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.

Kaposiform haemangioendothelioma of the spine associated with fixed hyperlordotic deformity and Kasabach-Merritt Syndrome: a case report and literature review.

Kaposiform haemangioendothelioma (KHE) is a rare childhood disease classified by the International Society for the Study of Vascular Anomalies (ISSVA) as a locally aggressive vascular tumor. It has been reported to affect any site, with a predilection for the extremities and trunk. Although it typically manifests as an enlarging cutaneous or soft tissue lesion, less than 10% of cases have no skin involvement, with the retroperitoneum being the most frequently involved extracutaneous site. Approximately twenty cases of KHE with bony involvement have been reported in the literature to date, with only five of those cases involving the spine specifically.We present a, rare case of KHE who presented with progressive fixed hyperlordotic deformity, multiple non-specific spinal lesions, and abnormal blood tests, posing a clinical and radiological diagnostic challenge. Additionally, we conducted a thorough review of the literature to compare and contrast the various multimodality imaging manifestations of KHE involving the spine.

18F-FDG PET/CT Findings in a Woman With Kaposiform Hemangioendothelioma in the Sacrum.

ABSTRACT: Kaposiform hemangioendothelioma is a rare vascular tumor with borderline malignancy and is typically diagnosed in infancy or early childhood. It most commonly affects cutaneous tissues, whereas the subtype with only primary bone involvement is extremely rare. Herein, we report a case of Kaposiform hemangioendothelioma involving the sacrum in a 37-year-old woman, with intense 18F-FDG accumulation in the lytic lesion on PET/CT. This case indicates that Kaposiform hemangioendothelioma with the primary bone involvement should be taken into consideration as a rare differential diagnosis for lytic lesions with increased 18F-FDG uptake on PET/CT.

Kaposiform hemangioendothelioma in an unusual site: A report of two cases in children.

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor usually seen in children. It is frequently associated with Kasabach-Merritt phenomenon. Here we report two cases of KHE: the first case being an 11-month-old boy who came with massive swelling on the face and violaceous discoloration. The second case was a 7-year-old boy who presented with respiratory distress and bleeding manifestations. CT scan chest showed a large mass involving the anterior mediastinum. Histologic examination of resected masses from both these cases showed features of KHE involving subcutaneous tissue and thymus, respectively. Although cutaneous and subcutaneous location is common, thymic involvement is unusual. It is important to distinguish KHE from infantile haemangioma, tufted angioma, spindle-cell haemangioma, verrucous malformation and Kaposi sarcoma. Histologic features, supportive immunohistochemistry and the clinical profile together are helpful to differentiate KHE from other vascular lesions.

Management of Refractory Mandibular Kaposiform Hemangioendothelioma with Sirolimus: A Case Report and Review of the Literature.

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm of intermediate malignancy that generally occurs in infancy and early childhood. Typically, the lesion arises from superficial or deep soft tissues of the extremities, trunk and retroperitoneum. The paucity of reported cases of head and neck KHEs is evidence of the rarity of the disease in this region. We report on the presentation and treatment of KHE in an 11-month-old boy who presented with a mandibular lesion. We include a brief discussion about the differential diagnosis of KHE. Management involved preoperative interventional radiology, surgical excision and chemotherapeutic treatment with Sirolimus. The lesion resolved without evidence of relapse 12 months later.

Splenic Artery Embolization for Treatment of Iatrogenic Kasabach-Merritt Phenomenon Following Failed Endovascular Splenic Artery Aneurysm Repair.

Stent grafts are utilized to treat and exclude visceral arterial aneurysms while preserving flow through the vessel. Stent-associated thrombocytopenia is a rare complication not typically seen with modern stents. The following case describes the clinical presentation of stent kinking and consumptive coagulopathy. Stent-associated microangiopathic hemolytic anemia was inferred from protracted workup and exclusion of alternative diagnoses. Despite the risk of arterial puncture in the setting of profound thrombocytopenia, the patient was successfully treated with stent embolization with near immediate rebound in platelet count. This case report documents the presentation of rare stent-associated thrombocytopenia leading to challenging diagnostic evaluation and necessitating high-risk intervention.

Kaposiform haemangioendothelioma: magnetic resonance imaging features in 64 cases.

