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
This section has been translated automatically.

Dabska, 1969

Definition
This section has been translated automatically.

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

You might also be interested in

Dermatology

Bulla repens

Acute, vesicular, superficial form of an acute paronychia. Bulla repens can also be described as a special ...

Advertisment

Manifestation
This section has been translated automatically.

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

Localization
This section has been translated automatically.

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

Clinical features
This section has been translated automatically.

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
This section has been translated automatically.

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
This section has been translated automatically.

Retiform Hemangioendothelioma

Progression/forecast
This section has been translated automatically.

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

Literature
This section has been translated automatically.

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