Hemangioma tufted D18.01

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 05.08.2021

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Synonym(s)

hypertrophic hemangioma; progressive capillary hemangioma; tufted angioma

History
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Nakagawa, 1949; Macmillan, 1971

Definition
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Variant of lobular capillary hemangioma with tufted angioma nodules without tendency to spontaneous regression. The "tufted angioma" has the same growth pattern and dignity as the"caposiform hemangioendothelioma", i.e. it is a semi-malignant locally aggressive growing vascular tumor (slightly less local aggressiveness). It may transform into a caposiform hemangioendothelioma .

Etiopathogenesis
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Probably autosomal dominant inheritance.

Manifestation
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Predominantly congenital or acquired in the first years of life, more rarely occurring in later life or during pregnancy (in this case spontaneous regression after birth) or under immunosuppressive therapy (e.g. after liver transplantation).

Localization
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Head, neck, upper torso.

Clinical features
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Onset with multiple, small, smooth-surfaced, red, soft papules that slowly enlarge and persist at a certain size. Frequently accompanied by painfulness and hyperhidrosis (about 30% of cases). Spontaneous regression is extremely rare.

Histology
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Multicentric pattern of nodular, mostly solid capillary, roundish-oval vascular convolutes, which penetrate the entire dermis, possibly also the subcutis, in a cluster-like manner (cannonball pattern). Capillary vascular clusters partially surrounded by crescentic vascular clefts. Endothelial atypia, papillae (multilayering) absent. Only scattered mitoses.

Immunohistology: positive for CD31 and Glut-1.

Differential diagnosis
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Kaposi-form infantile hemangioma, glomeruloid hemangioma, Kaposi's sarcoma.

Therapy
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Literature
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  1. Bernstein EF et al (1994) Tufted angioma of the thigh. J Am Acad Dermatol 31: 307-311
  2. Chu CY et al (2003) Transformation between Kaposiform Hemangioendothelioma and Tufted Angioma. Dermatology 206: 334-337
  3. Fließer M et al (2017) Acute complications of vascular anomalies in children. Dermatologist 68: 792-795
  4. Herron MD et al (2002) Tufted angiomas: variability of the clinical morphology. Pediatrist Dermatol 19: 394-401
  5. Mentzel T et al (1996) Tufted angioma. Dermatologist 47: 369-375
  6. Jones EW et al (1976) Malignant vascular tumors. Clin Exp Dermatol 1: 287-312
  7. Macmillan A (1971) Progressive capillary haemangioma. Br J Dermatol 85: 492-493
  8. Mahendran R (2002) Response of childhood tufted angioma to the pulsed-dye laser. J Am Acad Dermatol 47: 620-622
  9. Nakagawa K (1949) Case report of angioblastoma of the skin. Jpn J Dermatol 59: 92-94
  10. Full EC (2002) Congenital tufted angioma. Pediatric Dermatol 19: 445-447
  11. Tille JC (2003) Familial predisposition to tufted angioma: identification of blood and lymphatic vascular components. Clin Genet 63: 393-399
  12. Wong SN (2002) Tufted angioma: a report of five cases. Pediatric Dermatol 19: 388-393

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Last updated on: 05.08.2021