Pediatric Annals

Case Challenges 

A 2-month-old Girl with an Enlarging Lesion of the Nasal Root

Grace S. Sun, MS; Adekunle M. Adesina, MD; Ronald A. Rauch, MD

Abstract

A 2-month-old, otherwise healthy white girl presented with an enlarging lesion of the nasal root, slightly to the right of midline. The lesion was first seen on prenatal ultrasound at 7 months’ gestation. At birth, the lesion was a firm, darkly violaceous nodule. Her mother reported that immediately following birth, the lesion became smaller and lighter in color, but subsequently re-enlarged. Physical examination showed a 3-cm rubbery, pink nodule with peripheral, sparse hypertrichosis (see Figure 1). The lesion was not warm, tender, nor pulsatile. No pseudohypertelorism or visual obstruction was noted. Magnetic resonance imaging (MRI) and computerized tomography (CT) imaging was obtained (see Figure 2), following which the infant underwent complete surgical excision (see Figure 3).

Microscopic Findings

Histologic sections show subdermal and subcutaneous ectopic neural tissue with variable density of glial cells and ganglion cells within an eosinophilic neuropil-like stroma (see Figure 3). Many glial cells show plump astrocytic cytoplasm consistent with those of gemistocytic astrocytes.

No immature neuroectodermal tissue, other ectodermal derivatives, or mesenchymal/endodermal derivatives are seen. Immunostains show positivity for synaptophysin and MAP-2 in the neuropil and ganglion cells; GFAP positivity is diffuse and present in the astrocytic glial cells.

Radiologic Findings

MRI of the face and brain (see Figure 2) show a 16.5 x 21.9-mm mass protruding through the cribiform plate that appears contiguous with the fronto-orbital gyri in the floor of the anterior cranial fossa. CT (see Figure 2) shows an enhancing soft tissue mass distorting and overlying the nasal bones. The mass extends through the foramen cecum or the fonticulus frontalis, approaching but not extending through the dura.

Figure 1. Physical examination showed a 3-cm rubbery, pink nodule
Figure 1. Physical examination showed a 3-cm rubbery, pink nodule with peripheral, sparse hypertrichosis.
Figure 2. MRI of the face and brain
Figure 2. MRI of the face and brain (left) show a 16.5 x 21.9 mm mass protruding through the cribiform plate, which appears contiguous with the fronto-orbital gyri in the floor of the anterior cranial fossa. CT (right) shows an enhancing soft tissue mass distorting and overlying the nasal bones.

Diagnosis: Nasal Glioma

Nasal gliomas (NG), also known as nasal cerebral heterotopias or glial heterotopias, are rare, benign masses of glial tissue located near the nasal glabella that may be extranasal (60%), intranasal (30%), or both (10%).1 Their frequency is 1 in 20,000 to 40,000 live births,2 and lesions are more common in males than females by a 3:2 ratio.3

Figure 3. Histology of nasal glioma
Figure 3. Histology of nasal glioma showing subdermal neural tissue (A) composed of astrocytes, some of which are plump gemistocytes (arrowhead) and ganglion cells (arrow) (B). Immunostains for synaptophysin (C and D) and glial fibrillary acidic protein (E and F) define the neuronal and astrocytic components of the tissue, respectively. Magnification: A, C and E x40; B, D and F x400.

NG are forebrain extensions growing through the foramen cecum or fonticulus frontalis during embryogenesis, which eventually lose communication with the cranial cavity.4 If communication with the intracranial cavity remains intact, then the mass is an encephalocele. Encephaloceles present clinically with pulsation or expansion of the mass with actions increasing intracranial pressure.

Diagnostic workup includes MRI for defining the soft tissue mass and CT for determining the extent of the nasal bone distortion, and surgical intervention is required.5 Attempted aspiration or fine needle biopsy should be avoided due to the risk of encephalocele complications. Complete surgical removal is recommended.6 Incomplete removal accounts for the recurrence rates between 4% and 10%.7

The violaceous color of our patient’s lesion at birth, combined with the initial reduction in size and lightening of color, led to the inclusion of tufted angioma, congenital self-healing reticulohistiocytosis, and rapidly involuting congenital hemangioma (RICH) in our differential diagnosis. NG typically remain stable in size or enlarge;7 we speculate that birth trauma may have been accountable for the unusual color and enlargement noted in our patient’s lesion initially. Although there has been a report of eccrine ductal proliferation in association with NG,8 the presence of peripheral hypertrichosis associated with NG, to our knowledge, has not been reported previously. However, hypertrichosis is not uncommonly seen with other neural lesions, including encephaloceles.

