Case Of Kaposiform Hemangioendothelioma Research Articles

PA15: A vascular tumour at birth: a case of Kaposiform haemangioendothelioma with Kasabach–Merritt phenomenon

PA15: A vascular tumour at birth: a case of Kaposiform haemangioendothelioma with Kasabach–Merritt phenomenon

Effective surgical treatment of life-threatening huge vascular anomalies associated with thrombocytopenia and coagulopathy in infants unresponsive to drug therapy

BackgroundSystemic drug therapy is generally recommended for infant huge vascular anomalies associated with thrombocytopenia and coagulopathy, but some patients are not suitable due to drug unresponsiveness or life threatening conditions before the drug works, who will need to receive surgical treatment. This study retrospectively analyzed the clinical features, imaging features, and surgical outcomes of these patients.MethodsThe clinical data of 4 infants with huge vascular anomalies (2 vein malformations (VMs) and 2 kaposiform hemangioendothelioma (KHE)) associated with thrombocytopenia and coagulopathy treated from June 2016 to December 2017 were retrospectively analyzed. All patients received glucocorticoids, propranolol, vincristine or sirolimus treatment before admission, but the treatment was ineffective. Skin petechia, thrombocytopenia and coagulopathy were present at the time of admission. CT scanning was performed before operation. The patient’s general clinical data, hematological examination results, operation time, surgical bleeding volume, blood transfusion volume and surgical complications were collected for analysis. The patients were followed up for 10–26 months.ResultsCT scanning results of 2 patients showed special CT features without detectable enhancement within the lesion after CT enhanced scanning and multiple phleboliths formation. Four patients underwent surgical treatment successfully. Two patients underwent complete resection of the lesion, and 2 underwent cytoreductive surgery. Preoperative clinical symptoms such as skin petechia, thrombocytopenia and coagulopathy were normal at 1 week after surgery. Postoperative pathological results showed 2 cases of KHE and 2 cases of VMs. All patients were discharged from hospital without physical dysfunction, recurrence, or death.ConclusionsTimely and appropriate surgical intervention can achieve satisfactory results for infants with huge VMs and KHE who were unresponsive to drug therapy or suffering from life-threatening occasion before the drug become effective.

Primary kaposiform hemangioendothelioma of ulna

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that can be potentially low malignant and mainly affects infants and adolescents. This tumor usually is seen in the skin, soft tissue, and retroperitoneum. This study is a case of KHE in the long bone of the ulna. The subject was a 7-year-old female patient with osteolytic lesion in the forearm without cutaneous lesions. Histologically, the neoplasms comprised of nodules of spindle-to oval-shaped cells that grew in an infiltrative fashion. The results of 2-year follow-up of the patient after enblec resection were desirable and no recurrence was observed. This is the first study to report a case of KHE of the bone in Iran.
 Keywords: Bone, Hemangioma, Hemangioendothelioma, Kaposiform

Vincristine, aspirin, and prednisolone therapy in Kasabach–Merritt phenomenon: Response in 2 cases

Kasabach–Merritt phenomenon (KMP) is a severe thrombocytopenic coagulopathy which usually occurs in the presence of enlarging vascular tumors such as kaposiform hemangioendothelioma (KHE) and tufted angioma. The treatment for this potentially fatal condition is challenging without a consensus on appropriate management. The authors report two cases of KHE with KMP, wherein improvement in size of tumor and coagulopathy occurred after treatment with prednisolone, vincristine, and aspirin.

Clinical analysis on 13 cases of Kaposiform hemangioendothelioma

Objective To explore the diagnosis and treatment of Kaposiform hemangioendothelioma (KHE), deepen the understanding of KHE and Kasabach-Merritt phenomenon (KMP), and discuss the optimal treatment for KHE. Methods From January 2008 to August 2016, 13 cases of KHE were confirmed by surgery or biopsy pathology and admitted to the First Affiliated Hospital of Sun Yat-Sen University, and the clinical diagnosis and treatment were analyzed retrospectively. Results There were 13 patients(7 males and 6 females) with a median age of 1.0 years(0.2-10.0 years), and 84.6%(11/13 cases) were infants and young children, 76.9% (10/13 cases) involved with deep tissue, 23.1% (3/13 cases) were associated with KMP and they were younger than 1 year old, and 15.4% (2/13 cases) coexisted with hemangioma or lymphangioma.The location, extent and infiltration depth of the lesion were observed by imaging examinations and histopathology showed nodule shaped spindle tumor cells.Radical resection was considered if possible.Dose of Vincristine (0.5 mg/m2 weekly) and Propranolol [1 mg/(kg·d)] were administered.The prognosis was different in thirteen cases undergoing different treatments.After 3 months to 9 years follow-up, 41.7% (5/12 cases) survived after tumor treatment. Conclusions KHE happens mostly in infants and young children, with varying clinical manifestations and a high recurrence rate.The diagnosis of KHE is based on histological examination, computed tomography and magnetic resonance imaging while it still need explicit pathological diagnosis is needed.KHE may be accompanied by hemangioma or lymphangioma.Prognosis is affected by many factors and the comprehensive treatment is required.KMP should be remedied preferentially, individual treatment protocol and long term follow-up are necessary. Key words: Kaposiform hemangioendothelioma; Kasabach-Merritt phenomenon; Diagnosis; Treatment; Analysis

Identical Presentation of Scapular Osteolysis in Two Patients with Thoracic Kaposiform Hemangioendothelioma

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor that does not metastasize. We report two unique cases of KHE involving the right hemithorax and the upper ipsilateral extremity. Kasabach-Merrit phenomenon and osteolytic lesions in the scapula were observed in both cases.

