Available online at www.sciencedirect.com
British Journal of Oral and Maxillofacial Surgery 52 (2014) e4–e6
Short communication
Heterotopic central nervous system tissue arising in the palatopharyngeal region associated with cleft palate: case report Q. Zhang a,∗ , C.Y. Luo a , Y. Huang b , Y.J. Wang a a b
Department of Oral & Maxillofacial Surgery, Affiliated Stomatological Hospital of Nanchang University, Nanchang, China Department of Pathology, Fujian Provincial Hospital, Fuzhou, China
Received 22 July 2013; accepted 1 October 2013 Available online 31 October 2013
Abstract We present a rare case of heterotopic central nervous system tissue that arose in the palatopharyngeal region in a 4-year girl with a cleft palate. © 2013 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Heterotopic central nervous system tissue; Glial choristoma; Developmental malformation; Cleft palate
Introduction Heterotopic central nervous system tissue, also named glial choristoma, is an uncommon developmental malformation that usually occurs in midline structures of the head and neck1 and is rare in the palatopharyngeal region.2–4 Even more rarely other developmental anomalies, such as cleft palate, may also be present.5–7 Here we describe a case that arose in the palatopharyngeal region in a 4-year girl with a cleft palate.
Case report A 4-yeal-old girl was admitted to our department with a mass in the palatopharyngeal region during a palatoplasty at the local hospital 2 months previously. She had been born with a cleft uvula, but no history of hereditary or environmental ∗
Corresponding author. Tel.: +86 791 86361896. E-mail addresses: [email protected] (Q. Zhang), [email protected] (C.Y. Luo), [email protected] (Y. Huang), [email protected] (Y.J. Wang).
factors related to cleft palate. She was delivered normally at the end of a full-term pregnancy and was growing. She had no other medical problems, such as epistaxis or cerebrospinal fluid rhinorrhoea. On physical examination there was a mass measuring about 2.5 cm × 1.5 cm in the palatopharyngeal region adjacent to the posterior margin of the soft palate. The mass was lobulated and reddish, and was solid on palpation. There were no signs of nasal obstruction or respiratory embarrassment. The remainder of the physical examination was unremarkable. A computed tomographic scan (CT) (Fig. 1) showed a hypodense globular lesion with a clear boundary (30 mm × 40 mm) in the palatopharyngeal region. We could find no defect in the cranial bone or apparent connections to the adjacent structures of the brain. We took an excision biopsy specimen under general anaesthesia, histopathological examination of which showed neuroglial tissue composed of round to oval cells with uniform, basophilic nuclei that resembled astrocytes interspersed with an eosinophilic, fibrillar network and sparse oligodentrocytes or microgliocytes (Fig. 2). Choroid plexus tissue could be seen in some areas (Fig. 3), which made the diagnosis consistent with heterotopic central nervous system tissue.
0266-4356/$ – see front matter © 2013 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.bjoms.2013.10.002
Q. Zhang et al. / British Journal of Oral and Maxillofacial Surgery 52 (2014) e4–e6
e5
Fig. 3. Photomicrograph showing the choroid plexus nature of the papillary formations (haematoxylin and eosin, original magnification 200×).
Fig. 1. Computed tomographic scan showing a hypodense globular lesion (30 mm × 40 mm) in the palatopharyngeal region.
Her postoperative course was uneventful. Two years after operation there was no evidence of complications, in particular mental problems.
Discussion Heterotopic central nervous system tissue has been defined as a mass composed of mature brain tissue isolated from the cranial cavity or spinal canal,1 and it seems to have a female predominance. However, its pathogenesis has not been clearly explained, though 3 mechanisms have been proposed.1,8 The first presupposes protrusion of neural glial tissue from the developing
cerebral tissue that becomes isolated from the brain during later development. The second theory speculates that the displacement of neuroectodermal cells at an early stage of embryogenesis may subsequently differentiate into various cells of neuroglial tissue in ectopic sites. The third theory assumes that the lesion is a neoplasm. However, the lesion did not behave like a neoplasm, its growth rate being parallel to that of the surrounding tissues. We therefore think that the lesion should be classified as a developmental malformation rather than a neoplasm. Such growths are generally there at birth, or present within the first few years of life, which also suggests a congenital nature. Some authors have supported the first theory, which suggests protrusion of cranial tissue through an aperture in the cranium during embryogenesis. Such lesion would usually have a stalk connecting it with the subarachnoid space or the ventricular system, [2,3] but in our case there was no apparent evidence on CT that the lesion was connected to the skull. It can also be associated with other conditions.7 Uemura et al. reviewed 17 reported nasopharyngeal glial choristomas and found that 6 developed with a soft cleft palate in each case.8 Until now, only a few cases have been reported. The embryological basis for these malformations may be explained by the second theory. During embryogenesis, the neural tube fuses during the fifth week whereas the palate forms during weeks 9–12. These cases propose the model of a unifying sequence of developmental events whereby deformation of heterotopic central nervous system tissue results in secondary palatal clefting. However, it is still not clear why in most cases the palate remains normal.
