Very rare mediastinal location of Kaposiform haemangioendothelioma: A case report and a brief review of the previously published cases

Slaviša Đuričić,Dragomir Đokić,Adrijan Sarajlija,Radoje Simić

doi:10.5937/scriptamed51-25350

Abstract

Kaposiform haemangioendothelioma (KHE) is a rare, locally invasive vascular tumour that is commonly associated with the Kasabach-Merritt phenomenon (KMP). A case of a five-month-old female infant admitted for dyspnoea, stridor, and skin haematoma is presented. Computerised tomography of the chest showed a tumour mass occupying mediastinum and most of the left hemithorax, while laboratory analysis revealed a thrombocytopaenia and a consumption coagulopathy. Histology of tumour biopsy was characteristic of KHE with a component of tufted angioma. Corticosteroid treatment initially induced a reduction in tumour size, but progression occurred four weeks later and led to a fatal outcome despite additional chemotherapy. After a literature search, we found only 18 cases of mediastinal KHE published so far, with 21 % fatality rate. In the present case several risk factors for adverse outcome were present: onset of disease in early infancy, a large volume of the tumour, mediastinal location, KMP, and partial response to available therapy.

Highlights

Childhood tumours represent just over 1 % of all human tumours
Kaposiform haemangioendothelioma (KHE) is a rare, locally invasive vascular tumour that is commonly associated with the Kasabach-Merritt phenomenon (KMP)
In addition to the striking differences in clinical presentation, each of these tumours is different from KHE in the following histologic details: (1) spindle cell haemangioma has a biphasic composition with cavernous vascular spaces and more solid spindle-cell areas containing cytoplasmic vacuoles; (2) in nodular phase of Kaposi sarcoma there is no lobular arrangement of the spindle cells, which are invariably HHV-8 immunopositive; (3) spindle cell variant of angiosarcoma is characterised by an infiltrative growth and multilayering of more atypical endothelial cells at the periphery of the lesion.[7, 8, 15]

Summary

Introduction

Childhood tumours represent just over 1 % of all human tumours. They are different from tumours in adolescents and adults regarding histological types, biology, incidence, clinical features, prognosis, and response to treatment. Some tumour nodules bulged into the outer crescent-shaped thin-walled vascular spaces, which is a pattern corresponding to the tufted angioma (TA) structures These tumour components were associated with malformed, thin-walled, lymphatic vessels corresponding to the microcystic lymphatic malformation (Figure 1C). The tumour nodules were densely cellular with plump, spindled cells, arranged in haphazardly interlaced, sometimes tightly coiled, short fascicles interspersed with tiny capillaries and lining slit-like vascular channels filled with erythrocytes (Figure 1D). Immunohistochemical stains showed a diffuse CD31, CD34, and FLI-1 immunopositivity of tumour cells in the well-canalised and spindled areas (Figure 2A) These cells were GLUT-1 and HHV8 immunonegative, but they expressed podoplanin (D2-40), mainly at the periphery of the nodules (Figure 2B). After establishing the diagnosis of KHE, intravenous corticosteroid therapy was instituted (initial pulse dose of 100 mg methylprednisolone, followed by 5 mg/kg/day).

Findings

Discussion

Conclusion