Recent advances in the diagnosis and treatment of Kasabach-Merritt phenomenon / 国际儿科学杂志

Kasabach-Merritt phenomenon (KMP),also known as Kasabach-Merritt syndrome,is a rare syndrome associated with Kaposiform haemangioendothelioma or tufted angioma,and characterized by thrombocytopenia and consumptive coagulopathy.KMP onsets early and progresses quickly.If diagnosis and treatment delayed,it can be life-threatening.In this paper,the progress of clinical manifestations,pathogenesis,diagnosis and treatment of KMP are summarized.

Angioma en penacho: A propósito de un caso clínico / Tufted angioma: A case report

Se presenta el caso de una paciente de un año de edad, con una lesión tumoral congénita en dorso, eritemato-violácea. Se evidencia al cabo de un año durante el control evolutivo, aumento del volumen y cambio de coloración, por lo que se decide su exéresis completa. El examen histopatológico de la pieza concluyó con el diagnóstico de angioma en penacho (AP). El AP es un tumor vascular benigno, poco frecuente. Aparece sin predilección racial y es igual en ambos sexos. Puede ser congénito o adquirido en la primera infancia, ocasionalmente se presenta en la edad adulta. Su patogenia está poco dilucidada.

Presented the case of a patient of one year old with a congenital, erythematous-violaceous tumoral lesion on back. During the control evolutionary is evidence after a year increase of the volume and change of coloration by what is decides his removal complete. The histopathological examination of the piece ended with a diagnosis of tufted angioma (TA). The TA is a rare, benign vascular tumor. Appears no predilection racial and is equal in both sexes. It can be congenital or acquired in early childhood, it occurs occasionally in adulthood. Its pathogenesis is shortly to become.

Tufted Angioma Presenting with Polycyclic Features Mimicking Tinea Faciale / 대한피부과학회지

Tufted angioma is a rare benign vascular neoplasm that usually occurs in children. It appears as an erythematous, purplish, indurated papule or plaque on the trunk or neck. Although it can have variable clinical manifestations, the annular or serpiginous configuration of lesions resembling tinea infections has rarely been reported. A 47-year-old woman presented with an erythematous plaque enlarging by peripheral extension to form polycyclic or serpiginous figures on her left chin and buccal cheek for 7 months without any subjective symptoms. Tinea faciale was considered as the initial clinical diagnosis, but repeated KOH tests were all negative; therefore, skin biopsy was performed. Histopathology revealed discrete circumscribed foci of capillaries scattered throughout the dermis showing a cannon ball appearance, compatible with tufted angioma. Clinical features showed considerable improvement after 2 sessions of intense pulsed light and pulsed-dye laser (5 repetitions) without any complications. Herein, we report a rare case of polycyclic variant of tufted angioma, which developed in a woman in her 5th decade.

Angioma en penacho congénito: Presentación de un caso de aspecto típico / Congenital tuft angioma: A typical presentation

El angioma en penacho es un raro tumor vascular benigno de comportamiento localmente agresivo que aparece en la infancia temprana; puede complicarse con el fenómeno de Kasabach-Merritt. Presentamos el caso de un paciente masculino de 2 años de edad con la lesión clínica característica.

Tufted angioma is a rare benign vascular tumor with a locally aggressive behavior that appears in early childhood; it may be complicated by the Kasabach - Merritt phenomenon. We report a 2 year old boy with a characteristic clinical lesion.

Angioma en penacho / Tufted angioma

El Angioma en Penacho es un raro tumor vascular de la infancia, que comparte características con el hemangioendotelioma kaposiforme, teniendo ambos posibilidad de desarrollar Síndrome de Kasabach-Merrit. Presentamos una paciente portadora de un angioma en penacho desde el nacimiento, que hemos seguido por 13 años: clínicamente, con estudios de imágenes y biopsias. El caso presenta similitudes con la literatura, con algunas interesantes peculiaridades en su evolución. Consideramos que por el comportamiento clínico e histológico, el Angioma en Penacho es parte de la misma enfermedad que el Hemangioendotelioma Kaposiforme.

Tufted angioma is a rare vascular tumor of childhood that shares features with the Kaposiform hemangioendothelioma, having both tumors the possibility of developing Kasabach-Merrit phenomenon. We report the case of a patient with a tufted angioma since birth, which we have followed for 13 years: clinically, with imaging studies and biopsies. The case presents similarities with literature, with some interesting peculiarities in its evolution. We consider that because of the clinical and histological behavior, Tufted angioma is a part of the same disease that Kaposiform hemangioendothelioma.

