Congenital hemangiomas and infantile hemangioma: missing links

Rapid postnatal growth and slow involution in childhood characterize the common infantile hemangioma. There are other rare vascular tumors that present fully grown at birth and behave quite differently, as designated by the acronyms: rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH). RICH and NICH have similarities in appearance, location, size, equal sex ratio, and both have overlapping radiologic and histologic features with infantile hemangioma. However, neither type of congenital tumor immunostains for glucose transporter-1 protein, a marker of infantile hemangioma. This raises the question of whether these congenital vascular lesions are variations in a spectrum of hemangioma or are entirely different tumors. We describe two groups of patients that suggest a linkage between postnatal and congenital vascular tumors: Link I (n=5), children who had either RICH or NICH coexisting with infantile hemangioma, and Link II (n=10), children initially diagnosed as having RICH, but regression was incomplete and the residuum was that of NICH. We conclude that these infants exhibit "missing links" between the rare RICH and NICH, and the common infantile hemangioma.     

Rapidly Involuting Congenital Hemangioma with Fetal Involution

Uncommon congenital hemangiomas differ from common infantile hemangiomas in their appearance, postnatal behavior, histopathology, and immunohistologic staining. Two types are well described in the literature: noninvoluting congenital hemangioma (NICH) and rapidly involuting congenital hemangioma (RICH). We report a series of infants with another presentation of congenital hemangioma that arises prenatally and is nearly regressed at birth. This was a retrospective case series. We describe six infants with unusual congenital vascular tumors. Each lesion presented at birth as a violaceous, atrophic plaque with a surrounding pale halo. The lesions involuted in infancy, fading in color and becoming atrophic, with prominent central veins, similar to RICH in the final stage of regression. The distinctive morphology and behavior suggests that these tumors undergo a life cycle of proliferation and involution during fetal life. We describe a new variant of congenital hemangioma that we refer to as rapidly involuting congenital hemangioma with fetal involution.     

Use of dermal fat grafts for treating anetoderma with lipoatrophy following involution of hemangiomas

Infantile hemangioma (IH) is a benign vascular tumor that gradually involutes over several years. Rapidly involuting congenital hemangioma (RICH) is the relatively rare congenital vascular tumor that is fully grown at birth and does not undergo postnatal growth and involutes during the first year. However, after involution of both IH and RICH, some have severe sequelae, such as redundant skin or conspicuous scarring, requiring additional treatment. We present the case of a 6-year-old girl with a concave deformity due to subcutaneous atrophy, skin darkening, and altered skin texture of her left zygomatic region following involution of a hemangioma. We successfully treated this patient by transferring a dermal fat graft. This technique can be beneficial for atrophic sequelae after regression of a hemangioma and is easy to perform and cosmetically effective.     

Rapidly involuting congenital haemangioma associated with transient thrombocytopenia and coagulopathy: a case series

Rapidly involuting congenital haemangioma (RICH) may present with thrombocytopenia, low fibrinogen and elevated fibrin degradation products and D-dimers. Such complications have rarely been reported. We wished to define the clinical characteristics of the thrombocytopenia and coagulopathy associated with RICH, to emphasize the transient nature of this haematological complication and to distinguish these abnormalities from true Kasabach-Merritt phenomenon (KMP). We present a case series of seven patients with large RICH who presented with thrombocytopenia and coagulopathy during the first week of life. Clinical and haematological characteristics were recorded retrospectively. Two of the patients were treated with embolization due to early signs of high-output cardiac failure; four patients received oral corticosteroids in the range of 2 mg kg(-1) daily; one patient did not receive any treatment in the neonatal period, although the tumour was excised at 6 months of age. Two patients with platelet counts lower than 10 x 10(9) L(-1) received a platelet transfusion. There were no bleeding complications and only one patient presented with petechiae. In all seven patients, platelet counts started to increase at > 2 weeks of age and the coagulopathy resolved. We conclude that RICH may present with thrombocytopenia and coagulopathy similar to mild KMP early in the neonatal period. However, in contrast to true KMP, these abnormal laboratory findings are self-limited and are usually not complicated by bleeding problems.     

