Nasal glioma LEONARD F. HIRSH, M.D., SYLVAN E. STOOL, M.D., THOMAS W. LANGFITT, M.D., AND LUIS SCHUT, M.D.
Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania v~ Six cases of nasal gliomas, which are rare ectopic rests of neural tissue found at the root of the nose, are presented. It is important to distinguish nasal tumors from basofrontal encephaloceles to avoid inadvertent exposure of the brain during the surgical removal of mass lesions. Because of their related embryologic origins, the distinction between nasal gliomas and basofrontal encephaloceles may not be clear clinically. Nasal gliomas may be treated by several surgical specialties, and only a proper awareness of their relationship to encephaloceles can assure the selection of a flexible and adequate surgical approach. This paper emphasizes the salient clinical characteristics of nasal gliomas, their clinical distinction from and embryologic relationship to encephaloceles, and the options for treatment. KEY WORDS
9 nasal gliomas
'ASAL gliomas are rare ectopic rests of neural tissue found at the root of the nose, usually at birth. The differential diagnosis of congenital nasal masses includes ectodermal tumors such as dermoids or pilonidal cysts, mesodermal lesions such as hemangiomas, lipomas, rhabd o m y o s a r c o m a s , sinus pericranii, and n e u r o e c t o d e r m a l a b n o r m a l i t i e s such as neurofibromas and basal encephaloceles. Therapeutically, it is of importance to distinguish encephaloceles from other nasal tumors to avoid inadvertent exposure of the brain during the surgical removal of mass lesions. Because of their related embryologic origins, however, the distinction between nasal gliomas and n a s o f r o n t a l encephaloceles may not be clear or even possible clinically in spite of suggestions to the cont r a r y ? ~ These tumors fall within the province of several surgical specialties and may need to be appraised by all of these groups for the planning of adequate surgical therapy. Proper therapy can eradicate these benign neural rests, and, by being cognizant of their clinical
N
J. Neurosurg. / Volume 46 / January, 1977
9 encephaloceles
9 glial ectopias
similarity to basal encephaloceles, the surgeon may select an approach that will provide the maximum flexibility with which to deal with an unexpected exposure of the intracranial contents. We are presenting six cases of nasal gliomas, and emphasize the clinical characteristics, and their clinical distinction from and embryologic relationship to encephaloceles. The first apparent report of a nasal glioma associated with holoprosencephaly is included. Case Reports
Case 1 This baby was first seen at 14 days of age for a 2-cm mass presenting on the right side of the bridge of the nose (Fig. 1). The mass, which had been apparent at birth, was purplish in color and clinically seemed cystic. The t u m o r was removed by a direct incision along the lateral side of the nose extending up to the inner canthus of the right eye. The specimen revealed glial elements adjacent to 85
L. F. Hirsh, et al. normal muscle and fat. The scar was revised by a z-plasty at 7 months of age and a small amount of residual glial tissue was discovered and removed. Case 2
FIG. 1. Case 1 at the age of 14 days.
This baby was first seen at 1 day of life because of a congenital mass, 2 • 2 cm in size, underlying the skin of the nasion. The tumor was unattached to the skin, which appeared normal. A round nasofrontal bone defect with sclerotic borders was noted on plain skull films (Fig. 2). The child had a supernumerary digit attached to the right thumb and paralysis of the left hemidiaphragm. A bifrontal craniotomy was performed for a suspected nasofrontal encephalocele, and an entirely extradural nasal glioma was discovered that could be peeled from the dura. Histologically, the glial tissue contained some nerve bundles, fibrous connective tissue, peripheral striated muscle fibers, and a fragment of cartilage presumably from the orbicularis oculi muscle and nasal structures since the t u m o r lay between the eyes and had protruded through a glabellar defect. Case 3 This baby was seen at 3 days of age for a mass just to the left of the nasion, covered by normal skin. Skull films revealed a small defect at the root of the nose. Mild hypertelorism was present. The lesion was excised via an inferior frontal skin incision and a stalk was noted passing through the nasal bone. At the age of 6 months, the tumor had recurred and measured 2 X 2 cm. Again, the tumor was excised by a direct approach and was followed through the nasal bones to achieve a complete removal. The tumor did not extend through the dura. It consisted of nodules of neuropil, composed mainly of astrocytes, some o f which were multinucleated. N o mitotic figures were present. Small bands of peripheral striated muscle were also noted. Case 4
FIG. 2. Case 2. Plain skull film showing nasofrontal bone defect with sclerotic borders in extradural nasal glioma. 86
This baby was admitted on the second day of life because of a reddish-brown mass protruding from the left nostril and distorting the left ala (Fig. 3 left). Skull films J. Neurosurg. / Volume 46 / January, 1977
Nasal glioma
FIG. 3. Case 3. The patient at birth (left), and aged 7 years old (right). demonstrated some distortion of the nasal bones but no defect in the base of the skull. The mass was biopsied and this revealed a typical nasal glioma. The remainder of the t u m o r was removed via a rhinotomy. The t u m o r was entirely extracranial. A plastic revision of the scar was performed at the age of 4 1/3 years. When the patient was 7 years old, a plastic revision of the nasal tip was carried out and fibrous scar tissue, initially thought to be persistent tumor, was excised. The final result at 7 years old was quite satisfactory (Fig. 3 right).
Case 5 This baby was seen at 4 days of age because of respiratory distress. The nasopharynx was found obstructed by a grayish-pink t u m o r 1 X 3 cm in diameter. Skull films revealed a small midline sphenoid bone defect. The palate was split surgically and the t u m o r was removed along with its stalk, which was attached to the soft palate but not to the intracranial contents. T h e specimen revealed glial tissue with ependymal-lined canals, choroid, and a few nerves and ganglion cells (Fig. 4).