Role of master transcriptional factor Prox-1 in lymphatic endothelial differentiation of Kaposiform hemangioendothelioma

Objective: To analyze the clinical and pathological features of Kaposiform hemangioendothelioma (KHE), and to investigate the role of master transcriptional factor Prox-1 in the regulation of lymphatic differentiation. Methods: Nine cases of KHE (during the period from October 2009 to June 2016) were collected with clinical and pathological data. H&E stained section review and immunohistochemietry using the Dako EnVision method were performed. Results: There were 6 female and 3 male patients with age ranging from 2 months to 8 years (median 3 years and 4 months). The patients presented with either single subcutaneous soft tissue mass, or bone tumors, with the duration of disease onset ranging from 1 month to 1 year. The sites of involvement included the skins of neck (2 cases), nose root (1 case), inguinal (1 case), thigh root (1 case), humerus (2 cases), lumbar vertebrae(1 case), and mesentery (1 case). These tumors were histologically composed of nodules of densely packed spindle or ovoid cells and deformed small blood vessels in an invasive growth pattern. The tumor cells were immunohistochemically positive for both blood vessels and lymphatic endothelial markers, including Prox-1, the master transcriptional factor, and VEGFR-3. With followed-up from 1 to 60 months (median 26 months), two patients died of the disease, while the remaining patients were alive without recurrence. Conclusions: KHE is a rare vascular tumor with at least partial lymphatic endothelial differentiation, in which Prox-1 may act as a master regulator for such differentiation. KHE is an aggressive tumor of intermediate malignant potential, with local invasion and recurrence tendency, and long term follow-up is required.

Expansile Kaposiform Hemangioendothelioma Deformed Thoracic Cage in an Adult

Kaposiform hemangioendothelioma is a vascular tumor that commonly presents as a cutaneous mass, is observed in children, and is associated with Kasabach-Merritt phenomenon. Herein we report a case of kaposiform hemangioendothelioma with chest wall deformity in an adult who did not show the Kasabach-Merritt phenomenon or cutaneous lesions. To our knowledge, this is the first case of asymptomatic kaposiform hemangioendothelioma arising from the pleura and deforming the chest wall. The patient was treated with tumor excision and chest wall reconstruction.

Hémangioendothéliome kaposiforme associé à une leucémie aiguë lymphoblastique B

Herein, we report the first case of kaposiform haemangioendothelioma (KHE) associated with acute B-lymphoblastic leukemia (B-ALL). A five-month-old infant presented a plaque of angiomatous appearance on the forearm that had increased in volume since birth, as well as pallor and cutaneous haematomas. Kasabach-Merritt syndrome (KMS) was evoked despite hepatomegaly and considerable splenomegaly. Laboratory tests revealed severe anaemia and thrombocytopenia as well as major hyperleukocytosis with 90% blasts. Skin biopsy revealed vast vascular lobules containing cohesive fusiform endothelial cells not expressing Glut1, bound up in a dense infiltrate of B-lymphoblast cells. It was in fact KHE associated with B-ALL confirmed by the myelogram. The child was treated with the INTERFANT 2006 protocol followed by allograft of haematopoietic stem cells, which resulted in complete haematological remission. At the same time, almost total regression of KHE was noted. In this infant, KHE had an inflammatory appearance and was associated with thrombocytopenia, evocative of KMS. Analysis of blood and marrow samples resulted in a diagnosis of B-ALL. Histopathological examination of the angioma revealed a typical appearance of KHE associated with dense lymphoblastic proliferation. This appearance could have resulted either from passive contamination by circulating blast cells or from active recruitment of tumor cells at the KHE site. HK mimicking KMS may reveal B-ALL.

Kaposiform Hemangioendothelioma in the Uterine Cervix of a 5-Year Girl

The occurrence of the vascular tumors in the female genital tract, particularly in the uterine cervix, is extremely rare. This kind of tumor has mainly been reported as either cavernous hemangiomas or capillary hemangiomas. To date, no Kaposiform hemangioendothelioma has been reported in this site. Here we presented a case of Kaposiform hemangioendothelioma without Kasabach-Merritt syndrome in the cervix of a 5-year-old girl.

