Congenital Nasal Chondromesenchymal Hamartoma

Abstract

Objectives: To report the case of a congenital nasal chondromesenchymal hamartoma in a one-year-old female and review the literature, identifying problems encountered in confirming the diagnosis and in treatment of this patient.
 Methods:
 Design: Case Report 
 Setting: Tertiary Public General Hospital
 Patient: One
 Results: A one-year-old female with an intranasal mass noted at birth and with subsequent unilateral maxillary enlargement is described. Computed tomography showed calcifications and erosion of adjacent bony structures. Histopathology and immunohistochemistry of an intranasal biopsy were interpreted as chordoma, a malignant tumor. Following surgical excision, the final histopathologic diagnosis was chondroid hamartoma.
 Conclusion: Only 20 cases of nasal chondromesenchymal hamartoma have been reported in the literature worldwide. These tumors may present clinically, histopathologicaly and radiologically as malignant tumors and may mislead even the experts. The whole clinical picture should be taken together to avoid misdiagnosis as a malignancy and to facilitate appropriate management. 
 Keywords: nasal chondromesenchymal hamartoma, nasal masses in infancy, nasal chondroid lesions

Prenatal diagnosis and appearance of nasal chondromesenchymal hamartoma in a fetus: A case report

“…Thus, normal ultrasonographic examination in early pregnancy does not rule out the probability of a nasal tumor developing later pregnancy. A probable explanation is that the mass may enlarge very rapidly, 3 or the scanning of nasal cavity may not be a routine region in ultrasound examination.…”

“…1,2 Although benign, an NCMH often exhibits characteristics similar to malignant tumors, such as involvement of adjacent paranasal sinuses and erosion of surrounding bone. 3 Although the histopathologic and radiographic of NCMH after birth have been well described in literature, there has been rare discussion about prenatal diagnosis and appearance of NCMH. In this study, we report a fetal case of NCMH which was detected by ultrasound and magnetic resonance imaging (MRI) in prenatal stages that have not been detailed reported before.…”

“…The usual clinical presentations in infants range from asymptomatic to nasal obstruction, respiratory distress, and feeding difficulties 1,2 . Although benign, an NCMH often exhibits characteristics similar to malignant tumors, such as involvement of adjacent paranasal sinuses and erosion of surrounding bone 3 . Although the histopathologic and radiographic of NCMH after birth have been well described in literature, there has been rare discussion about prenatal diagnosis and appearance of NCMH.…”