Presentation
Lesions typically present during infancy or early childhood and are most commonly reported to persist and/or expand over time. Congenital presentations are rare, as are reports of spontaneous regression. [ncbi.nlm.nih.gov]
Entire Body System
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Hunting
Wassef M, Hunt SJ, Santa Cruz DJ, Barnhill RL. Vascular tumors and vascular malformations. In: Barnhill RL editor. Dermatopathology. 3 rd ed. New York: McGraw Hill; 2010; p. 805-6. 10. [e-ijd.org]
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Lymphadenopathy
No lymphadenopathy. Core needle biopsy showed haemngioendothelioma ( Figure 2 ). The immunohistochemical examination showed capillary haemangioma (Tufted Angioma, Angioblastoma of Nakagawa) ( Figure 3 ). [omicsonline.org]
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Weakness
UEA1 immunohistochemical staining of endothelial cells was strongly positive ( Figs. 1 and 3 ), but weak and less extensive staining was found with antibodies to VIII-RA. Stains for S-100 and actin were negative in all cells of the lesion. [cda-adc.ca]
Hematological
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Prolonged Bleeding
A female infant, born with a tufted angioma, developed a coagulopathy with prolonged bleeding time, with the risk of progression to Kasabach-Merritt phenomenon. [ncbi.nlm.nih.gov]
Skin
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Localized Hyperhidrosis
We found localized hyperhidrosis in two of our patients. Proliferation of eccrine glands near the tumour lobule was a consistent finding in the biopsy specimens from these four patients. [ncbi.nlm.nih.gov]
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Macula
This lesion was seen at the age of 7 as a small, asymptomatic red wine macula, which grew progressively. At present it is slightly painful and presents papular areas. [elsevier.es]
Eyes
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Eye Pain
PATIENT: A 73-year-old man presented with right eye pain, redness, burning sensation, swelling, and double vision. [ncbi.nlm.nih.gov]
Psychiatrical
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Suggestibility
The evolution of the lesion may then suggest a dermatologic origin. [ncbi.nlm.nih.gov]
Neurologic
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Burning Sensation
PATIENT: A 73-year-old man presented with right eye pain, redness, burning sensation, swelling, and double vision. [ncbi.nlm.nih.gov]
Treatment
Two patients developed grade 2 oral mucositis during treatment. CONCLUSION: Sirolimus as first-line therapy shows great promise in the treatment of kaposiform hemangioendothelioma and tufted angioma. 2018 Wiley Periodicals, Inc. [ncbi.nlm.nih.gov]
Prognosis
It has been associated with Kasabach-Merritt syndrome, a severe coagulopathy of poor prognosis. There have been very few published cases of oral tufted angiomas, and maxillary bone involvement has not been hitherto reported. [ncbi.nlm.nih.gov]
What is the Prognosis of Tufted Angioma? (Outcomes/Resolutions) The prognosis of Tufted Angiomas with adequate treatment is usually good. [dovemed.com]
Treatment and prognosis Various treatments are used, with varying degrees of success, in Kasabach-Merritt syndrome - systemic corticosteroids, radiation therapy, compression, embolization and/or pharmacotherapy (e.g. interferon, platelet aggregation inhibitors [radiopaedia.org]
Prognosis in patients with large or abdominal tumors is poor. Mortality is approximately 10% and is attributable to extensive local disease or Kasabach-Merritt phenomenon. [path.upmc.edu]
Etiology
Its etiology and pathogenesis are uncertain. Tufted angioma typically occurs during infancy or early childhood and displays various clinical patterns. [ncbi.nlm.nih.gov]
(Etiology) The exact cause of Tufted Angioma formation is unknown. [dovemed.com]
Etiology Kasabach-Merritt syndrome is usually caused by kaposiform hemangioendothelioma or tufted angioma. Other vascular tumors may be responsible, such as infantile hemangioma. The radiographic features are specific to each vascular tumor. [radiopaedia.org]
The etiology of kaposiform hemangioendothelioma remains unknown. It is not associated with HHV8 infection or Kaposi sarcoma. Tumors often involve soft tissue of extremities as ill-defined, violaceous lesions and may encase normal structures. [path.upmc.edu]
Epidemiology
Her main area of interest is cancer epidemiology, with a particular focus on melanoma and other skin cancers. Nagwa M. Elwan is Professor of Dermatology at the Faculty of Medicine, Tanta University, Egypt. [books.google.com]
[…] pattern Considered to be on the same neoplastic spectrum as kaposiform hemangioendothelioma ; some consider it to be the same entity Terminology Synonyms: Tufted angioma, Nakagawa’s angioblastoma, progressive capillary hemangioma, tufted hemangioma Epidemiology [pathologyoutlines.com]
Vascular markers (CD31 and CD34), vascular endothelial growth factor receptor-3 (VEGFR-3), and lymphatic markers (D2-40 and PROX1) on the neoplastic cells suggest they may be derived from the endothelial cells of lymphatic vessels. [3, 4] Epidemiology [emedicine.medscape.com]
We included a total of nine patients for whom we collected epidemiological data such as sex, age at onset and ethnicity; clinical data such as localisation, appearance, and clinical diagnosis; data on diagnostic testing, such as imaging studies, coagulation [analesdepediatria.org]
Other clinical findings include increase in local sensitivity, and, more rarely, hyperhydrosis and hypertricosis.9 All clinical and epidemiological data pof the present patient agree to those in literature. [scielo.br]
Pathophysiology
Tufted angiomas usually have a benign course, with only rare reports of aggressive behavior or local invasion. [1] However, tufted angiomas seldom self-involute. [2] Pathophysiology The pathogenesis of tufted angiomas is not well understood. [emedicine.medscape.com]
Prevention
Familiarity with distinctive histologic features of tufted angioma should prevent these lesions from being misdiagnosed as either Kaposi's sarcoma or as angiosarcoma. [ncbi.nlm.nih.gov]
Also, post-operative follow-up care with regular screening and check-ups are advised, especially to monitor for any recurrences How can Tufted Angioma be Prevented? Current medical research has not established a method of preventing Tufted Angioma. [dovemed.com]
Consequently, we chose radical excision of the lesion to prevent, if the medical treatment was ineffective, the lesion from reaching critical areas. Various options exist for medical treatment. [elsevier.es]
If a lesion responds to steroid, the dose should be reduced slowly to prevent recurrence. [synapse.koreamed.org]
One can also argue whether an early intervention with interferon for example, may prevent complications such as the Kasabach-Merritt syndrome, or the local aggressive infiltration of this tumor. [nature.com]