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.     

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.     

Kaposiform hemangioendothelioma with Kasabach-Merritt syndrome: a new indication for propranolol treatment

Kaposiform hemangioendothelioma is a rare vascular tumor in children. Especially, in association with the Kasabach-Merritt Phenomenon it can be life threatening. The management of these patients is very difficult and an aggressive treatment regime is required. Several multimodality and chemotherapeutic regimens have been described but with variable success and many side effects. We present a 6-week-old boy with Kaposiform hemangioendothelioma and Kasabach-Merritt Phenomenon. Ongoing propranolol treatment with only 4 initial courses of vincristine resulted in a remission that lasted at least 1 year.     

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.     

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.     

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.     

Hemangioendothelioma: A Rare Case of a Primary Intracardiac Tumor

Kaposiform hemangioendothelioma (KH) is a rare tumor of vascular origin that commonly affects the cutaneous tissues of the extremities. It can, however, affect the abdomen, thorax, head, or neck. Compared with juvenile hemangiomas, which tend to regress, KH tumors are locally aggressive and usually persist. Associated morbidity and mortality rates range from 12 to 30 % and typically are related to either compressive effects on surrounding vital structures or effects of the Kasabach–Merritt phenomenon [10, 11, 13]. To our knowledge, this report is the first to describe KH presenting as a primary intracardiac tumor.     

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     

Infantile hemangioendothelioma of the pelvis associated with Kasabach-Merritt syndrome

A case of infantile hemangioendothelioma of the pelvis in a newborn male infant is described. Shortly after delivery, a large abdominal mass was found by external examination. CT scan revealed a hypervascular retroperitoneal pelvic mass invading the lumbar spinal column. Exploratory laparotomy was performed and biopsy revealed infantile hemangioendothelioma. The tumor was associated with profound thrombocytopenia, intratumoral hemorrhage, and right hydroureteronephrosis. After treatment with high dose steroids, Cytoxan, and external beam radiation for 2 months, the patient was discharged with persistent thrombocytopenia requiring platelet transfusions. The hydroureteronephrosis has also not improved. The literature on this subject is reviewed.     

Infantile Hemangioendothelioma of the Pelvis Associated with Kasabach-merritt Syndrome

A case of infantile hemangioendothelioma of the pelvis in a newborn male infant is described. Shortly after delivery, a large abdominal mass was found by external examination. CT scan revealed a hypervascular retroperitoneal pelvic mass invading the lumbar spinal column. Exploratory laparotomy was performed and biopsy revealed infantile hemangioendothelioma. The tumor was associated with profound thrombocytopenia, intratumoral hemorrhage, and right hydroureteronephrosis. After treatment with high dose steroids, Cytoxan, and external beam radiation for 2 months, the patient was discharged with persistent thrombocytopenia requiring platelet transfusions. The hydroureteronephrosis has also not improved. The literature on this subject is reviewed.     

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。     

Infantile hemangioendothelioma of the thymus with massive pleural effusion and Kasabach-Merritt syndrome: Histopathological, flow cytometrical analysis of the tumor

Infantile hemangioendothelioma of the thymus is a rare disease. We describe a patient who developed a large anterior mediastinal mass, severe thrombocytopenia and massive pleural effusion at 1 month of age. Glucocorticosteroid and irradiation therapy had no effect on either the tumor size or clinical symptoms and the tumor was resected subtotally. Three months after the subtotal resection, the remaining tumor had almost disappeared and the symptoms had resolved. The patient has now been well for 1 year after surgery without evidence of recurrence. The tumor tissue was characterized by prominent vascular endothelial proliferation intermixed with a normal thymic structure, producing a picture consistent with that of an infantile hemangioendothelioma in the thymus, lmmunohistochemically, the tumor cells showed positive staining for vimentin, factor VIII and CD34. The DNA stemline and proliferative activity were examined by flow cytometry, which revealed a diploid stemline with a low growth fraction. DNA content and cell cycle analyses of the tumor tissue may be useful for predicting the biological behavior of the tumor.     

Ultrasonography in primary hepatic tumors in early childhood

Ultrasonography was useful in preoperative investigation of primary hepatic tumors in nine children, aged 4 weeks to 4 years. In all patients, the hepatic origin and the solid or mixed echogenic nature of the tumor was determined. In two children (one with hemangioendothelioma, one with cystic mesenchymoma) the ultrasonographic findings were even more specific when correlated with angiography and/or histology. Ultrasonography in children with hepatomegaly should be performed as a screening procedure before angiography, to determine if enlargement of the liver is of tumorous origin. For follow-up, ultrasonography should be preferred over radionuclide imaging because it does not require the use of ionizing energy.     

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.     

Splenic hemangioma with thrombocytopenia in a newborn

The case report of a newborn infant with a splenic hemangioendothelioma with the life-threatening complications of thrombocytopenia, anemia, and disseminated intravascular coagulation is presented together with a review of the literature. Removal of the tumor via splenectomy, despite the known risk of subsequent overwhelming sepsis due to encapsulated organisms in the young child, is the treatment of choice for splenic hemangiomas complicated by thrombocytopenia. The use of enhanced CT with delayed filling is a diagnostic tool in the workup of suspected hemangiomatous lesions.     

