Papillary intralymphatic angioendothelioma (PILA)

A report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels

Julie Fanburg-Smith, Michal Michal, Taina A. Partanen, Kari Alitalo, Markku Miettinen

Research output: Contribution to journalArticle

83 Citations (Scopus)

Abstract

Six childhood vascular tumors were designated as 'malignant endovascular papillary angioendothelioma' by Dabska in 1969. Since then, a few reports of similar cases were published, often called 'Dabska tumors.' Twelve similar cases were identified in review of vascular tumors from the authors' institutions. There were five men and seven women, including seven adults. Patient ages ranged from 8 to 59 years (mean, 30 years). The tumors occurred in the dermis or subcutis of the buttocks or thigh (n = 6), thumb or hand (n = 3), abdomen (n = 2), and heel (n = 1). The tumor sizes ranged from 1 to more than 40 cm (mean, 7.0 cm). The unifying feature of all cases was distinctive intravascular growth of well-differentiated endothelial cells presenting as a matchstick columnar configuration, sometimes with a large production of matrix that was positive for collagen type IV. In half the cases, these intravascular proliferations had an associated actin-positive pericytic proliferation. There was minimal cytologic atypia and rare to absent mitotic activity. Two cases had an adjacent lymphangioma, and two additional cases had clusters of lymphatic vessels adjacent to the tumor. All but two of the cases showed varying degrees of stromal or intraluminal lymphocytes. Occasional epithelioid endothelial cells were seen, but no cases had features typical of epithelioid, spindle cell, or retiform hemangioendothelioma. Tumor cells were positive for vimentin, von Willebrand factor, CD31, and locally for CD34 and were negative for keratins, epithelial membrane antigen, S-100 protein, and desmin. Vascular endothelial cell growth factor receptor type 3, a recently introduced marker for lymphatic endothelia, was positive in all eight cases that were studied, supporting a lymphatic phenotype. Follow-up in 8 of the 12 cases showed no evidence of recurrences, metastases, or residual disease during follow-ups ranging from 1 to 17 years (mean, 9 years). Based on the proliferative borderline features and the lymphatic phenotype, we propose to designate these tumors as papillary intralymphatic angioendothelioma. Additional cases with extensive follow-up should be studied to rule out variants with malignant potential.

Original languageEnglish (US)
Pages (from-to)1004-1010
Number of pages7
JournalAmerican Journal of Surgical Pathology
Volume23
Issue number9
DOIs
StatePublished - Sep 1 1999

Fingerprint

Hemangioendothelioma
Lymphatic Vessels
Blood Vessels
Neoplasms
Epithelioid Cells
S100 Proteins
Endothelial Cells
Lymphatic Endothelium
Vascular Endothelial Growth Factor Receptor-3
Phenotype
Lymphangioma
Mucin-1
Buttocks
Vascular Endothelial Growth Factor Receptor
Desmin
Collagen Type IV
Heel
Thumb
von Willebrand Factor
Vimentin

All Science Journal Classification (ASJC) codes

  • Anatomy
  • Pathology and Forensic Medicine

Cite this

@article{dbbea5fcfb2b4bf1af4cb096b7537565,
title = "Papillary intralymphatic angioendothelioma (PILA): A report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels",
abstract = "Six childhood vascular tumors were designated as 'malignant endovascular papillary angioendothelioma' by Dabska in 1969. Since then, a few reports of similar cases were published, often called 'Dabska tumors.' Twelve similar cases were identified in review of vascular tumors from the authors' institutions. There were five men and seven women, including seven adults. Patient ages ranged from 8 to 59 years (mean, 30 years). The tumors occurred in the dermis or subcutis of the buttocks or thigh (n = 6), thumb or hand (n = 3), abdomen (n = 2), and heel (n = 1). The tumor sizes ranged from 1 to more than 40 cm (mean, 7.0 cm). The unifying feature of all cases was distinctive intravascular growth of well-differentiated endothelial cells presenting as a matchstick columnar configuration, sometimes with a large production of matrix that was positive for collagen type IV. In half the cases, these intravascular proliferations had an associated actin-positive pericytic proliferation. There was minimal cytologic atypia and rare to absent mitotic activity. Two cases had an adjacent lymphangioma, and two additional cases had clusters of lymphatic vessels adjacent to the tumor. All but two of the cases showed varying degrees of stromal or intraluminal lymphocytes. Occasional epithelioid endothelial cells were seen, but no cases had features typical of epithelioid, spindle cell, or retiform hemangioendothelioma. Tumor cells were positive for vimentin, von Willebrand factor, CD31, and locally for CD34 and were negative for keratins, epithelial membrane antigen, S-100 protein, and desmin. Vascular endothelial cell growth factor receptor type 3, a recently introduced marker for lymphatic endothelia, was positive in all eight cases that were studied, supporting a lymphatic phenotype. Follow-up in 8 of the 12 cases showed no evidence of recurrences, metastases, or residual disease during follow-ups ranging from 1 to 17 years (mean, 9 years). Based on the proliferative borderline features and the lymphatic phenotype, we propose to designate these tumors as papillary intralymphatic angioendothelioma. Additional cases with extensive follow-up should be studied to rule out variants with malignant potential.",
author = "Julie Fanburg-Smith and Michal Michal and Partanen, {Taina A.} and Kari Alitalo and Markku Miettinen",
year = "1999",
month = "9",
day = "1",
doi = "10.1097/00000478-199909000-00002",
language = "English (US)",
volume = "23",
pages = "1004--1010",
journal = "American Journal of Surgical Pathology",
issn = "0147-5185",
publisher = "Lippincott Williams and Wilkins",
number = "9",

}

Papillary intralymphatic angioendothelioma (PILA) : A report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels. / Fanburg-Smith, Julie; Michal, Michal; Partanen, Taina A.; Alitalo, Kari; Miettinen, Markku.

