Rare adult Kaposiform hemangioendothelioma with multiple-bone invasion - clinical experience and literature review.

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a borderline vascular tumor between hemangioma and malignant angiosarcoma. While KHE has strong local invasion with rare spontaneous regression, it is not observed with distant metastasis. Even if KHE is asymptomatic or without the Kasabach-Merritt phenomenon (KMP), bone or joint invasion should clearly receive proactive treatment. KHE commonly affects infants/children but is rarely seen in adults. CASE REPORT: We reported a rare adult KHE case with an invasion of >10 separate forearm/hand bones, who underwent multiple-lesion resection and finger amputation after tumor recurrence. Tumor recurrence and KMP were not observed during the 6-month follow-up after the final operation. During the hospitalization and follow-up period, the patient only received medications for infection prevention and pain relief. CONCLUSIONS: Multiple resectable lesions were found in the distal limb, for which complete resection might not present typical features (high-intensity T2-weighted MRI), which might fail to detect all KHE lesions. Therefore, complete excision is not optimal for multiple resectable KHE lesions.

Kaposiform hemangioendothelioma of skull base with dura invasion in a pediatric patient: a case report.

Kaposiform hemangioendothelioma is an extremely rare vascular tumor which shows aggressive local growth. We present a case of rapid growing vascular skull tumor with dura invasion in a pediatric patient with neurofibromatosis type 1. A 14-year-old male complained of headache and dizziness for 1 month after minor head trauma. Brain magnetic resonance imaging (MRI) revealed a 5-cm-sized tumor in the left frontotemporal bone with internal hemorrhage and cystic changes. The gross total resection of tumor was done. At the 7-month follow-up, brain MRI revealed a recurrent skull tumor with intracranial dura mass. He underwent second surgery, and the pathologic diagnosis was suggestive of Kaposiform hemangioendothelioma. For this vascular proliferative tumor, mTOR inhibitor was treated for 6 months, and there was the recurred nodular-enhancing mass along the sphenoid ridge. After additional 2 months of medication, the following MRI revealed a decreased nodular-enhancing mass.

Liver Transplantation for Giant Hemangioma Complicated by Kasabach-Merritt Syndrome: A Case Report and Literature Review.

BACKGROUND Liver hemangiomas are the most common benign liver tumor. Giant hepatic hemangiomas are hemangiomas that are greater than 4 cm in diameter. While asymptomatic giant hepatic hemangioma patients can be monitored without intervention, patients that experience complications can be managed by trans-arterial embolization, radiofrequency ablation, surgical resection, or enucleation. Although there is no consensus on definite medical treatment or optimal timing of surgery, liver transplantation is rarely indicated. Among giant hepatic hemangioma patients who received liver transplantation, Kasabach-Merritt syndrome (KMS), a consumptive coagulopathy associated with hemangiomas, is one of the most common indications. We present a case of giant hepatic hemangioma complicated by Kasabach-Merritt syndrome, which was successfully treated by orthotopic liver transplantation. CASE REPORT The patient was a 39-year-old woman with a known history of multiple giant hepatic hemangiomas who presented with abdominal pain and distension. She had life-threatening intra-abdominal hemorrhages caused by benign endometriomas due to hepatic hemangiomas complicated by Kasabach-Merritt syndrome. Despite interventional radiology embolization of a bleeding uterine artery and aggressive resuscitation with fluid and blood products, the patient's status continued to decline. Emergent orthotopic liver transplantation was applied with subsequent resolution of the consumptive coagulopathy. She remained well at 2-month follow-up, with normal liver enzyme levels and intact liver allograft function. CONCLUSIONS Liver transplantation is indicated for selected patients with giant hepatic hemangioma complicated by KMS; despite the high surgical risk, outcomes seem favorable.

Kaposiform Hemangioendothelioma of the Oral Cavity: A Rare Tumor with an Unusual Location.

Kaposiform hemangioendothelioma is a rare neoplasm with intermediate malignant behavior, mainly affecting infants and children. Involvement head and neck is uncommon, and there are only four cases reported in the oral cavity and oropharynx. Microscopically, it is characterized by a vascular proliferation permeated by spindle-to-ovoid cells resembling Kaposi sarcoma. Immunohistochemically, the tumor is positive for CD31, CD34 and negative for D2-40. Herein we present a rare case of intraoral Kaposiform hemangioendothelioma in a 10-year-old boy.

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

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. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. CONCLUSIONS: KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

Kaposiform haemangioendothelioma of duodenum in a neonate.