H?mangiome. Neue Aspekte zu Pathogenese, Differenzialdiagnosen und Therapie

With a prevalence of 10–12% infantile hemangiomas (IH) represent the most common skin tumor in infancy. They are characterized by a sequence of growth and, in 90%, spontaneous regression. Special manifestations of IH include hemangiomatosis, congenital hemangioma (RICH/NICH), reticular and segmental hemangiomas (PHACE, PELVIS/SACRAL syndrome). The latter represent a transition between vascular tumor and vascular malformation. Important differential diagnoses of IH include pyogenic granuloma (PG) and hemangioendothelioma (HAE) of which PG is a common, benign vascular tumor with a predilection for the facial region and a proneness to bleed whereas HAE is a slowly growing tumor exhibiting infiltrative growth; unlike IH it is associated with the Kasabach-Merritt syndrome of consumptive coagulopathy. The majority of IHs do not require therapy, however, therapy is absolutely indicated for ulcerating or obstructive IH where propranolol is considered the first-line therapy. Non-obstructive facial IH represents a relative indication for therapy. Cryotherapy is most suitable for IH with diameters below 1 cm and a depth < 4 mm.     

Sonographic findings in a series of rapidly involuting congenital hemangiomas (RICH)

We report 10 patients with lesions consistent with the clinical entity of rapidly involuting congenital hemangioma (RICH). These are congenital vascular tumors bearing some resemblance to infantile hemangiomas, but with important clinical differences. The lesions in our patients were firm, red or purple plaques or tumors, sometimes with surface telangiectasia and almost all with a pale or blanched halo. They were fully developed at birth and underwent no further expansion. All promptly began to resolve and this progressed rapidly over the early months of life, with complete resolution, sometimes with residual atrophy, occurring at less than 1 year of age in most of the patients. Sonography was performed in all of our cases. A remarkably consistent picture was demonstrated. The lesions were uniformly hypoechoic and mostly confined to the subcutaneous fat. They were diffusely vascular, being traversed by multiple tubular vascular channels. Some of the channels were compressible, with a venous flow signal, while others demonstrated low resistant arterial flow. These features are consistent with recently described histopathologic findings in these rapidly involuting lesions. We feel that this entity can be reliably diagnosed in most cases with a consideration of the unique clinical features and with the use of noninvasive imaging studies.     

Multifocal infantile hemangiomas associated with concomitant giant hepatic rapidly involuting congenital hemangioma

We present a 9-day-old girl with multifocal cutaneous and hepatic infantile hemangiomas as well as a hepatic rapidly involuting congenital hemangioma. These two distinct vascular tumors have rarely been reported to co-occur. We additionally review the sonographic features that distinguish a hepatic congenital hemangioma from the hepatic infantile hemangioma.     

ICAM-1在先天性血管瘤激光干预前后的表达及意义

目的探讨细胞间黏附分子-1(ICAM-1)在先天性血管瘤患儿长脉宽1064nm Nd:YAG激光干预前后表达变化的临床意义。方法采用ELISA及免疫组化方法测定50例先天性血管瘤患儿激光治疗前及治疗1个月后血清与血管瘤组织中ICAM-1的水平及健康对照组患儿血清和皮肤组织中的ICAM-1水平,将先天性血管瘤患儿术前分别与健康体检者,激光干预后sICAM-1及mICAM-1的表达水平进行比较。结果激光干预前先天性血管瘤患儿血清sICAM-1的水平(566.92±51.39)μg/L明显高于健康对照组(218.01±38.43)μg/L,差异有统计学意义(P<0.01);干预后1个月sICAM-1水平(418.40±55.89)μg/L较干预前有显著下降,差异有统计学意义(P<0.01)。干预前血管瘤组织中mICAM-1显著高于干预后及正常皮肤组织,干预后1个月mICAM-1表达较干预前明显减弱,差异均有统计学意义(P均<0.01)。结论 ICAM-1可能是先天性血管瘤分期诊断和疗效观察的一个临床生物检测指标,进一步的研究有助于婴幼儿先天性血管瘤的诊断和治疗。     

