Abstract

Congenital hemangiomas are rare benign vascular tumors that develop in utero and are fully formed by the time of birth. Depending on the ability to involution, there are three subtypes: RICH (repidly involuting congenital hemangioma), NICH (non involuting congenital hemangioma), PICH (partially involuting congenital hemangioma). PICH may be accompanied by thrombocytopenia and consumption coagulopathy. Despite clearly defined clinical and histological characteristics, it can be difficult to make a differential diagnosis between congenital hemangiomas and other vascular tumors (infantile hemangioma, kaposiform hemangioendothelioma/“fascicular” angioma and others). The clinical case in the article of a vascular tumor in a newborn complicated by thrombocytopenia and consumption coagulopathy was regarded as Kazabach-Merritt syndrome, which is based on kaposiform hemangioendothelioma/“fascicular” angioma. Rapid regression of the tumor and recovery of hemogram and coagulogram parameters, as well as anamnesis of the disease and initial characteristics of the tumor forced to reconsider the diagnosis. Based on the histological picture, the diagnosis of congenital hemangioma, RICH, was confirmed. Verification of the diagnosis made it possible to change therapeutic tactics and avoid chemotherapy. A giant hemangioma, accompanied by thrombocytopenia and consumption coagulopathy, may have a very favorable outcome – a complete resolution of the pathological process inherent in its natural course. The patient's parents agreed to use the information, including the child's photo, in scientific research and publications.

Highlights

  • Врожденные гемангиомы – это редкие доброкачественные сосудистые опухоли, развивающиеся внутриутробно и полностью сформированные к моменту рождения

  • Congenital hemangiomas are rare benign vascular tumors that develop in utero and are fully formed by the time of birth

  • PICH may be accompanied by thrombocytopenia and consumption coagulopathy

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Summary

Repidly involuting congenital hemangioma

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Healthcare of Russian Federation, Moscow. PICH may be accompanied by thrombocytopenia and consumption coagulopathy. Despite clearly defined clinical and histological characteristics, it can be difficult to make a differential diagnosis between congenital hemangiomas and other vascular tumors (infantile hemangioma, kaposiform hemangioendothelioma/“fascicular” angioma and others). The clinical case in the article of a vascular tumor in a newborn complicated by thrombocytopenia and consumption coagulopathy was regarded as Kazabach-Merritt syndrome, which is based on kaposiform hemangioendothelioma/“fascicular” angioma. Based on the histological picture, the diagnosis of congenital hemangioma, RICH, was confirmed. A giant hemangioma, accompanied by thrombocytopenia and consumption coagulopathy, may have a very favorable outcome – a complete resolution of the pathological process inherent in its natural course. Khachatryan L.A., et al Pediatric Hematology/Oncology and Immunopathology. Она основывалась на биологии и пролиферативном потенциале сосудистых аномалий [2]

КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ
Arteriovenous malformation
КЛИНИЧЕСКИЙ СЛУЧАЙ
ИГ Infantile hemangioma
СКМ Risk of SCM
Характеристика Characteristics
Капошиформный лимфангиоматоз Kaposiform lymphangiomatosis
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