Adult Kaposiform Hemangioendothelioma Of The Leg Complicated By Kasabakh Meritt Phenomenon: Case Report (original) (raw)
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Adult Kaposiform Hemangioendothelioma of the Leg Complicated by Kasabakh Meritt Phenomenon
International Journal of Advanced Research
Kaposiform hemangioendothelioma is a rare, locally aggressive vascular neoplasm that mainly occurs during childhood. the tumor is often located on the skin but may be deeper. The development of KHE in adults is very rare, and its evolution towards KMP is exceptional. The KMP is defined as the combination of a deep thrombocytopenia and a vascular tumor, usually a KHE or a tuftted angioma. It is a life threatening pathology, because of the major risk of bleeding, and compression of vital structure. A multidisciplinary approach to treatment has to be employed.
Diagnostic Pathology, 2021
Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. Methods We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. Results Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. ...
Clinical and imaging features of Kaposiform Hemangioendothelioma
Kaposiform hemangioendothelioma (KHE) is rare, and classified as a locally aggressive vascular tumor by International Society for the Study of Vascular Anomalies. 1 It was composed of spindled endothelial cells, and immumnopositivity for both vascular and lymphatic endothelial markers, absence of mitosis and nuclear atypia. Numerous KHE in a broad range of locations have been reported (e.g. mediastinum, retroperitoneum, extremity, trunk, gut wall, and even long bone). 2-6 KHE is composed of both vascular and lymphatic components, characterized by infiltrative spindle endothelial nodules. This vascular tumor occurs mostly in children, 2-6 and exceedingly rarely in adult. 7,8 KHE is usually accompanied by Kasabach-Merritt phenomenon (KMP), characterized by severe thrombocytopenia (or with hypofibrinogenemia, raised D dimers), even microangiopathic anemia in the presence of a rapidly growing tumor. 2,3 Its prognosis is usually poor due to hemorrhage and infiltration of vital organ, and the mortality rate of about 10-30%. 2,5,9 Therefore, a timely diagnosis and treatment of KHE is crucial. Ultrasound is the modality of choice for small and superficial vascular tumor. 9 CT is valuable in assessing bony involvement. 10 MRI has most value in evaluating the extent of vascular anomalies and differentiate between vascular tumors and vascular malformations. 9,11
Kaposiform hemangioendothelioma with associated Kasabach-Merritt phenomenon
The Journal of Pediatrics, 1998
A 24-day-old boy presented with a 2-hour history of swelling and bluish discoloration of the left lower limb, genitalia, and buttocks. Physical examination revealed swelling of the left lower limb, extending from the inguinal area to the ankle. The mass was indurated, irregular, covered with ecchymosis, and spared the foot (Fig 1). A complete blood count revealed hemoglobin of 10.5 g/dL and platelet count of 10 × 10 9 /L. There was evidence of a micro-angiopathic process on the blood smear. Coagulation studies revealed a low fibrinogen level (93 mg/dL) and a positive D-dimer test result. The coronal magnetic resonance imaging sequence (T1 inversion recovery [short tau inversion recovery]) (Fig 2) showed abnormal bright material infiltrating the muscle and subcutaneous fat in the pelvis and the left leg. The patient was treated with high doses of intravenous steroids (methylprednisolone, 6 mg/kg per day) for 4 to 6 weeks with marked diminution in the size of the mass and normalization of his platelet, hemoglobin, and fibrinogen values.
Curative effect of surgical resection in the treatment of Kaposiform hemangioendothelioma
2020
Background The clinical features and surgical outcomes of patients with Kaposiform hemangioendothelioma (KHE) is not fully clear. The purpose of this study is to better understand the therapeutic effect and hematological indices of surgical therapy for KHE. Procedure A retrospective study was conducted to review the medical documents of 43 patients with KHE who were treated with surgical resection at our hospital between February 2016 and November 2019. Multiple anatomical sites were involved. The curative effect was evaluated according to the tumor volume shrinkage, color shade, and blood examinations including platelet count, hemoglobin level, red blood cell number, clotting time and D-dimer concentration. Results For all cases underwent surgical treatment, the curative effects accounted for 100%. At the end of the treatment, 9 children were followed up for 6 months, and no evidence of the recurrence of KHE was not found in any case. The number of red blood cells and clotting time were maintained at normal levels during different stage of treatment. The concentrations of hemoglobin and fibrinogen and the average number of platelets increased significantly after surgical treatment. The concentrations of D-dimer were much higher than normal value during admission with a high variability, and significantly decreased after surgery treatment. Further analysis found that the changing trends of D-dimer was positively correlated with fibrinogen protein or platelet number, but not clotting time. Conclusions The surgical treatment method is highly effective and of great significance for KHE therapy in pediatric clinic.
Pediatric Dermatology, 2020
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy and childhood that presents as a locally infiltrative, indurated violaceous mass. Initially, KHE can be confused with congenital hemangiomas, in particular rapidly involuting congenital hemangioma (RICH). RICH can also present as a warm red violaceous mass, and both vascular tumors may have an associated consumptive coagulopathy. The similarities shared by both entities can pose a diagnostic challenge. We present a complex case of a proliferative vascular tumor with a clinical evolution resembling RICH, but with histology and imaging diagnostic of KHE.
Surgical treatment of a huge kaposiform hemangioendothelioma in the chest wall: A case study
SAGE Open Medical Case Reports, 2016
Kaposiform hemangioendothelioma, a rare vascular pediatric tumor often associated with Kasabach–Merritt phenomenon, is characterized by severe thrombocytopenia and consumptive coagulopathy. Kaposiform hemangioendothelioma is a severe disease and may progress quickly, resulting in a high mortality. However, standard treatment regimens for Kasabach–Merritt phenomenon have not yet been established. We reported here an infant with a large congenital kaposiform hemangioendothelioma in his chest wall who responded extremely well to surgical excision.