Büschelartiges Hämangiom (“tufted angioma”) des Unterarms Fallbericht und Literaturübersicht

Background: Tufted angioma represents a benign vascular lesion of the skin. The knowledge of this distinctive entity is important in distinction of clinically more aggressive and malignant neoplasms respectively. Case report. We report a 8-months-old girl who developed a deep seated tufted haemangioma on her right forearm. Sonography and MRI showed a good vascularized tumor with interseptal spreading. Histological examination of the incisional biopsy revealed a cellular capillary haemangioma with morphological features of rare tufted haemangioma. Discussion. Characteristic morphological features of tufted haemangioma allow accurate diagnosis and differential diagnosis to clinically more aggressive and malignant neoplasms of skin and soft tissues, wheras radiological methods are rather unspecific and do not distinguish reliably between these vascular lesions. It is of special clinical importance that tufted haemangioma represents an enlarging but biologically benign vascular neoplasm.     

丛状血管瘤的临床病理学观察

目的 观察了解丛状血管瘤的临床病理特点.方法 对16例丛状血管瘤患儿进行临床病理学观察,并结合文献进行讨论.结果 16例丛状血管瘤患儿,年龄为15 d至11岁1个月,平均29.3个月龄;男13例,女3例;其中头部5例,躯干3例,肢体8例.全部病例均为单发,采用手术切除.临床表现为单发或多发淡红色或紫红色的斑片、丘疹、结节或斑块,可伴有深在的皮下结节.组织病理学见:真皮及皮下组织可见成簇分布的瘤样毛细血管丛,低倍镜下呈"炮弹"样外观,细胞无异型性.免疫组织化学标记:CD31(+)、CD34(+)、FⅧAg(+)、SMA(+)、VEGF(+)、Vimentin(+)和Glut1(-).随访3个月至7年1个月,仅有1例右耳后丛状血管瘤术后3个月复发,改用平阳霉素瘤体注射治疗,间隔1个月,注射3次后痊愈,至今未见复发.结论 丛状血管瘤有一定自限性,局部切除可治愈,但有复发倾向,保守观察和术后的患儿应监测血小板和纤维蛋白原数量,若有发现潜在侵袭性,有向Kaposiform血管内皮瘤转化倾向,应行扩大手术切除,术后病理检查确诊.     

丛状血管瘤的临床病理学观察

目的 观察了解丛状血管瘤的临床病理特点.方法 对16例丛状血管瘤患儿进行临床病理学观察,并结合文献进行讨论.结果 16例丛状血管瘤患儿,年龄为15 d至11岁1个月,平均29.3个月龄;男13例,女3例;其中头部5例,躯干3例,肢体8例.全部病例均为单发,采用手术切除.临床表现为单发或多发淡红色或紫红色的斑片、丘疹、结节或斑块,可伴有深在的皮下结节.组织病理学见:真皮及皮下组织可见成簇分布的瘤样毛细血管丛,低倍镜下呈"炮弹"样外观,细胞无异型性.免疫组织化学标记:CD31(+)、CD34(+)、FⅧAg(+)、SMA(+)、VEGF(+)、Vimentin(+)和Glut1(-).随访3个月至7年1个月,仅有1例右耳后丛状血管瘤术后3个月复发,改用平阳霉素瘤体注射治疗,间隔1个月,注射3次后痊愈,至今未见复发.结论 丛状血管瘤有一定自限性,局部切除可治愈,但有复发倾向,保守观察和术后的患儿应监测血小板和纤维蛋白原数量,若有发现潜在侵袭性,有向Kaposiform血管内皮瘤转化倾向,应行扩大手术切除,术后病理检查确诊.     

丛状血管瘤的临床病理学观察

目的 观察了解丛状血管瘤的临床病理特点.方法 对16例丛状血管瘤患儿进行临床病理学观察,并结合文献进行讨论.结果 16例丛状血管瘤患儿,年龄为15 d至11岁1个月,平均29.3个月龄;男13例,女3例;其中头部5例,躯干3例,肢体8例.全部病例均为单发,采用手术切除.临床表现为单发或多发淡红色或紫红色的斑片、丘疹、结节或斑块,可伴有深在的皮下结节.组织病理学见:真皮及皮下组织可见成簇分布的瘤样毛细血管丛,低倍镜下呈"炮弹"样外观,细胞无异型性.免疫组织化学标记:CD31(+)、CD34(+)、FⅧAg(+)、SMA(+)、VEGF(+)、Vimentin(+)和Glut1(-).随访3个月至7年1个月,仅有1例右耳后丛状血管瘤术后3个月复发,改用平阳霉素瘤体注射治疗,间隔1个月,注射3次后痊愈,至今未见复发.结论 丛状血管瘤有一定自限性,局部切除可治愈,但有复发倾向,保守观察和术后的患儿应监测血小板和纤维蛋白原数量,若有发现潜在侵袭性,有向Kaposiform血管内皮瘤转化倾向,应行扩大手术切除,术后病理检查确诊.     

