Intracranial kaposiform hemangioendothelioma presenting as epistaxis: a rare case report with review of literature
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CASE REPORT
Intracranial kaposiform hemangioendothelioma presenting as epistaxis: a rare case report with review of literature Soutrik Das 1 & Harsh Deora 2 & Shilpa Rao 1
&
Sandeep Kandregula 2 & Suma Mysore Narayana 3
Received: 13 May 2020 / Accepted: 24 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Introduction Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignancy with tendency for local invasion and recurrence. The tumor almost exclusively occurs in children, especially in infants. Intracranial KHE are extremely rare with only two cases reported in the literature. Report We report the clinical and pathological features of this rare tumor arising from basitemporal region in a 21-month child. Our case did not present with Kasabach-Merritt phenomenon. Histopathological examination confirmed the diagnosis of KHE. Conclusion KHE should be considered in the differential diagnosis of intracranial extra-axial neoplasm in children, and histopathological examination plays an important role in distinguishing KHE from its morphologic mimics. It is essential to diagnose KHE due to its locally aggressive nature. Keywords Kaposiform hemangioendothelioma . Rare . Intracranial . Basitemporal . Histopathology
Introduction Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of intermediate malignancy, which was first described by Zukerberg et al in 1993 [1]. Though the name implies, it is never associated with Kaposi sarcoma or HHV8 infection. The tumor almost always affects children,
* Shilpa Rao [email protected] Soutrik Das [email protected] Harsh Deora [email protected] Sandeep Kandregula [email protected] Suma Mysore Narayana [email protected] 1
Departments of Neuropathology, National Institute of Mental Health and Neurosciences, Bangalore, India
2
Departments of Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, India
3
Department of Pathology, Kidwai Memorial Institute of Oncology, Bangalore, India
most commonly during infancy [2]. However, rare adult cases are also reported. Most cases arise in soft tissues of the limbs followed by retroperitoneum. There are only a few published cases of KHE in the head and neck region [3–6]. Intracranial KHE are extremely rare with only two cases reported in the literature. The first intracranial case of KHE was reported in a 6-month-old male infant [3]. The second case report was published 3 years later, the site of origin being tentorium cerebelli in a 13-month-old boy [7]. KHE is often associated with the Kasabach-Merritt phenomenon (KMP) which is characterized by thrombocytopenia and consumptive coagulopathy due to localized intravascular coagulation [8]. Pathologically, the tumor has features mimicking both capillary hemangioma and Kaposi sarcoma. We report the clinical and pathological features of this rare tumor in a 21-month child.
Case report A 21-month-old male child presented with a history of epistaxis from
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