Vissa Shanthi etal ,J Biosci Tech, Vol 2 (3),2011, 318-323

SUPERFICIAL ANGIOMYXOMA WITH EPITHELIAL COMPONENT – A RARE CUTANEOUS MYXOID TUMOR *Vissa Shanthi, Baddukonda Appala Rama Krishna, Nandam Mohan Rao, Raj Sekhar reddy, B. H. Poorna Chandra Sekhar, Chenna Venkata Harish, Dr. NTR University of Health sciences, Vijayawada, Andhra Pradesh, India *E.mail: [email protected] ABSTRACT:

Superficial angiomyxoma is a benign soft tissue tumor that has a KEY WORDS: predilection for the head and neck, trunk and lower extremities. We report a case of 30 year old female who presented with a history of nodular swelling Superficial angiomyxoma, on the medial side of the right toe. Pathological analysis confirmed the cutaneous . lesion to be superficial angiomyxoma. We use this case to highlight the features of this rare entity, which is still a poorly recognized cutaneous tumor, despite its description since 1988. 2 years. She denied any trauma at that site. 1. INTRODUCTION: Physical examination revealed a firm Superficial angiomyxoma, also known as immobile, ovoid nodule attached on the is a rare myxoid tumor of medial aspect of the right toe. The overlying the skin and subcutis, which was first skin was unremarkable. No further lesions described by Carney and colleagues in 1986.[1] were seen . The clinical diagnosis of This benign tumor usually presents in middle neurofibroma was made. aged adults as single nodule or polypoid lesion that may be clinically confused with skin tag Local resection was performed and at surgery or neurofibroma.[2] When this lesion occurs in the tumor was centred in subcutaneous tissue. the setting of Carney’s complex (Cutaneous Macroscopically, the resected lesional tissue and cardiac , spotty pigmentation received was a single firm nodule which was and endocrine overactivity), they are often skin covered measuring 2.5 X 2.5 X 1.5cms. multiple and frequently occur in the eyelid or Cut surface of the lesion was gray to white external ear.[3] Histologically this tumor has a with glistening surface (Figure-1). lobular low power appearance with a circumscribed border that involves the dermis Microscopic examination revealed a and sometimes cutis. Distinctive histologic circumscribed myxoid lesion within the features included extensive myxoid stroma dermis . Lesion has a lobular appearance at with stellate or bipolar fibroblastic cells, small low magnification. The lobules are separated blood vessels, and mixed inflammatory cell by fibrous septa (Figure-2). The lesional cells infiltrate with notable neutrophils.[4] are small, cytologically bland and spindled to Cutaneous myxoma should be distinguished stellate in shape embedded in the vascularized from the other cutaneous myxoid lesions with myxoid stroma (Figure-3). In some foci, the which it may be confused because it has a lesion was seen extending into the epidermis propensity for local recurrence. (Figure-4). Entrapped adnexal glands (Figure- 5) and prominent neutrophilic infiltrate were

also seen. Immunohistochemistry showed 2. CASE REPORT: A 30 year old female presented with a painless focal positivity with CD34 (Figure-3). The soft tissue mass on the medial aspect of the histological and immunohistochemical right toe, which had been slowly growing for features were consistent with superficial angiomyxoma.

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Vissa Shanthi etal ,J Biosci Tech, Vol 2 (3),2011, 318-323

3. DISCUSSION: channels seen in myxoid stroma. A mixed Superficial angiomyxoma (Cutaneous inflammatory infiltrate is common, myxoma) is benign soft tissue tumor which particularly stromal neutrophils, a feature was first described by Carney and later more unique to this tumor when compared to other fully characterized by Allen et al in 1988 [2] cutaneous myxoid lesions Upto one quarter of and Calonje and colleagues in 1999.[4] these tumors have epithelial structures consisting of basoloid buds, epithelial strands Cutaneous myxoma arises more commonly in or epidermoid cyst, possibly as a result of males, predominantly middle aged adults with entrapment of adnexal structures by the a peak incidence between 20 and 40 years of neoplasm. age.[5] Rare congenital examples have been described.[6] These lesions can arise Immunohistochemically, the tumor cells essentially anywhere in the superficial tissue express vimentin and CD34, but rarely stain but there is a predilection for the trunk, lower for cytokeratin or S-100 protein . Some cells extremities, and head and neck. Some arise in stain for smooth muscle actin, desmin, the genital region of both males and females possibly indicating focal myofibroblastic (vulva, mons pubis, scrotum/ inguinal).[7] differentiation.[10]

