Kaposiform hemangioendothelioma: A locally aggressive vascular tumor

doi:10.1016/S0190-9622(98)70461-X

Journal of the American Academy of Dermatology

Volume 38, Issue 5, Supplement, May 1998, Pages 799-802

https://doi.org/10.1016/S0190-9622(98)70461-X Get rights and content

Abstract

Kaposiform hemangioendothelioma is a locally aggressive, endothelial-derived spindle cell neoplasm that occurs exclusively in infants and adolescents. Lesions are characterized by rapid growth and extension, and are often associated with Kasabach-Merritt syndrome and lymphangiomatosis. Clinically nonspecific, they can appear as tender rapidly expanding red plaques, nodules, grouped papules, or telangiectasias. The histology is distinctive, however, as it combines features of tufted angioma, progressive lymphangioma, and Kaposi's sarcoma in a characteristic pattern. We describe a patient with kaposiform hemangioendothelioma currently controlled with systemic prednisone. (J Am Acad Dermatol 1998;38:799-802.)

Section snippets

CASE REPORT

A 14-year-old white boy had a tender telangiectatic plaque on his left arm that had rapidly enlarged during the past 8 months. A telangiectatic 1 × 2 cm macule was present since infancy and clinically diagnosed at 1 year of age as a hemangioma. He was otherwise healthy with no family history of similar lesions.

Examination revealed a partially blanchable, poorly defined erythematous plaque extending from the left axilla to elbow (Fig. 1).

. Kaposiform hemangioendothelioma involving left arm and

DISCUSSION

Kaposiform hemangioendothelioma is a distinct, rare vascular tumor that occurs almost exclusively in children. Only 14 cases have being previously reported,1, 2, 4, 5, 6, 7, 8 seven by Zukerberg et al.⁶ The oldest patient was a 19-year-old girl.⁵ These neoplasms demonstrate locally aggressive behavior, rapidly invading surrounding tissues and structures. All but two cases extended beyond the subcutis,⁶ involving deep tissues and even bone.⁸ All kaposiform hemangioendotheliomas were

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Kaposiform hemangioendothelioma (KHE) is a rare, locally invasive vascular tumour of childhood that may occur in soft tissue or bones, and is associated with cutaneous plaques and Kasabach-Merritt phenomenon (KMP). We present an instance of a 9-year-old girl with primary vertebral involvement of KHE, whose clinical presentation was with painless, progressive scoliosis alone, sans cutaneous markers. We highlight the imaging features of this rare manifestation and importance of histopathological diagnosis for optimal management.
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Non-hematopoietic tumors of the skin and superficial soft tissues of the head and neck comprise a large group of lesions, both benign and malignant. They include surface-epithelial and adnexal-epithelial neoplasms, neuroendocrine tumors, mesenchymal proliferations, and melanocytic neoplasms. This chapter synopsizes the clinicopathologic features of those lesions, providing information on their pathologic differential diagnosis.
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Large lesions of subcutaneous tissues, mediastinum, and retroperitoneum cannot be completely excised and may cause fatal complications due to the associated Kasabach–Merritt syndrome.131 In these cases, several medical therapies have been assayed, including systemic corticosteroids132; interferon alfa-2a108,109; combined treatments with systemic corticosteroids, alfa interferon, vincristine, and radiotherapy110; embolization, systemic interferon, cyclophosphamide, epsilon aminocaproic acid, and compression therapy111; combined chemotherapy with vincristine, cyclophosphamide, and actinomycin-D113; vincristine and ticlopidine133; only vincristine134; and combined with sirolimus.135 Small lesions and those with superficial location are better managed with surgical excision.
Hemangioendothelioma is the term used to name those vascular neoplasms that show a borderline biological behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Although originally spindle cell hemangioendothelioma was proposed as a specific clinicopathologic variant of hemangioendothelioma, currently, it is considered as an entirely benign lesion, and thus, the name spindle cell hemangioma seems to be the most accurate for this lesion. Authentic hemangioendotheliomas involving the skin and soft tissues include papillary intralymphatic angioendothelioma (also known as Dabska tumor), retiform hemangioendothelioma, kaposiform hemangioendothelioma, epithelioid hemangioendothelioma, pseudomyogenic hemangioendothelioma (also known as epithelioid sarcoma-like hemangioendothelioma), and composite hemangioendothelioma. Each of these neoplasms exhibit characteristic histopathologic features. The most characteristic finding of papillary intralymphatic hemangioendothelioma consists of papillary tufts, with a central hyaline core lined by hobnail-like endothelial cells protruding into the lumina. Retiform hemangioendothelioma is an infiltrative neoplasm composed of elongated arborizing vessels, arranged in an anastomosing pattern that resembles that of the rete testis, and lined by a single layer of hobnail-like endothelial cells that protrude within the narrow lumina. Kaposiform hemangioendothelioma is composed of several solid poorly circumscribed nodules, and each nodule is composed of a mixture of small capillaries and solid lobules of endothelial cells arranged in a glomeruloid pattern. A frequent finding consists of the presence of areas of lymphangiomatosis adjacent to the solid nodules. Epithelioid hemangioendothelioma is composed of cords, strands, and solid aggregates of round, oval, and polygonal cells, with abundant pale eosinophilic cytoplasm, vesicular nuclei, and inconspicuous nucleoli, embedded in a fibromyxoid or sclerotic stroma. Many neoplastic cells exhibit prominent cytoplasmic vacuolization as an expression of primitive vascular differentiation. Pseudomyogenic hemangioendothelioma is a poorly circumscribed, fascicular lesion with infiltrative borders composed of round or oval neoplastic cells, with vesicular nuclei and inconspicuous nucleoli, and ample homogeneous eosinophilic cytoplasm, giving them a rhabdomyoblastic appearance. Finally, composite hemangioendothelioma is the term used to name locally aggressive vascular neoplasms of low-grade malignancy showing varying combinations of benign, low-grade malignant, and high-grade malignant vascular components. From the immunohistochemical point of view, proliferating cells of all hemangioendotheliomas express a lymphatic endothelial cell immunophenotype. Most hemangioendotheliomas are low-grade vascular neoplasms, with a tendency to recur locally and a low metastatic potential, mostly to regional lymph nodes. Epithelioid hemangioendothelioma, especially large lesions and those located in deep soft tissues, seems to have a more aggressive biological behavior.
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Several therapies have been reported for KHE/TA, but none have been uniformly effective. Although surgical removal of the tumor has been associated with immediate normalization of hemostasis and hematologic abnormalities,20 it is rarely attempted because of the large size and infiltrating nature of KHE/TA associated with KMP. Tumor embolization has been used with some success in combination with medical and surgical therapies.8

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Kaposiform hemangioendothelioma: A locally aggressive vascular tumor☆,☆☆,★

Abstract

Section snippets

CASE REPORT

DISCUSSION

J Am Acad Dermatol

Hemangioma with Kaposi's sarcoma-like features: report of two cases

Pediatr Pathol

Kaposi-like infantile hemangioendothelioma

Am J Surg Pathol

Capillary hemangioma with extensive purpura: report of a case

Am J Dis Child

Congenital retroperitoneal hemangioendothelioma with Kasabach-Merritt syndrome

South Med J

Cyclophosphamide and radiation therapy in the treatment of hemangioendothelioma with disseminated intravascular clotting

Cancer

Kaposiform hemangioendothelioma of infancy and childhood

Am J Surg Pathol

Hemangioendothe-lioma with intravascular coagulation and ischemic colitis

Cancer