Soft tissue
Lymphangioma
Lymphangiomatous papules / plaques, post radiation

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 30 September 2016, last major update November 2012

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed search: lymphangioma papules OR plaques post-radiation

Cite this page: Lymphangiomatous papules / plaques, post radiation. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuelymphangiomaradiation.html. Accessed December 2nd, 2016.
Definition / General
  • Due to obstruction / destruction of lymphatic drainage, often post radiation therapy (Histopathology 1999;35:319) or idiopathic in elderly
Terminology
  • In breast, often called lymphangioma circumscriptum, although the classic lesion of lymphangioma circumscriptum appears at birth or in the early years, and most breast cases are actually post surgery or radiation therapy within the field of radiation (Am J Clin Pathol 1994;102:757)
Epidemiology
  • In women, age 33 - 72 years
  • Arises 3 - 20 years after radiation therapy for breast (93%) or ovarian (7%) carcinoma
  • Associated with pain, chronic drainage, cellulitis (South Med J 1999;92:69)
Clinical Features
Case Reports
Treatment
  • Cryosurgery, electrocautery or laser therapy to vaporize surface lymphatics (Dermatol Surg 1998;24:893); frequently recurs and causes substantial morbidity so must monitor for recurrence
Clinical Images
Images hosted on other servers:

Axilla and shoulder lesions

Breast: multiple small vesicles


Breast: multiple vesicles of lymphangioma circumscriptum

Chest: asymptomatic vesicular eruption

Scrotum: vesicular rash

Gross Description
  • One or more circumscribed papules, small vesicles or erythematous plaques, usually in irradiated field, median 0.5 cm
  • Frequently multiple synchronous lesions with discoloration
Micro Description
  • Irregular dilated vascular spaces with branching and anastomosing pattern in superficial and deep dermis
  • Thin walls, lymphatic appearance
  • Vascular channels lined by single discontinuous layer of endothelial cells with numerous small stromal papillary formations and flattened nuclei; also lined by endothelial cells projecting into lumina (lymphatic counterpart of intravascular papillary endothelial hyperplasia / Masson’s tumor)
  • May have poorly circumscribed and focally infiltrating irregular jagged vascular spaces involving the entire dermis, lined by inconspicuous endothelial cells, dissecting collagen bundles of the dermis and mimicking Kaposi's sarcoma (Am J Surg Pathol 2002;26:328)
  • No necrosis, no mitotic figures, no blood lakes

  • Lymphatic type: predominantly thin walled, variably anastomosing lymphatics primarily in superficial dermis
  • Vascular type: predominantly small, irregularly dispersed, capillary type vessels, invested by pericytes, often blood filled, in superficial or deep dermis; associated with extravasated red blood cells or hemosiderin, minor lymphatic type component
Micro Images

Figures 1 and 2: benign lymphangio- endothelioma- like lesions

Figures 3 and 4: superficial lymphangioma- like patterns

Figure 5: mixed histologic patterns

Figure 6 and 7: nuclear hyperchromasia and prominent papillary projections

Figure 8: D2-40+ lymphangioma-like lesion


Breast: ectatic lymphatic spaces in papillary dermis

Chest: asymptomatic vesicular eruption #1

#2

Positive Stains
Negative Stains
Electron Microscopy Description
  • Endothelial cells have cytoplasmic microfilaments and pinocytotic vesicles; intermediate junctions are present between adjacent cells (Jpn J Clin Oncol 1991;21:129)
Differential Diagnosis