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Soft tissue tumors

Lymphangioma

Lymphangiomatous papules / plaques, post-radiation


Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 26 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

Definition
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● Due to obstruction/destruction of lymphatic drainage, often post-radiation therapy (Histopathology 1999;35:319) or idiopathic in elderly

Terminology
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● 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
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● 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
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● 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
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● 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 post radiotherapy 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
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● 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
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3Axilla and shoulder lesions

Breast: multiple small vesicles

Breast: multiple vesicles of lymphangioma circumscriptum

Chest: asymptomatic vesicular eruption

Scrotum: vesicular rash

Gross description
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● 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
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● 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 / Massonís 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
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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
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● CD31; D2-40 (40%)

Negative stains
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● CD34 (or focally positive), smooth muscle actin (no peripheral ring of stain characteristic of pericytes)
● Ki-67

Electron microscopy description
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● Endothelial cells have cytoplasmic microfilaments and pinocytotic vesicles; intermediate junctions are present between adjacent cells (Jpn J Clin Oncol 1991;21:129)

Differential diagnosis
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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

End of Soft tissue tumors > Lymphangioma > Lymphangiomatous papules / plaques, post-radiation


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