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.     

A case of axillary kaposiform hemangioendothelioma resembles a soft tissue sarcoma

Kaposiform hemangioendothelioma is a rare, aggressive vascular proliferation in children that is clinically and histologically distinct from hemangioma of infancy. It is often complicated with Kasabach-Merritt syndrome. The authors describe a 2-month-old girl who developed a rapidly enlarging deep subcutaneous solid tumor in the left axilla. The tumor size was about 10x7 cm and there was no skin change over the mass. The results of laboratory tests were significant for a moderate anemia (7.0 g/dL) and a profound thrombocytopenia (3x10/L). The preoperative radiologic findings were suspicious for a soft tissue sarcoma with scapular erosion. Anemia and thrombocytopenia were corrected by preoperative corticosteroid therapy and blood component transfusion. The tumor was excised totally and the pathologic findings were consistent with Kaposiform hemangioendothelioma. Serial blood examinations showed that the hemoglobin and platelet count returned to the normal range after surgery.     

小儿卡波西型血管内皮瘤19例临床病理学特点

目的 探讨小儿卡波西型血管内皮瘤的临床病理学特点.方法 收集上海儿童医学中心及新华医院收治的19例卡波西型血管内皮瘤的临床特点及随访结果,应用HE及免疫组织化学染色,观察组织病理学特点,同时统计临床及随访资料.19例患儿中,男11例,女8例;平均年龄19个月,标准差为22.7个月,中位年龄10个月.14例位于皮肤及皮下软组织,3例原发于盆腔,2例原发于骨.临床上多出现局部肿块,少部分因发生凝血异常症状而就诊.实验室检查,8例血小板明显低于正常,7例凝血时间延长及纤维蛋白原降低.结果 本组病理大体标本为不规则实性肿块,无包膜,最大径0.3～1.5 cm.镜下肿瘤形成小叶状结构,边界不清,浸润性生长,小叶由增生的血管和梭形细胞组成,可见新月形和裂隙状血管腔,部分腔内可见微血栓.19例免疫组织化学检测肿瘤细胞表达CD31、CD34和D2-40,不表达Glut-1.19例患儿均经手术切除,其中8例伴有血小板减少的病例,经过对症治疗后再手术切除.随访时间3～75个月,18例术后无复发,1例并发致死性消耗性凝血(kasabach-merritt,KM)现象,患儿死亡.结论 卡波西型血管内皮瘤是少见的交界性儿童血管肿瘤,有特殊的组织学特点,因其可合并KM现象,所以明确诊断对临床意义重大.     

A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging

Kaposiform hemangioendothelioma (KH) is an endothelial-derived spindle cell neoplasm often associated with Kasabach-Merritt syndrome. Most cases arise in infancy and childhood and are soft-tissue tumors. The tumor displays an appearance between capillary hemangioma and Kaposi's sarcoma. We report a case of KH in a 1-year-old girl involving a mass that showed abnormal enhancement of soft tissue superficial to the right temporal bone with partial destruction of the temporal bone, the temporomandibular joint, mandibular condyle, and occipital bone. The physical finding of a discolored mass led clinicians to consider a hemangiomatous lesion, whereas the radiological picture suggested a more aggressive diagnosis of rhabdomyosarcoma and aggressive fibromatosis. Received: 8 March 2001/Accepted: 6 August 2001     

Kaposiformes kongenitales Hämangioendotheliom als Geburtshindernis und postpartaler Notfall

Kaposiform hemangioendothelioma (KHE) is a rare, weakly malignant vascular tumor that occurs nearly exclusively during infancy and childhood. It is often complicated by the Kasabach-Merritt phenomenon (KMP), severe thrombocytopenia and consumptive coagulopathy which can lead to severe hemorrhagic complications. This article describes the case of a term newborn with a protracted birth caused by a fulminant swollen, red-colored right arm and a threatening circulation disorder of the ipsilateral hand. The clinical, radiological and laboratory findings including histological analysis were in line with the diagnosis of congenital KHE with KMP. High-dose administration of prednisolone, acetylsalicylic acid and later sirolimus therapy resulted in a clear reduction of the tumor mass and recovery from thrombocytopenia. During the time of treatment the tumor became steroid-dependent and in the further course also vincristine-dependent; therefore, laser therapy was initiated, which successfully caused the tumor to disappear. Additional treatment with propranolol showed no positive effects. Neonatal KHE can present as an emergency and as in this case requires immediate treatment due to thrombocytopenia and imminent loss of hand function from circulation failure.     

