Soft Tissue Tumors 3 - Lymphangioma

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Soft Tissue Tumors Part 3

Muscle, Vascular, Nerve, Other

Lymphangiomatous papules / plaques, post-radiation

Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.

Revised: 11 October 2009, last major update - September 2009

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 (AJCP 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

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● Multiple papules, small vesicles or erythematous plaques in irradiated field

● Usually benign behavior (Cancer 2007;109:1584, AJSP 2005;29:983), but vascular type may progress to angiosarcoma (AJSP 2008;32:943)

Case reports

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● 40 year old woman with multiple vesicles (The Internet Journal of Dermatology 2006;3(2))

● 48 year old woman (J Cutan Pathol 1995;22:164)

● 50 year old woman (Acta Dermatovenerol Alp Panonica Adriat 2009;18:24)

● 68 year old woman (Dermatology Online Journal 10(1):9)

● 72 year old woman with lesions associated with ovarian fibroma (J Am Acad Dermatol 2007;56:S41)

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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Breast vesicles

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 / 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 (AJSP 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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Papillary dermis shows Superficial dermal lesion with slit-like vascular

ectatic vascular space spaces with pseudoangiosarcomatous pattern

Mixed patterns: (a) dilated vascular Lymphangioma circumscriptum-like: dilated vascular

spaces in the superficial dermis; spaces with proteinaceous material in superficial dermis,

(b) slit-like vascular spaces dissect resulting in a dome-shaped, exophytic papule

collagen fibers in deep dermis

Dilated vascular channels with small Narrow vascular spaces and

stromal projections into lumina moderate chronic inflammation

(a) Well-circumscribed, dermal, vascular proliferation with lymphangioendothelioma-like appearance

(b) Some vascular spaces are lined by endothelial cells with enlarged, hyperchromatic nuclei

Prominent papillary projections lined Lymphangioma-like lesion: slit-like vascular

by nonhyperchromatic endothelial spaces lined by D2-40+ endothelial cells

cells, resembling Dabska tumor/

retiform hemangioendothelioma

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

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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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● Benign lymphangioendothelioma

● Hobnail hemangioma

● Kaposi’s sarcoma-lymphangioma-like variant: HHV8+, not related to radiation therapy

● Well differentiated angiosarcoma - may be part of morphologic continuum with atypical vascular lesions (J Am Acad Dermatol 2007;57:126)

End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Lymphangioma > Lymphangiomatous papules / plaques, post-radiation

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