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Glial choristoma in the middle ear and mastoid bone: a case report.

Lee JI, Kim KK, Park YK, Eah KY, Kim JR - J. Korean Med. Sci. (2004)

Bottom Line: During operation, definite cranial bone defect or cerebrospinal fluid leakage was not found.Histologically, the lesion was composed of exclusively mature, disorganized glial tissue with fibrovascular elements in a rather loose fibrillary background.Glial tissue showed diffuse positive reaction for glial fibrillar acidic protein and S100 protein on immunohistochemical study.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, College of Medicine, Dongguk University, 707 Sukjang-dong, Gyeongju 780-714, Korea. leego@mail.dongguk.ac.kr

ABSTRACT
Heterotopic brain tissue usually involves extracranial midline structures of the head and neck such as nose, nasopharynx, and oral cavity. Its occurrence in the non-midline structures, including middle ear, is rare. We described a 50-yr-old-man with heterotopic glial tissue in the middle ear and mastoid bone. The patient presented with progressive hearing loss for 8 yr. There was no history of congenital anomalies, trauma, or ear surgery. Computed tomography revealed a mass-like lesion with soft tissue density occupying the middle ear cavity and mastoid antrum. At the operation, a gray-white fibrotic mass was detected in the epitympanic area. Mesotympanum and ossicles were intact. The patient underwent left simple mastoidectomy with type I tympanoplasty. During operation, definite cranial bone defect or cerebrospinal fluid leakage was not found. Histologically, the lesion was composed of exclusively mature, disorganized glial tissue with fibrovascular elements in a rather loose fibrillary background. Glial tissue showed diffuse positive reaction for glial fibrillar acidic protein and S100 protein on immunohistochemical study.

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Related in: MedlinePlus

Coronal (A, B) and axial (C, D) computed tomography demonstrate a mass-like lesion with soft tissue density occupying the middle ear and mastoid cavity of the left ear (B, D).
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Figure 2: Coronal (A, B) and axial (C, D) computed tomography demonstrate a mass-like lesion with soft tissue density occupying the middle ear and mastoid cavity of the left ear (B, D).

Mentions: A 50-yr-old man presented with progressive hearing loss and otorrhea of the left ear. Previously, he had frequently complained of ear fullness and hearing disturbance for 8 yr. Intermittent otorrhea was developed about 4 months ago. There was no history of congenital anomalies, trauma, or ear surgery. Physical examination revealed otorrhea in the external auditory canal of the left ear. The right ear was normal. Pure tone audiometry showed conductive hearing loss on the left ear (Fig. 1). The right ear was normal. Otorrhea was improved after treatment of otitis externa. The patient had suffered from progressive hearing disturbance. Tympanic membrane showed attic retraction. Computed tomography revealed a mass-like lesion with soft tissue density in the middle ear cavity and mastoid antrum. Bony erosions were suspected in tegmen tympani and long process of incus of the left ear (Fig. 2). Preoperative diagnosis was attic cholesteatoma. At operation, a graywhite fibrotic mass was detected in the epitympanic area. Mesotympanum was intact. There was no bony erosions in tegmen and incus. Malleus and stapes were unremarkable. After the surgical removal of the epitympanic mass, subsequent examination about ossicular movement was normal. The patient underwent left simple mastoidectomy with type I tympanoplasty. During operation, there was no evidence of cranial bone defect or leakage of cerebrospinal fluid. Microscopic examination of the hematoxylin-eosin stained section revealed a poorly circumscribed pale staining lesion with low cellularity. The lesion consisted of exclusively mature, disorganized glial tissue with fibrovascular tissue in a rather loose fibrillary background (Fig. 3A). No mitotic figures or cellular atypia were noted. Scant lymphocytes were scattered within the glial tissue. Dystrophic calcifications were noted in degenerative foci. Small distended glands, lined by ciliated, flat or cuboidal epithelium, were present at the periphery of the lesion (Fig. 3B). Other brain components, such as neurons, oligodendroglia, and meningeal, ependymal, or choroid elements were not detected. No clearly defined endodermal or mesodermal tissue could be identified. Additional sections were stained for glial fibrillar acidic protein (GFAP), S100 protein, cytokeratin (CK), and microtubule associated protein (MAP-2). The glial tissue revealed diffuse positive reaction for GFAP (Fig. 4A) and S100 protein (Fig. 4B), but negative reaction for MAP-2 and CK.