Update on hemangiomas and vascular malformations.

Hemangiomas and vascular malformations are frequently encountered in pediatric practice, especially hemangiomas and port-wine stains. These lesions may cause physical and psychological complications and it is important to recognize which lesions need to be treated and how. Great progress has been made in the classification of vascular anomalies. Angiogenesis and molecular genetics are areas of active research; recent findings relating to hemangiomas and vascular malformations are presented. New clinical features of hemangiomas are described, such as association of extensive facial hemangiomas with various malformations and the occurrence of Kasabach-Merritt phenomenon, not with common hemangiomas, but with other vascular tumors (Kaposiform hemangioendothelioma and tufted angioma). Interferon alfa is effective for treatment of complicated hemangiomas but may cause serious neurological side effects. It is to be hoped that early diagnosis of Sturge-Weber syndrome will soon be possible with new, noninvasive, functional imaging techniques. New issues surrounding pulsed dye laser therapy for port-wine stains are also discussed in this article.     

Kasabach-Merritt phenomenon

The objective of this article is to provide a comprehensive overview of the Kasabach-Merritt Phenomenon. The clinical presentation, laboratory findings, vascular pathology, and pathophysiology are discussed.     

Spindle cell (Kaposiform) hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with α-2A interferon

A two-month-old infant developed a vascular tumor of the right flank which, upon biopsy, proved to be a spindle cell hemangioendothelioma. The increased capillary bed characterizing the neoplasm caused a severe thrombocytopenia together with a consumption coagulopathy (Kasabach-Merritt syndrome). The patient, who was dependent on platelet transfusions, improved quickly after interferon α-2a was given at the dosage of 3,000,000 U/m², with resolution of the Kasabach-Merritt syndrome after three weeks and a 75% decrease of the tumor volume within three months of treatment. Med. Pediatr. Oncol. 28:358–361, 1997. © 1997 Wiley-Liss, Inc.     

Successful management of a retroperitoneal kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using alpha-interferon.

It has been shown recently that Kasabach-Merritt phenomenon, the association of a vascular tumour and consumption coagulopathy, does not--as previously thought--complicate "classical" infantile hemangiomas but distinctive entities called kaposiform hemangioendothelioma (KHE) and tufted angioma (TA), both tumours on the same neoplastic spectrum. These tumours have been found in the neck, face, thorax, abdomen, retroperitoneum and limbs and are associated with a mortality rate of as high as 30 %. Several therapeutic modalities, including alpha-interferon, vincristine, radiotherapy and surgery have been reported in the literature. We report a case of retroperitoneal kaposiform hemangioendothelioma regression using alpha-interferon and discuss the current knowledge of this entity and its treatment.     

TREATING CHILDREN WITH CHRONIC HEPATITIS C AFTER MALIGNANCY IN A SINGLE CENTER

Kasabach-Merritt phenomenon (KMP) is a serious coagulopathy with severe thrombocytopenia (<10,000/mm³) that occurs in the presence of an enlarging vascular tumor such as kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). The natural history and treatment of these lesions remain controversial. The authors report a KHE case and a TA case that presented with KMP, describing their successful pharmacological management with vincristine, ticlopidine, and aspirin.     

Intra-abdominal Kaposiform hemangioendothelioma and the benefits of laparoscopic surveillance

We report a case of a neonate presenting with the rare vascular tumor, Kaposiform hemangioendothelioma. She had a lesion arising from the left ovary with multiple intraperitoneal metastases causing small bowel obstruction. We managed this case with primary surgical resection followed by laparoscopic surveillance of the peritoneal cavity and metastectomy. The child is fit and well and free of tumor after 32 months. Neither this presentation of this tumor nor this management approach has been previously described in the literature.     