References

  1. Pensler JM, Ivescu AS, Ciletti SJ, Yokoo KM, Byrd SE. Cranifacial gliomas. Plas Reconstr Surg. 1996;98(1):27-30.
  2. Hughes GB, Sharpino G, Hunt W, Tucker HM. Management of the congenital midline nasal masses: a review. Head Neck Surg. 1980;2(3):222-233.
  3. Strauss RB, Callicott JH, Hargett IR. Intranasal heterotopia. So-called nasal glioma. Am J Dis Child. 1966;111(3):317-320.
  4. Patterson K, Kapur S, Chandra RS. Nasal gliomas and related brain heterotopias: a pathologist’s perspective. Pediatr Pathol. 1986;5(3-4):353-362.
  5. Puppala B, Mangurten HH, McFadden J, Lygizos N, Taxy J, Pellettiere E. Nasal glioma. Presenting as neonatal respiratory distress. Definition of the tumor mass by MRI. Clin Pediatr (Phila). 1990;29(1):49-52.
  6. Niedzielska G, Niedzielski A, Kotowski M. Nasal ganglioglioma — difficulties in radiological imaging. Int J Pediatr Otorhinolaryngol. 2008;72(2):285-287.
  7. Dasgupta NR, Bentz ML. Nasal gliomas: identification and differentiation from hemangiomas. J Craniofac Surg. 2003;14(5):736-738.
  8. Gambini C, Rongioletti F, Rebora A. Proliferation of eccrine sweat ducts associated with heterotopic neural tissue (nasal glioma). Am J Dermatopathol. 2000;22(2):179-182.

ABOUT THE AUTHORS

Grace S. Sun, MS, is with Baylor College of Medicine, Houston. Adekunle M. Adesina, MD, is with the Department of Pathology, Texas Children’s Hospital, Baylor College of Medicine. Ronald A. Rauch, MD, is with the Department of Radiology, Texas Children’s Hospital, Baylor College of Medicine. Denise W. Metry, MD, is with the Department of Dermatology, Texas Children’s Hospital, Baylor College of Medicine.

Address correspondence to: Denise W. Metry, MD, Department of Dermatology, Texas Children’s Hospital, 6621 Fannin St., CC 620.16, Houston, TX 77030; fax: 832-825-3722; or e-mail dmetry@bcm.tmc.edu.

Ms. Sun, Dr. Adesine, Dr. Rauch, and Dr. Metry have disclosed no relevant financial relationships.

Abstract

A 2-month-old, otherwise healthy white girl presented with an enlarging lesion of the nasal root, slightly to the right of midline. The lesion was first seen on prenatal ultrasound at 7 months’ gestation. At birth, the lesion was a firm, darkly violaceous nodule. Her mother reported that immediately following birth, the lesion became smaller and lighter in color, but subsequently re-enlarged. Physical examination showed a 3-cm rubbery, pink nodule with peripheral, sparse hypertrichosis (see Figure 1). The lesion was not warm, tender, nor pulsatile. No pseudohypertelorism or visual obstruction was noted. Magnetic resonance imaging (MRI) and computerized tomography (CT) imaging was obtained (see Figure 2), following which the infant underwent complete surgical excision (see Figure 3).

Microscopic Findings

Histologic sections show subdermal and subcutaneous ectopic neural tissue with variable density of glial cells and ganglion cells within an eosinophilic neuropil-like stroma (see Figure 3). Many glial cells show plump astrocytic cytoplasm consistent with those of gemistocytic astrocytes.

No immature neuroectodermal tissue, other ectodermal derivatives, or mesenchymal/endodermal derivatives are seen. Immunostains show positivity for synaptophysin and MAP-2 in the neuropil and ganglion cells; GFAP positivity is diffuse and present in the astrocytic glial cells.

