Intraoral superficial angiomyxoma

Intraoral superficial angiomyxoma

Intraoral superficial angiomyxoma Shabnum Meer, BChD, MDent,a and Ian Beavon, MBBCh, FCPath(SA),b Johannesburg, South Africa UNIVERSITY OF THE WITWATE...

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Intraoral superficial angiomyxoma Shabnum Meer, BChD, MDent,a and Ian Beavon, MBBCh, FCPath(SA),b Johannesburg, South Africa UNIVERSITY OF THE WITWATERSRAND AND LANCET LABORATORIES

Superficial angiomyxoma is an unusual benign myxoid tumor of the trunk, head and neck, extremities, and genitalia. We report the third case of intraoral superficial angiomyxoma, which occurred within the buccal mucosa of a 37-year-old woman. The symptomless slow-growing mass was present for 2 years. The lobulated, paucicellular myxoid tumor showed prominent vascularity, stromal inflammation, including neutrophils, and immunopositivity for CD34, vimentin, and muscle-specific actin. Cure is effected by localized excision, with follow-up, owing to the high recurrence rate of the cutaneous tumors, which has not been substantiated for the few reported intraoral cases. Superficial angiomyxoma does indeed occur intraorally and should be included in the differential diagnosis of myxoid intraoral soft tissue neoplasms. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106:e20-e23)

Superficial angiomyxoma, also known as cutaneous myxoma,1-3 is now a well documented entity since its initial description in 19884 as a distinctive benign cutaneous myxoid soft tissue neoplasm with a tendency for local recurrence. Although there have been many reported cases in the head and neck, including sites such as the chin, lip, cheek, nose, ear, forehead, face, and neck,5 truly intraoral cases are extremely rare, with, to the best of our knowledge, only 2 previously reported cases to date.6,7 The histologic features are characteristic, yet superficial angiomyomas are often underrecognized and acknowledged.5 We report an additional case of an intraoral superficial angiomyxoma, occurring within the buccal mucosa. CASE REPORTS A 37-year-old black woman patient presented with a painless, slow-growing, and soft mass in the left buccal mucosa, present for 2 years. Clinically, the lesion appeared well encapsulated, well demarcated, and unfixed, with a vague lobulated appearance. The clinical impression was that of a lipoma or other myxoid benign soft tissue neoplasm. The lesion was excised, the postoperative healing was uneventful and there was no sign of recurrence after 9 months. Gross examination showed a lobulated gray-white soft tumor mass measuring 45 ⫻ 32 ⫻ 20 mm, with a gelatinous, myxoid, and glistening cut surface (Fig. 1). Histologically, there were vaguely circumscribed irregular portions of pauci-

a

Senior Specialist Consultant, Division of Oral Pathology, University of the Witwatersrand. b Senior Specialist Consultant, Department of Histopathology, Lancet Laboratories. Received for publication Feb 25, 2008; returned for revision Apr 28, 2008; accepted for publication Jun 12, 2008. 1079-2104/$ - see front matter © 2008 Mosby, Inc. All rights reserved. doi:10.1016/j.tripleo.2008.06.011

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cellular myxoid soft tissue composed of scattered bland spindle-shaped and stellate cells set in copious amounts of myxoid stroma (Fig. 2, B). Focal areas of acellular mucinous pools (Fig. 2, A) as well as areas of collagenization were noted. There was no cellular or nuclear atypia or hyperchromasia, and mitotic activity and necrosis were not present. Small, congested, thin-walled, curvilinear blood vessels were prominent throughout the tumor mass (Fig. 3). Very occasional mixed stromal inflammatory cells and numerous mast cells were identified. Neutrophils were present though not conspicuous (Fig. 4). An epithelial component was absent. The tumor was immunopositive for CD34 and very focally immunopositive for muscle actin–specific monoclonal Ab-4 (clone HHF35), a marker for ␣ actins (smooth and skeletal muscle) and ␥–smooth muscle actin; and it was negative for desmin (clone D33), epithelial membrane antigen, S100 protein, and ␣–smooth muscle actin (Dako, Glostrup, Denmark). More than 70% of the tumor cells showed strong nuclear and cytoplasmic immunoreactivity for CD34 (Fig. 4), with less than 10% of the lesional cells demonstrating weak cytoplasmic staining with muscle-specific actin.

DISCUSSION Superficial angiomyxomas are recognized as rare distinctive, benign, cutaneous soft tissue lesions with a predilection for the trunk and the head and neck. Other reported sites include the lower extremities and the genital area.3,5 However, the present case reiterates the fact that although rare, these tumors can occur within the oral cavity. Cutaneous superficial angiomyxomas typically present as polypoid or papulonodular lesions, with a slight male predilection, in middle age, with a few rare congenital lesions being reported.7 Table I briefly outlines the pertinent clinical features of the reported intraoral superficial angiomyxomas. There are now 2 cases of superficial angiomyxoma documented in the buccal mucosa and 1 in the floor of the mouth.6,7 The relatively small, painless, lobulated

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Fig. 1. Macroscopy revealed a lobulated, gray-white, soft tumor mass, measuring 45 ⫻ 32 ⫻ 20 mm, with a gelatinous, myxoid and glistening cut surface (scale bar ⫽ 1 cm).