Conflict of interest Fig. 2. Photomicrograph showing the mature glial tissue interspersed with collagen (haematoxylin and eosin, original magnification 400×).
There is no conflict of interest exists.
e6
Q. Zhang et al. / British Journal of Oral and Maxillofacial Surgery 52 (2014) e4–e6
References 1. Sun LS, Sun ZP, Ma XC, et al. Glial choristoma in the oral and maxillofacial region: a clinicopathologic study of 6 cases. Arch Pathol Lab Med 2008;132:984–8. 2. Chen CY, Huang JH, Choi WM, et al. Parapharyngeal neuroglial heterotopia presenting as a growing single locular cyst: MR imaging findings. Am J Neuroradiol 2005;26:96–9. 3. Huisman TA, Brehmer U, Zeilinger G, et al. Parapharyngeal neuroglial heterotopia extending through the skull base in a neonate with airway obstruction. J Pediatr Surg 2007;42:1764–7. 4. Longo D, Menchini L, Delfino LN, et al. Parapharyngeal neuroglial heterotopia in Pierre Robin sequence: MR imaging findings. Int J Pediatr Otorhinolaryngol 2009;73:1308–10.
5. Giannas JE, Bayat A, Davenport PJ. Heterotopic nasopharyngeal brain tissue associated with cleft palate. Br J Plast Surg 2005;58: 862–4. 6. Gold AH, Sharer LR, Walden RH. Central nervous system heterotopia in association with cleft palate. Plast Reconstr Surg 1980;66: 434–41. 7. Noyola-Frias MA, Toranzo-Fernandez JM, Aguirre-Castillo A, et al. Heterotopic neuroglial tissue associated with bilateral palatine cleft. J Clin Pediatr Dent 2008;32:305–8. 8. Uemura T, Yoshikawa A, Onizuka T, et al. Heterotopic nasopharyngeal brain tissue associated with cleft palate. Cleft Palate Craniofac J 1999;36:248–51.
British Journal of Oral and Maxillofacial Surgery 52 (2014) e4–e6
Short communication
Heterotopic central nervous system tissue arising in the palatopharyngeal region associated with cleft palate: case report Q. Zhang a,∗ , C.Y. Luo a , Y. Huang b , Y.J. Wang a a b
Department of Oral & Maxillofacial Surgery, Affiliated Stomatological Hospital of Nanchang University, Nanchang, China Department of Pathology, Fujian Provincial Hospital, Fuzhou, China
Received 22 July 2013; accepted 1 October 2013 Available online 31 October 2013
Abstract We present a rare case of heterotopic central nervous system tissue that arose in the palatopharyngeal region in a 4-year girl with a cleft palate. © 2013 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Heterotopic central nervous system tissue; Glial choristoma; Developmental malformation; Cleft palate
Introduction Heterotopic central nervous system tissue, also named glial choristoma, is an uncommon developmental malformation that usually occurs in midline structures of the head and neck1 and is rare in the palatopharyngeal region.2–4 Even more rarely other developmental anomalies, such as cleft palate, may also be present.5–7 Here we describe a case that arose in the palatopharyngeal region in a 4-year girl with a cleft palate.
Case report A 4-yeal-old girl was admitted to our department with a mass in the palatopharyngeal region during a palatoplasty at the local hospital 2 months previously. She had been born with a cleft uvula, but no history of hereditary or environmental ∗
Corresponding author. Tel.: +86 791 86361896. E-mail addresses: [email protected] (Q. Zhang), [email protected] (C.Y. Luo), [email protected] (Y. Huang), [email protected] (Y.J. Wang).
factors related to cleft palate. She was delivered normally at the end of a full-term pregnancy and was growing. She had no other medical problems, such as epistaxis or cerebrospinal fluid rhinorrhoea. On physical examination there was a mass measuring about 2.5 cm × 1.5 cm in the palatopharyngeal region adjacent to the posterior margin of the soft palate. The mass was lobulated and reddish, and was solid on palpation. There were no signs of nasal obstruction or respiratory embarrassment. The remainder of the physical examination was unremarkable. A computed tomographic scan (CT) (Fig. 1) showed a hypodense globular lesion with a clear boundary (30 mm × 40 mm) in the palatopharyngeal region. We could find no defect in the cranial bone or apparent connections to the adjacent structures of the brain. We took an excision biopsy specimen under general anaesthesia, histopathological examination of which showed neuroglial tissue composed of round to oval cells with uniform, basophilic nuclei that resembled astrocytes interspersed with an eosinophilic, fibrillar network and sparse oligodentrocytes or microgliocytes (Fig. 2). Choroid plexus tissue could be seen in some areas (Fig. 3), which made the diagnosis consistent with heterotopic central nervous system tissue.