Radiologic Findings

MRI of the face and brain (see Figure 2) show a 16.5 x 21.9-mm mass protruding through the cribiform plate that appears contiguous with the fronto-orbital gyri in the floor of the anterior cranial fossa. CT (see Figure 2) shows an enhancing soft tissue mass distorting and overlying the nasal bones. The mass extends through the foramen cecum or the fonticulus frontalis, approaching but not extending through the dura.

Figure 1. Physical examination showed a 3-cm rubbery, pink nodule
Figure 1. Physical examination showed a 3-cm rubbery, pink nodule with peripheral, sparse hypertrichosis.
Figure 2. MRI of the face and brain
Figure 2. MRI of the face and brain (left) show a 16.5 x 21.9 mm mass protruding through the cribiform plate, which appears contiguous with the fronto-orbital gyri in the floor of the anterior cranial fossa. CT (right) shows an enhancing soft tissue mass distorting and overlying the nasal bones.

Diagnosis: Nasal Glioma

Nasal gliomas (NG), also known as nasal cerebral heterotopias or glial heterotopias, are rare, benign masses of glial tissue located near the nasal glabella that may be extranasal (60%), intranasal (30%), or both (10%).1 Their frequency is 1 in 20,000 to 40,000 live births,2 and lesions are more common in males than females by a 3:2 ratio.3

Figure 3. Histology of nasal glioma
Figure 3. Histology of nasal glioma showing subdermal neural tissue (A) composed of astrocytes, some of which are plump gemistocytes (arrowhead) and ganglion cells (arrow) (B). Immunostains for synaptophysin (C and D) and glial fibrillary acidic protein (E and F) define the neuronal and astrocytic components of the tissue, respectively. Magnification: A, C and E x40; B, D and F x400.

NG are forebrain extensions growing through the foramen cecum or fonticulus frontalis during embryogenesis, which eventually lose communication with the cranial cavity.4 If communication with the intracranial cavity remains intact, then the mass is an encephalocele. Encephaloceles present clinically with pulsation or expansion of the mass with actions increasing intracranial pressure.

Diagnostic workup includes MRI for defining the soft tissue mass and CT for determining the extent of the nasal bone distortion, and surgical intervention is required.5 Attempted aspiration or fine needle biopsy should be avoided due to the risk of encephalocele complications. Complete surgical removal is recommended.6 Incomplete removal accounts for the recurrence rates between 4% and 10%.7

The violaceous color of our patient’s lesion at birth, combined with the initial reduction in size and lightening of color, led to the inclusion of tufted angioma, congenital self-healing reticulohistiocytosis, and rapidly involuting congenital hemangioma (RICH) in our differential diagnosis. NG typically remain stable in size or enlarge;7 we speculate that birth trauma may have been accountable for the unusual color and enlargement noted in our patient’s lesion initially. Although there has been a report of eccrine ductal proliferation in association with NG,8 the presence of peripheral hypertrichosis associated with NG, to our knowledge, has not been reported previously. However, hypertrichosis is not uncommonly seen with other neural lesions, including encephaloceles.

References

  1. Pensler JM, Ivescu AS, Ciletti SJ, Yokoo KM, Byrd SE. Cranifacial gliomas. Plas Reconstr Surg. 1996;98(1):27-30.
  2. Hughes GB, Sharpino G, Hunt W, Tucker HM. Management of the congenital midline nasal masses: a review. Head Neck Surg. 1980;2(3):222-233.
  3. Strauss RB, Callicott JH, Hargett IR. Intranasal heterotopia. So-called nasal glioma. Am J Dis Child. 1966;111(3):317-320.
  4. Patterson K, Kapur S, Chandra RS. Nasal gliomas and related brain heterotopias: a pathologist’s perspective. Pediatr Pathol. 1986;5(3-4):353-362.
  5. Puppala B, Mangurten HH, McFadden J, Lygizos N, Taxy J, Pellettiere E. Nasal glioma. Presenting as neonatal respiratory distress. Definition of the tumor mass by MRI. Clin Pediatr (Phila). 1990;29(1):49-52.
  6. Niedzielska G, Niedzielski A, Kotowski M. Nasal ganglioglioma — difficulties in radiological imaging. Int J Pediatr Otorhinolaryngol. 2008;72(2):285-287.
  7. Dasgupta NR, Bentz ML. Nasal gliomas: identification and differentiation from hemangiomas. J Craniofac Surg. 2003;14(5):736-738.
  8. Gambini C, Rongioletti F, Rebora A. Proliferation of eccrine sweat ducts associated with heterotopic neural tissue (nasal glioma). Am J Dermatopathol. 2000;22(2):179-182.