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.     

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.     

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.     

Variable response to propranolol treatment of kaposiform hemangioendothelioma, tufted angioma, and Kasabach-Merritt phenomenon

Propranolol is a non‐selective beta‐adrenergic antagonist successfully used in a case of kaposiform hemangioendothelioma (KHE) associated with Kasabach–Merritt phenomenon (KMP). We report 11 patients treated with propranolol for KHE and the related variant tufted angioma (TA), six of whom also had KMP. The varied responses to treatment, with only 36% responding in our series, demonstrate the need for further study of this medication before routine use for these indications. Pediatr Blood Cancer 2012; 59: 934–938. © 2012 Wiley Periodicals, Inc.     

Clinical and sonographic features of pediatric soft-tissue vascular anomalies part 1: classification,sonographic approach and vascular tumors

Sonography can be used in the management of pediatric soft-tissue vascular anomalies for diagnosing, for assessing lesion extent and for evaluating complications and response to therapy. The sonographic technique includes a combination of gray-scale imaging with color and spectral Doppler techniques. However the interpretation of the sonographic findings requires correlation with the clinical findings, some of which can be easily obtained at the time of scanning. This has to be combined with the use of appropriate nomenclature and the most updated classification in order to categorize these children into the appropriate management pathway. In this article, which is part 1 of a two-part series, the authors review the current classification of vascular anomalies, provide a clinical and a sonographic approach to these lesions, and review the most relevant clinical and sonographic features of vascular tumors including infantile and congenital hemangiomas, tufted angioma, kaposiform hemangioendothelioma, pyogenic granuloma, intramuscular capillary-type hemangioma and angiosarcoma.     

Kasabach-merritt phenomenon: a retrospective study of treatment with vincristine

PURPOSE: Kasabach-Merritt phenomenon (KMP) is characterized by profound thrombocytopenia, microangiopathic hemolytic anemia, a consumptive coagulopathy, and an enlarging vascular lesion. The syndrome develops in infancy and is associated with a high morbidity and mortality rate. The purpose of this study was to assess the effectiveness of vincristine in the treatment of KMP. METHODS: We retrospectively reviewed the clinical and laboratory data of 15 patients with KMP treated with vincristine at 9 institutions across the United States, South America, and Europe. RESULTS: All 15 patients had profound thrombocytopenia and consumption of fibrinogen at presentation. Ten patients had biopsies of their lesions, and results included five (33.3%) kaposiform hemangioendotheliomas, three (20%) tufted angiomas, one lesion (6.7%) with features of both kaposiform hemangioendothelioma and tufted angioma, and one (6.7%) unclassified vascular tumor. All 15 patients had an increase in platelet count of at least 20,000 with an average response time of 4.0 weeks after initiation of vincristine therapy. Thirteen patients had an increase in fibrinogen level of 50 mg/dL with an average response time of 3.4 weeks. In 13 patients there was a significant decrease in the size of the vascular lesion. The average duration of treatment was 21.5 (+/-12.6) weeks. Four patients (26%) relapsed. All four were successfully treated with a second course of vincristine. Complications included one patient with abdominal pain, one patient with transient loss of deep tendon reflexes, and one patient with irritability. CONCLUSION: Vincristine presents a safe and sometimes effective treatment option in the management of KMP.     