FIG. 4. Case 5. Photomicrographs of the nasal glioma. Left: Neurophil and epithelium resembling ependyma. H & E, X 100. Right: Neurons can be identified in nasal glioma. H & E, X 450.
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L. F. Hirsh, et al. Case 6 This baby was transferred on the first day of life for evaluation of a cleft lip, single midline naris, and a mass overlying the bridge of the nose. The child's growth was retarded, and he developed seizures. He died at 9 months of age of pneumonia. An autopsy revealed holoprosencephaly with midline facial defects and a glabellar nasal glioma. Discussion Clinical Features Nasal gliomas were first described by Reid in 1852, 34 and again by Berger in 1890, 4 and labeled by Schmidt in 1900. s7 They have been called fibrogliomas, encephalochoristomas, and encephalomas. On examination, nasal gliomas are firm to palpation, and, if they are extranasal, appear unattached to the overlying skin. The skin may be discolore& or telangiectatic? The mass seldom bulges or pulsates with crying or straining, 6 and this has been described as a technique for distinguishing nasal gliomas from nasal encephaloceles: the Furstenberg test? ~ However, this test may be falsely positive or negative. TM There may be excessive lacrimation on the involved side. 4s Intranasal gliomas appear pink to red in color, 4x and polypoid, 4~ and are easily confused with nasal polyps, which, however, are rare under the age of 5 years? 5 Nasal gliomas are usually attached to the middle turbinate bone or to the lateral wall of the nasal fossa, in contrast to the true intranasal encephaloceles, which are more often attached to the nasal septum? Aspiration of a nasal mass in an attempt to distinguish solid nasal gliomas from encephaloceles may be dangerous, and can cause meningitis. 15,24 Recovery of spinal fluid may also be misleading since an occasional nasal glioma may secrete cerebrospinal fluid and yet be unattached to the intracranial c o n t e n t s ) The root of the nose is often described as widened, 6'12 and h y p e r t e l o r i s m may be present? ,45 Smith, et al.,S~ found that 14 of the 93 cases collected from the literature were connected to the intracranial contents t h r o u g h a c r a n i a l defect. Bone defects, however, are only occasionally seen even with laminography, x~ and this cannot be the criterion by which nasal gliomas are distinguished preoperatively from true enceph88
aloceles? ,45 Three of our six cases had cranial bone defects. Radiography may show septal and nasal bone displacement '5 or soft tissue masses. Calcification has not been seen radiographically in these lesions but sinus ostia obstruction may result in sinus opacification? b Other congenital abnormalities ordinarily do not occur, *s although they were present in our Cases 2 and 6. A familial incidence has not been reported. 1.'28'43 Histologically, nasal gliomas consist of g l i o m a t o u s tissue found e x t r a c r a n i a l l y , interspersed with, and at times covered by, fibrous tissue? ,3~ The glial tissue is composed almost entirely of astrocytes in a fine fibrillary matrix. Rarely, ganglion cells 6,l~176 or axons 2s'89 may be seen. Ependymal cells and ependymal-lined canals have been described. 8,8,2a Mitotic figures are rare 6,4s and metastases do not occur. 41 The fibrous septa are often vascular and melanin pigment may be observed, s~ Microscopically, calcium flecks may be present. 43 A typical specimen is illustrated in Fig. 4, demonstrating the astrocytic matrix with neurons and ependyma. The muscle and cartilage found adjacent to the glial rests in Cases l, 2, and 3 were not mixed with the nervous elements, and appeared normal. They most probably represent local normal tissues removed at surgery. These growths have been found from birth to 62 years of age, TM but most lesions are present at birth, sg All of our cases presented during the neonatal period. There is no sexual predilection. 2,6,ss Nasal gliomas occur over the bridge of the nose off the midline in 60% of the cases, intranasally in 30%, and both intranasally and extranasally in 10%. s9 Walker and Resler, 4s however, reviewed 33 cases reported in the literature between 1950 and 1963, and found that 39% were extranasal, 33% intranasal, and 27% both intranasal and extranasal. A few rare cases have been described involving the throat, 25 or the soft palate a,za,44 as in Case 5, or associated with an alar defect, aa Multiple cutaneous cranial glial nodules unattached to the intracranial contents or even to the galea have been found in association with nasal gliomas) ~ Pathogenesis M a c o m b e r and Wang 27 and Katz and Lewis 2~ have suggested that all congenital n a s o f r o n t a l tumors, such as dermoids, h e m a n g i o m a s , lipomas, and even basoJ. Neurosurg. / Volume 46 / January, 1977
Nasal glioma frontal encephaloceles, have a similar etiology: a trapping of tissue by the developing cranial bones with later growth of the ectopic nodule. A similar origin for nasal gliomas was suggested by Schmidt in 1900) 7 During embryologic life the frontal and nasal bones are bridged by a fibrous membrane, the fonticulus nasofrontalis (Fig. 5), that separates the dura from the developing skin? Protrusion of dura and brain through this membrane with obliteration of the connecting stalk by the growing frontal and nasal bones could result in subcutaneous extranasal ectopic glial rests (Fig. 5, a r r o w A). Failure of complete destruction of the stalk could lead to a persistent nasofrontal bone defect, through which an atrophic fibrous stalk connects the nasal glioma to the brain, a typical pathological finding.48 Persistence of an intact stalk might result in a glabellar encephalocele. In some cases, partial stalk atrophy might be expected with persistence of some glial tissue in the stalk itself, and this has been found?~ The original mesenchymal dural protrusion might become the fibrous capsule of the nasal glioma. Intranasal encephaloceles and glial rests may originate in a similar manner from the embryologic projection of dura and brain through the foramen cecum into the prenasal space (Fig. 5, a r r o w B)) 2 These mechanical explanations of the pathogenesis of nasal gliomas are supported by the fact that they rarely are associated with other congenital defects. 48 If this theory is correct, it should explain the presence of not only glial cells but also of choroid and ependyma in these tumors. The usual absence of ganglion cells must also be explained? Herniation of mature brain from the frontal lobes could be expected to produce a tumor mass consisting only of nerve, ganglion cells, and glial tissue. The presence of ependymal cells might be due to the herniation of immature brain containing the vestiges of the ependymal walls which may be found in the cerebral hemispheres. 2~ The choroidal tissue may develop from herniated totipotential cells. Smith, et al., 8~ have suggested that the lack of ganglion cells may be due to inadequate nutritional support. Other hypothetical etiologies for nasal gliomas do not depend on the above mechanical explanation. These theories are based on aberrant migration of glial cells into J. Neurosurg. / Volume 46 / January, 1977