Congenital Hemangioma: A Report of Evolution from Rapidly Involuting to Noninvoluting Congenital Hemangioma with Aberrant Mongolian Spots

Abstract:   Congenital hemangiomas are unusual vascular tumors that are fully developed at the time of delivery. We report a case of an infant with an exophytic congenital hemangioma with features of a rapidly involuting hemangioma on the arm which over 3 years decreased in volume but continued to persist with features of a noninvoluting congenital hemangioma. He also had aberrant, persistent Mongolian spots on both legs. To our knowledge, this association has not been reported to date.     

Verrucous hemangioma: a report of two cases and review of the literature

Verrucous hemangioma represents a rare congenital vascular proliferation that may be mistaken for angiokeratoma. Histopathological examination of a deep biopsy is necessary to confirm the diagnosis of verrucous hemangioma based on its involvement of the deep dermis and subcutaneous tissue. We present two cases of verrucous hemangioma and discuss the clinicopathologic and immunohistochemical findings.     

Verrucous lymphovascular malformation versus verrucous hemangioma: controversial nomenclature

The International Society for the Study of Vascular Anomalies (ISSVA) divides congenital vascular anomalies into malformations and tumors and subclassified hemangiomas under tumors. However, evidence shows this accepted classification has not been widely employed. Particularly troublesome is the use of the term hemangioma, commonly used to describe a variety of vascular lesions (both malformations and tumors). The term verrucous hemangioma has been used to describe a congenital vascular anomaly with a progressive verrucous epidermal surface persisting throughout life unless surgically excised. Recent evidence suggests that some of these lesions may share histologic features of both hemangiomas and malformations, thereby causing nosologic confusion. We report a 15-year-old adolescent girl with such a lesion and review the literature and controversy of verrucous hemangiomas. In our case, the most appropriate diagnosis is verrucous lymphovascular malformation. Further testing of similar lesions will be necessary to fully understand the nature and classification of these lesions.     

Congenital Capillary Hemangioma and Its Therapeutic Approach in Infants: A Case Report

Hemangiomas, common congenital lesions in infants and children, are thought to arise when islands of angioblastic tissue fail to connect with the developing vascular system. They are not usually life-threatening. A case of congenital capillary hemangioma in an infant, which was surgically excised, is reported, and therapeutic approaches are discussed.     

Congenital infantile fibrosarcoma: a masquerader of ulcerated hemangioma

Congenital infantile fibrosarcoma, a rare malignant tumor of childhood, may present as a highly vascularized mass that is clinically difficult to distinguish from a hemangioma. When ulcerated, significant hemorrhage, anemia, and thrombocytopenia may occur in children with these lesions. This report describes three infants with ulcerated congenital infantile fibrosarcomas of the hand. As appropriate medical and surgical management hinges on timely and appropriate diagnosis, we review the clinical manifestations of these lesions.     

Congenital Infantile Fibrosarcoma of the Lip

Infantile congenital fibrosarcomas are rare neoplasms that usually present on the extremities, and although they are locally invasive, they rarely metastasize. They are commonly misdiagnosed as hemangiomas or other vascular tumors, so further evaluation by pathology is required for proper diagnosis. We describe a newborn with a neoplastic growth of the lower lip that was thought to be an infantile hemangioma that did not respond to therapy. When the child was 2 months old, an incisional biopsy demonstrated a fibrosarcoma. This case highlights congenital infantile fibrosarcoma as a mimic of infantile hemangioma. To our knowledge this is first case report of congenital infantile fibrosarcoma involving the lip.     

