British Journal of Oral and Maxillofacial Surgery 45 (2007) 228–230
Short communication
Leiomyomatous hamartoma presenting as a congenital epulis Omar Kujan a , Stuart Clark b , Philip Sloan a,∗ a b
Unit of Oral Pathology, School of Dentistry, University of Manchester, Higher Cambridge Street, Manchester, M15 6FH, UK Unit of Oral Surgery, School of Dentistry, University of Manchester, Higher Cambridge Street, Manchester, M15 6FH, UK
Accepted 29 July 2005 Available online 12 September 2005
Abstract An otherwise-healthy 11-month-old white girl presented with a polyp-like lesion on the anteromedial part of the maxillary alveolar ridge. It looked like a congenital epulis, but histological examination showed fascicles of smooth muscle cells dispersed in collagenous stroma with a few peripheral nerve bundles that were intermingled with smooth muscle fibres. The muscle cells stained strongly for desmin and ␣-smooth-muscle actin. However, S-100 was found only in peripheral nerve bundles. It was therefore a leiomyomatous hamartoma. © 2005 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Congenital; Leiomyomatous hamartoma; Epulis
Introduction Congenital epulides usually occur in the anterior maxillary alveolus of baby girls, and the usual histological pattern is the granular cell type.1 Other rare histological variants (fibrous and leiomyomatous) of congenital epulis have been reported.2–4 We present a case of congenital leiomyomatous hamartoma, with immunohistochemical findings, which presented as an epulis in a healthy 11-month-old girl.
Case report An 11-month-old, otherwise-healthy girl was referred to the oral surgery out-patient clinic for investigation and management of a painless gingival polypoid mass on the anteromedial part of the maxillary alveolar ridge that had been present since birth and had slowly enlarged. We found a lobular, sessile, soft tissue polyp, roughly 10 mm long on the palatal side of the midline of the gingiva. ∗ Corresponding author. Tel.: +44 161 275 6788; fax: +44 161 275 6797/6640. E-mail address:
[email protected] (P. Sloan).
The nodule was firm on palpation and was covered by normal mucosa. There were no palpable lymph nodes in the neck. The lesion was excised under local anaesthesia, and the specimen examined histologically. It measured 10 mm × 5 mm × 5 mm and had a greyish-white cut surface. It was embedded in paraffin, and sectioned for routine histopathological and immunohistochemical staining. A standard indirect immunoperoxidase method with labelled streptavidin biotin (LSAB Kit, DAKO, Denmark) was used to stain for the following markers: desmin (monoclonal; DAKO), ␣-smooth-muscle actin (1:200 monoclonal; DAKO, Carpinteria, CA, USA), and S100 (1:2000 polyclonal; DAKO). Microscopy showed well-defined fascicles of smooth muscle cells intermingled with thick-walled blood vessels and a few nerve fibres. The islands of smooth muscle were dispersed in a fibrous stroma surrounding a central blood vessel. The covering epithelium was unremarkable (Fig. 1). There was no cytological atypia, and no granular cells or odontogenic epithelium in the lesion. Immunoperoxidase staining for desmin (Fig. 2A), and ␣-smooth-muscle actin (Fig. 2B) showed large concentrations in the smooth muscle bundles. The hamartomatous smooth muscle fasicles stained more strongly for desmin than the vascular smooth muscle. However, S-100 was detected in only a few peripheral nerve bundles intermingled with smooth muscle fibres (Fig. 2C).
0266-4356/$ – see front matter © 2005 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
doi:10.1016/j.bjoms.2005.07.019
O. Kujan et al. / British Journal of Oral and Maxillofacial Surgery 45 (2007) 228–230
229
Table 1 Clinical features of reported cases of intraoral leiomyomatous hamartoma
Fig. 1. Longitudinal section of the polyp showing the central vessel, fasicles of smooth muscle and the normal covering epithelium (haematoxylin and eosin, original magnification ×20).
Discussion Neumann was the first to describe (in 1871) the congenital gingival tumour as “congenital epulis”.5 Granular cell tumour is the most common type of congenital epulis.5 However, rare variants have been reported with fibrous and leiomyomatous histological features.2–4 The condition is found more often in baby girls than boys and most cases occur on the anterior
Case
First authors, reference number
Age
Sex
Site
1
Semba3
24 months
M
2 3 4 5
Goldsmith9 Napier7 Rosa-Garcia8 Takeda4
16 months 5 years 6 years 10 months
M F M M
6 7 8
Correa6 Kobayashi10 Present case
6 years 3 months 11 months
F M F
Anterior portion of maxillary alveolar ridge Posterior part of tongue Hard palate Anterior part of tongue Anterior portion of maxillary alveolar ridge Incisive papilla Mid-dorsum of the tongue Anterior portion of maxillary alveolar ridge
part of the maxillary alveolar ridge. Excision is the preferred treatment and the lesions do not recur.3,4,6–10 The clinical features in the present case were typical of congenital epulis in appearance, location, sex, lack of pain, and pattern of growth. The clinical and histological features were identical to those presented by Semba et al.,3 Takeda et al.,4 and Correa et al.6 and are consistent with a histological diagnosis of leiomyomatous hamartoma (Table 1).
Fig. 2. Immunohistochemical findings. (A) Desmin immunoreactivity, (B) ␣-smooth-muscle actin immunoreactivity, and (C) immunoreactivity of S100. The hamartomatous smooth muscle stains more strongly for desmin than the vascular smooth muscle, but both types show equal intensity for ␣-smooth-muscle actin (original magnification ×25).
230
O. Kujan et al. / British Journal of Oral and Maxillofacial Surgery 45 (2007) 228–230
References 1. Soames JV, Southam JC. Oral pathology. 3rd ed. Oxford: Oxford University Press; 1998. pp. 119–122. 2. Majid ZA, Siar CH, Ling KC. Congenital fibrous epulis: a case report. Med J Malaysia 1986;41:179–82. 3. Semba I, Kitano M, Mimura T. Gingival leiomyomatous hamartoma: immunohistochemical and ultrastructural observations. J Oral Pathol Med 1993;22:468–70. 4. Takeda Y, Satoh M, Nakamura S, Matsumoto D. Congenital leiomyomatous epulis: a case report with immunohistochemical study. Pathol Int 2000;50:999–1002. 5. Lack E, Worsham GF, Callihan MD, Crawford BE, Vawter GF. Gingival granular cell tumor of the newborn (congenital “epulis”): a clinical and pathologic study of 21 patients. Am J Surg Pathol 1981;5:37–46.
6. Correa L, Lotufo M, Martins MT, Sugaya N, de Sousa SC. Leiomyomatous hamartoma of the incisive papilla. J Clin Pediatr Dent 2001;25:157–9. 7. Napier SS, Devine JC, Rennie JS, Lamey PJ. Unusual leiomyomatous hamartoma of the hard palate: a case report. Oral Surg Oral Med Oral Pathol 1996;82:305–7. 8. de la Rosa-Garcia E, Mosqueda-Taylor A. Leiomyomatous hamartoma of the anterior tongue: report of a case and review of the literature. Int J Paediatr Dent 1999;9:129–32. 9. Goldsmith P, Soames JV, Meikle D. Leiomyomatous hamartoma of the posterior tongue: a case report. J Laryngol Otol 1995;109: 1190–1. 10. Kobayashi A, Amagasa T, Okada N. Leiomyomatous hamartoma of the tongue: case report. J Oral Maxillofac Surg 2001;59:337–40.
