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Chap 11 - MYXOMAS AND OSSIFYING FIBROMYXOID TUMOR

Published online by Cambridge University Press:  01 March 2011

By
Markku Miettinen
Edited by
Markku Miettinen
Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

Myxomas encompass a heterogeneous group of clinicopathologic entities, common to which is prominent content of extracellular mucin. Soft tissue myxomas are fibroblastic-myofibroblastic neoplasms, or in some cases possibly non-neoplastic proliferations.

Cardiac myxoma is thought to originate from primitive, multipotential endocardial cells. In addition to myxomas, ossifying fibromyxoid tumor, a mesenchymal tumor of uncertain histogenesis with potentially malignant variants, is discussed in this chapter.

Common to all myxomas is the production of a mucoid intercellular substance by the fibroblastic or myofibroblastic tumor cells. Although the chemical composition of the mucins is known to vary, its analysis is not of great practical significance. The constituents include glycoproteins and proteolgycans that include neutral and acidic carbohydrate components, such as hyaluronic acid.

The apparent character of the mucoid material on hematoxylin and eosin (HE) stain depends not only on its composition but also on fixation and staining. The mucinophilic (metachromatic) quality of HE stains varies. Mucin also becomes progressively extracted into the fixative over time, and therefore specimens kept longer in the fixative typically lose mucins and the stain is less metachromatic.

INTRAMUSCULAR MYXOMA

Clinical Features

This relatively rare tumor typically presents in middle-aged adults between 40 and 70 years, with a 2:1 female predominance (Fig. 11.1). Nearly one half of the lesions occur in the thigh, with the arm and buttocks being other common locations. A wide variety of extremity locations and chest wall are among other possible sites (Fig. 11.2).

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Chapter
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Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 308 - 334
Publisher: Cambridge University Press
Print publication year: 2010

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References

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Shenker, A, Weinstein, LS, Sweet, , Spiegel, AM. An activating Gs Alpha mutation is present in fibrous dysplasia of bone in the McCune-Albright syndrome. J Clin Endocr Metab 1994;79:750–755.Google ScholarPubMed
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Berker, D, Lawrence, C. Ganglion of the distal interphalangeal joint (myxoid cyst). Arch Dermatol 2001;137:607–610.Google Scholar
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Carney, JA. Carney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Semin Dermatol 1995;14:90–98.CrossRefGoogle ScholarPubMed
Boikos, SA, Stratakis, CA. Carney complex: the first 20 years. Curr Opin Oncol 2007;19:24–29.CrossRefGoogle ScholarPubMed
Atherton, DJ, Pitcher, DW, Wells, RS, MacDonald, DM. A syndrome of various cutaneous pigmented lesions, myxoid neurofibromata, and atrial myxoma: the NAME syndrome. Br J Dermatol 1980;103:421–429.CrossRefGoogle ScholarPubMed
Koopman, RJJ, Happle, R. Autosomal dominant transmission of the NAME syndrome (nevi, atrial myxoma, mucinosis of the skin and endocrine overactivity). Hum Genet 1991;86:300–304.CrossRefGoogle Scholar
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Calonje, E, Guerin, D, McCormick, D, Fletcher, CD. Superficial angiomyxoma: clinico-pathologic analysis of a series of a distinctive but poorly recognized cutaneous tumors with tendency for recurrence. Am J Surg Pathol 1999;23:910–917.CrossRefGoogle Scholar
Goldstein, MM, Casey, M, Carney, JA, Basson, CT. Molecular genetic diagnosis of the familial myxoma syndrome (Carney complex). Am J Med Genet 1999;86:62–65.3.0.CO;2-1>CrossRefGoogle Scholar
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