FIG. 5. Schematic embryologic development of the nasofrontal region, showing the route of development of extranasal tumors (arrow A ) and that of intranasal tumors (arrow B). the nose along the olfactory nerve processes" or through a separation in the frontoethmoidal suture, 3 or upon the persistence of the nervus terminalis. 1a,19 Our cases support the mechanical pathogenetic theory discussed above and demonstrate several tumor types predicted by the theory. Case 1 is an example of a subcutaneous glial rest without either stalk or cranial defect. Cases 2 and 3 are examples of ectopic glial nodules connected by atrophic fibrous stalks to the intracranial contents or dura through persistent skull defects. No pure encephaloceles were included in this report. Cases 4 and 5 are intranasal glial rests. Two of our six cases were associated with other congenital anomalies. The associated supernumerary digit in Case 2 could not have been predicted by the mechanical pathogenetic theory. Because of the rarity of such additional congenital anomalies, this occurrence may have been due to chance alone. On the other hand, it could be predicted that nasal gliomas, as one manifestation of anomalous midline development, would be associated with other congenital midline defects. Case 6, a nasal glioma associated 89
L. F. H i r s h , e t al. with holoprosencephaly, may be such an example. Treatment
Surgery is the treatment of choice for nasal gliomas. Growth of untreated lesions occurs as evidenced by their known tendency to recur and their appearance in later life. Excessive delay before definitive treatment may lead to distortion of the septum and nasal bones. 22,4~ If the lesions are extranasal or transnasal and extracranial, they may be approached directly ~~ as was done in all of our operated cases. One must always be prepared to treat an encephalocele in spite of a preoperative diagnosis of a nasal glioma. The difficulty of distinguishing preoperatively the two lesions by clinical or radiological criteria has been thoroughly emphasized. This is not surprising considering their presumed related embryologic origins. These cases should be treated by the other surgical specialties only when adequate neurosurgical coverage is available. Meningitis, cerebrospinal fluid rhinorrhea, or radiographic evidence of possible transcranial extension suggest connections between the nasal glioma and the central nervous system and require primary intracranial exploration. 39 Intranasal gliomas may also be treated by primary craniotomy followed by intranasal resection to avoid any risk of opening an occult communication with the intracranial space. 3~,4s An occasional negative intracranial exploration may be a reasonable price to pay for avoiding subsequent central nervous system infections. TM Smith, et al., in 19638~ found that of 93 operated cases reported in the literature, only 11 recurred and all but one of these was cured by subsequent surgery. Lowe, et al., ~6 estimated that 10% of nasal gliomas recurred postoperatively. Two of our six operated cases recurred due to incomplete initial excision, and both were cured by subsequent surgery. Although recurrence is most likely due to incomplete initial excision, ~,~~ the continued growth following partial removal may suggest 'that nasal gliomas have the potential of low-grade neoplasms. 6 Some tumors, certainly, have been shown to have growth potential independent of that of the brain, 2'8'xa'27 and so are more than ectopic glial rests. Rubinstei# 5 also notes the difficulty in deciding whether these lesions are 90
purely hamartomatous or really neoplastic. Radiation therapy is not suggested, although it has been used occasionally,z,13 and perhaps with some success. Conclusions
These six cases have been presented to show the variety of ways in which nasal gliomas may present. The first report of a nasal glioma associated with holoprosencephaly has been included. The pathogenesis of these lesions has been attributed to a mechanical anomaly of the developing cranium. The reported cases support such a theory. The clinical features and treatment of nasal gliomas have been reviewed. Nasal gliomas are often seen and treated by various surgical specialists. Although these lesions are rare, they are entirely benign and must, therefore, be handled properly. Awareness of the embryologic etiology of these growths and their relationship to nasofrontal encephaloceles should alert the surgeon preoperatively to possible complications involving the meninges and the central nervous system. In this manner, a safe and definitive surgical approach can be selected. References
1. Agarwal S, Gandagule VN, Chouhan SS: Neurogenic tumors of the nose. Indian J Cancer 5:127-131, 1968 2. Altany FE, Pickrell KL: Nasal gliomas. Arch Surg 71:275-278, 1955 3. Anglade M, Philip: Le gliome des fosses nasales, l~tude clinique et anatomopathologique. Nouv Presse Med 28:464, 1920 4. Berger P: Consid6rations sur l'origine, le mode de d6veloppement et le traitement de certaines enc6phaloc~les. Rev ChiT 10: 269-321, 1890 5. Birnbaum LM, Owsley JQ Jr: Frontonasal tumors of neurogenic origin. Plast Recnnstr Surg 41:462-470, 1968 6. Black BK, Smith DE: Nasal glioma. Two cases with recurrence. Arch Neural Psychiatry 64:614-630, 1950 7. Borsanyi S" Nasal glioma. Arch Otolaryngol 72:376-379, 1960 8. Bratton AB, Robinson SHG: Gliomas of the nose and oral cavity. A report of two cases. J Pathol Bacteriol 58:643-648, 1946 9. Brunner H, Harned JW: Dermoid cysts of the dorsum of the nose. Arch Otolaryngol 36:86-94, 1942 10. Cassidy WA, Wahl JW" Congenital displacement of central nervous system tissue in the J. Neurosurg. / Volume 46 / January, 1977
Nasal glioma 11. 12.