Non-Immune Fetal Hydrops with Hepatic Hemangioendothelioma and Kasabach-Merritt Syndrome: a Case Report

A premature infant presented with non-immune hydrops fetalis, a liver mass, thrombocytopenia, and hypofibrinogenemia. Histologic examination of the liver tumor showed an infantile hemangioendothelioma. The clinical features of this case can be explained by anemia, hypoalbuminemia, and coagulopathy. The association with Kasabach-Merritt syndrome, the pathophysiology of non-immune hydrops fetalis, and primary hepatic neoplasms of the neonate are discussed.     

Non-immune fetal hydrops with hepatic hemangioendothelioma and Kasabach-Merritt syndrome: a case report

A premature infant presented with non-immune hydrops fetalis, a liver mass, thrombocytopenia, and hypofibrinogenemia. Histologic examination of the liver tumor showed an infantile hemangioendothelioma. The clinical features of this case can be explained by anemia, hypoalbuminemia, and coagulopathy. The association with Kasabach-Merritt syndrome, the pathophysiology of non-immune hydrops fetalis, and primary hepatic neoplasms of the neonate are discussed.     

Long‐term outcome for kaposiform hemangioendothelioma: A report of two cases

Kaposiform hemangioendothelioma (KHE) is a rare aggressive vascular tumor of skin and deep soft tissues that typically presents in infancy and may be associated with a potentially life‐threatening coagulopathy known as Kasabach–Merrit phenomenon (KMP). Recent advances in medical therapy have successfully treated many patients. However, our knowledge regarding the natural history of these lesions and optimum surveillance strategies remains rudimentary. We report two young women who had KHE with KMP treated in infancy and presented in adolescence with comorbidities related to their KHE tumor. This presentation supports the need for long‐term surveillance in these patients.     

Komplizierter Verlauf eines unklaren Halstumors

Our patient demonstrated a left-cervical palpable strand, which subsequently showed a progressive increase in size. On the basis of magnetic resonance tomography, sample biopsy and blood values at 3?months of age, the immovable, bluish shimmering submandibular swelling in the neck (no tenderness on palpation), with intermittent biphasic stridor was diagnosed as Kasabach-Merritt phenomenon associated with kaposiform hemangioendothelioma. Elimination of the tumor also eliminates the consumptive coagulopathy.     

Mediastinal Kaposiform Hemangioendothelioma and Kasabach-Merritt Phenomenon in a Patient with no Skin Changes and a Normal Chest CT

A 16-month-old previously healthy boy was admitted to the hospital with respiratory distress and thrombocytopenia. Initial workup demonstrated large pleural and pericardial effusions. The patient had no cutaneous abnormality on physical examination, and his initial chest CT (computed tomography) was nondiagnostic. He required multiple platelet transfusions, chest tube placement, and pericardiocentesis. Sixteen days after admission, a chest MRI (magnetic resonance imaging) revealed a large infiltrative mass of the superior mediastinum, consistent with kaposiform hemangioendothelioma (KHE). The patient's thrombocytopenia was due to associated Kasabach-Merritt phenomenon (KMP). The patient now has complete resolution of KMP after medical treatment with prednisolone, aminocaproic acid, vincristine, and aspirin.     