Development and treatments of Kasabach-Merritt phenomenon / 中华实用儿科临床杂志

Kasabach-Merritt phenomenon (KMP) is characterized by thrombocytopenia with enlarging vascular tumour,KMP usually develops in infancy and is associated with significant morbidity and mortality,the mortality rate is reported as high as 30％.It commonly reported sites of tumor include extremities,trunk,retroperitoneum and neck.There is no consensus in treatment and various regimens have been used by different authors.This report is aim to learn the pathophysiology of the KMP and its diagnosis and treatment.

Un caso congénito de angioma en penacho / A case of congenital tufted angioma

El angioma en penacho (AP) o tufted angioma es un tumor vascular benigno poco frecuente. Aparece principalmente durante el primer año de vida y muy rara vez es congénito. Comunicamos el caso de una niña de 13 años de edad que presenta un tumor vascular congénito a nivel de la región preesternal. La biopsia confirmó un AP...

Tufted angioma is a rare benign vascular tumor. It usually appears in infancy and early childhood, however, rare cases of TA presenting at birth have been published. We report a case of a 13-year-old girl with a congenital vascular tumor on the upper trunk. A skin biopsy confirmed a tufted angioma. Key words: congenital tufted angioma; tufted angioma; vascular tumors...

Primary Cutaneous Epithelioid Hemangioendothelioma That Mimicked Tufted Angioma Histopathologically / 대한피부과학회지

Epithelioid hemangioendothelioma is a rare vascular tumor of endothelial origin that commonly occurs in the deep soft tissue, bone, lung, and liver, although cutaneous forms have rarely been reported. Tufted angioma (TA) is a rare slowly progressive vascular lesion characterized by multiple capillary tufts with the characteristic "canon-ball" appearance scattered throughout the dermis. A 35-year-old male presented a single, 2.5x1.5 cm sized, purpuric mass on the back. A biopsy specimen showed multiple lobules composed of epithelioid endotheial cells with intracytoplasmic vacuoles in a hyalinized and myxoid stroma, and large dilated vessels. The tumor cells were positive for CD31, CD34, and factor VIII-related Antigen, and the crescent shaped dilated vessels were negative for D2-40. We could not find any metastatic lesions on radiologic examinations. The patient was diagnosed with primary cutaneous epithelioid hemangioendothelioma. Herein, we present a rare and interesting case of primary cutaneous epithelioid hemangioendothelioma resembling TA histopathologically.

Kasabach-Merritt Syndrome Arising from Tufted Angioma Successfully Treated with Systemic Corticosteroid

We report a case of Kasabach-Merritt syndrome arising from a tufted angioma successfully treated with systemic corticosteroid. A 2-month-old male infant presented with a palm-sized, erythematous induration on his left pubis. The lesion was diagnosed as tufted angioma histopathologically. After 1 month, the lesion suddenly expanded to the abdomen and scrotum. Initial laboratory tests were consistent with consumptive coagulopathy. He was diagnosed with Kasabach-Merritt syndrome and treated with intravenous dexamethasone at 0.32 mg/kg/day (equivalent to prednisolone 2.0 mg/kg/day). Two days after initiating the treatment, his platelet counts recovered and the lesion ceased to expand. Steroid therapy was converted to oral prednisolone and the dosage was subsequently tapered, and the lesion gradually involuted with no signs of recurrence for a year.

Multiple Annular Tufted Angioma / 대한피부과학회지

Tufted angioma is a rare, slow-growing, benign, vascular tumor that usually occurs in children. It appeared as an erythematous to red-brown, indurated papule on the trunk or neck. Histopathologic examination shows multiple capillary lobules in a cannon ball appearance scattered throughout the dermis. We present here a 32-year-old woman with multiple annular lesions on her right thigh, which started as red papules and they progressed with annular extension and central regression for 3 years. The histopathologic findings were compatible with tufted angioma. The lesions were treated with 3 sessions of sclerotherapy. We report here on an unusual tufted angioma that occurred as multiple lesions which showed annular extension with central regression.