13. 14. 15. 16. 17.
18. 19. 20. 21.
22. 23. 24. 25. 26. 27. 28. 29.
nasal fossae. Report of two cases. Arch Otolaryngol 59:93-99, 1954 Crosby JF: Unusual nasal tumors in children. Glioma and rhabdomyosarcoma. Plast Reconstr Surg 19:143-149, 1957 Davis CH Jr, Alexander E Jr: Congenital nasofrontal encephalomeningoceles and teratomas. Review of seven cases. J Neurosurg 16:365-377, 1959 Davis EW: Gliomatous tumors in the nasal region. J Neuropathol Exp Neurol 1:312-319, 1942 Dawson RLG, Muir JFK: The frontonasal glioma. Br J Plast Surg 8:136-143, 1955-1956 Deutsch H J: Intranasal glioma. Ann Otol Rhinol Laryngol 74:637-644, 1965 Guthrie D, Dott N: The occurrence of braintissue within the nose: so-called nasal glioma. J Laryngol Otol 42:733-745, 1927 Hage J: Surgical approach to the external and internal nose: with a supplementary report on two cases of nasal glioma. Br J Plast Surg 12:327-339, 1960 Hamilton W J, Boyd JD, Mossman HW: Human Embryology. Baltimore: Williams & Wilkins, 1964, p 368 Jamieson KG: Nasal glioma. Pediatrics 35:342-344, 1965 Katz A, Lewis JS: Nasal gliomas. Arch Otolaryngol 94:351-355, 1971 Kernohan JW, Sayre GP: Tumors of the central nervous system, in: Atlas of Tumor Pathology, Section X, Fascicle 35. Washington, DC: Armed Forces Institute of Pathology, 1952, p 43 Kubo K, Garrett WS Jr, Musgrove RH: Nasal gliomas. Plast Reconstr Surg 52:47-51, 1973 Lampertico P, Ibanez ML: Nasal glioma (encephalochoristoma nasofrontalis). Arch Otolaryngol 79:628-631, 1964 Lister J: Nasal glioma. J Laryngol Otol 77:34-42, 1963 Low NL, Scheinberg L, Andersen DH" Brain tissue in the nose and throat. Pediatrics 18:254-259, 1956 Lowe RS, Robinson DW, Ketchum LD, et al: Nasal gliomata. Plast Reconstr Surg 47:1-5, 1971 Macomber WB, Wang MK: Congenital neoplasms of the nose. Plast Reconstr Surg 11:215-229, 1953 Matheson DN: Nasal glioma. Texas J Meal 60:640-641, 1964 Morley GH, Cross RM: Nasal glioma. J Pathol Bacteriol 76:590-592, 1958
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30. Musser AW, Campbell R: Nasal glioma. Report of an unusual case associated with multiple spragaleal glial nodules. Arch Otolaryngol 73:732-736, 1961 31. O'Brien P: The surgical approach to nasal glioma. Br J Plast Surg 23:30-35, 1970 32. Pollock JA, Newton TH, Hoyt WF: Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology 90:442-453, 1968 33. Ramakrishnan MS, Dayalan N: Nasal glioma associated with defect of the nasal ala. J Pediatr Surg 6:491, 1971 34. Reid: cited in reference 16 35. Rubinstein L J: Tumors of the central nervous system, in: Atlas of Tumor Pathology, Second Series, Fascicle 6. Washington, DC: Armed Forces Institute of Pathology, 1972 36. Russell DS, Rubinstein L J: Pathology of Tumors of the Nervous System, ed 3. London: Edward Arnold, 1971, p 30 37. Schmidt MB: Ueber seltene Spaltbildungen im Bereiche des mittleren Stirnfortsatzes. Virchows Arch [Pathol Aunt] 162:340-370, 1900 38. Shapiro M J, Mix BS: Heterotopic brain tissue of the palate. A report of two cases. Arch Otolaryngol 87:522-526, 1968 39. Smith KR Jr, Schwartz HG, Luse SA, et al: Nasal gliomas: a report of five cases with electron microscopy of one. J Neurosurg 20:968-982, 1963 40. Strauss RB, Callicott JH Jr, Hargett IR: Intranasal neuroglial heterotopia. So-called nasal glioma. Am J Dis Child 111:317-320, 1966 41. Villar R del: Astrocytomas of the nose. Arch Otolaryngoi 63:466-473, 1956 42. Wadsworth P: Nasal glioma. J Laryngol Otoi 83:87-88, 1969 43. Walker AE Jr, Resler DR: Nasal glioma. Laryngoscope 73:93-107, 1963 44. Zarem HA, Gray GF Jr, Morehead D, et al: Heterotopic brain in the nasopharynx and soft palate: report of two cases. Surgery 61:483-486, 1967 45. Ziter FMH Jr, Bramwit DN: Nasal encephaloceles and gliomas. Br J Radioi 43:136-138, 1970
Address reprint requests to: Leonard F. Hirsh, M.D., Hahnemann Medical College and Hospital, 230 North Broad Street, Philadelphia, Pennsylvania 19102.