Conventional ultrasonography and elastography for the diagnosis of congenital and infantile hemangiomas

Infantile and congenital hemangiomas are difficult to distinguish in infants. The aim of this study was to compare the conventional ultrasonographic (US) and elastographic features of infantile and congenital hemangiomas. The US findings in 118 patients with congenital hemangioma (58 non-involuting, 36 rapidly involuting, 24 partially involuting) and 111 with 120 infantile hemangioma were retrospectively evaluated. On US imaging, 31.7% of infantile hemangiomas were hyperechoic, 31.7% hypoechoic and 36.6% mixed-echoic with hyperechoic and hypoechoic areas; 57.6% of congenital hemangiomas were mixed-echoic with a hypoechoic area and many vessels visible, 39.0% hypoechoic and 3.4% were mixed-echoic with hyperechoic and hypoechoic area. Calcifications were present in 6.8% and visible vessels involving muscle in 24.6% of congenital hemangiomas. All infantile hemangiomas and 82.2% of congenital hemangiomas were well-defined. All congenital hemangiomas were subcutaneous whereas 17.5% of the infantile hemangiomas were superficial. The maximum diameter and vascular density were greater in congenital hemangiomas. Elastography demonstrated that the congenital hemangiomas were softer than the infantile hemangiomas. The maximum diameter (including of visible vessels), thickness, vascular density, venous blood flow velocity and elasticity scores were greater for rapidly and partially involuting congenital hemangiomas than for non-involuting ones. The density of visible vessels in congenital hemangiomas decreased in the order of non-involuting, partially involuting and rapidly involuting. In conclusion, congenital hemangiomas have distinctive US imaging characteristics, including a greater maximum diameter, vascular density, number of visible vessels, visible vessels involving muscle, calcifications and elasticity score.     

Infantile fibrosarcoma mimicking rapidly involuting congenital haemangioma (RICH)

BACKGROUND: While haemangioma is common, an unusual appearance or course should alert the clinician's concern. Congenital haemangioma, particularly rapidly involuting congenital haemangioma (RICH), may carry a risk of misdiagnosis as congenital malignant tumours such as infantile fibrosarcoma (also known as congenital infantile fibrosarcoma). In this case, histological diagnosis may prove inconclusive, as in the case reported herein. PATIENTS AND METHODS: At birth, a newborn baby presented angiomatous lesions on the sole of the left foot that was initially considered as congenital haemangioma. Histopathological examination suggested highly remodelled immature infantile haemangioma. After surgery, the tumour increased in size within eight weeks. Reanalysis of the histology slides resulted in a diagnosis of infantile fibrosarcoma. This diagnosis was confirmed by the presence of a specific translocation seen in infantile fibrosarcoma (ETV6/NTRK3). CONCLUSION: There is a risk of erroneous diagnosis in newborn infants between angiomatous tumour in RICH and malignant congenital tumours (particularly infantile fibrosarcoma). Clinicians should be attentive for this type of lesion and take all necessary diagnostic measures.     

Massive hemorrhage: A rare complication of rapidly involuting congenital hemangioma

Rapidly involuting congenital hemangioma is a subtype of congenital hemangioma. Ulceration and bleeding are rarely reported in rapidly involuting congenital hemangioma, with only four cases reported in the literature to our knowledge. We describe a case of a newborn girl who presented with rapidly involuting congenital hemangioma complicated by ulceration and severe bleeding and discuss treatment.     

Childhood cutaneous hemangiomas

An hemangioma is a common benign growth of vascular endothelium that may have multiple clinical manifestations. Either identified at birth or shortly thereafter, hemangiomas have both proliferative and involutional phases. Certain varieties are associated with congenital anomalies. Numerous therapy options are discussed.     

Multifocal Lymphangioendotheliomatosis with Thrombocytopenia: Presentation of Two Cases Treated with Sirolimus

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a rare disease characterized by congenital and progressive vascular lesions of the skin and gastrointestinal tract that may be associated with thrombocytopenia and possibly life‐threatening gastrointestinal bleeding. Reports published on the disease and treatment strategies are scarce. We present two cases of MLT treated with sirolimus.     

Verrucous Hemangioma

Two patients with verrucous hemangioma, a congenital vascular malformation, were seen. The typical lesion is a unilateral group of hyperkeratotic papules and nodules on a lower extremity. Unlike the more common capillary or cavernous hemangiomas, verrucous hemangiomas tend to enlarge and spread with time, and to recur after excision.