Giant chondrolipoma of the tongue in a 14-year-old child with mandibular hypertrophy. Isolated lesion or regional overgrowth syndrome

Abstract

Chondrolipoma is a rare condition; especially in the oral cavity. The authors described a giant chondrolipoma of the tongue, associated with mandibular and lower lip overgrowth, in a 14-year-old boy. After tumour excision, histopathological examination showed mature cartilage within lobules of mature adipocytes. This is the first case of giant chondrolipoma associated with facial overgrowth. The aetiology and the association with a localized Proteus syndrome are discussed.

Chondrolipoma is a rare, benign mesenchymal tumour. It occurs in almost any region of the body, particularly in the soft tissues of the skeletal system, breast, pharynx, and nasopharynx. It is relatively uncommon in oral tissues . Only five cases of tongue chondrolipoma have been reported in the literature and only one paediatric case .

The authors present a case of giant tongue chondrolipoma, associated with three lower lip lipomas, and mandibular overgrowth. To the authors’ knowledge this is the first case reported in the literature of giant tongue chondrolipoma in a child, associated with facial overgrowth. The authors suggest that these lesions represent a mild, regional form of Proteus syndrome.

Case report

A 14-year-old boy without remarkable medical or family history underwent surgery 6 years ago for a small lesion to the lower gum without histological analysis. The gingival and tongue lesions have been growing slowly, progressively and asymptomatically. When he was referred to the authors’ hospital, intra-oral examination revealed a large, well-circumscribed mass at the left lateral border of the tongue. On palpation, the mass was tender, painless and contained a hard area on the tip of the tongue. No ulceration was noted ( Fig. 1 ). Consecutive to this extremely large macroglossia, there was an advancement and opening of the mandibular angle downwards. The patient presented an anterior open bite with labial incompetence.

Fig. 1
Frontal photographs. (A) Large, well circumscribed mass at the left lateral border of the tongue. (B) Intra-oral view showing mandibular and gingival overgrowth.

Intra-oral examination found three other painless, 1 cm, soft tissue nodules on the lower lip, a massive bone formation of the mandibular symphysis and a gingival hyperplasia ( Fig. 1 ). This progressive mandibular and gingival overgrowth covered and displaced all the teeth from 34 to 44 ( Fig. 2 ). A solitary café au lait spot was present on his forehead. Otherwise the physical examination and laboratory findings were unremarkable. Karyotype analysis was normal. Radiological investigations (computed tomography scan and magnetic resonance imaging) showed a giant well-circumscribed fatty mass in the left side of the tongue leading to partial obstruction of the upper airway. Bone erosion and abnormal asymmetric overgrowth of the anterior mandible were noted ( Fig. 2 ). An abdominal ultrasound scan was normal. Ophthalmological investigation showed no ocular anomalies.

Fig. 2
Radiological examination. (A) Orthopantomography showing mandibular overgrowth with displacement of the anterior teeth. (B) Lateral cephalogram showing mandibular overgrowth, and advancement and opening of the mandibular angle. (C) CT scan showing a 16 cm fatty mass into the tongue, partial upper way obstruction and mandibular hyperplasia. (D) Sagittal MRI view (T1) showing a giant fatty tumour of the tongue and mandibular overgrowth.

Under general anaesthesia and using an intraoral approach, the tongue and lower lip lesions were completely excised ( Fig. 3 ). Biopsies of the gingival and alveolar hypertrophy were carried out. Macroscopically, section of the 234 g tongue samples showed a soft and yellow lesion.

Fig. 3
(A) 230 g, 16 cm, surgical specimen of the tongue showing a large lobulated lesion. (B) No recurrence 4 years after surgery.

Pathological examination showed lobules of mature adipose tissue dissociating the tongue muscles with one focus of mature cartilage underneath the tongue mucosa. No evidence of malignant change was found and chondrolipoma of the tongue was diagnosed ( Fig. 4 ). Histological examination of the lower lip nodules found normal surface mucosa and regular infiltrating mature adipose tissue. The gingival and mandibular biopsies revealed gingival and alveolar fibromatous hypertrophy. Four years after surgery, there has been no recurrence ( Fig. 3 ).

Jan 26, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Giant chondrolipoma of the tongue in a 14-year-old child with mandibular hypertrophy. Isolated lesion or regional overgrowth syndrome
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