9]
Division of Neurosurgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania v~ Six cases of nasal gliomas, which are rare ectopic rests of neural tissue found at the root of the nose, are presented. It is important to distinguish nasal tumors from basofrontal encephaloceles to avoid inadvertent exposure of the brain during the surgical removal of mass lesions. Because of their related embryologic origins, the distinction between nasal gliomas and basofrontal encephaloceles may not be clear clinically. Nasal gliomas may be treated by several surgical specialties, and only a proper awareness of their relationship to encephaloceles can assure the selection of a flexible and adequate surgical approach. This paper emphasizes the salient clinical characteristics of nasal gliomas, their clinical distinction from and embryologic relationship to encephaloceles, and the options for treatment. KEY WORDS
9 nasal gliomas
'ASAL gliomas are rare ectopic rests of neural tissue found at the root of the nose, usually at birth. The differential diagnosis of congenital nasal masses includes ectodermal tumors such as dermoids or pilonidal cysts, mesodermal lesions such as hemangiomas, lipomas, rhabd o m y o s a r c o m a s , sinus pericranii, and n e u r o e c t o d e r m a l a b n o r m a l i t i e s such as neurofibromas and basal encephaloceles. Therapeutically, it is of importance to distinguish encephaloceles from other nasal tumors to avoid inadvertent exposure of the brain during the surgical removal of mass lesions. Because of their related embryologic origins, however, the distinction between nasal gliomas and n a s o f r o n t a l encephaloceles may not be clear or even possible clinically in spite of suggestions to the cont r a r y ? ~ These tumors fall within the province of several surgical specialties and may need to be appraised by all of these groups for the planning of adequate surgical therapy. Proper therapy can eradicate these benign neural rests, and, by being cognizant of their clinical
N
J. Neurosurg. / Volume 46 / January, 1977
9 encephaloceles
9 glial ectopias
similarity to basal encephaloceles, the surgeon may select an approach that will provide the maximum flexibility with which to deal with an unexpected exposure of the intracranial contents. We are presenting six cases of nasal gliomas, and emphasize the clinical characteristics, and their clinical distinction from and embryologic relationship to encephaloceles. The first apparent report of a nasal glioma associated with holoprosencephaly is included. Case Reports
Case 1 This baby was first seen at 14 days of age for a 2-cm mass presenting on the right side of the bridge of the nose (Fig. 1). The mass, which had been apparent at birth, was purplish in color and clinically seemed cystic. The t u m o r was removed by a direct incision along the lateral side of the nose extending up to the inner canthus of the right eye. The specimen revealed glial elements adjacent to 85
L. F. Hirsh, et al. normal muscle and fat. The scar was revised by a z-plasty at 7 months of age and a small amount of residual glial tissue was discovered and removed. Case 2
FIG. 1. Case 1 at the age of 14 days.
This baby was first seen at 1 day of life because of a congenital mass, 2 • 2 cm in size, underlying the skin of the nasion. The tumor was unattached to the skin, which appeared normal. A round nasofrontal bone defect with sclerotic borders was noted on plain skull films (Fig. 2). The child had a supernumerary digit attached to the right thumb and paralysis of the left hemidiaphragm. A bifrontal craniotomy was performed for a suspected nasofrontal encephalocele, and an entirely extradural nasal glioma was discovered that could be peeled from the dura. Histologically, the glial tissue contained some nerve bundles, fibrous connective tissue, peripheral striated muscle fibers, and a fragment of cartilage presumably from the orbicularis oculi muscle and nasal structures since the t u m o r lay between the eyes and had protruded through a glabellar defect. Case 3 This baby was seen at 3 days of age for a mass just to the left of the nasion, covered by normal skin. Skull films revealed a small defect at the root of the nose. Mild hypertelorism was present. The lesion was excised via an inferior frontal skin incision and a stalk was noted passing through the nasal bone. At the age of 6 months, the tumor had recurred and measured 2 X 2 cm. Again, the tumor was excised by a direct approach and was followed through the nasal bones to achieve a complete removal. The tumor did not extend through the dura. It consisted of nodules of neuropil, composed mainly of astrocytes, some o f which were multinucleated. N o mitotic figures were present. Small bands of peripheral striated muscle were also noted. Case 4
FIG. 2. Case 2. Plain skull film showing nasofrontal bone defect with sclerotic borders in extradural nasal glioma. 86
This baby was admitted on the second day of life because of a reddish-brown mass protruding from the left nostril and distorting the left ala (Fig. 3 left). Skull films J. Neurosurg. / Volume 46 / January, 1977
Nasal glioma
FIG. 3. Case 3. The patient at birth (left), and aged 7 years old (right). demonstrated some distortion of the nasal bones but no defect in the base of the skull. The mass was biopsied and this revealed a typical nasal glioma. The remainder of the t u m o r was removed via a rhinotomy. The t u m o r was entirely extracranial. A plastic revision of the scar was performed at the age of 4 1/3 years. When the patient was 7 years old, a plastic revision of the nasal tip was carried out and fibrous scar tissue, initially thought to be persistent tumor, was excised. The final result at 7 years old was quite satisfactory (Fig. 3 right).
Case 5 This baby was seen at 4 days of age because of respiratory distress. The nasopharynx was found obstructed by a grayish-pink t u m o r 1 X 3 cm in diameter. Skull films revealed a small midline sphenoid bone defect. The palate was split surgically and the t u m o r was removed along with its stalk, which was attached to the soft palate but not to the intracranial contents. T h e specimen revealed glial tissue with ependymal-lined canals, choroid, and a few nerves and ganglion cells (Fig. 4).
