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Soft Tissue Tumors Part 3

Muscle, Vascular, Nerve, Other

Angioleiomyoma

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

Revised: 4 August 2009, last major update - August 2009

Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.

Definition

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● Benign, painful, subcutaneous or deep dermal tumor of smooth muscle and vessels

● Part of morphologic spectrum with myopericytoma (Hum Pathol 2007;38:645) and myofibroma

● Arises from smooth muscle of blood vessels without elastic fibers

Terminology

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● Also called angiomyoma, vascular leiomyoma

● Do NOT diagnose as “superficial angiomyolipoma” even if it contains some fat

Epidemiology

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● Relatively common, usually females, ages 30-60 years

Clinical

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● Solitary, slow growing nodule

● Often in soft tissue of lower limbs, but tumors in males are more common in upper extremity, head and neck (Laryngoscope 2004;114:661)

● Other painful nodules are glomus tumor, traumatic neuroma, eccrine spiradenoma and angiolipoma

● Pain n 60% (Int J Clin Pract 2004;58:587), due to stretching of nerves in tumor or capsule or release of mediators from mast cells; pain may be exacerbated by wind, cold, pressure, menses

Case reports

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● 51 year old man with nasal turbinate mass (Pathology Case of Week #138)

● 58 year old woman with small intestinal tumor (World J Surg Oncol 2007 Nov 8;5:129)

● 66 year old woman with posterior mediastinal tumor (Yonsei Med J 2008;49:666)

Treatment and prognosis

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● Simple excision; does not recur

Gross description

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● Firm, sharply circumscribed gray-white-brown nodules

● Usually 2 cm or less

Gross images

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Well dermacated tumors

Micro description

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● Well circumscribed fascicles of mature smooth muscle cells surrounding vascular lumina, lined by normal appearing endothelium but with no elastic lamina present

● Subtypes are solid (closely compacted smooth muscle bundles), venous (vessels have thick muscular walls that merge with smooth muscle bundles) and cavernous (dilated vascular channels with minimal smooth muscle that merges with smooth muscle bundles), but subtypes have no clinical significance (Cancer 1984;54:126)

● May have foci of cartilaginous or adipose metaplasia

● May have bizarre degenerative type cells similar to symplastic leiomyoma of uterus

● Rarely prominent calcification in acral sites (J Am Acad Dermatol 2008;59:1000)

● Rarely epithelioid cells (Am J Dermatopathol 1998;20:213)

● No hemorrhage, no necrosis, no mitotic activity, no vasculitis, no fibromuscular dysplasia

Micro images

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Smooth muscle                  Smooth muscle in vessel walls                                      Occasional bizarre

and vessels, often              merges into smooth muscle of lesion                          degenerative type cells

thick walled

Closely compacted eosinophilic smooth muscle cells blended in with blood vessels

Cutaneous tumor

Nasal turbinate mass

Actin

Cytology description

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● Moderate or sparse cellularity

● Usually uniform spindle cells mixed with smooth muscle cells and fragments of collagenous tissue in varying proportions

● Occasional macrophages, fat cells, ganglion-like cells (Diagn Cytopathol 2002;27:161)

Positive stains

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● Alpha smooth muscle actin, desmin

● Also vimentin, type IV collagen, S100 in small nerve fibers

● Variable PR in nasal cavity cases (Acta Otolaryngol 2002;122:408, Chinese Medical Journal 2007;120:350)

Negative stains

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● HMB45, ER

Differential diagnosis

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● Leiomyoma with bizarre nuclei - no prominent vascular component

● Angiomyolipoma - prominent adipose tissue component, HMB45+

End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Angioleiomyoma

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