A Case of Acquired Tufted Angioma That Mimicked Pyogenic Granuloma and It Was Precipitated by Trauma / 대한피부과학회지

Tufted angioma (TA) is a rare benign vascular tumor that is histologically characterized by circumscribed angiomatous vascular aggregates in the dermis and its "cannon-ball" pattern. The acquired type is known as angioblastoma of Nakagawa and it was first described in 1949. TA usually occurs on the trunk and neck in children, but TA sometimes occurs at birth or adulthood. The cutaneous manifestations include slowly spreading erythematous macules, papules and nodules that are generally without any other symptoms. We examined a 75-year old man with a bean sized solitary hemorrhagic crusted nodule on the tip of his 3rd finger, and the lesion was clinically similar to the pyogenic granuloma. The nodule developed at the site of a local injury that happened 6 months ago. The histopathological findings were consistent with tufted angioma. He was treated with surgical excision and it did not recur during the next 12 months. We report here on a case of tufted angioma that mimicked pyogenic granuloma, and this lesion occurred at an unusual location and it was precipitated by trauma.

Six Cases of Congenital Tufted Angioma / 대한피부과학회지

Tufted angioma is a rare slowly progressive vascular tumor, characterized by histopathologic findings of numerous angiomatous lobules of "cannonball" appearance within the dermis. It affects children but occurs infrequently at birth. It appears as a clinical imitator of hemangioma of infancy (HOI), so it needs to be differentiated from HOI. Clinically, lesions most often present as red to purple, indurated or nodular plaque, and are usually located on the neck, upper trunk or proximal limb. We report six cases of tufted angioma of congenital onset. We undertook immunohistochemical study using CD31, D2-40 and GLUT1 to rule out other vascular tumors or malformations, particularly HOI. These cases may demonstrate the keys of differential diagnosis between HOI and tufted angioma, which share common features clinically and rarely histopathologically.

A Case of Tufted Angioma: An Annular Variant / 대한피부과학회지

Tufted angioma is a relatively rare hemangioma which mainly occurs in pediatric age. It is slowly-growing over years, with a clinical picture of papule to plaque. Development with an annular configuration is very rare and only five such cases have been reported worldwide. A 64-year-old woman presented with an egg-sized, red, annular lesion on her abdomen which had started as a papule 8 years previously. The biopsy revealed typical histopathological features of tufted angioma. The lesion was treated three times with surgery or cryotherapy and showed recurrence each time over the seven years. During the course it was apparent that the lesion was showing annular extension. We believe the case is the first report of annular tufted angioma showing central regression in Korea. It was also interesting that the benign lesion showed recurrence twice after complete subsidence.

Tufted Angioma:Clinicopathologic Surveys and the Response to Intralesional Steroid

BACKGROUND: Tufted angioma is an uncommon slowly progressive vascular tumor found typically in infants and young children with characteristic histologic findings, so called "cannonball" appearance. OBJECTIVE: The purpose of this study was aimed to investigate the clinical and histopathological characteristics of tufted angioma and the response to intralesional steroid. METHODS: Clinical information of 10 patients with tufted angioma diagnosed in Severance hospital and Pundang CHA hospital from 1983 to 1999 was obtained from the medical records and clinical follow-ups. We re-evaluated 10 biopsy specimens obtained from them with routine H&E staining. RESULTS: Five male and five female patients were included. In 9 patients the lesion appeared before 2 months of age. Four had a lesion at birth. The thigh was the most common site. The clinical symptoms were diverse, but characteristically tenderness was present in most cases. In all the patients the lesions had a tendency to spread progressively. Microscopically, numerous, distinct, variably sized, tightly packed capillary and endothelial cellular lobules were scattered in the dermis. There were characteristic semilunar spaces adjacent to the capillary tufts. Six patients received intralesional triamcinolone. This treatment was found to be effective in 5 patients who experienced remarkable improvement. The improved cases had similar histologic findings which were composed of cellular mass more than lumen formation. We classified our specimens into two categories, one with more cellular mass and the other with more lumen formation in relative proportion. The former was different from the latter in that it had more solid appearance and more definite margin. And we realized that it was useful to divide into these two categories since its response to treatment could be different. CONCLUSIONS: Tufted angioma is a relatively uncommon disease with characteristic histopathologic findings. It seems not to regress spontaneously. So early treatment is required to pre-vent further spreading up to the extent. We treated 6 patients with intralesional injection of triamcinolone and 5 patients experienced marked improvement which had more cellular mass more than lumen formation histopathologically.

Two Cases of Tufted Angioma Improved with Intralesional Injection of Steroid / 대한피부과학회지

Tufted angioma is a rare, acquired vascular tumor, most commonly presenting in the first year of life, which was first described by Wilson Jones in 1979. Spontaneous resolution of this tumor are known to be very rare and local recurrence often occurs unless the tumor is excised completely. We present two cases of tufted angioma developed in two 1-year-old girls. Skin biopsy in a case showed compatible findings with tufted angioma and the other was diagnosed at an other hospital. The lesions were treated with intralesional injections of triamcinolone acetonide and showed marked improvement. During follow up period, the lesions kept improving.