FIG. 4. Case 5. Photomicrographs of the nasal glioma. Left: Neurophil and epithelium resembling ependyma. H & E, X 100. Right: Neurons can be identified in nasal glioma. H & E, X 450.
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L. F. Hirsh, et al. Case 6 This baby was transferred on the first day of life for evaluation of a cleft lip, single midline naris, and a mass overlying the bridge of the nose. The child's growth was retarded, and he developed seizures. He died at 9 months of age of pneumonia. An autopsy revealed holoprosencephaly with midline facial defects and a glabellar nasal glioma. Discussion Clinical Features Nasal gliomas were first described by Reid in 1852, 34 and again by Berger in 1890, 4 and labeled by Schmidt in 1900. s7 They have been called fibrogliomas, encephalochoristomas, and encephalomas. On examination, nasal gliomas are firm to palpation, and, if they are extranasal, appear unattached to the overlying skin. The skin may be discolore& or telangiectatic? The mass seldom bulges or pulsates with crying or straining, 6 and this has been described as a technique for distinguishing nasal gliomas from nasal encephaloceles: the Furstenberg test? ~ However, this test may be falsely positive or negative. TM There may be excessive lacrimation on the involved side. 4s Intranasal gliomas appear pink to red in color, 4x and polypoid, 4~ and are easily confused with nasal polyps, which, however, are rare under the age of 5 years? 5 Nasal gliomas are usually attached to the middle turbinate bone or to the lateral wall of the nasal fossa, in contrast to the true intranasal encephaloceles, which are more often attached to the nasal septum? Aspiration of a nasal mass in an attempt to distinguish solid nasal gliomas from encephaloceles may be dangerous, and can cause meningitis. 15,24 Recovery of spinal fluid may also be misleading since an occasional nasal glioma may secrete cerebrospinal fluid and yet be unattached to the intracranial c o n t e n t s ) The root of the nose is often described as widened, 6'12 and h y p e r t e l o r i s m may be present? ,45 Smith, et al.,S~ found that 14 of the 93 cases collected from the literature were connected to the intracranial contents t h r o u g h a c r a n i a l defect. Bone defects, however, are only occasionally seen even with laminography, x~ and this cannot be the criterion by which nasal gliomas are distinguished preoperatively from true enceph88
aloceles? ,45 Three of our six cases had cranial bone defects. Radiography may show septal and nasal bone displacement '5 or soft tissue masses. Calcification has not been seen radiographically in these lesions but sinus ostia obstruction may result in sinus opacification? b Other congenital abnormalities ordinarily do not occur, *s although they were present in our Cases 2 and 6. A familial incidence has not been reported. 1.'28'43 Histologically, nasal gliomas consist of g l i o m a t o u s tissue found e x t r a c r a n i a l l y , interspersed with, and at times covered by, fibrous tissue? ,3~ The glial tissue is composed almost entirely of astrocytes in a fine fibrillary matrix. Rarely, ganglion cells 6,l~176 or axons 2s'89 may be seen. Ependymal cells and ependymal-lined canals have been described. 8,8,2a Mitotic figures are rare 6,4s and metastases do not occur. 41 The fibrous septa are often vascular and melanin pigment may be observed, s~ Microscopically, calcium flecks may be present. 43 A typical specimen is illustrated in Fig. 4, demonstrating the astrocytic matrix with neurons and ependyma. The muscle and cartilage found adjacent to the glial rests in Cases l, 2, and 3 were not mixed with the nervous elements, and appeared normal. They most probably represent local normal tissues removed at surgery. These growths have been found from birth to 62 years of age, TM but most lesions are present at birth, sg All of our cases presented during the neonatal period. There is no sexual predilection. 2,6,ss Nasal gliomas occur over the bridge of the nose off the midline in 60% of the cases, intranasally in 30%, and both intranasally and extranasally in 10%. s9 Walker and Resler, 4s however, reviewed 33 cases reported in the literature between 1950 and 1963, and found that 39% were extranasal, 33% intranasal, and 27% both intranasal and extranasal. A few rare cases have been described involving the throat, 25 or the soft palate a,za,44 as in Case 5, or associated with an alar defect, aa Multiple cutaneous cranial glial nodules unattached to the intracranial contents or even to the galea have been found in association with nasal gliomas) ~ Pathogenesis M a c o m b e r and Wang 27 and Katz and Lewis 2~ have suggested that all congenital n a s o f r o n t a l tumors, such as dermoids, h e m a n g i o m a s , lipomas, and even basoJ. Neurosurg. / Volume 46 / January, 1977