Kaposiform hemangioendothelioma arising in the deltoid muscle without the Kasabach–Merritt phenomenon

Kaposiform hemangioendothelioma (KHE) is a rare tumor that occurs nearly exclusively during infancy and childhood. It has features common to both capillary hemangioma and Kaposi sarcoma and for that reason many terms have been used for these tumors including "Kaposi-like infantile hemangioendothelioma" and "hemangioma with Kaposi sarcoma-like features." KHE typically presents as an ill-defined, red to purple, indurated plaque and is often complicated by the Kasabach-Merritt phenomenon (KMP), a condition of severe thrombocytopenia and consumptive coagulopathy. Knowledge of the radiological findings of this uncommon tumor might be helpful for diagnosis. We present the MRI features of a case of KHE with neither typical skin lesions nor the Kasabach-Merritt phenomenon.

Case Report: Kaposiform Hemangioendothelioma in Multiple Spinal Levels Without Skin Changes

Kaposiform hemangioendothelioma is a rare vascular tumor of childhood that is locally aggressive but has little metastatic potential and by itself is not known to be lethal. It most commonly presents as a superficial or deep soft tissue mass with associated cutaneous lesions. Kasabach-Merritt phenomenon, a condition characterized by profound thrombocytopenia and life-threatening hemorrhage, often is associated with kaposiform hemangioendothelioma. Six cases of kaposiform hemangioendothelioma have been reported in bone, two of which were located in extracraniofacial bones. We report a diagnostically challenging case of a 6-year-old girl with kaposiform hemangioendothelioma of the thoracolumbar spine without Kasabach-Merritt phenomenon or cutaneous lesions.

MRI of multifocal kaposiform haemangioendothelioma without Kasabach–Merritt phenomenon

Kaposiform haemangioendothelioma is a rare soft-tissue tumour of infants and children, and presents as a moderately aggressive malignancy. We present the MRI findings of a histologically proven case of Kaposiform haemangioendothelioma without Kasabach-Merritt phenomenon or typical skin changes. Our case also reveals that the multiple foci of the cutaneous tumour have different MRI morphologies. These findings have not been reported in the literature to date.

Usefulness of D2‐40 immunohistochemistry for differentiation between kaposiform hemangioendothelioma and tufted angioma

Recent investigations have demonstrated the utility of the monoclonal antibody D2-40 as a marker for lymphatic endothelium. D2-40 can be used on formalin-fixed and paraffin-embedded materials. Our objective was to elucidate, using D2-40 immunohistochemistry, the differences among capillary hemangiomas, and especially between kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). We studied four cases of KHE, nine cases of TA, and 31 cases of other vascular tumors. Antibodies against CD31, CD34, factor VIII-related antigen, and GLUT1 were also applied. In KHE, D2-40 was markedly reactive for three and partially for one of four cases in the peripheral area of Kaposi's sarcoma-like proliferative capillaries and negative in the surrounding dilated vessels. In TA, D2-40 was partially positive in the surrounding dilated vessels and negative in cannonball-like proliferative capillaries. Our results suggest that D2-40 is a useful antibody for immunohistochemical discrimination between KHE and TA. In addition, the difference of immunostaining pattern of D2-40 is limited to the peripheral area of capillary proliferation and surrounding dilated vessels; therefore, it is suggested that KHE and TA may reflect different stages in the evolution of a single entity. Namely, they may originate from stem cells possessing the characteristics of both lymphatic and blood vessel endothelial lineages.

Clinicopathologic features of kaposiform hemangioendothelioma

To explore the clinicopathologic features of kaposiform hemangioendothelioma (KHE). The clinicopathologic data were studied in three cases of KHE and review the literatures. Two cases were female and one was male. All cases occurred in infancy. Two tumor located in axillary chest wall and one in lumbar region. All of the three patients had Kasabach-Merritt syndrome. Histologically, the tumor was composed of spindle-shaped cells. in all cases nodular growth pattern was seen. Immunohistochemically, Neoplastic spindled cells expressed CD34 and CD31. Associated lymphangiomatosis was present in two cases. Two tumors were resected completely, one was resected partly. the follow-up period ranged from 6 months to 3 years, and all were alive. Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that mainly occurred in early infancy. It is frequently complicated by Kasabach-Merritt syndrome, and it has features common to both capillary hemangioma and Kaposi sarcoma. The prognosis of KHE is determined by the size, location and the hemorrhage degree of vascular tumor. Better outcome might be achieved in patients with KHE of the skin and in the soft tissues under the skin. It appears that the main treated measure should be wide local excision.

Kaposiform Hemangioendothelioma Presenting with Kasabach-Merritt Syndrome

The double eponym Kasabach-Meritt has been used since the case report in 1940 for a clinical presentation of giant hemangioma with thrombocytopenia, petechia, and bleeding. However, recently two retrospective studies revealed that KMS is not associated with hemangiomas but rather with KHE or tufted angioma (TA). Kaposiform hemangioendothelioma (KHE) is a pathologically benign and clinically aggressive vascular neoplasm in children which was first described by Zukerburg et al. in 1993. We present a case of KHE manifested with KMS and the pathological picture of KHE.