A Case of Tufted Angioma Developing After Electrocautery / 대한피부과학회지

Tufted angioma is a benign, slowly progressive angioma with typical histologic findings of small, circumscribed angiomatous tufts and lobules scattered in the dermis. More than 50% of cases occur during the first year of life, but rarely it develops late in life. There was only one reported case of tufted angioma after trauma in the English literature. We report a case of tufted angioma, which developed after electrocautery in a 61-year-old woman showing histologic features of typical tufted angioma.

Clinical and Histopathologic Study on Tufted Angioma / 대한피부과학회지

BACKGROUND: Tufted angioma, a rare vascular disorder found typically in children, usually involves in the trunk and neck with characteristic "cannonball" distributed vascular tuft. OBJECTIVES: The purpose of this study was aimed to characterize the clinical and histopathologic features of tufted angioma. METHODS: Clinical examinations were performed on 7 patients with tufted angioma regarding the age, sex, duration, sites, clinical morphology, associated symptoms and treatments and disease course. Histopathologic studies of 8 specimens obtained from 7 patients were performed with routine hematoxylin and eosin staining and immunohistochemical staining with anti-factor VIII- related antigen antibody. RESULTS: 1. Male-female ratio was 1:0.75 (male patients were 4 and female patients were 3). Onset of the skin lesions ranged from birth to 67 years of age. Over 40 years of age were 4, 2 patients had the skin lesion at birth and 1 patient found the lesion at 67 years of age on the preexisting nevus flammeus. 2. Typical clinical features showing patch with papules were seen in 4 patients, patch only was seen in three patients. 3. 4 patients had the lesion on the proximal extremities, 2 on the trunk, 1 on the finger and one. Mild tenderness was noted in 3 patients, mild hyperhidrosis in 1 and severe tenderness and pain in 1 patient. 4. Histopathologic studies revealed typical vascular tufts with "cannonball" distribution in the dermis. Immunohistochemical staining performed on 2 cases showed weak positivity to factor VIII-related antigen. 5. In 1 patient, because of small size of the skin lesion, a simple excision was possible. Another 2 patients were being treated with dye laser with minimal improvement, 1 patient showed spontaneous complete regression. However in the 2 patients without treatment, the skin lesion was persisted in 1 patient and the other died of underlying malignancy. CONCLUSION: Though there was no statistical significance for the short number of patients, tufted angioma occurred not only in early childhood but also in adulthood with the predilection sites of the proximal extremities and the trunk. Clinical manifestations were erythematous papulo-patch or patch. Histopathologically, typical proliferation of the blood vessel with the array of cannonball was observed. As reported, several treatments have been tried, and there was a case of self-remission.

A Case of Acquired Tufted Angioma / 대한피부과학회지

Acquired tufted angioma is a benign, progressive vascular lesion that has a distinctive histopathologic appearance. We report a 31-year-old man with acquireed tufted angioma. Several reddish papules and plaque were present on the neck. The lesion had been present for 6 months. They had enlarged slowly, and were slightly tender. Histopathologic examination of the biopsy specimen showed round to ovoid cellular tufts of capillaries, most prominent in the middle to lower dermis.

Painful Tufted Angioma on the Thumb / 대한피부과학회지

Tufted angioma is a rare slowly progressive vascular disorder found typically in children and young adults, usually involving the trunk and neck. We report a case of painful tufted angioma in a 58-year-old man, who had suffered from a painful reddish patch on the dorsum of the right thumb for 2 years. On histopathologic examination, the numerous round lobules of vascular proliferation showed the typical "cannonball" distribution in the dermis. The unusual location and spontaneous pain are rare manifestations of tufted angioma.

A Case of Tufted Angioma Showing a Good Response to Interferon-alpha / 대한피부과학회지

A 13-month-old boy had irregularly shaped, erythematous to dusky red, slightly raised patches and plaques on the left antecubital fossa and arm for 7 months. The lesions were firm and tender. Histopathologically the lesion consisted of small, circumscribed, angiomatous tufts and lobules with a cannonball appearance scattered throughout dermis, a characteristic feature of tufted angioma. For 15 weeks, interferon-a intralesional injections have been performed and the lesions showed an improvement.