New and emerging therapies show safety and new ways of administration Thank you!!