ABOUT THE AUTHORS

Grace S. Sun, MS, is with Baylor College of Medicine, Houston. Adekunle M. Adesina, MD, is with the Department of Pathology, Texas Children’s Hospital, Baylor College of Medicine. Ronald A. Rauch, MD, is with the Department of Radiology, Texas Children’s Hospital, Baylor College of Medicine. Denise W. Metry, MD, is with the Department of Dermatology, Texas Children’s Hospital, Baylor College of Medicine.

Address correspondence to: Denise W. Metry, MD, Department of Dermatology, Texas Children’s Hospital, 6621 Fannin St., CC 620.16, Houston, TX 77030; fax: 832-825-3722; or e-mail dmetry@bcm.tmc.edu.

Ms. Sun, Dr. Adesine, Dr. Rauch, and Dr. Metry have disclosed no relevant financial relationships.

A 2-month-old, otherwise healthy white girl presented with an enlarging lesion of the nasal root, slightly to the right of midline. The lesion was first seen on prenatal ultrasound at 7 months’ gestation. At birth, the lesion was a firm, darkly violaceous nodule. Her mother reported that immediately following birth, the lesion became smaller and lighter in color, but subsequently re-enlarged. Physical examination showed a 3-cm rubbery, pink nodule with peripheral, sparse hypertrichosis (see Figure 1). The lesion was not warm, tender, nor pulsatile. No pseudohypertelorism or visual obstruction was noted. Magnetic resonance imaging (MRI) and computerized tomography (CT) imaging was obtained (see Figure 2), following which the infant underwent complete surgical excision (see Figure 3).

Microscopic Findings

Histologic sections show subdermal and subcutaneous ectopic neural tissue with variable density of glial cells and ganglion cells within an eosinophilic neuropil-like stroma (see Figure 3). Many glial cells show plump astrocytic cytoplasm consistent with those of gemistocytic astrocytes.

No immature neuroectodermal tissue, other ectodermal derivatives, or mesenchymal/endodermal derivatives are seen. Immunostains show positivity for synaptophysin and MAP-2 in the neuropil and ganglion cells; GFAP positivity is diffuse and present in the astrocytic glial cells.

Radiologic Findings

MRI of the face and brain (see Figure 2) show a 16.5 x 21.9-mm mass protruding through the cribiform plate that appears contiguous with the fronto-orbital gyri in the floor of the anterior cranial fossa. CT (see Figure 2) shows an enhancing soft tissue mass distorting and overlying the nasal bones. The mass extends through the foramen cecum or the fonticulus frontalis, approaching but not extending through the dura.

Figure 1. Physical examination showed a 3-cm rubbery, pink nodule
Figure 1. Physical examination showed a 3-cm rubbery, pink nodule with peripheral, sparse hypertrichosis.
Figure 2. MRI of the face and brain
Figure 2. MRI of the face and brain (left) show a 16.5 x 21.9 mm mass protruding through the cribiform plate, which appears contiguous with the fronto-orbital gyri in the floor of the anterior cranial fossa. CT (right) shows an enhancing soft tissue mass distorting and overlying the nasal bones.

Diagnosis: Nasal Glioma

Nasal gliomas (NG), also known as nasal cerebral heterotopias or glial heterotopias, are rare, benign masses of glial tissue located near the nasal glabella that may be extranasal (60%), intranasal (30%), or both (10%).1 Their frequency is 1 in 20,000 to 40,000 live births,2 and lesions are more common in males than females by a 3:2 ratio.3

Figure 3. Histology of nasal glioma
Figure 3. Histology of nasal glioma showing subdermal neural tissue (A) composed of astrocytes, some of which are plump gemistocytes (arrowhead) and ganglion cells (arrow) (B). Immunostains for synaptophysin (C and D) and glial fibrillary acidic protein (E and F) define the neuronal and astrocytic components of the tissue, respectively. Magnification: A, C and E x40; B, D and F x400.

