Soft tissue tumors - Lymphangiomyoma

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Soft tissue tumors

Lymphangioma

Lymphangiomyoma

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 25 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Benign, most commonly in women, very rarely males
● Formerly known as lymphangiopericytoma
● Localized form present in mediastinum and retroperitoneum, associated with thoracic duct, causes chylothorax, chylous ascities, chyluria, rarely chylopericardium (Korean J Thorac Cardiovasc Surg 2011;44:377)
● Rarely found in kidney as a multiloculated mass (AJR Am J Roentgenol 1989;152:307)
● Diffuse form is lymphangioleiomyomatosis

Case reports
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● 26 year old woman with painless shoulder mass (Acta Orthop Belg 2008;74:114)

Treatment
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● Progesterone, oophorectomy

Micro description
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● Proliferation of intermingled blood vessels and smooth muscle
● Tumor cells plumper and paler than leiomyoma

Micro images
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Slit-like vascular spaces and lymphoid tissue

Positive stains
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● Actin, desmin, HMB45

Differential diagnosis
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● Angioleiomyoma

End of Soft tissue tumors > Lymphangioma > Lymphangiomyoma

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