Background

This soft tissue tumor must be distinguished from its deep soft tissue counterpart. It most commonly presents as a solitary and slowly growing subcutaneous nodule ranging in size from 3-4 cm. It arises in the trunk, genital region, head and neck, and limbs. There is a definite association with the Carney Complex.

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGY	CHARACTERIZATION
AGE RANGE-MEDIAN	4-6th decades Rare cases in children
CONGENITAL
Congenital superficial angiomyxoma. Bedlow AJ, Sampson SA, Holden CA. Department of Dermatology, St. Helier Hospital, Carshalton, Surrey, UK.	Clin Exp Dermatol 1997 Sep;22(5):237-9 Abstract quote We describe a 12-year-old girl who presented with a nodular swelling in the midline of her scalp that had been present since birth. Histological examination revealed an ill-defined myxoid lesion within the dermis, comprising spindle cells, blood vessels and occasional multi-nucleate giant cells. Immunohistochemical staining was negative for S-100, cytokeratin and smooth muscle actin, but focally positive for CD34. Our patient is unusual in that the angiomyxoma was present at birth, which has not previously been described. The importance of screening patients with cutaneous myxomas for cardiac lesions is discussed.
SEX (M:F)	Equal

 

DISEASE ASSOCIATIONS	CHARACTERIZATION
CARNEY'S COMPLEX	Arch Dermatol 1986;122:790-798 Multiple tumors
Carney complex: report of a Japanese case associated with cutaneous superficial angiomyxomas, labial lentigines, and a pituitary adenoma. Takahashi H, Hida T. Department of Dermatology, Sapporo Kosei General Hospital, Sapporo, Japan.	J Dermatol. 2002 Dec;29(12):790-6. Abstract quote   We report the case of a 12-year-old female patient who manifested multiple cutaneous angiomyxomas and labial pigmented lesions. Although the familial history was not confirmed in the present case, autosomal dominant inheritance has been reported to be involved in the pathogenesis of this condition. In addition to the cutaneous complications, magnetic resonance (MR) images revealed the presence of a pituitary adenoma, which provoked an elevation of serum growth hormone (GH) level. On the other hand, no significant symptoms such as cardiac myxoma, myxoid fibroadenoma of the breast, or adrenocortical complaints suggesting Cushing syndrome, were detected. In the Japanese literature, only a few cases of this disorder have been described in the form of brief reports. There have been only a few similar cases described in the dermatological field, except for one report diagnosed as Carney complex in 1990. Therefore, the present case seems to be the first Japanese case of typical Carney complex manifesting major clinical complications, including angiomyxomas, lentigines, and a pituitary adenoma, which induced endocrine overactivity.
Myxomas of the external ear and their significance Ferreiro JA and Carney JA.	Am J Surg Pathol 1994;18:274-280 If these tumors occur here, it is almost pathognomonic of Carney's complex

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
EYELID
Solitary Superficial Angiomyxoma in the Eyelid. Yuen HK, Cheuk W, Luk FO, Wat CS, Auyeung KC, Lam DS. Department of Ophthalmology & Visual Sciences, The Chinese University of Hong Kong, Hong Kong Eye Hospital, Kowloon, Hong Kong, SAR, People's Republic of China; Hong Kong Eye Hospital, Hospital Authority Ophthalmic Services, Hong Kong, SAR, People's Republic of China.	Am J Ophthalmol. 2005 Jun;139(6):1141-1142. Abstract quote   PURPOSE: To report the clinicopathologic features of a solitary superficial angiomyxoma arising in the eyelid. DESIGN: Case report. METHODS: A retrospective review of the clinical and pathologic features of a patient with solitary superficial angiomyxoma in the eyelid. RESULTS: A 47-year-old male presented with a right upper lid mass for 6 months. Excisional biopsy was performed, and microscopic examination revealed a tumor comprising loose spindle or stellate-shaped cells in myxoid stroma sprinkled with small numbers of neutrophils. The tumor cells were negative for smooth muscle actin, desmin, S-100 protein, and CD34 on immunostaining. CONCLUSIONS: Ophthalmologists should be aware of superficial angiomyxoma as a rare cutaneous tumor with a tendency for local recurrence. Multiple lesions and occurrence in the external ear can be associated with the Carney's complex.
GENITAL
Superficial angiomyxoma (cutaneous myxoma): a clinicopathologic study of 17 cases arising in the genital region. Fetsch JF, Laskin WB, Tavassoli FA. Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.	Int J Gynecol Pathol. 1997 Oct;16(4):325-34. Abstract quote   Seventeen cases of superficial angiomyxoma (cutaneous myxoma) of the genital region are reported. Thirteen patients were female (age range: 15-33 years; mean: 21 years) and four were male (age range: 18-55 years; mean: 39 years). The sites of involvement in females were the labium majus or labium, not otherwise specified (n = 6), vulva (n = 4), groin (n = 2), and mons pubis (n = 1). All lesions in male patients involved the scrotum. The tumors were present from 2 months to 4 years before resection and ranged from 0.9 to 6 centimeters in maximal dimension; 10 tumors were 3 centimeters or less in size. The predominant reason for seeking medical attention was a slow growing painless mass. All lesions were locally excised. Follow-up was obtained for 9 patients with a mean and median follow-up interval of 135 and 95 months, respectively. A recurrence developed in three patients at 8 months, 7 years 11 months, and 20 years. No patient has been shown to have Carney's complex. The tumors were immunoreactive for vimentin (11/11), CD34 (11/11), muscle-specific actin (8/12), smooth muscle actin (9/11), S100 protein (5/13), and Factor XIIIa (5/9). No immunoreactivity was present for desmin (DE-R- 11), glial fibrillary acidic protein, estrogen receptor or progesterone receptor. Superficial angiomyxomas are probably derived from fibroblast-like cells capable of antigen modulation