Fig. 2. A, The abundant myxoid matrix formed prominent acellular mucin pools and clefts, producing an “angiectoid” pattern (hematoxylin-eosin stain, original magnification ⫻200), B, High-power photomicrograph showing a paucicellular proliferation of bland spindle-shaped and stellate cells in a copious myxoid stroma (hematoxylin-eosin stain, original magnification ⫻400).

Fig. 3. Intervening arborizing small thin-walled curvilinear blood vessels (arrows) were present throughout the myxoid tumor (hematoxylin-eosin stain, original magnification ⫻400).

aggressive angiomyxoma of the floor of the mouth described by Yamashita et al.8 appears no more infiltrative than other reported cases of superficial angiomyxoma.9 Furthermore, the vascularity of the oral floor tumor described and illustrated by Yamashita et al.8 lacks the large blood vessels typical of aggressive angiomyxoma, and the arborizing vascularity of the irregular distributed smaller vessels within multiple poorly defined myxoid nodules is more reminiscent of that

seen in superficial angiomyxoma. There are still too few documented intraoral cases to draw any meaningful clinical comparisons; however, the histologic and immunohistochemical profiles of the intraoral tumors are distinctive and mimic those of their cutaneous counterparts. The distinctive histologic features of superficial angiomyxoma include a poorly circumscribed tumor with a multilobular growth pattern composed of spindle-

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Fig. 4. The tumor showed strong nuclear and cytoplasmic immunoreactivity for CD34 in more than 70% of the tumor cells (original magnification ⫻400). Although not conspicuous, very occasional stromal neutrophils (inset), unrelated to foci of ulceration or necrosis, were scattered within the myxoid matrix.

shaped to stellate cells in a profusely myxoid stroma. There is no nuclear atypia or hyperchromasia. Small thin-walled vessels are prominent. An important diagnostic clue is the presence of stromal inflammatory cells, especially neutrophils.5 As with most myxoid tumours, Calonje et al.5 reported an increased number of stromal mast cells in 51% of their cases. An epithelial component probably derived from entrapped hyperplastic adnexal structures has been described in cutaneous variants.9 The histologic diagnosis of superficial angiomyxomas is not complex. The main histologic differential diagnosis for intraoral tumors includes soft tissue myxoma, myxoid nerve sheath tumor (neurothekeoma), myxoid neurofibroma, oral focal mucinosis, and myxofibroma or odontogenic myxoma. Soft tissue myxoma is a hypocellular tumor, with tumor cells embedded in an abundant myxoid or mucous background. Unlike superficial angiomyxoma, the presence of blood vessels is rare.10 There have been many reports of soft tissue myxomas arising within the head and neck area, including the oral regions,10-12 some of which comprehensively detail the differential diagnosis of soft tissue myxoma.9,13 Nerve sheath myxoma has smaller individual nodules, is less vascular with cells arranged concentrically like a Pacinian corpuscle, and contains occasional eosinophilic histiocytic cells.4 The cells of myxoid neurofibroma are typically slender with wavy nuclei and intralesional nerve bundles and exhibit S100 protein positivity.11,13 Oral focal mucinosis is typically acellular with very few blood vessels, lack of a lobular

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architectural pattern and no stromal inflammation. In addition, it is characterized by a lack of reticulin fibers in a mucoid stroma, which is clearly defined from the surrounding tissues.10 Myxofibromas or odontogenic myxomas are central lesions that are diffuse and nonlobulated, with no stromal inflammation, and may contain odontogenic epithelial rests.11 Histologically, identical lesions to superficial angiomyxomas have been described in the Carney complex, particularly those that are multiple and arise in the eyelids and external ear.1 Carney complex myxomas include cardiac, cutaneous, and mammary myxomas.9 In a small minority of cases, superficial angiomyxomas are a marker for the Carney complex. The presence of multiple cutaneous angiomyxomas in a young patient may be the earliest manifestation of the complex. It is therefore crucial to realize their significance and to investigate patients so affected.3 Superficial angiomyxomas are generally immunoreactive with vimentin and CD34, variably immunoreactive for muscle-specific actin, and consistently negative for S100 protein and desmin.3,5 Fetsch et al.3 reported S100 protein, muscle-specific actin and ␣–smooth muscle actin immunopositivity in 39%, 67%, and 82% of their cases, respectively. The focal ␣–smooth muscle actin positivity reported is suggestive of focal myofibroblastic differentiation.3,6,7 Ultrastructural features include a proteoglycan-type matrix admixed with collagen fibers and sparsely distributed stromal fibroblasts.9 Many myxoid soft tissue neoplasms lack a distinct immunohistochemical profile. Although no conclusive specific translocation has been reported in superficial angiomyxoma, the use of cytogenetic and/or reverse transcriptase polymerase chain reaction analyses, as well as fluorescence in situ hybridization, are valuable tools applicable in the differential diagnosis of myxoid soft tissue neoplasms.14 For example, distinct chromosomal translocations have been identified in many myxoid sarcomas, including t(12;16)(q13;p11) FUS-DDIT3 in myxoid liposarcoma and t(7;16)(q34;p11) FUSCREB3L2 in low-grade fibromyxoid sarcoma. Cure is effected by localized surgical excision, with careful follow-up owing to its high rate of local recurrence, which is reported to range between 20% and 40%.4,5,7 Superficial angiomyxomas have an overall good prognosis. CONCLUSION Our findings further demonstrate that superficial angiomyxoma may indeed occur within the oral cavity and should be included in the differential diagnosis of myxoid intraoral soft tissue neoplasms.

