Outcome of radiation therapy for patients with Kasabach-Merritt syndrome

Giant cavernous haemangioma with Kasabach-Merritt syndrome: a case report and review

“…Recent non-pharmacological treatment options include contact cryosurgery [22], laser treatment [28] for small and superficial lesions, embolisation [13], surgical excision [12,26] and radiotherapy [18]. Surgical intervention remains controversial.…”

“…Large, unresectable haemangiomas of infancy with a deep component and KMS, which did not benefit from pharmacological treatment, have shown dramatic response to radiation therapy [18,23]. In treating patients with KMS, radiotherapy combined with corticosteroids has been shown to be effective on long-term follow-up [17].…”

“…Because of the risk of growth delay, scarring and secondary malignancy [11], the efficiency of radiation therapy in KMS is somewhat contraversial. To minimise those side-effects, Mitsuhashi et al [18] recommended a maximum total dose of 8-10 Gy for initiating a reduction of the vascular tumour mass and recovery from haematological disorders. Thus the treatment of giant haemangiomas, if required, should be adapted to their individual location and extension.…”

Kasabach–Merritt Syndrome

“…The total radiation doses were 8±10 Gy. 27 Although all patients survived with no hematological abnormalities nor evidence of hemangioma on followup, limb-shortening was noted in three patients. 27 The known longterm eects of radiation and its failure to reliably correct the DIC in other reports make this treatment questionable.…”

Kasabach-Merritt-Syndrom bei Riesenhämangiom der Leber Ein Fallbericht

“…Die überwiegende Anzahl der bisher beschriebenen Fälle fand sich bei Kindern und wurde ausgelöst durch Krankheiten wie Klippel-Trénaunay-Weber-Syndrom [2,13], kaposiformes Hämangioendothe-liom [1,14], diffuse Hämangiomatose [15] oder multifokale kongenitale Häm-angioperizytome [3], aber nicht durch normale juvenile Hämangiome [4,14]. Die Empfehlungen zur Behandlung richten sich nach der Grunderkrankung und beinhalten Bestrahlung [10], Äthanolembolisierung [17,21], Interferontherapie [11] oder eine Behandlung mit hochdosiert Methylprednisolon [12].…”