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Endolymphatic sac tumor: case report and review of the literature.

Sun YH, Wen W, Wu JH, Song JM, Guan H, Wang KX, Xu MQ - Diagn Pathol (2012)

Bottom Line: Neither the symptoms nor a family history of VHL disease were found in the patient.Because of the rarity of this tumor, it can easily be confused with other tumors such as paraganglioma, middle ear adenoma, adenocarcinoma, papillary carcinoma of thyroid or choroid plexus papilloma.Owing to its locally aggressive nature, it is difficult to extirpate surgically when it is large.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pathology, Shenzhen Second People's Hospital, 3002 Sungang West Road, Shenzhen 518035, China.

ABSTRACT

Unlabelled: Endolymphatic sac tumor (ELST) is a rare neoplasm which can be encountered sporadically or in Von Hippel-Lindau (VHL) disease. Here we report a sporadic case of ELST in 31-year-old man. Neither the symptoms nor a family history of VHL disease were found in the patient. CT imaging demonstrated an expansile lytic lesion of the mastoid process of the left petrous bone. MR scanning revealed a 5.2 cm × 4.7 cm × 4.2 cm mass which showed hyperintensity on T1- and T2-weighted images. Histologic sections showed a papillary, cystic or glandular architecture. The papillary and glandular structures were lined by a single layer of flattened cuboidal-to-columnar cells. The stroma of the papillary fronds was richly vascularized and chronically inflamed. The tumor showed diffusely positive reactivity with cytokeratin (Pan), cytokeratin 19, cytokeratin 5/6, cytokeratin 7, EMA, vimentin, CD56, and NSE and also showed variable reactivity with glial fibrillary acidic protein (GFAP) and VEGF. The Ki-67 immunostain showed a proliferation index of < 1%. Because the mass was large, it was difficult to extirpate surgically. After surgery, the patient underwent gamma-knife radiosurgery for residual tumor. The findings indicate that ELST is a rare neoplasm with benign histopathological appearance and clinically destructive behavior. Because of the rarity of this tumor, it can easily be confused with other tumors such as paraganglioma, middle ear adenoma, adenocarcinoma, papillary carcinoma of thyroid or choroid plexus papilloma. Owing to its locally aggressive nature, it is difficult to extirpate surgically when it is large.

Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/7973320646763012.

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Radiologic characterization of endolymphatic sac tumor. A. CT imaging demonstrated an expansile lytic lesion of the mastoid process of the left petrous bone extending to involve both the left medial mastoid as well as the middle ear. B. MR scan showed a 5.2 cm × 4.7 cm × 4.2 cm mass which was irregular, heterogeneous, and lobulated and showed hyperintensity on T1-weighted images. C. The periphery of the lesion appeared markedly hyperintense on the T2-weighted images. D. Axial postcontrast T1-weighted MR scan showed a heterogeneous enhancing mass.
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Figure 1: Radiologic characterization of endolymphatic sac tumor. A. CT imaging demonstrated an expansile lytic lesion of the mastoid process of the left petrous bone extending to involve both the left medial mastoid as well as the middle ear. B. MR scan showed a 5.2 cm × 4.7 cm × 4.2 cm mass which was irregular, heterogeneous, and lobulated and showed hyperintensity on T1-weighted images. C. The periphery of the lesion appeared markedly hyperintense on the T2-weighted images. D. Axial postcontrast T1-weighted MR scan showed a heterogeneous enhancing mass.

Mentions: The patient presented with a chief complaints of progressive hearing loss of approximately 4-year duration. In the past month, the symptom increased accompanied by headaches. There was no tinnitus, otalgia, otorrhea, vertigo or facial nerve paralysis. There was no history of trauma or surgeries. On physical examination, the hearing of left ear disappeared. The right ear was normal. Examination of the facial nerves, nasopharynx, oral cavity, larynx and neck was normal. Neither the symptoms nor a family history of VHL disease were found in the patient. Computed tomography (CT) demonstrated a large, hypodense, and expansile lytic lesion of the mastoid process of the left petrous bone extending to involve both the left medial mastoid as well as the middle ear (Figure 1A). Magnetic resonance (MR) revealed a 5.2 cm × 4.7 cm × 4.2 cm mass in the mastoid process of the left petrous bone which was irregular, heterogeneous, and lobulated and showed hyperintensity on T1- and T2-weighted images (Figure 1B). The periphery of the lesion appeared markedly hyperintense on the T2-weighted images (Figure 1C). This might represent hemorrhage (extracellular methemoglobin) or cystic areas with high protein within the lesion. Axial postcontrast T1-weighted MR scan showed a heterogeneous enhancing mass (Figure 1D). Surgical excision was performed. Because the mass was too large, it was difficult to extirpate surgically. After surgery, the patient underwent gamma-knife radiosurgery for residual tumor.