In: American Journal of Surgical Pathology, Vol. 23, No. 9, 01.09.1999, p. 1004-1010.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Papillary intralymphatic angioendothelioma (PILA)

T2 - A report of twelve cases of a distinctive vascular tumor with phenotypic features of lymphatic vessels

AU - Fanburg-Smith, Julie

AU - Michal, Michal

AU - Partanen, Taina A.

AU - Alitalo, Kari

AU - Miettinen, Markku

PY - 1999/9/1

Y1 - 1999/9/1

N2 - Six childhood vascular tumors were designated as 'malignant endovascular papillary angioendothelioma' by Dabska in 1969. Since then, a few reports of similar cases were published, often called 'Dabska tumors.' Twelve similar cases were identified in review of vascular tumors from the authors' institutions. There were five men and seven women, including seven adults. Patient ages ranged from 8 to 59 years (mean, 30 years). The tumors occurred in the dermis or subcutis of the buttocks or thigh (n = 6), thumb or hand (n = 3), abdomen (n = 2), and heel (n = 1). The tumor sizes ranged from 1 to more than 40 cm (mean, 7.0 cm). The unifying feature of all cases was distinctive intravascular growth of well-differentiated endothelial cells presenting as a matchstick columnar configuration, sometimes with a large production of matrix that was positive for collagen type IV. In half the cases, these intravascular proliferations had an associated actin-positive pericytic proliferation. There was minimal cytologic atypia and rare to absent mitotic activity. Two cases had an adjacent lymphangioma, and two additional cases had clusters of lymphatic vessels adjacent to the tumor. All but two of the cases showed varying degrees of stromal or intraluminal lymphocytes. Occasional epithelioid endothelial cells were seen, but no cases had features typical of epithelioid, spindle cell, or retiform hemangioendothelioma. Tumor cells were positive for vimentin, von Willebrand factor, CD31, and locally for CD34 and were negative for keratins, epithelial membrane antigen, S-100 protein, and desmin. Vascular endothelial cell growth factor receptor type 3, a recently introduced marker for lymphatic endothelia, was positive in all eight cases that were studied, supporting a lymphatic phenotype. Follow-up in 8 of the 12 cases showed no evidence of recurrences, metastases, or residual disease during follow-ups ranging from 1 to 17 years (mean, 9 years). Based on the proliferative borderline features and the lymphatic phenotype, we propose to designate these tumors as papillary intralymphatic angioendothelioma. Additional cases with extensive follow-up should be studied to rule out variants with malignant potential.

AB - Six childhood vascular tumors were designated as 'malignant endovascular papillary angioendothelioma' by Dabska in 1969. Since then, a few reports of similar cases were published, often called 'Dabska tumors.' Twelve similar cases were identified in review of vascular tumors from the authors' institutions. There were five men and seven women, including seven adults. Patient ages ranged from 8 to 59 years (mean, 30 years). The tumors occurred in the dermis or subcutis of the buttocks or thigh (n = 6), thumb or hand (n = 3), abdomen (n = 2), and heel (n = 1). The tumor sizes ranged from 1 to more than 40 cm (mean, 7.0 cm). The unifying feature of all cases was distinctive intravascular growth of well-differentiated endothelial cells presenting as a matchstick columnar configuration, sometimes with a large production of matrix that was positive for collagen type IV. In half the cases, these intravascular proliferations had an associated actin-positive pericytic proliferation. There was minimal cytologic atypia and rare to absent mitotic activity. Two cases had an adjacent lymphangioma, and two additional cases had clusters of lymphatic vessels adjacent to the tumor. All but two of the cases showed varying degrees of stromal or intraluminal lymphocytes. Occasional epithelioid endothelial cells were seen, but no cases had features typical of epithelioid, spindle cell, or retiform hemangioendothelioma. Tumor cells were positive for vimentin, von Willebrand factor, CD31, and locally for CD34 and were negative for keratins, epithelial membrane antigen, S-100 protein, and desmin. Vascular endothelial cell growth factor receptor type 3, a recently introduced marker for lymphatic endothelia, was positive in all eight cases that were studied, supporting a lymphatic phenotype. Follow-up in 8 of the 12 cases showed no evidence of recurrences, metastases, or residual disease during follow-ups ranging from 1 to 17 years (mean, 9 years). Based on the proliferative borderline features and the lymphatic phenotype, we propose to designate these tumors as papillary intralymphatic angioendothelioma. Additional cases with extensive follow-up should be studied to rule out variants with malignant potential.

UR - http://www.scopus.com/inward/record.url?scp=0032823030&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0032823030&partnerID=8YFLogxK

U2 - 10.1097/00000478-199909000-00002

DO - 10.1097/00000478-199909000-00002

M3 - Article

VL - 23

SP - 1004

EP - 1010

JO - American Journal of Surgical Pathology

JF - American Journal of Surgical Pathology

SN - 0147-5185

IS - 9

ER -