Kaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals

doi:10.1016/j.jpeds.2012.06.044

The Journal of Pediatrics

Volume 162, Issue 1, January 2013, Pages 142-147

https://doi.org/10.1016/j.jpeds.2012.06.044 Get rights and content

Objective

To examine the presentation characteristics of patients with Kaposiform hemangioendothelioma (KHE) to describe the spectrum of disease and risk factors for Kasabach-Merritt phenomenon (KMP).

Study design

A retrospective review of 163 patients referred to the Vascular Anomalies Center at Children's Hospital Boston for KHE between 1991 and 2009 identified 107 patients with sufficient data for inclusion.

Results

The prevalence of KHE in Massachusetts is ∼0.91 case per 100 000 children. KHE manifested in infancy in 93% of cases, with 60% as neonates. Common presenting features included enlarging cutaneous lesion (75%), thrombocytopenia (56%), and musculoskeletal pain or decreased function (23%). Cutaneous KHE favored the extremities, especially overlying joints. In our cohort, 71% developed KMP (11% after initial presentation), and 11% of patients lacked cutaneous findings. Retroperitoneal and intrathoracic lesions, though less common, were complicated by KMP in 85% and 100% of cases, respectively. Compared with superficial lesions, KHE infiltrating into muscle or deeper was 6.3-fold more likely to manifest KMP and 18-fold higher if retroperitoneal or intrathoracic. KHE limited to bone or presenting after infancy did not manifest KMP.

Conclusion

An enlarging cutaneous lesion is the most common presenting feature of KHE in infancy. Older patients with KHE or those lacking cutaneous manifestations present with musculoskeletal complaints or atypical symptoms. The risk of KMP increases dramatically when tumor infiltrates muscle or when KHE arises in the retroperitoneum or mediastinum.

Section snippets

Methods

We reviewed the medical records and database of the Vascular Anomalies Center at Children's Hospital Boston from 1991 to 2009 using the search terms “Kaposiform hemangioendothelioma,” “KHE,” “Kasabach-Merritt phenomenon,” “KMP,” “Kasabach-Merritt syndrome,” and “KMS” to define a cohort of patients with probable KHE. Our institutional review board approved this retrospective review. Our interdisciplinary team reviewed all cases and reached consensus on the diagnosis of KHE based on review of

Epidemiology

This KHE cohort represented referrals from 30 states and 15 countries. We assume that our center was involved in the vast majority of cases from Massachusetts. Given the estimated 1.4 million children <18 years old in Massachusetts in 2009¹³ and 13 Massachusetts children with KHE in the same year, we estimate the prevalence of KHE as 0.91 case per 100 000 children. Over the past decade, there has been about one new case of KHE diagnosed in Massachusetts per year, yielding an incidence of 0.071

Discussion

KHE involves a spectrum of lesions from small, superficial tumors without KMP to large, infiltrative lesions with life-threatening complications including KMP. We have characterized the largest cohort of patients with KHE to date. Most patients have KMP and present in infancy with classic cutaneous lesions. Patients without cutaneous lesions present with atypical signs and symptoms and tend to be older. Numerous anatomic locations have been reported: cervicofacial region (sinuses,14, 15

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Based on the rarity and significant heterogeneity of KHE patients with bone destruction, we should give full play to the role of multidisciplinary teams in addressing disease to reduce the long-term complications of KHE with bone destruction and improve the quality of life of patients.
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: Supported by a Lovejoy Resident Research and Education Award and an American Society of Hematology Trainee Research grant (to S.C.) and by the National Institutes of Health/National Heart, Lung, and Blood Institute (K08 HL089509 to C.T.). The authors declare no conflicts of interest.

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Original ArticleKaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals

Objective

Study design

Results

Conclusion

Section snippets

Methods

Epidemiology

Discussion

J Pediatr Surg

Mod Pathol

Mod Pathol

J Pediatr

J Pediatr Surg

Kaohsiung J Med Sci

Auris Nasus Larynx

J Am Acad Dermatol

Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis

Am J Surg Pathol

Residual lesions after Kasabach-Merritt phenomenon in 41 patients

J Am Acad Dermatol

Multifocal kaposiform haemangioendothelioma

Virchows Arch

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

Plast Reconstr Surg

Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma

Am J Surg Pathol

Original Article
Kaposiform Hemangioendothelioma: Atypical Features and Risks of Kasabach-Merritt Phenomenon in 107 Referrals