Glial choristoma in the middle ear and mastoid bone: a case report.

Lee JI, Kim KK, Park YK, Eah KY, Kim JR - J. Korean Med. Sci. (2004)

Coronal (A, B) and axial (C, D) computed tomography demonstrate a mass-like lesion with soft tissue density occupying the middle ear and mastoid cavity of the left ear (B, D).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2822257&req=5

Figure 2: Coronal (A, B) and axial (C, D) computed tomography demonstrate a mass-like lesion with soft tissue density occupying the middle ear and mastoid cavity of the left ear (B, D).
Mentions: A 50-yr-old man presented with progressive hearing loss and otorrhea of the left ear. Previously, he had frequently complained of ear fullness and hearing disturbance for 8 yr. Intermittent otorrhea was developed about 4 months ago. There was no history of congenital anomalies, trauma, or ear surgery. Physical examination revealed otorrhea in the external auditory canal of the left ear. The right ear was normal. Pure tone audiometry showed conductive hearing loss on the left ear (Fig. 1). The right ear was normal. Otorrhea was improved after treatment of otitis externa. The patient had suffered from progressive hearing disturbance. Tympanic membrane showed attic retraction. Computed tomography revealed a mass-like lesion with soft tissue density in the middle ear cavity and mastoid antrum. Bony erosions were suspected in tegmen tympani and long process of incus of the left ear (Fig. 2). Preoperative diagnosis was attic cholesteatoma. At operation, a graywhite fibrotic mass was detected in the epitympanic area. Mesotympanum was intact. There was no bony erosions in tegmen and incus. Malleus and stapes were unremarkable. After the surgical removal of the epitympanic mass, subsequent examination about ossicular movement was normal. The patient underwent left simple mastoidectomy with type I tympanoplasty. During operation, there was no evidence of cranial bone defect or leakage of cerebrospinal fluid. Microscopic examination of the hematoxylin-eosin stained section revealed a poorly circumscribed pale staining lesion with low cellularity. The lesion consisted of exclusively mature, disorganized glial tissue with fibrovascular tissue in a rather loose fibrillary background (Fig. 3A). No mitotic figures or cellular atypia were noted. Scant lymphocytes were scattered within the glial tissue. Dystrophic calcifications were noted in degenerative foci. Small distended glands, lined by ciliated, flat or cuboidal epithelium, were present at the periphery of the lesion (Fig. 3B). Other brain components, such as neurons, oligodendroglia, and meningeal, ependymal, or choroid elements were not detected. No clearly defined endodermal or mesodermal tissue could be identified. Additional sections were stained for glial fibrillar acidic protein (GFAP), S100 protein, cytokeratin (CK), and microtubule associated protein (MAP-2). The glial tissue revealed diffuse positive reaction for GFAP (Fig. 4A) and S100 protein (Fig. 4B), but negative reaction for MAP-2 and CK.

Bottom Line: During operation, definite cranial bone defect or cerebrospinal fluid leakage was not found.Histologically, the lesion was composed of exclusively mature, disorganized glial tissue with fibrovascular elements in a rather loose fibrillary background.Glial tissue showed diffuse positive reaction for glial fibrillar acidic protein and S100 protein on immunohistochemical study.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, College of Medicine, Dongguk University, 707 Sukjang-dong, Gyeongju 780-714, Korea. leego@mail.dongguk.ac.kr

ABSTRACT
Heterotopic brain tissue usually involves extracranial midline structures of the head and neck such as nose, nasopharynx, and oral cavity. Its occurrence in the non-midline structures, including middle ear, is rare. We described a 50-yr-old-man with heterotopic glial tissue in the middle ear and mastoid bone. The patient presented with progressive hearing loss for 8 yr. There was no history of congenital anomalies, trauma, or ear surgery. Computed tomography revealed a mass-like lesion with soft tissue density occupying the middle ear cavity and mastoid antrum. At the operation, a gray-white fibrotic mass was detected in the epitympanic area. Mesotympanum and ossicles were intact. The patient underwent left simple mastoidectomy with type I tympanoplasty. During operation, definite cranial bone defect or cerebrospinal fluid leakage was not found. Histologically, the lesion was composed of exclusively mature, disorganized glial tissue with fibrovascular elements in a rather loose fibrillary background. Glial tissue showed diffuse positive reaction for glial fibrillar acidic protein and S100 protein on immunohistochemical study.

Show MeSH
Related in: MedlinePlus