0266-4356/$ – see front matter © 2013 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.bjoms.2013.10.002
Q. Zhang et al. / British Journal of Oral and Maxillofacial Surgery 52 (2014) e4–e6
e5
Fig. 3. Photomicrograph showing the choroid plexus nature of the papillary formations (haematoxylin and eosin, original magnification 200×).
Fig. 1. Computed tomographic scan showing a hypodense globular lesion (30 mm × 40 mm) in the palatopharyngeal region.
Her postoperative course was uneventful. Two years after operation there was no evidence of complications, in particular mental problems.
Discussion Heterotopic central nervous system tissue has been defined as a mass composed of mature brain tissue isolated from the cranial cavity or spinal canal,1 and it seems to have a female predominance. However, its pathogenesis has not been clearly explained, though 3 mechanisms have been proposed.1,8 The first presupposes protrusion of neural glial tissue from the developing
cerebral tissue that becomes isolated from the brain during later development. The second theory speculates that the displacement of neuroectodermal cells at an early stage of embryogenesis may subsequently differentiate into various cells of neuroglial tissue in ectopic sites. The third theory assumes that the lesion is a neoplasm. However, the lesion did not behave like a neoplasm, its growth rate being parallel to that of the surrounding tissues. We therefore think that the lesion should be classified as a developmental malformation rather than a neoplasm. Such growths are generally there at birth, or present within the first few years of life, which also suggests a congenital nature. Some authors have supported the first theory, which suggests protrusion of cranial tissue through an aperture in the cranium during embryogenesis. Such lesion would usually have a stalk connecting it with the subarachnoid space or the ventricular system, [2,3] but in our case there was no apparent evidence on CT that the lesion was connected to the skull. It can also be associated with other conditions.7 Uemura et al. reviewed 17 reported nasopharyngeal glial choristomas and found that 6 developed with a soft cleft palate in each case.8 Until now, only a few cases have been reported. The embryological basis for these malformations may be explained by the second theory. During embryogenesis, the neural tube fuses during the fifth week whereas the palate forms during weeks 9–12. These cases propose the model of a unifying sequence of developmental events whereby deformation of heterotopic central nervous system tissue results in secondary palatal clefting. However, it is still not clear why in most cases the palate remains normal.
Conflict of interest Fig. 2. Photomicrograph showing the mature glial tissue interspersed with collagen (haematoxylin and eosin, original magnification 400×).
There is no conflict of interest exists.
e6
Q. Zhang et al. / British Journal of Oral and Maxillofacial Surgery 52 (2014) e4–e6
References 1. Sun LS, Sun ZP, Ma XC, et al. Glial choristoma in the oral and maxillofacial region: a clinicopathologic study of 6 cases. Arch Pathol Lab Med 2008;132:984–8. 2. Chen CY, Huang JH, Choi WM, et al. Parapharyngeal neuroglial heterotopia presenting as a growing single locular cyst: MR imaging findings. Am J Neuroradiol 2005;26:96–9. 3. Huisman TA, Brehmer U, Zeilinger G, et al. Parapharyngeal neuroglial heterotopia extending through the skull base in a neonate with airway obstruction. J Pediatr Surg 2007;42:1764–7. 4. Longo D, Menchini L, Delfino LN, et al. Parapharyngeal neuroglial heterotopia in Pierre Robin sequence: MR imaging findings. Int J Pediatr Otorhinolaryngol 2009;73:1308–10.
5. Giannas JE, Bayat A, Davenport PJ. Heterotopic nasopharyngeal brain tissue associated with cleft palate. Br J Plast Surg 2005;58: 862–4. 6. Gold AH, Sharer LR, Walden RH. Central nervous system heterotopia in association with cleft palate. Plast Reconstr Surg 1980;66: 434–41. 7. Noyola-Frias MA, Toranzo-Fernandez JM, Aguirre-Castillo A, et al. Heterotopic neuroglial tissue associated with bilateral palatine cleft. J Clin Pediatr Dent 2008;32:305–8. 8. Uemura T, Yoshikawa A, Onizuka T, et al. Heterotopic nasopharyngeal brain tissue associated with cleft palate. Cleft Palate Craniofac J 1999;36:248–51.
