Papillary intralymphatic angioendothelioma
Citation, DOI, disclosures and article data
At the time the article was created Daniel J Bell had no recorded disclosures.View Daniel J Bell's current disclosures
At the time the article was last revised Daniel J Bell had no recorded disclosures.View Daniel J Bell's current disclosures
Papillary intralymphatic angioendothelioma (PILA) (also known - especially historically - as a Dabska tumor) is a rare, low-grade soft tissue tumor 1. The lesion is borderline-malignant and metastasis is distinctly unusual.
On this page:
Less than 40 cases have been reported in the global literature 1. In this small cohort it was generally a disease of children and young adults, although it has been seen in the elderly. No gender bias has been identified.
Most patients present with a steadily enlarging skin/subcutaneous nodule, usually maximizing at 2-3 cm. Occasionally they lesions can be painful 5. Nodal metastasis is very rare.
Papillary intralymphatic angioendothelioma is a neoplasm of vascular origin with distinctive papillary formations of atypical endothelial cells.
The cause of papillary intralymphatic angioendotheliomas remains unknown.
Greater than 90% of the reported cases were in the skin or subcutaneous soft tissue, with the exception of three cases localized to the bone 1. Most cases are sited in the extremities, a truncal or head and neck location is less usual. Occasional cases have been found deeper in the body, including spleen, testis and tongue 1,5.
Soft tissue papillary intralymphatic angioendotheliomas may be seen incidentally, but generally imaging has little role to play.
A recent case report of an extremely rare bone-based lesion presented as several well-circumscribed lucent lesions, each looking similar to an osteoid osteoma 1.
Treatment and prognosis
Surgical excision is the standard approach and in general prognosis is extremely good.
History and etymology
This entity was first described in 1969 by the distinguished Polish pathologist, Maria Dąbska (1921-2014) who was a leading member of the "Polish School of Pathology". It is (sadly) thought to be one of only two neoplasms named after a female scientist (the other is a Spitz nevus) 2-4.
NB: Dąbska is written using a diacritic under the a, which resembles a tail, giving it a nasal pronunciation 2
- 1. Gambarotti M, Righi A, Sbaraglia M, Bianchi G, Picci P, Vanel D, Tos APD. Intraosseous papillary intralymphatic angioendothelioma (PILA): one new case and review of the literature. (2018) Clinical sarcoma research. 8: 1. doi:10.1186/s13569-018-0087-9 - Pubmed
- 2. Schwartz RA, Janniger EJ. On being a pathologist: Maria Dąbska--the woman behind the eponym, a pioneer in pathology. (2011) Human pathology. 42 (7): 913-7. doi:10.1016/j.humpath.2011.04.006 - Pubmed
- 3. Nasierowska-Guttmejer A. In memoriam professor Maria Dabska (20.07.1921- 20.07.2014). (2015) Polish journal of pathology : official journal of the Polish Society of Pathologists. 66 (1): 1-2. Pubmed
- 4. Dabska M. Malignant endovascular papillary angioendothelioma of the skin in childhood. Clinicopathologic study of 6 cases. (1969) Cancer. 24 (3): 503-10. Pubmed
- 5. Schultheis AM, Sandmann M, Steurer S. Strong ERG Positivity in Papillary Intralymphatic Angioendothelioma of the Testis of a 24-Year-Old Male: A Case Report. (2013) Case reports in pathology. 2013: 531479. doi:10.1155/2013/531479 - Pubmed