Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon: Successful Treatment with Embolization and Vincristine in Two Newborns

doi:10.1016/j.jvir.2011.12.007

Journal of Vascular and Interventional Radiology

Volume 23, Issue 3, March 2012, Pages 417-422

https://doi.org/10.1016/j.jvir.2011.12.007 Get rights and content

Abstract

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor and has a high mortality in newborns when associated with Kasabach-Merritt syndrome (KMS). In two newborns with KHE and severe KMS refractory to medical treatment, emergency embolization led to clinical improvement in the acute neonatal setting by reducing tumor volume, increasing the platelet count, and improving other clotting parameters. Systemic vincristine treatment was added for further tumor control. Both patients remained symptom-free at long-term follow-up.

Section snippets

Case 1

A female fetus of 37 weeks' gestational age with a prenatal ultrasound diagnosis of a right lower limb vascular tumor was delivered by cesarean section. Apgar score was 9/10 at birth, and the neonate weighed 2,700 g. The patient was referred to our hospital on day 1 of life. On arrival, physical examination showed an extensive soft tissue tumor affecting the right side of the vulva, gluteus, thigh, and knee, with purpuric changes in the skin extending from the pelvis to the foot. The tumor was

Case 2

A female fetus of 39 weeks' gestational age with a prenatal ultrasound diagnosis of a right lower limb vascular tumor was delivered by cesarean section. Apgar score was 8/10 at birth, and the neonate weighed 3,300 g. A large (10-cm) soft tissue tumor located in the right thigh was noted at birth. The tumor gradually increased in size the following weeks. The concomitant appearance of hematuria and petechiae prompted referral to our service on day 26. After admission to our hospital, physical

Discussion

KHE is a rare, locally aggressive vascular tumor, commonly associated with KMS, a thrombocytopenic consumptive coagulopathy (1, 2). It is congenital in about 60% of cases, but it may also affect infants or children and, less frequently, young adults (3, 4). It is a serious clinical condition, with a reported mortality of 24% and even higher in patients < 6 months of age (4). Death most often results from expansive tumor growth and life-threatening hemorrhage (2, 4).

KHE usually manifests as an

Acknowledgment

The authors thank Dr. J. Mulliken and Dr. A. Alomari for their assistance in the preparation of this article and Dr. M. R. Cordisco for her clinical advice in patient management for case 1.

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A few contemporary reports have documented favorable experiences in using embolization as upfront therapy [49]. Embolization has been used in conjunction with medical treatments to decrease blood flow and reduce the size of large tumors [50,51]. Although tumors involute over time, either spontaneously or with treatment, complete resolution of KHE/TA is rarely seen.
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The separation of vascular anomalies into two distinct groups, vascular tumors and vascular malformations, was followed by the characterization that their respective coagulopathies were due to either a derangement of platelets or to a disequilibrium of the patient's coagulation/fibrinolytic process. This configuration of coagulopathies will be the foundation for this two-chapter review series. In the initial review, coagulopathies where thrombocytopenia is the main feature will be characterized, whereas the second review will focus on vascular malformations that have a coagulation disorder secondary to some degree of coagulation consumption and/or fibrinolytic pathway derangement.
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Kaposiform haemangioendothelioma (KHE) is a benign vascular tumour. While it is not associated with distant spread, it can be locally aggressive and associated with Kasabach-Merritt syndrome (KMS). There were only 2 prior reports of KHE isolated to the kidney, with both having been managed with neoadjuvant chemotherapy. We present a case report of renal KHE in a paediatric patient managed instead with upfront surgical resection, and discuss the close interdisciplinary diagnostic and management approach undertaken to facilitate this, and the considerations in the approach.
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In 17 patients, 36 treatment cycles were successfully performed. The platelet count for all patients increased to ≥ 100 × 10⁹/L for the first time at 6.0 days ± 3.5; the platelet level of 15 infants was maintained at levels > 150 × 10⁹/L at 57.5 days ± 16.5. Before treatment, two infants had a normal fibrinogen level (2.21 g/L and 2.34 g/L); the fibrinogen level in the other 15 infants was first found to be increased to ≥ 2.0 g/L at 7.0 days ± 3.4 and was stabilized at levels > 2.0 g/L at 55.9 days ± 13.8 after treatment. Complications were graded as major in four cases and as minor in 13 cases.
TAE plus vincristine can rapidly improve levels of platelets and fibrinogen, and it is an effective method for treatment of corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon in infants.
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Brief reportKaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon: Successful Treatment with Embolization and Vincristine in Two Newborns

Abstract

Section snippets

Case 1

Case 2

Discussion

Acknowledgment

Thrombocytopenic coagulopathy (Kasabach-Merritt phenomenon) is associated with kaposiform hemangioendothelioma and not with common infantile hemangioma

Plast Reconstr Surg

Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas

J Pediatr

Mediastinal and neck kaposiform hemangioendothelioma: report of three cases

Pediatr Dermatol

Case records of the Massachusetts General HospitalWeekly clinicopathological exercises. Case 13-2004. A newborn girl with a large cutaneous lesion, thrombocytopenia, and anemia

N Engl J Med

Successful multimodal therapy for kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: case report and review of the literature

Pediatr Hematol Oncol

Embolization therapy in the management of infantile hemangioma with Kasabach Merritt syndrome

Pediatr Radiol

Kasabach-Merritt syndrome treated by therapeutic embolization with polyvinyl alcohol

Am J Pediatr Hematol Oncol

Brief report
Kaposiform Hemangioendothelioma with Kasabach-Merritt Phenomenon: Successful Treatment with Embolization and Vincristine in Two Newborns