Endolymphatic sac tumor: case report and review of the literature.

Sun YH, Wen W, Wu JH, Song JM, Guan H, Wang KX, Xu MQ - Diagn Pathol (2012)

Radiologic characterization of endolymphatic sac tumor. A. CT imaging demonstrated an expansile lytic lesion of the mastoid process of the left petrous bone extending to involve both the left medial mastoid as well as the middle ear. B. MR scan showed a 5.2 cm × 4.7 cm × 4.2 cm mass which was irregular, heterogeneous, and lobulated and showed hyperintensity on T1-weighted images. C. The periphery of the lesion appeared markedly hyperintense on the T2-weighted images. D. Axial postcontrast T1-weighted MR scan showed a heterogeneous enhancing mass.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3368761&req=5

Figure 1: Radiologic characterization of endolymphatic sac tumor. A. CT imaging demonstrated an expansile lytic lesion of the mastoid process of the left petrous bone extending to involve both the left medial mastoid as well as the middle ear. B. MR scan showed a 5.2 cm × 4.7 cm × 4.2 cm mass which was irregular, heterogeneous, and lobulated and showed hyperintensity on T1-weighted images. C. The periphery of the lesion appeared markedly hyperintense on the T2-weighted images. D. Axial postcontrast T1-weighted MR scan showed a heterogeneous enhancing mass.
Mentions: The patient presented with a chief complaints of progressive hearing loss of approximately 4-year duration. In the past month, the symptom increased accompanied by headaches. There was no tinnitus, otalgia, otorrhea, vertigo or facial nerve paralysis. There was no history of trauma or surgeries. On physical examination, the hearing of left ear disappeared. The right ear was normal. Examination of the facial nerves, nasopharynx, oral cavity, larynx and neck was normal. Neither the symptoms nor a family history of VHL disease were found in the patient. Computed tomography (CT) demonstrated a large, hypodense, and expansile lytic lesion of the mastoid process of the left petrous bone extending to involve both the left medial mastoid as well as the middle ear (Figure 1A). Magnetic resonance (MR) revealed a 5.2 cm × 4.7 cm × 4.2 cm mass in the mastoid process of the left petrous bone which was irregular, heterogeneous, and lobulated and showed hyperintensity on T1- and T2-weighted images (Figure 1B). The periphery of the lesion appeared markedly hyperintense on the T2-weighted images (Figure 1C). This might represent hemorrhage (extracellular methemoglobin) or cystic areas with high protein within the lesion. Axial postcontrast T1-weighted MR scan showed a heterogeneous enhancing mass (Figure 1D). Surgical excision was performed. Because the mass was too large, it was difficult to extirpate surgically. After surgery, the patient underwent gamma-knife radiosurgery for residual tumor.

Bottom Line: Neither the symptoms nor a family history of VHL disease were found in the patient.Because of the rarity of this tumor, it can easily be confused with other tumors such as paraganglioma, middle ear adenoma, adenocarcinoma, papillary carcinoma of thyroid or choroid plexus papilloma.Owing to its locally aggressive nature, it is difficult to extirpate surgically when it is large.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pathology, Shenzhen Second People's Hospital, 3002 Sungang West Road, Shenzhen 518035, China.

ABSTRACT

Unlabelled: Endolymphatic sac tumor (ELST) is a rare neoplasm which can be encountered sporadically or in Von Hippel-Lindau (VHL) disease. Here we report a sporadic case of ELST in 31-year-old man. Neither the symptoms nor a family history of VHL disease were found in the patient. CT imaging demonstrated an expansile lytic lesion of the mastoid process of the left petrous bone. MR scanning revealed a 5.2 cm × 4.7 cm × 4.2 cm mass which showed hyperintensity on T1- and T2-weighted images. Histologic sections showed a papillary, cystic or glandular architecture. The papillary and glandular structures were lined by a single layer of flattened cuboidal-to-columnar cells. The stroma of the papillary fronds was richly vascularized and chronically inflamed. The tumor showed diffusely positive reactivity with cytokeratin (Pan), cytokeratin 19, cytokeratin 5/6, cytokeratin 7, EMA, vimentin, CD56, and NSE and also showed variable reactivity with glial fibrillary acidic protein (GFAP) and VEGF. The Ki-67 immunostain showed a proliferation index of < 1%. Because the mass was large, it was difficult to extirpate surgically. After surgery, the patient underwent gamma-knife radiosurgery for residual tumor. The findings indicate that ELST is a rare neoplasm with benign histopathological appearance and clinically destructive behavior. Because of the rarity of this tumor, it can easily be confused with other tumors such as paraganglioma, middle ear adenoma, adenocarcinoma, papillary carcinoma of thyroid or choroid plexus papilloma. Owing to its locally aggressive nature, it is difficult to extirpate surgically when it is large.

Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/7973320646763012.

Show MeSH
Related in: MedlinePlus