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Table I. Reported cases of intraoral superficial angiomyxoma Reference

Site

Age (yrs)

Gender

Race

Size (mm)

Presentation Slow growing painless lump present for 2 years Slow growing painless lump present for 3 years Slow growing painless lump present for 2 years

Chen6

Right buccal mucosa

19

Male

Chinese

50 ⫻ 35 ⫻ 30

Gardner7

Left floor of mouth

69

Female

European

10 ⫻ 12 ⫻ 12

Meer (this study)

Right buccal mucosa

37

Female

African

45 ⫻ 32 ⫻ 20

The authors thank Dr. Jameel Desai [BSc, BDS, PDD, MDent, FCMFOS(SA)], a maxillofacial and oral surgeon in private practice, Newcastle, South Africa, for his contribution of the clinical case. They also thank Dr. Salim Kaskar [BSc, BChD, MChD (Ortho)] and Ms. Amina Kaskar for their help with the electronic imaging. REFERENCES 1. Weiss SW, Goldblum JR. Cutaneous myxoma. In: Enzinger and Weiss’s soft tissue tumors. Ed 4. St. Louis: Mosby; 2001. p. 1437-40. 2. Miettinen MM, Diagnostic soft tissue pathology. New York: Churchill Livingstone; 2003. p. 159. 3. Fetsch JF, Laskin WB, Tavassoli FA. Superficial angiomyxoma (cutaneous myxoma). A clinicopathologic study of 17 cases arising in the genital region. Int J Gynecol Pathol 1997;16: 325-34. 4. Allen PW, Dymock RB, MacCormac LB. Superficial angiomyxomas with and without epithelial components. Report of 30 tumors in 28 patients. Am J Surg Pathol 1988;12:519-30. 5. Calonje E, Guerin D, McCormick D, Fletcher C. Superficial angiomyxoma: clinicopathologic analysis of a series of distinctive but poorly recognized cutaneous tumors with tendency for recurrence. Am J Surg Pathol 1999;23:910-7. 6. Chen YK, Lin LM, Lin CC, Yan YH. Myxoid tumor of the oral cavity with features of superficial angiomyxoma: report of a case. J Oral Maxillofac Surg 1998;56:379-82. 7. Gardner AW. Superficial angiomyxoma of the floor of the mouth–a case report. Br J Oral Maxillofac Surg 2007;45:418-9.

Clinical impression

Treatment

Soft tissue tumor

Excised, no recurrence

Lipoma

Excised, no recurrence

Lipoma

Excised, no recurrence

8. Yamashita Y, Tokunaga O, Goto M. Aggressive angiomyxoma of the oral floor: report of a case. J Oral Maxillofac Surg 2004;62:1429-31. 9. Allen PW. Myxoma is not a single entity: a review of the concept of myxoma. Ann Diagn Pathol 2000;4:99-123. 10. Epivatianos A, Iordanidis S, Zaraboukas T. Myxoma of the oral soft tissues: report of a case and literature review. J Oral Maxillofac Surg 2007;65:317-20. 11. Perrotti V, Rubini C, Fioroni M, Piattelli A. Soft tissue myxoma: report of an unusual case located on the gingiva. J Clin Periodontol 2006;33:76-8. 12. van Roggen JFG, McMenamin ME, Fletcher CDM. Cellular myxoma of soft tissue: a clinicopathological study of 38 cases confirming indolent clinical behaviour. Histopathology 2001;39: 287-97. 13. van Roggen JFG, Hogendoorn PCW, Fletcher CDM. Myxoid tumours of soft tissue. Histopathology 1999;35:291-312. 14. Downs-Kelly E, Goldblum JR, Patel RM, Weiss SW, Folpe AL, Mertens F, et al. The utility of fluorescence in situ hybridization (FISH) in the diagnosis of myxoid soft tissue neoplasms. Am J Surg Pathol 2008;32:8-13. Reprint requests: Dr. Shabnum Meer, BChD, MDent Division of Oral Pathology School of Oral Health Sciences Private Bag 3 WITS, 2050 South Africa. [email protected]