Cutaneous Myxoma and Angiomyxoma

 a.k.a. NAME (nevi, atrial myxomas, myxoid neurofibromas, and ephelides) and LAMB (lentigines, atrial myxomas, and blue nevi) syndromes


image If patient has multiple cutaneous myxomas, should consider Carney complex





Clinical Issues




• Uncommon lesions, which typically present in adults

• Papule, nodule, or polypoid lesion

• Benign but local recurrence common (up to ~ 20%)

• Excision is curative


Microscopic




• Relatively hypocellular proliferation of bland, spindled to stellate fibroblasts

• Abundant myxoid stroma

• Many thin-walled blood vessels present

• Perivascular neutrophils common

• May have associated epithelial proliferation, usually resulting from entrapped adnexal structures


Top Differential Diagnoses




• Focal dermal mucinosis
image Lacks associated vasculature

• Myxoid dermatofibrosarcoma protuberans
image More uniformly cellular

• Myxoid neurofibroma
image May show abundant myxoid stroma, but areas of collagenous stroma also present

• Myxoid neurothekeoma (dermal nerve sheath myxoma)

• Superficial acral fibromyxoma

image
Cutaneous Myxoma
Cutaneous myxomas are dermal-based tumors characterized by a hypocellular proliferation of bland, spindled cells and abundant myxoid stroma.


image
Cutaneous Myxoma With Prominent Myxoid Stroma and Vessels
Intermediate magnification shows that the spindled cells are associated with a capillary vasculature image. Often, there are subtly more cellular image and less cellular image areas.

image
Cutaneous Myxoma With Basaloid Proliferation
Angiomyxomas may induce a superficial proliferation of basaloid epithelium image, similar to dermatofibroma.

image
High Magnification of Myxoma With Stromal Neutrophils
Stromal neutrophils image are often present within cutaneous myxomas.


TERMINOLOGY


Synonyms




• Superficial angiomyxoma


Definitions




• Benign, mesenchymal proliferation of spindled to stellate fibroblasts in abundant myxoid stroma


ETIOLOGY/PATHOGENESIS


Some Cases Associated With Carney Complex




• Carney complex
image a.k.a. NAME (nevi, atrial myxomas, myxoid neurofibromas, and ephelides) and LAMB (lentigines, atrial myxomas, and blue nevi) syndromes

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Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Cutaneous Myxoma and Angiomyxoma

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