Papillary intralymphatic angioendothelioma C49.-

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 07.08.2021

Synonym(s)

Angioendothelioma papillary intralymphatic; dabska tumor; Endovascular papillary angioendothelioma; Hemangioendothelioma type Dabska; malignant endovascular papillary angioendothelioma; Papillary intralymphatic angioendothelioma

History
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Dabska, 1969

Definition
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Extremely rare, intralymphatically growing vascular tumor of childhood of unclear dignity.

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Manifestation
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Occurs mainly in early childhood; less frequently congenital; less frequently in older patients (Ø 30 years).

Localization
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Head, neck, neck, torso; more rarely extremities.

Clinical features
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Dermatologically uncharacteristic. Firm, red to blue-red nodular or flat tuberous swellings of the skin and subcutaneous fat tissue up to 40 cm Ø. Frequently arising in pre-existent lymphatic malformations or in lymphangiomas.

Histology
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Intralymphatic proliferates located in the dermis or subcutis but also affecting internal organ systems (e.g. bone structures) consisting of grape-shaped papillae (dabskoid tufts) with a homogeneous connective tissue base (immunoreactive for collagen type IV) covered with hobnail endothelia . This results in a glomeruloid (kidney glomerula-like) aspect. Focal lymphoid cell infiltrates.

Immunohistology: Smooth muscle actin-positive pericytes are absent (suggests lymphatic genesis); endothelia express among others VEGFR-3 (marker for lymphatic endothelia) and podoplanin.

Differential diagnosis
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Retiform Hemangioendothelioma

Progression/forecast
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Dignity doubtful. After surgical removal high recurrence rate (40%); regional lymph node metastases are possible but rare, very rare distant metastases.

Literature
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Chundriger Q et al (2021) Retiform hemangioendothelioma: a case series and review of the literature. J Med Case Rep 15:69.
Dabska M et al (1969) Malignant endovascular papillary angioendothelioma of the skin in childhood. Clinicopathologic study of 6 cases. Cancer 24: 503-510
Fanburg-Smith JC et al (1999) Papillary intralymphatic angioendothelioma (PILA): a report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels. Am J Surg Pathol 23: 1004-1010
Ide F et al (2002) Endovascular papillary hemangioma of the lip.J Oral Pathol Med 31: 565-566.
Manivel JC et al (1986) Endovascular papillary angioendothelioma of childhood: a vascular lesion possibly characterized by high endothelial cell differentiation. Hum Pathol 17: 1240-1244
Quecedo E et al (1996) Dabska tumor developing within a preexisting vascular malformation. Am J Dermatopathol 18: 302-307
Schwartz RA et al (2001) The Dabska tumor: a thirty-year retrospect. Dermatology 201: 1-5
Takaoka K et al (2003) Endovascular papillary angioendothelioma (Dabska tumor) of the tongue: report of a case. J Oral Pathol Med 32: 492-495