16例新生儿卡梅综合征临床分析

目的 分析总结新生儿卡梅综合征（Kasabach-Merritt syndrome,KMS）的临床特点、治疗及预后,为优化该病的诊断及治疗提供参考依据。方法 回顾性分析安徽医科大学附属省儿童医院2016年1月至2020年12月收治的16例KMS新生儿的住院及随访资料。结果 16例KMS患儿中,男13例（81%）,女3例（19%）,入院年龄为1 h至10 d。13例（81%）为皮肤血管瘤（头面部2例、躯干5例、四肢6例）,3例（19%）为肝脏血管瘤。10例（62%）临床表现以出血倾向、全身散在瘀点瘀斑为主。16例患儿均有不同程度的血小板减少及凝血功能异常。所有患儿入院后均采取糖皮质激素保守治疗,7例（44%）有效,其中复发4例。糖皮质激素治疗无效患儿中3例接受西罗莫司治疗,1例治疗4周后瘤体缩小58.8%,血小板计数及凝血功能恢复正常;2例瘤体无明显缩小,血小板计数无明显回升,联合博来霉素动脉栓塞治疗4周后瘤体缩小（43.7±0.4）%,血小板计数及凝血功能恢复正常。4例单独接受博来霉素动脉栓塞治疗4周后瘤体缩小（52.0±3.4）%,血小板计数及凝血功能恢复正常。2例接受外科钝性+锐性剥离切除术,术中切除全部瘤体,术后无感染及复发,血小板计数及凝血功能恢复正常,1例术后病理结果为卡波西样血管内皮瘤。结论 KMS具有特征性临床表现、组织病理特征及实验室检查结果。对激素反应不敏感的KMS通过动脉栓塞和西罗莫司治疗可取得较好的疗效。     

后腹膜卡波西样血管内皮瘤诊疗分析

目的 通过分析后腹膜卡波西样血管内皮瘤的诊治过程,探讨该类疾病的诊断与治疗方法.方法 复旦大学附属儿科医院外科近年来成功治疗后腹膜卡波西洋血管内皮瘤伴血小板减少患儿2例,回顾性分析2例患儿的病史及诊疗经过.结果 1例患儿肿瘤位于后腹膜,表现为后腹膜巨大占位性病变,伴血小板减少,被误诊为神经母细胞瘤.1例表现为顽固血便,血小板减少,被误诊为出血性肠炎.2例患儿诊断过程复杂,经多科会诊得以确诊.在治疗初期激素治疗有效,但迅速出现耐药和病情反复.采用长春新碱（Vincristine,VCR）治疗后病情得到控制,撤离激素,血小板均恢复至正常范围,肿块消失.结论 后腹膜卡波西样血管内皮瘤临床罕见,最常见的并发症是血小板减少.该病诊断困难,容易误诊.VCR治疗有效,部分患儿可完全缓解.规范化和个体化的治疗非常必要.     

Liver transplant in an infant with bilateral cystic neuroblastoma complicated by hepatic metastases and life-threatening consumption coagulopathy

Abstract:  Here we report a patient with stage-4 bilateral cystic adrenal neuroblastomas with disseminated liver metastases and consumption coagulopathy who underwent liver transplant. Our patient was initially diagnosed with infantile hepatic hemangioendothelioma and bleeding into the adrenal glands secondary to consumption coagulopathy (Kasabach-Merritt syndrome). Liver transplant was performed as a life-saving procedure under this diagnosis. We discuss this unique patient because of the diagnostic pitfalls of this rare disease and the successful clinical outcome after LT and subsequent chemotherapy for neuroblastoma.     

Kaposiform hemangioendothelioma associated with Kasabach-Merritt syndrome

OBJECTIVE: To describe a case of kaposiform hemangioendothelioma, the only malignant tumor of vascular origin specific of childhood.METHODS: We report a case of a 40-days-old girl who presented with a giant hemangioma of the face. Rapid enlargement of the tumor lead to laryngeal compression with severe respiratory distress. She had also a consumptive thrombocytopenic coagulopathy (Kasabach-Merritt syndrome).RESULTS: She was admitted to the pediatric intensive care unit of the Hospital das Clínicas da Universidade Estadual de Campinas and mechanical ventilation was introduced. A ten day course of dexamethasone was only partially effective. We then started alpha-2a interferon at a dose of 1.8 million units/m(2)/day by subcutaneous route, but she died four days after the onset of this therapy. The microscopic features showed a kaposiform hemangioendothelioma.CONCLUSION: We discuss this unusual fatal evolution of a rapidly growing hemangioma and its hematological complications.