Transfusion-associated fall in platelet count in very low birthweight infants

Thirty-three infants with a birthweight of less than 1500 g were investigated retrospectively for the incidence and aetiology of thrombocytopenia occurring during the first week of life. The platelet count fell below 100 x 10(9)/l in 16 infants (48%). There was a moderately strong inverse correlation between the platelet count at its nadir during the first week or the first value below 100 x 10(9)/l and the percentage of blood volume transfused prior to this (r = -0.61; P less than 0.0001). When the platelet count was expressed as a percentage of the initial count the correlation was -0.74 (P less than 0.0001). The results were not affected by the elimination of the 10 infants with clinical conditions regarded as a probable cause of thrombocytopenia. The fitted least-squares regression line suggests that a transfusion equal to 10% of the blood volume on average reduced the platelet count by 19 x 10(9)/l or by 7% in these very low birthweight infants during the first week of life.     

Peripheral blood stem cell transplantation in young children: experience with harvesting, mobilization and engraftment

The purpose of this study was to determine the feasibility and assess optimal timing of harvesting peripheral blood stem cells (PBSC) for transplantation in young children. Thirteen children with body weight less than 25 kg, mean age of 3.9 years (1-9 yrs) who had recurrent solid tumors and leukemia were given tumor specific chemotherapy followed by i.v. rhG-CSF (5 microg/kg/d) for stem cell mobilization. Cytaphereses were done through a central venous line (CVL) during the marrow recovery phase (WBC >0.5 x 10(9)/l). The phereses were analyzed separately and assigned to three groups depending on the WBC at the time of the pheresis: Group I (WBC <1.0 x 10(9)/l), Group II [WBC in the range 1.0-3.0 x 10(9)/l] and Group III (WBC >3.0 x 10(9)/l). Samples from each harvest were assayed for cell count, CFU-GM, BFU-E, CD34+ cell count, and tumor cell immunocytology in patients with neuroblastoma (NBL). A median of 3.2 x 10(8) mononuclear cells per kg (MNC/kg), [mean 2.8 x 10(8) MNC/kg, standard error of the mean (SEM) +/- 0.74 (1.1-4.7)] were infused following myeloablative therapy. 78 phereses were performed in 13 children with a median weight of 18 kg (10-25 kg). A median of 5 phereses were performed per patient. There were no significant differences in the percentage and number of CD34+ cells, CFU-GM or BFU-E colonies assayed by plating 0.5 x 10(5) cells. Differences could be found in the total number of MNC (p<0.008) and the number of MNC/kg (p<0.001) between Groups II and III. No tumor cell contamination was detected in the NBL patients by immunocytology. All patients were rescued with PBSC and achieved sustained white cell engraftment (ANC >0.5 x 10(9)/l) at a median of 13.5 d (10-25 d) and platelet engraftment (untransfused platelet count >20.0 x 10(9)/l) at a median of 29 d (12-63 d). The only toxicity encountered during the phereses was thrombocytopenia in 4 patients whose median post-pheresis platelet count was 6.0 x 10(9)/l (3.0-9.01). It is concluded that collection of PBSC in young children is feasible and safe and can be performed through a cuffed CVL at the time of WBC recovery post mobilization with chemotherapy and G-CSF. Cytopheresis can be effectively performed when the peripheral WBC count approaches 1.0 x 10(9)/l. Following stem cell infusion, engraftment was prompt and durable.     

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.     

Initial bone marrow aspiration in childhood idiopathic thrombocytopenia: decision analysis

PURPOSE: Bone marrow aspiration (BMA) is routinely performed before starting steroid therapy in children with idiopathic thrombocytopenia, primarily to rule out leukemia. METHODS: A decision tree for the initial management of a child older than age 6 months, presenting with idiopathic thrombocytopenia, without blasts on the peripheral smear was constructed. The three strategies are: 1) initial BMA in all patients; 2) initial BMA only in patients at high risk; and 3) empiric therapy for all patients without initial BMA. High-risk criteria include any of: platelet count >50 x 10(9)/L; hemoglobin <100 g/L (age younger than 12 months) or <110 g/L (age older than 12 months): white blood cell count <5 x 10(9)/L (younger than 6 years) or <4 x 10(9)/L (older than 6 years); or absolute neutrophil count <1.5 x 10(9)/L (younger than 6 years) or <2 x 10(9)/L (older than 6 years). The results are expressed as quality-adjusted life years (QALYs), a measure that estimates the overall life expectancy in years for patients receiving a particular treatment strategy, corrected for the patient's quality of life. RESULTS: The base case results are: 1) BMA all = 69.649 QALYs; 2) high-risk BMA = 69.652 QALYs; and 3) empiric therapy = 69.644 QALYs. These results indicate a three-way toss-up because there is less than a 4-day quality-adjusted difference (0.01) between strategies. CONCLUSION: This study indicates that the initial BMA does not significantly change the overall QALYs of a child presenting with thrombocytopenia and, consequently, is not mandatory in every patient before starting steroids.     

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.