Table of Contents
Definition / general | Terminology | Epidemiology | Clinical features | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Differential diagnosisCite this page: Shankar V. Atypical vascular lesion post radiation. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuelymphangiomaradiation.html. Accessed December 31st, 2024.
Definition / general
- Due to obstruction / destruction of lymphatic drainage, often postradiation 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 postsurgery 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
- Multiple papules, small vesicles or erythematous plaques in irradiated field
- Usually benign behavior (Cancer 2007;109:1584, Am J Surg Pathol 2005;29:983), but vascular type may progress to angiosarcoma (Am J Surg Pathol 2008;32:943)
Case reports
- 39 year old woman with enlarging erythema and blisters on axilla (Indian J Dermatol Venereol Leprol 2012;78:121)
- 40 year old woman with multiple vesicles (The Internet Journal of Dermatology 2006;3(2))
- 48 year old woman with acquired progressive lymphangioma of skin following radiotherapy for breast carcinoma (J Cutan Pathol 1995;22:164)
- 50 year old woman with inguinal papules postradiotherapy for uterine rhabdomyosarcoma (Acta Dermatovenerol Alp Panonica Adriat 2009;18:24)
- 68 year old woman with multiple small vesicular lesions on breast (Dermatol Online J 2004;10:9)
- 72 year old woman with lesions associated with ovarian fibroma (J Am Acad Dermatol 2007;56:S41)
- Asymptomatic vesicular eruption on the chest (Arch Dermatol 2011;147:1443)
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
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
Microscopic (histologic) 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
Microscopic (histologic) images
Negative stains
- CD34 (or focally positive), smooth muscle actin (no peripheral ring of stain characteristic of pericytes)
- Ki67
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
- Angiosarcoma - well differentiated: may be part of morphologic continuum with atypical vascular lesions (J Am Acad Dermatol 2007;57:126)
- Hobnail hemangioma
- Kaposi sarcoma: lymphangioma-like variant is HHV8+, not related to radiation therapy
- Lymphangioendothelioma