Cutaneous myxomas associated with The differential diagnosis of cutaneous Carney’s syndrome are often multiple and myxomas is extensive and includes benign involves the eyelids and ear.[8] and low grade malignant myxoid lesions including , focal The clinical history is typically that of a long cutaneous mucinosis,myxoid neurothekeomas standing painless nodule or mass. Most of (dermal nerve sheath myxoma) , myxoid them are solitary lesions varying in size from neurofibroma, superficial acral 1-5cms. Grossly, cutaneous myxomas are fibromyxomaand . usually well circumscribed tumors. They have a gray white, glistening, gelatinous cut Aggressive angiomyxoma usually tends to be surface. Thin fibrous septa traverse the larger, involves deeper structures, usually in neoplasm, resulting in a vaguely multinodular the female pelvic region and has a vascular tumor. Cysts that are sometimes filled with pattern that differs from that of cutaneous keratinous debris may be identified grossly.[9] myxoma. Focal cutaneous mucinosis lacks Histologically, this lesion has a lobular or lobular architecture, stromal neutrophils, and multinodular appearance at low magnification. epithelial structures found in cutaneous Most are histologically poorly circumscribed myxoma. Superficial acral fribromyxoma with extension into the underlying arises almost exclusively on the fingers and subcutaneous tissue and rarely skeletal toes of middle aged adults and lacks muscle. A sparse proliferation of spindled to neutrophilic infiltrate.[11] Myxoid stellate shaped cells are deposited in an neurothekeoma has a more pronounced extensive myxoid stroma, sometimes forming lobular growth pattern and is characterized by cysts or irregular clefts, that is sensitive to more pl;umper cells that are usually positive hyaluronidase digestion. The cells have for S-100 protein. Myxoid neurofibroma is indistinct cell borders and nuclei with composed of cells with wavy or buckled inconspicuous nucleoli. Mitotic figures are nuclei that are also S-100 positive. rare. Binucleated and multinucleated cells may be seen. There are often small vascular 319

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Myxoid liposarcomas are usually more deeply [2] Allen PW, Dymock RB, Mac Cormac LB. located and larger than cutaneous myxomas Superficial angiomyxomas with or without and is characterized by a “chicken-wire” epithelial components. Report of 30 tumors in 28 plexiform vasculature with scattered patients. Am J Surg Pathol, 1988:12(7):519-530. lipoblasts. Myxofibrosarcoma has a greater [3] Ferreiro JA, Carney JA. Myxomas of external ear degree of nuclear atypia and hyperchromasia and their significance. Am J Surg Pathol 1994, as well as curvilinear vessels often lined by 18(3): 274-280. hyperchromatic tumor cells. [4] Calonje E, Guerin D, Mc Cormick D, Fletcher CD. Superficial angiomyxoma. Clinicopathologic 4. CONCLUSION: analysis of a series of distinctive but poorly Superficial angiomyxoma is a rare recognized cutaneous tumors with tendency for clinicopathologic entity with recurrence recurrence. Am J Surg Pathol 1999 Aug; potential. Hence it should be distinguished 23(8):910-917. from other myxoid lesions of the skin. [5] Allen PW. Myxoma is not a single entity: a Appropriate diagnosis, complete surgical review of the concept of myxoma. Ann Diagn removal and close follow up of the patient is Pathol 2000; 4: 99. recommended. [6] Bedlow AJ, Sampson SA, Holden CA. Congenital superficial angiomyxoma. Clin Exp 5. REFERENCES: Dermatol 1997; 22: 237. [1] Carney JA, Gordon H. Carpenter PC, et al. The [7] Fetsch JF, Laskin MB, Tavassoli FA. Superficial complex of myxomas, spotty pigmentation, and angiomyxoma (Cutaneous myxoma): A endocrine overactivity. Medicine (Baltimore) clinicopathologic study of 17 cases arising in the 1985: 64-270. genital region. Int J Gynecol Pathol 1997; 16: 325.

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[8] Yuen HK, Cheuk W, Luk FO, Wat CS, Auyeung KC, Lam DS. Solitary Superficial angiomyxoma in the eyelid. Am J Opthalmol 2005 Jun ; 139 (6): 1141-1142. [9] Takahashi H, Hida T. Corney complex: Report of a Japanese case associated with cutaneous superficial angiomyxomas, labial lentigines, and a pituitary adenoma. J Dermatol 2002 Dec; 29(12): 790-6. [10] Wilk M, Schmoeckel C, Kaiser HW, Hepple R, Kreysel HW. Cutaneous angiomyxoma: a benign neoplasm distinct from cutaneous focal mucinosis. J Am Aead Dermatol 1995 Aug ; 33(2pt2): 352-5.

[11] Fetsch JF, Laskin MB, Miettinen M. Superficial

acral fibromyxoma: A clinicopathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumors with a predilection fingers and toes.

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