Kasabach-Merritt综合征治疗中自体原位植皮术的应用

目的 对Kasabach-Merritt综合征手术治疗过程中使用自体原位植皮术,评价其治疗效果,提出一种新的手术治疗方式.方法 回顾性分析2010年至2015年间自体原位植皮手术治疗的Kasabach-Merritt综合征患儿23例,病例纳入标准:均为瘤体分布弥散,皮肤大面积(＞5 cm×5 cm)异常,手术切除瘤体后创面缝合困难;非手术治疗方法无效;血小板值在2×109/L～31×109/L.23例Kasabach-Merritt综合征患儿瘤体均位于四肢,上肢6例,下肢17例;男9例,女14例;年龄7 d～9个月.总结该手术治疗方法的手术要点及术中、术后处理,分析治愈率及术后并发症.术后均经病理学检查证实为卡波西样血管内皮瘤(kaposiform hemangioendothelioma,KHE)20例,丛状血管瘤(tufted angioma,TA)3例.结果 23例患儿均在术后1周内血小板值恢复正常,随访1～5年,治愈率为100％;18例患儿自体皮片原位移植全部成活,有3例成活面积约90％,经后续换药处理后愈合,2例原位皮片移植成活约60％,行二期皮肤移植修补手术后治愈,术后并发症少.结论 手术治疗Kasabach-Merritt综合征可作为有效的治疗方法,本组资料显示,自体原位植皮术作为一种新的手术方式,治愈率100％,术后并发症少,其对符合适应证者是一种可选择治疗手段.     

Pre- and postnatal imaging of early cerebral damage in Sturge-Weber syndrome

A case of prenatal diagnosis of Sturge-Weber syndrome associated with polymicrogyria is reported. The diagnosis was based on a unique association with unilateral hemispheric gyriform calcification, focal hemispheric atrophy and white matter changes on prenatal imaging including ultrasound and MRI. Polymicrogyria, which is exceptionally associated with Sturge-Weber syndrome, is suggestive of and reinforces the hypothesis of early impairment of the cerebral microvasculature related to leptomeningeal angioma, which may lead to abnormal cerebral development as early as the second trimester of pregnancy.     

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??Abstract??Objective??To evaluate the diagnostic value of susceptibility weighted imaging ??SWI?? in cerebral vascular malformation of children. Methods??The imaging data of MRI were retrospectively analyzed in all twenty-seven cases?? which included 16 cases of venous angioma?? 8 cases of cavernous angioma and 3 cases of arterio-venous malformation. The comparison of SWI and conventional MR sequences was obtained. Results??The dilated draining veins and medulla veins were displayed well on SWI sequence. More brain cavernous angioma lesions could be detected on SWI than those on conventional MR sequences??P < 0.05??. Conclusion??SWI can detect lesions of venous angioma?? cavernous angioma and arterio-venous malformation more sensitively than other sequences?? which is a reliable diagnostic technique for cerebral vascular malformation of children. SWI has higher clinical value?? and should be the routine sequence in the MRI examination.     

Histopathology of Vascular Lesions Found in Kasabach-Merritt Syndrome: Review Based on 13 Cases

We reviewed the histopathology of 13 cases of Kasabach-Merrit Syndrome (KMS). In 4 (31%) cases the predominant morphology was that of a tufted angioma (TA). Six (46%) cases were Kaposiform hemangioendotheliomas (KHE), and 3 (23%) cases showed an infantile (juvenile) hemangioma only. Immunostaining for CD34 and actin (HHF-35) was helpful in defining these types of hemangiomas. The TA was characterized by a proliferation of endothelial cells positive for CD34 with a minimal component of actin-positive cells. KHE showed a paucity of immunoreactive cells; only the luminal endothelial cells were positive for CD34. In three cases with the morphology of infantile hemangiomas, actin-positive cells outnumbered the CD34-positive cells. Our findings confirm the observation that the underlying vascular lesion in KMS is usually not an infantile hemangioma as was originally thought, but variants of hemangiomas such as TA and KHE (77% of 13 KMS cases). Infantile hemangioma was the phenotypic substrate of KMS in only 3 of 13 cases. Received March 1, 1999; accepted September 30, 1999.     

Cerebral arteriovenous malformations (CAVM)]

Two infants with endocranic A-V malformations and irreversible picture of congestive cardiac failure are presented: the first is a newborn with a very large angioma, the second is a newborn with a large aneurysm of the vein of Galen. A review of the literature is presented: the salient age-related features required to make a diagnosis are discussed.     

Fatal giant pediatric intracranial cavernous angioma

Cavernous angioma is a benign vascular lesion that may occur in the central nervous system. The symptoms of raised intracranial pressure or consciousness alteration are usually related to acute hemorrhage. A previously healthy four-year-old girl was admitted with sudden loss of consciousness, vomiting and clonic seizures. Her Glasgow coma score (GCS) was 7 at presentation (5 m 1v 1e). Anisocoria and mydriasis were present on the right. Computerized tomography revealed a giant spherical, hyperdense intraaxial left frontoparietal lesion. The findings of surrounding vasogenic edema and compression of the adjacent lateral ventricle were seen on computerized tomography (CT). She was taken to operation and the mass was grossly excised. The GCS remained unchanged. A diagnosis of brain death was made. A cavernous hemangioma was diagnosed with pathologic examination. In conclusion, a cavernous angioma may occasionally follow a rapid and fatal course by causing gross hemorrhage in the pediatric age group. Early recognition by CT or magnetic resonance imaging (MRI) and prompt surgical evacuation are necessary.     