Nasal glioma frontal encephaloceles, have a similar etiology: a trapping of tissue by the developing cranial bones with later growth of the ectopic nodule. A similar origin for nasal gliomas was suggested by Schmidt in 1900) 7 During embryologic life the frontal and nasal bones are bridged by a fibrous membrane, the fonticulus nasofrontalis (Fig. 5), that separates the dura from the developing skin? Protrusion of dura and brain through this membrane with obliteration of the connecting stalk by the growing frontal and nasal bones could result in subcutaneous extranasal ectopic glial rests (Fig. 5, a r r o w A). Failure of complete destruction of the stalk could lead to a persistent nasofrontal bone defect, through which an atrophic fibrous stalk connects the nasal glioma to the brain, a typical pathological finding.48 Persistence of an intact stalk might result in a glabellar encephalocele. In some cases, partial stalk atrophy might be expected with persistence of some glial tissue in the stalk itself, and this has been found?~ The original mesenchymal dural protrusion might become the fibrous capsule of the nasal glioma. Intranasal encephaloceles and glial rests may originate in a similar manner from the embryologic projection of dura and brain through the foramen cecum into the prenasal space (Fig. 5, a r r o w B)) 2 These mechanical explanations of the pathogenesis of nasal gliomas are supported by the fact that they rarely are associated with other congenital defects. 48 If this theory is correct, it should explain the presence of not only glial cells but also of choroid and ependyma in these tumors. The usual absence of ganglion cells must also be explained? Herniation of mature brain from the frontal lobes could be expected to produce a tumor mass consisting only of nerve, ganglion cells, and glial tissue. The presence of ependymal cells might be due to the herniation of immature brain containing the vestiges of the ependymal walls which may be found in the cerebral hemispheres. 2~ The choroidal tissue may develop from herniated totipotential cells. Smith, et al., 8~ have suggested that the lack of ganglion cells may be due to inadequate nutritional support. Other hypothetical etiologies for nasal gliomas do not depend on the above mechanical explanation. These theories are based on aberrant migration of glial cells into J. Neurosurg. / Volume 46 / January, 1977
FIG. 5. Schematic embryologic development of the nasofrontal region, showing the route of development of extranasal tumors (arrow A ) and that of intranasal tumors (arrow B). the nose along the olfactory nerve processes" or through a separation in the frontoethmoidal suture, 3 or upon the persistence of the nervus terminalis. 1a,19 Our cases support the mechanical pathogenetic theory discussed above and demonstrate several tumor types predicted by the theory. Case 1 is an example of a subcutaneous glial rest without either stalk or cranial defect. Cases 2 and 3 are examples of ectopic glial nodules connected by atrophic fibrous stalks to the intracranial contents or dura through persistent skull defects. No pure encephaloceles were included in this report. Cases 4 and 5 are intranasal glial rests. Two of our six cases were associated with other congenital anomalies. The associated supernumerary digit in Case 2 could not have been predicted by the mechanical pathogenetic theory. Because of the rarity of such additional congenital anomalies, this occurrence may have been due to chance alone. On the other hand, it could be predicted that nasal gliomas, as one manifestation of anomalous midline development, would be associated with other congenital midline defects. Case 6, a nasal glioma associated 89
L. F. H i r s h , e t al. with holoprosencephaly, may be such an example. Treatment
Surgery is the treatment of choice for nasal gliomas. Growth of untreated lesions occurs as evidenced by their known tendency to recur and their appearance in later life. Excessive delay before definitive treatment may lead to distortion of the septum and nasal bones. 22,4~ If the lesions are extranasal or transnasal and extracranial, they may be approached directly ~~ as was done in all of our operated cases. One must always be prepared to treat an encephalocele in spite of a preoperative diagnosis of a nasal glioma. The difficulty of distinguishing preoperatively the two lesions by clinical or radiological criteria has been thoroughly emphasized. This is not surprising considering their presumed related embryologic origins. These cases should be treated by the other surgical specialties only when adequate neurosurgical coverage is available. Meningitis, cerebrospinal fluid rhinorrhea, or radiographic evidence of possible transcranial extension suggest connections between the nasal glioma and the central nervous system and require primary intracranial exploration. 39 Intranasal gliomas may also be treated by primary craniotomy followed by intranasal resection to avoid any risk of opening an occult communication with the intracranial space. 3~,4s An occasional negative intracranial exploration may be a reasonable price to pay for avoiding subsequent central nervous system infections. TM Smith, et al., in 19638~ found that of 93 operated cases reported in the literature, only 11 recurred and all but one of these was cured by subsequent surgery. Lowe, et al., ~6 estimated that 10% of nasal gliomas recurred postoperatively. Two of our six operated cases recurred due to incomplete initial excision, and both were cured by subsequent surgery. Although recurrence is most likely due to incomplete initial excision, ~,~~ the continued growth following partial removal may suggest 'that nasal gliomas have the potential of low-grade neoplasms. 6 Some tumors, certainly, have been shown to have growth potential independent of that of the brain, 2'8'xa'27 and so are more than ectopic glial rests. Rubinstei# 5 also notes the difficulty in deciding whether these lesions are 90
purely hamartomatous or really neoplastic. Radiation therapy is not suggested, although it has been used occasionally,z,13 and perhaps with some success. Conclusions
