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Rhabdomyomatous mesenchymal hamartoma of the tongue
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  1. M Vaidyanathan1,
  2. C E C S Williams1,
  3. P R Morgan2
  1. 1Department of Paediatric Dentistry, Guys and St Thomas’ NHS Foundation Trust, London, UK
  2. 2Department of Oral Pathology, Guys and St Thomas’ NHS Foundation Trust, London, UK
  1. Correspondence to M Vaidyanathan, minavaidyanathan{at}hotmail.co.uk

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Description

Presenting problem

A healthy 14-month-old South American girl presented with a soft tissue swelling on the left lateral border of her tongue, which had been present at birth and had gradually increased in size. The lesion was white in colour, 1.5 cm in size, well circumscribed and mobile (figure 1).

Figure 1

Clinical photo showing lesion on lateral border of the tongue.

Clinical management

Following consultation with the Department of Oral Surgery, surgical excision of the swelling was carried out under general anaesthesia. The swelling was sent for histological examination and a diagnosis of rhabdomyomatous (mesenchymal) hamartoma of the tongue (RMH) was made (figures 2 and 3). At the most recent follow-up appointment 2 years postsurgery there has been no reoccurrence.

Figure 2

Histological slide of H&E staining – low power.

Figure 3

Desmin staining to identify striated muscle characteristically found in the connective tissue in these lesions (brown staining).

Discussion

RMH is a rare congenital tumour occurring on the face and in the head and neck. Like other hamartomas they are composed of a mixture of tissues including mature adipose tissue, skeletal muscle, adnexal structures and sometimes blood vessels and nerves. The aetiology is unknown but theories proposed include migration of the mesodermally derived tissues or genetic factors. It can also be associated with congenital abnormalities such as Delleman’s syndrome. RMH very rarely presents intraorally on the tongue with isolated cases reported in the literature.1 2 Reoccurrence of RMH in the oral cavity is very rare. This case highlights the importance of including hamartomas as a differential diagnosis for soft tissue intraoral swellings in children.

References

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.