Diagnosis and treatment of blue rubber bleb nevus syndrome in children

Background  Blue rubber bleb nevus syndrome (BRBNS) is characterized by distinctive vascular malformations of skin and the gastrointestinal tract, often leading to chronic anemia and intestinal bleeding. It usually presents right after birth or during early infancy. Though the disease is inherent, its occurrence is sporadic. Thus it is usually not timely diagnosed. We analyzed the clinical characteristics and treatment of this disorder in order to improve the diagnosis and treatment. Methods  Three patients with BRBNS treated at our hospital during 2002–2003 and 39 patients from the literature reported during 1965–2003 were reviewed in terms of the diagnosis and treatment. BRBNS may be diagnosed as cutaneous cavernous hemangioma associated with the same lesion of the gastrointestinal tract and other organs. Results  Our 3 patients suffered from cutaneous angioma and gastrointestinal hemangioma. In 39 patients reported in the literature, cutaneous angioma was observed in all of them, and gastrointestinal hemangioma in 31. Additionally, the lesions were also found in other organs such as the brain (7 patients), joint (2), liver (2), eye (1), kidney (1) and spleen (1). Cutaneous angioma was located on the surface of the skin, including body (93%), limbs (86%), hip (36%) and face (26%). Gastrointestinal hemangioma was more common in the small intestine (100%) than in the colon (74%) and stomach (26%). When the joint was involved by hemangioma, pathologic fracture or overgrowth of bone needed traction and amputation (1 patient respectively). For significant gastrointestinal bleeding, endoscopic techniques (8 patients), surgical excision (5), or both (1) were performed. Recurrent bleeding was successfully treated by endoscopic laser combined with steroid or interferon in one patient. Conclusions  BRBNS in children presents atypical symptom and systemic complications. It should be dealt with seriously if gastrointestinal bleeding or orthopedic complication occurs. Treatment includes conservative, endoscopic and surgical options. Its recurrence with new angioma in the gastrointestinal tract needs laser-steroid therapy.     

Pediatric littoral cell angioma of the spleen: multimodality imaging including diffusion-weighted imaging

Littoral cell angioma (LCA) is a rare primary splenic vascular tumor originating from littoral cells lining the splenic red pulp sinuses. LCAs are rarely seen in children. We present the US, CT, and MRI findings including diffusion-weighted imaging (DWI) in a 2-year-old boy with histologically proven LCA. Previous studies on liver lesions have shown that DWI allows differentiation of vascular tumors from primary neoplasms and metastatic disease. The current case indicates that increased ADC values within the splenic lesions suggest a vascular etiology, which might help narrow the differential diagnosis.     

Being alive: Building on the work of Anne Alvarez

The paper illustrates the capacity for development in a mother and her baby which is linked, in the author's view with the capacity to be in contact with reality and with supportive internal objects. The observed baby was born with a livid angioma which marked her face and affected the inside of her mouth. Her mother, who had not been able to prepare herself psychically for the birth, was enabled, by her own mother's experience of the real baby, to face the shock and narcissistic disappointment of not having her fantasied perfect baby, and to be able to make a loving and growing relationship with her little daughter whose beauty could be seen and appreciated despite the blow of having a baby with an angioma.     

Clinical features of Sturge–Weber syndrome without facial nevus: Five novel cases

Classic Sturge–Weber syndrome (SWS) is characterized by presence of flammeus nevus involving the first sensory branch of trigeminal nerve, ipsilateral leptomeningeal angiomatosis, and choroidal angioma. Sporadic cases of SWS without facial nevus (SWS type III) have been rarely reported. Here we report the clinical and neuroradiological findings of five patients with SWS type III and compare their findings with those described in the literature. This study confirmed that SWS type III should be considered in any child or young adult presenting with seizures or complicated migraine and intracranial unilateral calcification. The diagnosis must be confirmed with contrast-enhanced MRI images of the brain. Surgical therapy should be considered in patients with drug-resistant and persistent epileptic seizures.