International Journal of Pediatric Otorhinolaryngology Extra
Volume 4, Issue 3 , Pages 111-113, September 2009

Nasal chondromesenchymal hamartoma in an adolescent

Department of Otolaryngology, Head and Neck Surgery, Graduate School of Medicine, Kyoto University, Kawaharacho 54, Shogoin, Sakyo-ku, 606-8507 Kyoto, Japan

Received 22 July 2008; accepted 9 September 2008. published online 24 October 2008.

Article Outline

Summary 

Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign tumour that was first described in 1998. It has been considered to be a disease of infancy. Here we report a case of NCMH in a 12-year-old boy who presented with a tumour in the left nasal cavity. Computed tomography and magnetic-resonance imaging revealed a heterogeneous and polypoid mass. The patient underwent endoscopic surgical excision. Histological analyses of the tumour were consistent with NCMH. This case report indicates that NCMH should be included in the differential diagnosis of paediatric nasal cavity lesions.

Keywords: Endoscopic surgery, Hamartoma, Nose, Paediatric tumour

 

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1. Introduction 

A hamartoma is an overgrowth of tissues native to the site in which it occurs. Nasal chondromesenchymal hamartoma (NCMH) is a rare and benign neoplasm, with only 21 cases having been reported in the literature since it was first described by McDermott et al. in 1998 [1]. Patients typically present with a nasal mass or local symptoms of obstruction [1], [2]. Skull base and orbital extension, but not aggressive bony destruction, has frequently been documented [1], [2], [3], [4], [5], [6], [7]. Although previous reports have stated that NCMH is essentially a disease of infancy, there are an increasing number of reports in adolescents and adults. Here we report a case of NCMH in a 12-year-old boy who presented with a tumour in the left nasal cavity. To our knowledge, nine of the 22 cases (including the current case) have occurred in patients over 10 years of age [6], [8], [9], [10], [11]. The knowledge that NCMH can occur in an older age group is essential for correct diagnosis and treatment.

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2. Case report 

A 12-year-old boy presented with left-sided severe nasal obstruction. He was otherwise healthy. A polypoid mass was identified, and endonasal resection was performed. The histopathological diagnosis was NCMH. Two months after the resection, tumour recurrence was identified, and the patient was sent to Kyoto University Hospital, Japan, for a consultation. Rhinoscopy revealed a polypoid mass in the roof of the left side of the nose, which extended to the nasal floor and the sphenoethmoidal recess (Fig. 1). The findings upon examination of the reminder of the head and neck were unremarkable.

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  • Fig. 1. 

    Endoscopic view of the tumour. An arrow indicates the tumour in the left nasal cavity. The location of the middle turbinate is indicated by an arrowhead, and that of the inferior turbinate is indicated by an asterisk.

Computed tomography (CT) scans revealed a heterogeneous and solid mass extending to the sphenoid sinus; soft-tissue densities in the left posterior ethmoid sinuses and bilateral maxillary sinuses. Fig. 2a indicated the presence of a tumour extending from the roof of the nasal cavity to the sphenoid and posterior ethmoid sinuses. Sagittal views of the CT scan showed bony erosion of the roof of the nasal cavity (Fig. 2b). No calcification was identified in the mass lesion. T2-weighted magnetic-resonance images demonstrated a heterogeneous and slightly high-intense mass (Fig. 2c). T1-weighted magnetic-resonance images demonstrated an iso-density mass that was enhanced by gadolinium (Fig. 2d and e).

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  • Fig. 2. 

    CT and MR images. Coronal (a) and sagittal (b) non-contrast CT images and coronal T2-weighted (c) axial T1-weighted (d) and gadolinium-enhanced coronal T1-weighted (e) images show a heterogenous mass (arrows) in the nasal cavity. An arrowhead in (b) indicates bony erosion of the roof of the nasal cavity, and an arrowhead in (c) shows a high-intense lesion in the ethomoid sinus.

The patient underwent endoscopic endonasal excision of the tumour. Initially, partial resection of the tumour was performed to visualize its neck, which was located in the roof of the nasal cavity. Ethmoidectomy and sphenoidotomy was then performed, which confirmed a lack of tumour invasion into the sphenoid and posterior ethmoid sinuses. The superior turbinate was resected, and the tumour was completely excised. The removal of the tumour from the roof of the nasal cavity was smooth, and no intracranial invasion was identified. There was no cerebrospinal leak during the surgical procedures. The resected lesion measured approximately 1.5cm in its maximum dimension. On gross examination, the tumour appeared to be a soft, white mass with areas of cartilage. Microscopic examination identified a cartilaginous lesion with respiratory mucosa, and no evidence of malignancy (Fig. 3). The patient had an uneventful postoperative course, and no further recurrence was detected during the 5-month follow-up period.

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3. Discussion 

Johnson et al. summarized the CT imaging characteristics of NCMH [11]. Internal calcification and cystic components are common CT findings. In the current case, preoperative CT and magnetic-resonance imaging revealed a heterogeneous solid mass without internal calcification or cystic components. However, internal calcification and/or cystic components are not specific to NCMH. In preference to the tumour characteristics, an anatomical extent including skull base or orbital extension is considered more important, as it is suggestive of malignancies. Because NCMH is considered to be benign, correct diagnosis is imperative to avoid potentially harmful therapies. An incisional biopsy of the lesion is therefore crucial. Complete surgical excision is the treatment of choice for NCMH. If the tumour is limited to the nasal cavity and/or paranasal sinuses, endoscopic sinus surgery is an appropriate choice. Even in cases with intracranial extension, endoscopic skull-base surgery might be included among the various therapeutic options.

NCMH comprises various mesenchymal components, predominantly mature or immature cartilage. The lesion is typically composed of large and lobular islands of hyaline cartilage with myxoid fibrous stroma. The histopathological differential diagnoses include fibrous dysplasia, chondrosarcoma and osteosarcoma. However, mature or immature cartilage is unusual for fibrous dysplasia. Although the cartilaginous component of NCMH is sometimes hypercellular, it lacks significant atypia, which is useful for differential diagnosis from chondrosarcoma or osteosarcoma. Histological diagnosis is thus relatively simple, although difficulties were documented in an early case that was misdiagnosed as mesenchymal chondrosarcoma [12].

To our knowledge, this is the second case of NCMH to be reported in Japan. Although it is rare, it is a well-recognized lesion of the nose and paranasal sinuses. As the current case arose in a 12-year-old boy, NCMH should be considered in the differential diagnosis of adolescent tumours in the nasal and/or paranasal sinuses. Complete excision is the treatment of choice. An accurate diagnosis of this benign lesion is required to avoid misdiagnosing the malignancy and to ensure appropriate surgery.

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Conflict of interest 

None.

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References 

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PII: S1871-4048(08)00066-X

doi:10.1016/j.pedex.2008.09.002

International Journal of Pediatric Otorhinolaryngology Extra
Volume 4, Issue 3 , Pages 111-113, September 2009

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