NG are forebrain extensions growing through the foramen cecum or fonticulus frontalis during embryogenesis, which eventually lose communication with the cranial cavity.4 If communication with the intracranial cavity remains intact, then the mass is an encephalocele. Encephaloceles present clinically with pulsation or expansion of the mass with actions increasing intracranial pressure.

Diagnostic workup includes MRI for defining the soft tissue mass and CT for determining the extent of the nasal bone distortion, and surgical intervention is required.5 Attempted aspiration or fine needle biopsy should be avoided due to the risk of encephalocele complications. Complete surgical removal is recommended.6 Incomplete removal accounts for the recurrence rates between 4% and 10%.7

The violaceous color of our patient’s lesion at birth, combined with the initial reduction in size and lightening of color, led to the inclusion of tufted angioma, congenital self-healing reticulohistiocytosis, and rapidly involuting congenital hemangioma (RICH) in our differential diagnosis. NG typically remain stable in size or enlarge;7 we speculate that birth trauma may have been accountable for the unusual color and enlargement noted in our patient’s lesion initially. Although there has been a report of eccrine ductal proliferation in association with NG,8 the presence of peripheral hypertrichosis associated with NG, to our knowledge, has not been reported previously. However, hypertrichosis is not uncommonly seen with other neural lesions, including encephaloceles.

References

  1. Pensler JM, Ivescu AS, Ciletti SJ, Yokoo KM, Byrd SE. Cranifacial gliomas. Plas Reconstr Surg. 1996;98(1):27-30.
  2. Hughes GB, Sharpino G, Hunt W, Tucker HM. Management of the congenital midline nasal masses: a review. Head Neck Surg. 1980;2(3):222-233.
  3. Strauss RB, Callicott JH, Hargett IR. Intranasal heterotopia. So-called nasal glioma. Am J Dis Child. 1966;111(3):317-320.
  4. Patterson K, Kapur S, Chandra RS. Nasal gliomas and related brain heterotopias: a pathologist’s perspective. Pediatr Pathol. 1986;5(3-4):353-362.
  5. Puppala B, Mangurten HH, McFadden J, Lygizos N, Taxy J, Pellettiere E. Nasal glioma. Presenting as neonatal respiratory distress. Definition of the tumor mass by MRI. Clin Pediatr (Phila). 1990;29(1):49-52.
  6. Niedzielska G, Niedzielski A, Kotowski M. Nasal ganglioglioma — difficulties in radiological imaging. Int J Pediatr Otorhinolaryngol. 2008;72(2):285-287.
  7. Dasgupta NR, Bentz ML. Nasal gliomas: identification and differentiation from hemangiomas. J Craniofac Surg. 2003;14(5):736-738.
  8. Gambini C, Rongioletti F, Rebora A. Proliferation of eccrine sweat ducts associated with heterotopic neural tissue (nasal glioma). Am J Dermatopathol. 2000;22(2):179-182.

ABOUT THE AUTHORS

Grace S. Sun, MS, is with Baylor College of Medicine, Houston. Adekunle M. Adesina, MD, is with the Department of Pathology, Texas Children’s Hospital, Baylor College of Medicine. Ronald A. Rauch, MD, is with the Department of Radiology, Texas Children’s Hospital, Baylor College of Medicine. Denise W. Metry, MD, is with the Department of Dermatology, Texas Children’s Hospital, Baylor College of Medicine.

Address correspondence to: Denise W. Metry, MD, Department of Dermatology, Texas Children’s Hospital, 6621 Fannin St., CC 620.16, Houston, TX 77030; fax: 832-825-3722; or e-mail dmetry@bcm.tmc.edu.

Ms. Sun, Dr. Adesine, Dr. Rauch, and Dr. Metry have disclosed no relevant financial relationships.

10.3928/00904481-20090201-02

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