Superficial Angiomyxoma

Abstract

Despite being first described in 1988, superficial angiomyxoma is still a poorly recognized cutaneous tumor. Although its histologic features are distinctive, its existence seems not to be widely accepted. We analyzed the clinicopathologic and immunohistochemical features in a series of 39 cases. Twenty-five patients were males; age range was birth to 82 years (median, 45.5 years). Most cases presented as cutaneous papules, nodules, or polypoid lesions. Seventeen tumors arose on the trunk, 14 on the head and neck, and seven on the lower limbs. All cases were treated by local excision, and eight recurred locally. In four of the latter cases, there were two recurrences. Histologically, the lesions were dermal with variable involvement of the subcutis. Tumors were poorly circumscribed, but a focal lobular outline was always identified. Distinctive histologic features included extensive myxoid stroma, numerous small blood vessels, varying cellularity, acellular mucin pools, stellate or bipolar fibroblastic cells, muciphages, a sparse, mixed inflammatory cell infiltrate with notable neutrophils, and occasional plumper cells with eosinophilic cytoplasm. Cytologic atypia was mild at most, and mitotic figures were rare. In approximately 20% of cases, the primary lesion or its recurrence contained epithelial structures, including epidermoid cysts, thin strands of squamous epithelium, and small buds of basaloid cells. Immunohistochemically, tumor cells were negative for S-100 protein, smooth muscle actin, and pan-keratin. We support the concept of superficial angiomyxoma as a distinctive clinicopathologic entity that should be included in the differential diagnosis of other myxoid cutaneous tumors, including dermal nerve sheath myxoma, trichodiscoma and trichofolliculoma, and low-grade myxofibrosarcoma.

Superficial Angiomyxoma causing Torsion of an Extra-Digit

“…[1] A lesion in an extra digit, as reported here, of a newborn is rare and to date no reports have been found to exist in the literature. To our knowledge, no metastasis or malignancy of superficial angiomyxoma has been reported.…”

Solitary superficial acral angiomyxoma: An infrequently reported soft tissue tumor

“…Cutaneous focal mucinosis is largely acellular with very few blood cells, and it lacks a lobular architecture and stromal neutrophils, thus those findings do not correlate with the features observed in our patient's lesion. [2] Another myxoid lesion that can occur in the finger, namely, acral persistent mucinosis, can be easily ruled out because of its characteristic symmetrical distribution of multiple papular lesions on the back of the hands and forearms. In conclusion, the features most useful to differentiate a SA from other myxoid tumors include its superficial location, lack of atypia, stromal inflammatory infiltrate, and a frequent association with an entrapped epithelial component.…”

“…[12] Our patient had a recurrence after 3 months of excision possibly because of incomplete removal of the tumor. Close follow up is also mandated since SA can represent the earliest and only manifestation of the Carney's syndrome.…”

“…Originally proposed to be synonymous with cutaneous focal mucinosis, trichofolliculomas and trichodiscomas, Calonje et al . [2] disagreed with this hypothesis concluding that SA was a distinct entity. They have only been infrequently described on the digit mimicking a digital myxoid cyst.…”

A Case Report on Angiomyxoma

“…Tumour size is often underestimated by physical examination. Most AA are big, size may vary from 1-60 cm 3 . These tumours are macroscopically lobulated and may adhere to surrounding soft tissue.…”