Kaposiform Hemangioendothelioma: A Study of 33 Cases Emphasizing Its Pathologic, Immunophenotypic, and Biologic Uniqueness From Juvenile Hemangioma

  title={Kaposiform Hemangioendothelioma: A Study of 33 Cases Emphasizing Its Pathologic, Immunophenotypic, and Biologic Uniqueness From Juvenile Hemangioma},
  author={Lisa L Lyons and Paula E. North and Fernand Mac-Moune Lai and Mark H. Stoler and Andrew L. Folpe and Sharon Whelan Weiss},
  journal={The American Journal of Surgical Pathology},
Kaposiform hemangioendothelioma (KH) is a rare tumor of childhood often associated with Kasabach-Merritt phenomenon (KMP) and occasionally lymphangiomatosis. Although generally considered distinct from other vascular neoplasms, its rarity has precluded a thorough study of its immunophenotypic profile and long-term behavior. Thirty-three cases of KH were reviewed and immunostained for alpha-smooth muscle actin, various endothelial markers (CD31, CD34, vWf, FLI1), a platelet marker (CD61), and… 

Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature

The clinicopathological features of 8 cases of KHE are described which will be helpful in making their diagnosis and knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

Original Article Clinicopathological features of Kaposiform hemangioendothelioma

The results suggest that KHE can occur in the embryonic period, and patients with KMP often have earlier onset time and larger lesional size.

Clinicopathological features of Kaposiform hemangioendothelioma.

The results suggest that KHE can occur in the embryonic period, and patients with KMP often have earlier onset time and larger lesional size.

Adult-Onset Kaposiform Hemangioendothelioma in a Posttraumatic Site

A rare patient is presented with adult-onset KHE arising in a posttraumatic location and treatment consists of drug therapy with corticosteroid, interferon, or vincristine.

Kaposiform hemangioendothelioma with distant lymphangiomatosis without an association to Kasabach-Merritt-Syndrome in a female adult!

The association of KHE in a female adult with lymphangioma rather than KMS in this case supports the hypothesis that such an association may represent a benign subform of this disease in an adult and excision seems to be curative.

Intracranial kaposiform hemangioendothelioma: proposal of a new malignant variant.

A case of a KHE without Kasabach-Merritt syndrome arising from the right tentorium cerebelli in a 13-month-old male child is reported, which may be not only the first intracranial extraaxial KHE, but also the first truly malignant variant.

Kaposiform hemangioendothelioma/Kasabach–Merritt syndrome. Сlinical and laboratory characteristics. Analysis of clinical cases

Despite the presence of clear clinical and laboratory characteristics of KMS, some cases require differential diagnosis with other vascular anomalies accompanied by thrombocytopenia and consumption coagulopathy – with congenital hemangiomas (rapidly involuting congenitalhemangioma), multifocal lymphangioendotheliomatosis, kaposiform lymphangiom atosis, venous malformations.

Kaposiform hemangioendothelioma in tonsil of a child associated with cervical lymphangioma: a rare case report

This case forms the second documented case of KHE occurring in tonsil of a child, associated with a neck swelling, but unassociated with KMP, along with its unique association with neck lymphangioma.



Kaposiform Hemangioendothelioma of Infancy and Childhood: An Aggressive Neoplasm Associated with Kasabach-Merritt Syndrome and Lymphangiomatosis

Nine distinctive, but relatively unknown, vascular tumors of infancy and childhood presenting as soft tissue masses often associated with locally aggressive disease, lymphangiomatosis and Kasabach-Merritt syndrome are reported, and treatment appears to consist of wide local excision and supportive therapy for associated symptoms.

Histopathology of Vascular Lesions Found in Kasabach-Merritt Syndrome: Review Based on 13 Cases

The observation that the underlying vascular lesion in KMS is usually not an infantile hemangioma as was originally thought, but variants of hemang iomas such as TA and KHE (77% of 13 KMS cases) is confirmed.

Kaposiform hemangioendothelioma in adults. Clinicopathologic and immunohistochemical analysis of three cases.

In adults, the differential diagnosis of KHE comprises especially Kaposi's sarcoma and spindle cell hemangioendothelioma; further differential diagnoses include tufted hemangioma and cellular capillary hemang ioma, which occur rarely in adults.

Kaposiform Hemangioendothelioma: Five Patients with Cutaneous Lesion and Long Follow-Up

This series indicates that kaposiform hemangioendothelioma is incapable of metastasis, despite a protracted course of many decades with no tendency for spontaneous regression.

Kaposiform hemangioendothelioma of the thymus.

The case of a 1-month-old infant who presented with stridor and was found to have a diffusely infiltrating tumor in the thymus that extended into the pericardium and up the carotid sheaths is reported.

Thrombocytopenic Coagulopathy (Kasabach‐Merritt Phenomenon) Is Associated with Kaposiform Hemangioendothelioma and Not with Common Infantile Hemangioma

Variable response to current pharmacologic therapy underscores inadequate knowledge of the pathogenesis of thrombocytopenia in KHE.

Kaposiform hemangioendothelioma. An aggressive, locally invasive vascular tumor that can mimic hemangioma of infancy.

Cutaneous kaposiform hemangioendothelioma may appear in early infancy but often appears months to years later, and demonstrates aggressive local behavior with invasion but not distant metastasis.

Infants with Kasabach-Merritt syndrome do not have "true" hemangiomas.

GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas.

Residual lesions after Kasabach-Merritt phenomenon in 41 patients.

Residua of tumors associated with Kasabach-Merritt phenomenon are common after the resolution of thrombocytopenia and coagulopathy and differ markedly from involuted hemangioma.