Introduction: Hemangiomas are the most common tumors of infancy. Hemangiomas are present at birth
in up to 2,5% and more than 10% in the caucasic population show hemangiomas in their
first year of life. Hemangiomas, benign vascular lesions usually show a tendency to
grow rapidly during the first 8–12 months of life, followed by an involution phase
that could take several years. Most hemangiomas do not require specific treatment
because of the high rate of spontaneous regression. However, a small number of hemangiomas
require therapy including hemangiomas endangering vision, oral intake and organ functions.
Diffuse neonatal hemangiomatosis (DNH) is a rare, frequently fatal disorder characterized
by multiple cutaneous and visceral hemangiomas. In most cases of DNH hemangiomas are
either present at birth or develop during the first week of life. In addition to the
cutaneous hemangiomas other possibly involved organs include the liver, then less
frequently pleura, lungs, intestines, central nervous system and eyes. A combined
manifestation of skin and liver without other organs being involved is found in most
patients. Children with DNH have a grave prognosis (mortality rate 50–95%). The main
reason is high-output cardiac failure due to high-flow liver hemangiomas. Other complications
could be liver failure including consumption coagulopathy, as well as cutaneous, gastrointestinal
and cerebral bleeding.
Many efforts have been made in treating DNH. Published treatment regimes include systemic
corticosteroids, subcutaneous interferon alpha 2a or 2b, liver radiation, partial
liver resection, hepatic artery embolization and the use of antiangiogenetic chemotherapeutic
agents like vincristine and cyclophosphamide. Cyclophosphamide was reported to offer
promising results in so far 9 published cases of children suffering from DNH.
Case reports: We present two cases of neonates with multiple cutaneous lesions together with high-flow
hemangiomas of the liver and in one patient additional pulmonary and cerebellar angiomas.
We prescribed cyclophosphamide 10mg/kg/day on 4 consecutive days after failure of
corticosteroids. Cyclophosphamide induced a fast regression of the lesions with no
side effects in both infants. We could stop medication after 3 respectively 4 courses
of cyclophosphamide in both infants because of the excellent response.
Discussion: Concerning the high mortality rate of DNH, especially when associated
with high-output cardiac failure, we feel that an early and aggressive treatment is
warranted. Comparing therapy duration, side effects and mortality rates of the different
above mentioned therapeutic options cyclophosphamide offers some unique advantages
including low acute toxicity, rapid effect and low costs.
Conclusion: In our opinion cyclophosphamide should be considered as a reasonable safe, quick-working
and short therapy in children with corticosteroid-unresponsive life-threatening diffuse
neonatal hemangiomatosis.
