Neonatal Med.  2020 Feb;27(1):37-43. 10.5385/nm.2020.27.1.37.

Successful Management of Visceral Kaposiform Hemangioendothelioma with Kasabach‐Merritt Phenomenon Using Corticosteroids and Vincristine

• Gang MH
• Lim YJ
• Chang My

• KMID: 2502092
• DOI: http://doi.org/10.5385/nm.2020.27.1.37

Abstract

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm that often develops a coagulopathy known as Kasabach­Merritt phenomenon (KMP). Visceral involvement denotes a poor prognosis. We report a case of visceral KHE with KMP successfully treated with corticosteroids and vincristine. The infant had been born vaginally at 34 weeks 6 days’ gestation, weighing 2,360 g. He was admi tted for the management of respiratory failure. Blood tests showed anemia and thrombocytopenia 1 hour after delivery. Additional blood tests revealed a prothrombin time of 12.1 seconds, activated partial thromboplastin time of 60.6 seconds, fibrinogen levels of 72.4 mg/dL, and D­dimer levels >3,200 ng/mL. Despite supportive measures and daily transfusions, the clinical condition and coagulopathy gradually worsened. Renal ultrasonography performed to find the origin of the coagulopathy revealed an echogenic mass measuring >3 cm in the abdominal cavity. A magnetic resonance imaging scan showed an ill­marginated, infiltrative mass like lesion in the right anteromedial and posteromedial perirenal space that was hypointense with mild enhance ment on T1­ and T2­weighted images. Large vascular tumors adherent to other visceral organs were noted during exploratory laparotomy but could not be resected. Treatment with methylprednisolone was ineffective. Vincristine was administered weekly from the 17th hospital day, and the coagulation profile showed gradual impro vement after its initiation. Intravenous methylprednisolone was switched to oral prednisolone on the 57th hospital day. He was discharged on the 73rd hospital day and continued vincristine treatment every 2 weeks and oral prednisolone administration as an outpatient treatment for 8 weeks. He remained symptom­free at the 39­month follow­up.

Keyword

Hemangioendothelioma; Kasabach­Merritt syndrome; Vincristine; Systemic corticosteroid; Infant, Newborn

Figure

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  Figure 1. Hematologic laboratory values during the hospital course. D-dimer levels >3,200 ng/mL are indicated as 32×102 ng/mL in the graph.

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  Figure 2. Imaging studies of the patient. (A) Renal ultrasonography performed on the 6th day of life showing diffusely echogenic soft tissue surrounding the inferior vena cava, aorta, and its branches. (B) Renal ultrasonography performed on the 6th day of life showing diffusely increased parenchymal echogenicity of the right kidney with hydronephrosis. (C) Abdominopelvic computed tomography performed on the 7th day of life showing diffuse, ill-marginated enhancing lesion surrounding the right renal hilum and right inferomedial aspects of the perirenal space (indicated by arrows). (D) Abdominal magnetic resonance imaging (MRI) scan showing diffuse, ill-marginated infiltrative mass like lesion on the right anteromedial and posteromedial aspects of the perirenal space. (E) Abdominal MRI scan showing asymmetric thickening of right crura. (F) Abdominal MRI scan showing right hydronephrosis and hemorrhage/hematoma inside the right kidney. (G) Abdominal MRI scan showing the mesentery with diffuse high signal intensity on a T1-weighted image (indicated by arrowheads).

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  Figure 3. Follow-up magnetic resonance imaging performed at 39 months of age. (A, B) Unremarkable finding in the right perirenal space and the upper abdomen except for mild right hydronephrosis.

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