Superficial Angiomyxoma with a Polypoid Appearance

―578― 皮膚・第41巻・第5号・1999年10月 578-581

Hatsuki Shiraishi, M.D., Hiroko Gomi, M.D., Ph.D. and Itsuro Matsuo, M.D., Ph.D.

Department of Dermatology, Teikyo University School of Medicine, Ichihara Hospital 3426-3, Anesaki, Ichihara, Chiba 299-0111, Japan

Akira Kawada, M.D., Ph.D.

Department of Dermatology, Kinki University School of Medicine 377-2, Ohno-Higashi, Osaka-Sayama, Osaka 589-8511, Japan

Toshitaka Nagao, M.D., Ph.D.

Department of Pathology, Teikyo University School of Medicine, Ichihara Hospital 3426-3, Anesaki, Ichihara, Chiba 299-0111, Japan.

Key words: myxoma, superficial angiomyxoma, polypoid lesion

We describe a 66-year-old male patient with a bicornate polypiform nodule on the left dorsum of nose. The histological finding showed numerous ectatic blood vessels throughout mucinous stroma that was consistent with superficial angiomyxoma. The interstitial material was positive for colloid iron and mucicarmin staining methods and digested by hyaluronidase, suggesting the positive substance to be hyaluronic acid. Skin Research, 41: 578 581, 1999

Introduction Case Report

Superficial angiomyxoma is a rare, benign, der- A 66-year-old Japanese man was seen in Sep- mal and subcutaneous tumor1). We report here a tember 1998 with a bicornate polypiform nodule on case of superficial angiomyxoma with a polypoid the left dorsum of nose (Fig. 1) that had been appearance. noticed 6 months before and gradually increased in size. The each cornate lesion with telangiectasia was ―579― 皮膚・第41巻・第5号・1999年10月

Fig. 1 A Bicornate Polypoid Nodule on the Left Dorsum of Nose of the Patient

skin-colored, 0.5•~0.9cm, soft, and not tender. No

other skin and mucocutaneous lesions, cardiac, and

endocrine abnormalities were present. There was no

family history of similar skin lesions. No recurrence

was found in 5 months after the lesion was excised.

Histologic examination demonstrated a dermal myx-

oid lesion (Fig. 2-a) composing of fibroblast-like

cells varied in shape from spindle to stellate (Fig.

2-b) and multi-nucleate cells. Ectatic small blood

vessels were scattered throughout the lesion. No

keratinous cysts or epithelial strands were found in

the lesion. The interstitial material was positive for Fig. 2a: Histological View of a Diffuse Myxoid Lesion with

colloid iron and mucicarmin staining methods and Abundant Ectatic Small Blood Vessels in the Dermis

digested by hyaluronidase, indicating to be hyalur- (Hematoxylin and eosin.•~40) b: Fibroblast-like Cells and Stellate Cells in the Mucinous onic acid. Immunohistochemical staining of the Stroma (Hematoxylin and eosin.•~200) stromal cells was focally positive for CD-34 and