These six cases have been presented to show the variety of ways in which nasal gliomas may present. The first report of a nasal glioma associated with holoprosencephaly has been included. The pathogenesis of these lesions has been attributed to a mechanical anomaly of the developing cranium. The reported cases support such a theory. The clinical features and treatment of nasal gliomas have been reviewed. Nasal gliomas are often seen and treated by various surgical specialists. Although these lesions are rare, they are entirely benign and must, therefore, be handled properly. Awareness of the embryologic etiology of these growths and their relationship to nasofrontal encephaloceles should alert the surgeon preoperatively to possible complications involving the meninges and the central nervous system. In this manner, a safe and definitive surgical approach can be selected. References
1. Agarwal S, Gandagule VN, Chouhan SS: Neurogenic tumors of the nose. Indian J Cancer 5:127-131, 1968 2. Altany FE, Pickrell KL: Nasal gliomas. Arch Surg 71:275-278, 1955 3. Anglade M, Philip: Le gliome des fosses nasales, l~tude clinique et anatomopathologique. Nouv Presse Med 28:464, 1920 4. Berger P: Consid6rations sur l'origine, le mode de d6veloppement et le traitement de certaines enc6phaloc~les. Rev ChiT 10: 269-321, 1890 5. Birnbaum LM, Owsley JQ Jr: Frontonasal tumors of neurogenic origin. Plast Recnnstr Surg 41:462-470, 1968 6. Black BK, Smith DE: Nasal glioma. Two cases with recurrence. Arch Neural Psychiatry 64:614-630, 1950 7. Borsanyi S" Nasal glioma. Arch Otolaryngol 72:376-379, 1960 8. Bratton AB, Robinson SHG: Gliomas of the nose and oral cavity. A report of two cases. J Pathol Bacteriol 58:643-648, 1946 9. Brunner H, Harned JW: Dermoid cysts of the dorsum of the nose. Arch Otolaryngol 36:86-94, 1942 10. Cassidy WA, Wahl JW" Congenital displacement of central nervous system tissue in the J. Neurosurg. / Volume 46 / January, 1977
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nasal fossae. Report of two cases. Arch Otolaryngol 59:93-99, 1954 Crosby JF: Unusual nasal tumors in children. Glioma and rhabdomyosarcoma. Plast Reconstr Surg 19:143-149, 1957 Davis CH Jr, Alexander E Jr: Congenital nasofrontal encephalomeningoceles and teratomas. Review of seven cases. J Neurosurg 16:365-377, 1959 Davis EW: Gliomatous tumors in the nasal region. J Neuropathol Exp Neurol 1:312-319, 1942 Dawson RLG, Muir JFK: The frontonasal glioma. Br J Plast Surg 8:136-143, 1955-1956 Deutsch H J: Intranasal glioma. Ann Otol Rhinol Laryngol 74:637-644, 1965 Guthrie D, Dott N: The occurrence of braintissue within the nose: so-called nasal glioma. J Laryngol Otol 42:733-745, 1927 Hage J: Surgical approach to the external and internal nose: with a supplementary report on two cases of nasal glioma. Br J Plast Surg 12:327-339, 1960 Hamilton W J, Boyd JD, Mossman HW: Human Embryology. Baltimore: Williams & Wilkins, 1964, p 368 Jamieson KG: Nasal glioma. Pediatrics 35:342-344, 1965 Katz A, Lewis JS: Nasal gliomas. Arch Otolaryngol 94:351-355, 1971 Kernohan JW, Sayre GP: Tumors of the central nervous system, in: Atlas of Tumor Pathology, Section X, Fascicle 35. Washington, DC: Armed Forces Institute of Pathology, 1952, p 43 Kubo K, Garrett WS Jr, Musgrove RH: Nasal gliomas. Plast Reconstr Surg 52:47-51, 1973 Lampertico P, Ibanez ML: Nasal glioma (encephalochoristoma nasofrontalis). Arch Otolaryngol 79:628-631, 1964 Lister J: Nasal glioma. J Laryngol Otol 77:34-42, 1963 Low NL, Scheinberg L, Andersen DH" Brain tissue in the nose and throat. Pediatrics 18:254-259, 1956 Lowe RS, Robinson DW, Ketchum LD, et al: Nasal gliomata. Plast Reconstr Surg 47:1-5, 1971 Macomber WB, Wang MK: Congenital neoplasms of the nose. Plast Reconstr Surg 11:215-229, 1953 Matheson DN: Nasal glioma. Texas J Meal 60:640-641, 1964 Morley GH, Cross RM: Nasal glioma. J Pathol Bacteriol 76:590-592, 1958
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30. Musser AW, Campbell R: Nasal glioma. Report of an unusual case associated with multiple spragaleal glial nodules. Arch Otolaryngol 73:732-736, 1961 31. O'Brien P: The surgical approach to nasal glioma. Br J Plast Surg 23:30-35, 1970 32. Pollock JA, Newton TH, Hoyt WF: Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology 90:442-453, 1968 33. Ramakrishnan MS, Dayalan N: Nasal glioma associated with defect of the nasal ala. J Pediatr Surg 6:491, 1971 34. Reid: cited in reference 16 35. Rubinstein L J: Tumors of the central nervous system, in: Atlas of Tumor Pathology, Second Series, Fascicle 6. Washington, DC: Armed Forces Institute of Pathology, 1972 36. Russell DS, Rubinstein L J: Pathology of Tumors of the Nervous System, ed 3. London: Edward Arnold, 1971, p 30 37. Schmidt MB: Ueber seltene Spaltbildungen im Bereiche des mittleren Stirnfortsatzes. Virchows Arch [Pathol Aunt] 162:340-370, 1900 38. Shapiro M J, Mix BS: Heterotopic brain tissue of the palate. A report of two cases. Arch Otolaryngol 87:522-526, 1968 39. Smith KR Jr, Schwartz HG, Luse SA, et al: Nasal gliomas: a report of five cases with electron microscopy of one. J Neurosurg 20:968-982, 1963 40. Strauss RB, Callicott JH Jr, Hargett IR: Intranasal neuroglial heterotopia. So-called nasal glioma. Am J Dis Child 111:317-320, 1966 41. Villar R del: Astrocytomas of the nose. Arch Otolaryngoi 63:466-473, 1956 42. Wadsworth P: Nasal glioma. J Laryngol Otoi 83:87-88, 1969 43. Walker AE Jr, Resler DR: Nasal glioma. Laryngoscope 73:93-107, 1963 44. Zarem HA, Gray GF Jr, Morehead D, et al: Heterotopic brain in the nasopharynx and soft palate: report of two cases. Surgery 61:483-486, 1967 45. Ziter FMH Jr, Bramwit DN: Nasal encephaloceles and gliomas. Br J Radioi 43:136-138, 1970
Address reprint requests to: Leonard F. Hirsh, M.D., Hahnemann Medical College and Hospital, 230 North Broad Street, Philadelphia, Pennsylvania 19102.
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