 

HISTOLOGICAL TYPES	CHARACTERIZATION
Superficial angiomyxoma: clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. Calonje E, Guerin D, McCormick D, Fletcher CD. Department of Pathology, St. John's Institute of Dermatology, London, United Kingdom.	Am J Surg Pathol 1999 Aug;23(8):910-7 Abstract quote 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.
Cutaneous angiomyxoma: a benign neoplasm distinct from cutaneous focal mucinosis. Wilk M, Schmoeckel C, Kaiser HW, Hepple R, Kreysel HW. Department of Dermatology, University of Bonn, Germany.	J Am Acad Dermatol 1995 Aug;33(2 Pt 2):352-5 Abstract quote We describe a cutaneous angiomyxoma on the head of a 38-year-old man without evidence of Carney's complex. Complete excision of the tumor appeared to be curative. Histologic examination revealed fibroblast-like cells embedded in a well-demarcated, lobulate, mucinous, and vascularized stroma with a delicate reticulin network. Immunohistologically, the stromal cells were consistently positive for vimentin and focally positive for smooth muscle A-actin but were negative for desmin, KP1, MAC387, factor XIIIa, CD34, Leu-7, and S-100. Cutaneous angiomyxoma appears to represent a myofibroblastic neoplasm that should be distinguished from cutaneous focal mucinosis.
General	Dermal based tumor often extending into the subcutis Multilobulated growth pattern with poorly defined margins Bland spindle or stellate shaped fibroblasts with thin-walled vessels in myxoid matrix Scattered multinucleated fibroblasts No cytologic atypia or pleomorphism Scattered mitotic figures may be found Inconspicuous mixed inflammatory infiltrate with notable neutrophils but absent ulceration or necrosis 1/3 of cases may have epithelial component which may represent entrapped adnexal structures or adjacent squamous epithelium
VARIANTS
Superficial angiomyxomas with and without epithelial components. Report of 30 tumors in 28 patients. Allen PW, Dymock RB, MacCormac LB. Histopathology Department, Queen Elizabeth Hospital, Woodville, South Australia.	Am J Surg Pathol 1988 Jul;12(7):519-30 Abstract quote This paper describes 30 uncommon dermal and subcutaneous angiomyxoid tumors in 28 patients whose ages ranged from 4 to 78 years (mean, 39 years). There were 16 male patients and 12 female patients. Tumor size varied from 0.5 to 9 cm, with the majority measuring 1-5 cm. Eleven tumors (37%) were located on the trunk, ten (33%) on the lower extremity, five (17%) on the head or neck, and four (13%) on the arm. Microscopically, there were moderately to sparsely cellular angiomyxoid nodules with scattered small vessels. Nine tumors had an epidermal component that took the form of a keratin-filled cyst or epithelial strands. The angiomyxoid components of all 30 tumors were morphologically similar. Electron microscopy showed fibroblastic stromal cells, proteoglycan matrix, and collagen fibers. The S-100 protein stain was negative in two tumors, and the vimentin stain was positive in stromal cells in one tumor. Follow-up information obtained for 20 of the 28 patients included data on eight tumors with epithelial components. Five (63%) of those eight tumors recurred once; three had not recurred, and one patient developed a new and separate purely angiomyxoid tumor. Three (23%) of 13 tumors without epithelial components recurred. None recurred more than once, and none metastasized. We suspect that superficial angiomyxoma, cutaneous focal mucinosis, trichogenic myxoma, trichogenic adnexal tumors, trichodiscoma, myxoid perifollicular fibromas, trichofolliculomas and fibrofolliculomas, the Carney complex, NAME and LAMB syndromes are all closely related. We also believe that the solitary superficial angiomyxoma with no epithelial elements is the most common manifestation of these myxoid tumors.

 

SPECIAL STAINS/ IMMUNOPEROXIDASE/ OTHER	CHARACTERIZATION
Special stains
Immunoperoxidase	VIM+ Variable CD34, SMA, MSA, S100, and FXIIIa
Electron microscopy (EM)	Fibroblastic features

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
Dermal nerve sheath myxoma	More sharply circumscribed lobules with scattered epithelioid or multinucleate cells Paucivascular S100 positive
Low grade MFH (myxofibrosarcoma)	Limb location Obvious cytologic pleomorphism and nuclear atypia
Digital myxoma	Uninodular lesion and more cellular

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSIS
Recurrence	1/3 recur in locally non aggressive fashion, usually secondary to incomplete removal <5% recur repeatedly
Metastasis
TREATMENT	Complete surgical removal

Am J Surg Pathol 1988;12:519-530
Am J Surg Pathol 1999;23:910-917
Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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