Kaposiform haemangioendothelioma, an endothelial borderline tumour, is typically seen in childhood involving extremities. It has been closely associated with a consumptive coagulopathy state, Kasabach-Merritt phenomenon (KMP). Extracutaneous involvement is uncommon. Intestinal involvement is quite uncommon and can masquerade as an acute abdomen. A 24-day-old neonate presented with bilious vomiting and fever for 5 days. Sections from the resected gangrenous duodenum contained a submucosal tumour composed of infiltrating nodules of slit-like or crescentic CD34-positive spindled-to-flattened endothelial-lined vascular spaces along with zones of fibrosis. No nuclear pleomorphism or necrosis identified. The findings were classic example of kaposiform haemangioendothelioma with an absence of any deranged coagulation profile. The index case raises interest given its congenital incidental presentation at an uncommon site, like duodenum, and absence of coexistent KMP.

Síndrome de Kasabach-Merritt esplénico en adulto. Presentación de un caso y revisión del tema / Splenic Kasabach-Merritt syndrome in an adult. Presentation of a case and review of the topic / Síndrome de Kasabach-Merritt esplênica em adulto. Apresentação de um caso e revisão do tema

RESUMEN Se presentó un paciente de 22 años de edad, masculino, de la raza negra con gran esplenomegalia debido a un hemangioma gigante del bazo, el cual presentó trombocitopenia y trastornos de la coagulación. Se estudió y se diagnosticó un síndrome de Kasabach-Merrit. Se le realizó una esplenectomía total anterior regresando a la normalidad el conteo de plaquetas y los trastornos de la coagulación. Su evolución fue favorable al cabo de los 18 meses de seguimiento. Se recomienda el tratamiento quirúrgico, la esplenectomía. Se revisó el tema por lo infrecuente de la enfermedad.

ABSTRACT A 22-year-old male black patient presented with big splenomegaly due to a giant hemangioma of the spleen, showing thrombocytopenia and bleeding disorders. A Kasabach-Merrit syndrome was diagnosed. An anterior total splenectomy was performed, returning the platelet count and coagulation disorders back to normal. His evolution was favorable after 18 months of follow-up. Splenectomy surgical treatment was recommended. The topic was reviewed due to the infrequency of the disease.

RESUMO Foi apresentado paciente do sexo masculino, 22 anos de idade, de raça negra, apresentou grande esplenomegalia devido a hemangioma gigante do baço, que apresentava plaquetopenia e distúrbios de coagulação. Uma síndrome de Kasabach-Merrit foi estudada e diagnosticada. Uma esplenectomia total anterior foi realizada, retornando a contagem de plaquetas e distúrbios de coagulação ao normal. Sua evolução foi favorável após 18 meses de seguimento. Tratamento cirúrgico é recomendado, esplenectomia. O tema foi revisado devido à raridade da doença.

Coagulation issues in vascular anomalies.

Vascular anomalies, comprised of vascular tumors and malformations, are frequently associated with coagulopathy. Recognition of and familiarity with these vascular anomaly-associated hematologic abnormalities prior to surgery or interventional procedures is essential for pre-operative pre-operative planning. Complicated coagulopathies present within the framework of either Kasabach-Merritt phenomenon (KMP) or localized intravascular coagulopathy (LIC), and their management benefits from the expertise of a hematologist for optimal intra- and peri­operative care. Furthermore, with the recent broadening of understanding of vascular anomalies and the addition of new classification sub-groups, distinctions of these two classic coagulopathy phenotypes have been recognized. This review summarizes the main features of these coagulopathies, described according to their vascular anomaly type, highlighting clinical aspects relevant to surgical management.

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

BACKGROUND: Systemic 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. METHODS: The 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. RESULTS: CT 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. CONCLUSIONS: Timely 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.

Upper arm life-saving amputation of a 12 day-old neonate due to extensive vascular tumor of the upper extremity.

Vascular tumors in neonates are mostly benign; however, locally aggressive voluminous forms may destabilize the hemodynamics of a neonate. Herein, we present an unusual case of a neonatal giant vascular tumor in the right upper extremity, causing a consumption coagulopathy and acute deterioration of vital signs. The patient required mechanical ventilation, inotropic support, and administration of blood products by the seventh day. Vascular embolization attempts failed to improve the general condition of the patient. Due to the deteriorating and life-threatening general condition of the patient, amputation around the upper arm level occurred under emergency conditions on the twelfth day. The patient's hemodynamic parameters were regained immediately, with neither inotropic agents nor blood products required after the second postoperative day. Clinical and pathological diagnosis revealed kaposiform hemangioendothelioma. Patient monitoring proceeded until the age of 15 months, with no local recurrence around the stump or soft tissue coverage complications. Therefore, since other treatment options failed, the early amputation decision was life-saving.

Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma.

Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) primarily occurring in infants are difficult to distinguish. This study evaluated ultrasonography (US) and magnetic resonance imaging (MRI) features of KHE and TA. Pathologically proven TA (n = 21) and KHE (n = 40 [11 KHE + Kasabach-Merritt phenomenon [KMP]]) occurring between January 2015 and December 2017 were reviewed. US (n = 61) and MRI (n = 50) findings were retrospectively evaluated. On US, KHE and TA lesions were subcutaneous, while 40% of KHE exhibited an infiltrative pattern extending into adjacent muscles. Of TA lesions, 42.9% were hyperechoic and 96.15% of KHE lesions exhibited mixed echogenicity. Of TA lesions, 76.2% exhibited well-defined margins and all KHE lesions exhibited ill-defined margins. The depth and vascular density of KHE and KHE + KMP were significantly increased compared with TA. The arterial peak systolic blood flow velocity of KHE + KMP was significantly higher than that in TA and KHE. KHE and KHE + KMP were significantly harder than TA on elastography. 3-D color Doppler revealed branch-shape blood flow for KHE and KHE + KMP lesions; for TA, it revealed a dot-like and striped pattern. Considering MRI findings, KHE and KHE + KMP were more likely to exhibit diffuse heterogeneous enhancement after contrast than TA. KHE was infiltrative and more likely to be thick, hypoechoic, ill-defined, richly vascular and hard than TA on US. KHE lesions were subcutaneous and reticular, with heterogeneous enhancement on MRI. Awareness of these features should prompt radiologists in the differential diagnosis of pediatric masses.

Major liver resection in pregnancy: three cases with different etiologies and review of the literature.

BACKGROUND: Major liver resection during pregnancy is extremely rare. When required, the associated physiologic and anatomic changes pose specific challenges and greater risk for both mother and fetus Materials and methods: Three cases of major liver resection during pregnancy due to different etiologies are presented. The relevant literature is reviewed and discussed. RESULTS: We present three cases of major liver resection due to giant liver hemangioma with Kasabach-Merrit syndrome, giant hydatid cyst, and intrahepatic cholangiocarcinoma, at gestational week (GW) 17, 19, and 30, respectively. All patients had an uneventful postoperative course, continued the pregnancy and gave birth at GW 38. CONCLUSION: Major liver resection can be performed safely during pregnancy. A multidisciplinary team of surgeons, anesthesiologists and gynecologists, in a highly experienced tertiary hepatobiliary center, should be involved.

Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis.

CASE REPORT: A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt. After the surgery, the child's condition has markedly improved. DISCUSSION AND CONCLUSIONS: A network of intracranial lymphatics is presently being investigated. Neuroimaging excluded KLA infiltration of the skull and/or meninges, leaving as the most plausible explanation for the child's pseudotumor cerebri the existence of an increase in intracranial venous pressure by venous compression at the thorax. To our knowledge, our case constitutes the first report of pseudotumor cerebri occurring in the context of KLA.

Atypically presenting kaposiform hemangioendothelioma of the knee: ultrasound findings.

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of early childhood and infancy. Kasabach-Merritt phenomenon, a common complication of KHE, is characterized by life-threatening thrombocytopenia, hemolytic anemia, and consumption coagulopathy. There may be atypical cases that do not present with Kasabach-Merritt phenomenon and do have atypical imaging findings. Knowledge of atypical imaging features may assist radiologists in identifying KHE. In this report, we present a 4-year-old case of KHE with atypical ultrasound findings.

Intracranial intraaxial cerebral tufted angioma: case report.

Tufted angioma (TA) is a rare, slow-growing, vascular lesion that commonly presents as a solitary macule, papule, or nodule arising in the soft tissues of the torso, extremities, and head and neck in children and young adults. Adult-onset cases have been infrequently reported. While typically benign, TAs may be locally aggressive. Complete physical examination and hematological workup are recommended in patients with TA to exclude the presence of Kasabach-Merritt phenomenon (KMP). The authors describe the case of a 69-year-old man with a contrast-enhancing frontal lobe lesion, with surrounding vasogenic edema, which was treated by gross-total resection. Characteristic histological features of a TA were demonstrated, with multiple cannonball-like tufts of densely packed capillaries emanating from intraparenchymal vessels in cerebral cortex and adjacent white matter. Tumor recurrence was detected after 4 months and treated with adjuvant Gamma Knife radiosurgery. To the extent of the authors' knowledge, this case illustrates the first report of TA presenting in an adult as an intracranial intraaxial tumor without associated KMP. The fairly rapid regrowth of this tumor, requiring adjuvant treatment after resection, is consistent with a potential for locally aggressive growth in a TA occurring in the brain.