negative for S-100, keratin, or a -smooth muscle

actin. Angiomyxomas are termed as the lesions of cutaneous myxoma with a predominant vascular Discussion component. The 'superficial' angiomyxoma is dis- tinguishable from the unrelated aggressive angiomy- The cutaneous myxoma, a rare variant of myx- xoma (recurrent, infiltrative) of the pelvioperineal oma, is a demarcated nodule of the dermis or sub- region6). A few reports1,7,8) refers to superficial cutis, occurring as multiple lesions that resemble angiomyxomas. They occur in patients aged from 2 dermatofibroma2). Carney complex (NAME or to 78 years1,8) and are commonly located on the LAMB syndrome)3•`5) is well established as the trunk and lower extremity, and 17% on the head complex of multiple myxomas (cutaneous, cardiac, and neck. Tumor size mainly varies from 1 to 5 cm. and mammary), spotty pigmentation, and endocrine The clinical appearance is usually nodular swelling, overactivity. The epithelial component originated soft elevation, and subcutaneous lesion. They are from the outer root sheath of the hair follicle is solitary and locally recurrent, but there have been found in some of cutaneous myxomas in Carney no reports of metastatic spread8) Epithelial com- complex and is suggested to be trichofollicular ponents, e.g. keratin-filled cyst and epithelial hamartomas3). strands, are seen in 9 tumors of 30 superficial ―580― 皮膚・第41巻・第5号・1999年10月 angiomyxomas1). Our case as well as the two re- Skin (Elder D, Elenitsas R, Jaworsky C, John- ported cases7,8) did not show epidermal com- son B Jr, Eds), Lippincott-Raven, Philadelphia, ponents. Superficial angiomyxoma without epithelial 1997, 847-887 components is the most common manifestation1) and 3. Carney JA, Headington JT, Su WPD: Cutaneous may be different from cutaneous myxomas with myxomas. A major component of the complex of epidermal components in Carney complex. myxomas, spotty pigmentation, and endocrine The tumor should be histopathologically diffe- overactivity, Arch Dermatol, 122: 790-799, rentiated from mucinous dermal nodules. Vascular 1986 and myxoid fibromas of the fingers9) show warty 4. Atherton DJ, Pitcher DW, Wells RS, MacDonald lesions on the fingers and palms. Cutaneous focal DM: A syndrome of various cutaneous pig- mucinosis10) is a solitary, smooth-surfaced, white to mented lesions, myxoid neurofibromata and at- skin-colored papular nodule that does not have vas- rial myxoma: the NAME syndrome, Br J Der- cularity. matol, 103: 421-429, 1980 Histogenesis of superficial angiomyxoma is still 5. Rhodes AR, Siverman RA, Harrist TJ, unknown. Dermal nerve sheath myxoma with less Perez-Atayde AR: Mucocutaneous lentigines, vascularity and smaller lobules demonstrates posi- cardiomucocutaneous myxomas, and multiple tive S-100 expression and is suggested to be a neu- blue nevi: The "LAMB" syndrome, J Am Acad ral origin11). Five cases including our case1,7,8) Dermatol, 10: 72-82, 1984 showed negative expression of S-100, suggesting 6. Steeper TA, Rosai J: Aggressive angiomyxoma the origin of superficial angiomyxoma not to be neu- of the female pelvis and perineum. Report of ral. CD-34 was focally positive in our case as well nine cases of a distinctive type of gynecologic as a previously reported case of superficial soft-tissue neoplasm, Am J Surg Pathol, 7: 463 angiomyxoma8). CD-34 antigen is seen on hemato- 475, 1983 - poietic stem cells, leukemias, vascular tumors, nerve 7. Wilk M, Schmoeckel C, Kaiser HW, Hepple R, sheath and smooth muscle tumors, and dermatofib- Kreysel HW: Cutaneous angiomyxoma: A be- rosarcoma protuberans12). Further investigation in nign neoplasm distinct from cutaneous focal immunohistochemistry is needed. mucinosis, J Am Acad Dermatol, 33: 352- Some skin tumors, e. g. soft fibroma, neurofibro- 355, 1995 ma, pseudopyogenic granuloma, fibrous papule of 8. Bedlow AJ, Sampson SA, Holden CA: Congenit- the face, and polypoid fibrolipoma, show a polypi- al superficial angiomyxoma, Clin Exp Dermatol, form lesion. Superficial angiomyxoma as seen in our 22: 237-239, 1997. patient might be an addition to the clinical panel of 9. Coskey RJ, Mehregan AH, Lupulescu AP: Mul- cutaneous polypoid tumors. tiple vascular fibromas and myxoid fibromas of the fingers. A histologic and ultrastructural References1 study, J Am Acad Dermatol, 2: 425-431, 1980 . Allen PW, Dymock RB, MacCormac LB: Super- 10. Johnson WC, Helwig EB: Cutaneous focal muci- ficial angiomyxomas with and without epithelial nosis, Arch Dermatol, 93: 13-20, 1966 components. Report of 30 tumors in 28 11. Fletcher CDM, Chan JKC, McKee PH: Dermal patients, Am J Surg Pathol, 12: 519-530, nerve sheath myxoma: a study of three cases, 1988 Histopathology, 10: 135-145, 1986. 2. Heenan PJ: Tumors of the fibrous tissue in- 12. van de Rijn M, Rouse RV: CD34-A review, volving the skin, Lever's Histopathology of the Appl Immunohistochem, 2: 71-80, 1994 ―581― 皮膚・第41巻・第5号・1999年10月

Superficial Angiomyxoma with a Polypoid Appearance

白石葉月川田暁五味博子松尾聿朗長尾俊孝

キーワード : myxoma, superficial angiomyxoma, 有茎性腫瘍

66歳,男性の左鼻翼に生じたsuperficial angiomyxomaを報告した。臨床的には双角状,有茎性の柔らかい皮膚色の結節を示した。病理組織所見では,真皮のmucinousな間質に大小の多数の血管新生を認めた。間質はコロイド鉄とムチカルミン染色で陽性を示し,陽性物質はヒアルロニダーゼで消化され,ピ

アルロン酸と考えられた。(皮膚,41: 578-581, 1999)

Hatsuki SHIRAISHI, M. D., Hiroko GOMI, M.D., Ph.D. and Itsuro MATSUO, M. D., Ph.D. 帝京大学医学部附属市原病院皮膚科〒299-0111市原市姉崎3426-3 Akira KAWADA, M. D., Ph. D. 近畿大学医学部皮膚科学教室〒589-8511大阪府大阪狭山市大野東377-3 Toshitaka NAGAO, M. D., Ph. D. 帝京大学医学部附属市原病院病理部〒299-0111市原市姉崎3426-3 1999年9月25日掲載決定