BACKGROUND: Kaposiform haemangioendothelioma (KHE) is a rare, locally aggressive disorder. The presenting and imaging features of KHE can overlap with other vascular anomalies and tumours. We aimed to analyse the imaging findings of KHE disorder and highlight features most suggestive of this diagnosis. METHODS: The clinical features and imaging findings were retrospectively reviewed in 64 patients with pathological diagnosis of KHE. RESULTS: Of the 64 patients diagnosed with KHE, 36 patients were < 6 months and 28 patients were ≥ 6 months. The most common presenting features were Kasabach-Merritt phenomenon (KMP, 42.2 %), visible cutaneous lesions (90.6 %), oedema or swelling (43.8 %) and destructive changes or remodelling of adjacent bone (42.2 %). Compared with patients in the group ≥ 6 months, patients in the group < 6 months have higher odds of KMP (P = 0.000), infiltrative lesion with ill-defined borders (P = 0.044). The group ≥ 6 months have higher odds of destructive changes or remodelling of adjacent bone (P = 0.002). In all patients, the lesions in all of the 64 patients were hypointense or isointense compared with muscle on T1-weighted sequences, and hyperintense on T2-weighted or inversion-recovery sequences, nine patients (14.1 %) showed vascularity. There were 28 patients (43.8 %) with characteristic enhancing and infiltrative soft-tissue thickening. CONCLUSIONS: Presence of visible cutaneous lesions with ill-defined borders, destructive changes or remodelling of adjacent bone, severe thrombocytopenia and consumptive coagulopathy should favour the diagnosis of KHE.

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.

Linfangiomatosis kaposiforme: reporte de un caso y revisión de la literatura / Kaposiform lymphangiomatosis: a case report and literature review

La linfangiomatosis es una patología benigna, de etiología desconocida, que afecta especialmente a población pediátrica, caracterizándose por desarrollo de masas subcutáneas que involucran uno o más órganos. Cuando se asocia con coagulopatía y derrames quilosos, recibe el nombre de linfangiomatosis kaposiforme (LK), cuya evidencia disponible es escasa. El objetivo de este manuscrito es reportar un caso de LK pediátrico atendido en el Instituto del Cáncer SOLCA, Cuenca, Ecuador. Paciente femenina de 1 año 7 meses, con hernia inguinal bilaterales y alteración de los tiempos de coagulación. En los estudios de imagen se evidenció una masa mediastínica asociada a presencia de líquido en espacio pleural y cavidad abdomino-pélvica. Además, se evidenció la existencia de múltiples imágenes hipogénicas en el bazo. Se practicó timectomía mínima, toma de biopsia de linfonodos mediastínicos para establecer el diagnóstico, y se instaló un dren en el tórax para tratar el derrame pleural antes señalado. Se descartaron patologías similares (linfomas, lupus, inmunológicas, etc.). Dado el continúo deterioro respiratorio, coagulopatía, lesiones hipogénicas esplénicas y drenaje abundante del quilotórax por el tubo torácico, se planteó el diagnóstico de LK. Se inició apoyo nutricional vía parenteral (sin lípidos); se indicó vincristina, y se realizó una pleurodesis con bleomicina sin buena respuesta. La paciente falleció a los 16 días de su ingreso. Se trata de un caso de mal pronóstico, con evolución rápidamente progresiva hacia un desenlace fatal.

Lymphangiomatosis is a benign pathology, of unknown etiology. Affects especially to pediatric population and is characterized by development of subcutaneous masses that involve one or more organs. When it presents with coagulopathy and chylous effusions, it is called kaposiform lymphangiomatosis (KL). The available evidence of KL is scarce. The objective of this manuscript was to report a case of pediatric KL treated at SOLCA Cancer Institute, Cuenca, Ecuador. Female patient of 1 year and 7 months age. She had a bilateral inguinal hernia and altered coagulation times. Imaging studies revealed a mediastinal mass associated with fluid in the pleural space and in the abdominalpelvic cavity. Furthermore, the existence of multiple hypogenic images in the spleen was evidenced. Minimal thymectomy, biopsy of mediastinal lymph nodes was performed to establish the diagnosis, and a drain was installed in the chest to treat the aforementioned pleural effusion. Other pathologies like lymphomas, lupus, etc. were ruled out. Given the continuous respiratory deterioration, coagulopathy, splenic hypogenic lesions and abundant drainage of the chylothorax through the chest tube, the diagnosis of KL was raised. Parenteral nutrition (lipid-free) was started; Vincristine was indicated, and a pleurodesis with bleomycin was performed without a good response. The patient died 16 days after her admission. It is a case of very bad prognosis, with a rapidly progressive evolution towards a fatal outcome.

Ultrasonography of Pediatric Superficial Soft Tissue Tumors and Tumor-Like Lesions.

Ultrasonography (US) is usually the first imaging examination performed to evaluate palpable or visible superficial soft tissue lesions that are common in children. Although clinical assessments, such as age at presentation, clinical course, and overlying skin discoloration, are important for the differentiation of pediatric soft tissue lesions, US allows a specific diagnosis of some typical benign lesions and helps in guiding further investigation since it provides detailed information about the lesion location, characterization including solid versus cystic, vascularity, and compressibility. Therefore, sufficient knowledge of the normal anatomy, proper ultrasonographic techniques, and the imaging findings of common and uncommon soft tissue lesions in children are crucial for accurate assessment and management of patients. In this article, we review the techniques and imaging findings focusing on the ultrasonographic features of a variety of superficial soft tissue lesions detected in children.

Bifocal Tibial Kaposiform Hemangioendothelioma Responsive to Vincristine Therapy: A Case Report.

BACKGROUND Kaposiform hemangioendothelioma is a rare locally aggressive vascular endothelial-derived spindle cells neoplasm. Herein, we report a rare case of bifocal tibial kaposiform hemangioendothelioma. CASE REPORT A 9-year-old female presented with a 2-year history of pain and swelling in the left leg. The patient had a high plasma level of the D-dimer and fibrinogen. Radiography revealed a centric lytic lesion on the left proximal tibial metaphysis and an eccentric lateral distal tibial metaphyseal. Histopathologic examination of the sample taken from the distal tibia revealed a dense spindle cell tumor with lobular architecture composed of compact spindle cells compressing small slit-like vascular spaces, forming glomeruloid nests. No necrosis was identified. Based on these findings and the positive immunohistochemical staining for CD31, CD34, and D2-40, the patient was diagnosed with kaposiform hemangioendothelioma. Treatment was started by using vincristine chemotherapy, after which the patient developed temporary peroneal neuropathy, which improved over the next 3 months. CONCLUSIONS Bifocal tibial kaposiform hemangioendothelioma lesions are unique in pediatric patients and can be successfully treated with vincristine chemotherapy. In these cases, the treating physician should be aware of peroneal neuropathy as a potential complication of vincristine administration.

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.

Tacrolimus ointment for the treatment of superficial kaposiform hemangioendothelioma and tufted angioma.

Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare infiltrative vascular tumors. Currently, no standard treatment regimens exist for KHE/TA. The purpose of our study was to evaluate the efficacy and safety of topical application of tacrolimus for superficial KHE/TA. We examined six patients with superficial KHE/TA. All patients were treated with tacrolimus 0.1% ointment twice daily for at least 12 months. The response rate was 100%, including three nearly complete remissions. Only one patient experienced local pruritus during treatment. The data constituted an intriguing rationale for clinical trials of topical tacrolimus in the treatment of superficial KHE/TA.

Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.

BACKGROUND: Kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) are rare and aggressive vascular disorders. The aim of this study was to examine the clinical features and prognosis of KLA and KHE involving the thorax. METHODS: The clinical features, imaging and pathological findings, treatments and outcome were retrospectively reviewed for 6 patients with KLA and 7 patients with KHE involving the thorax. RESULTS: The mean ages at the time of the presentation of signs/symptoms were 26.7 months and 4.1 months for KLA and KHE, respectively. Respiratory symptoms, pericardial and pleural effusion, thrombocytopenia and coagulopathy were common in both KLA and KHE. Diffuse lesions involving the lung and extrathoracic sites were observed in KLA but not in KHE. Histopathologically, all lesions had spindled tumor cells, which were immunoreactive for CD31 and D2-40. In KLA, the spindle cells were distributed in sparse and poorly marginated clusters, whereas the spindle cells formed more defined and confluent vascularized nodules in KHE. Unlike the refractory behavior of KLA, the majority of patients with KHE responded to medical treatments with regression of the lesion and normalization of the hematologic parameters. CONCLUSIONS: The presenting and histological characteristics of KLA can overlap with those of KHE. The presence of diffuse vascular lesions in the mediastinum and lung with refractory thrombocytopenia and coagulopathy should suggest the diagnosis of KLA. Given the rarity and high morbidity and mortality of these disorders, the diagnostic process and therapeutic approach should include a multidisciplinary team review and consensus.