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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.The findings indicate that ELST is a rare neoplasm with benign histopathological appearance and clinically destructive behavior.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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Immunophenotype of endolymphatic sac tumor. A. The tumor cells showed positive reactivity with cytokeratin (Pan) (immunostaining, 400×). B. The tumor cells were positive to vimentin(immunostaining, 400×). C. The tumor cells exhibited CD56 expression(immunostaining, 400×).D. The tumor cells exhibited NSE expression (immunostaining, 400×).
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Figure 3: Immunophenotype of endolymphatic sac tumor. A. The tumor cells showed positive reactivity with cytokeratin (Pan) (immunostaining, 400×). B. The tumor cells were positive to vimentin(immunostaining, 400×). C. The tumor cells exhibited CD56 expression(immunostaining, 400×).D. The tumor cells exhibited NSE expression (immunostaining, 400×).

Mentions: The tumor showed diffusely positive reactivity with cytokeratin (Pan) (Figure 3A), cytokeratin 19, cytokeratin 5/6, cytokeratin 7, EMA, vimentin (Figure 3B), CD56 (Figure 3C), and NSE (Figure 3D) and also showed variable reactivity with GFAP and VEGF. Thyroglobulin, TTF-1, S-100, and calretinin immunoreactivity was not observed. The Ki-67 immunostain showed a proliferation index of < 1%. The colloid-like material showed positive reactivity with PAS.


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)

Immunophenotype of endolymphatic sac tumor. A. The tumor cells showed positive reactivity with cytokeratin (Pan) (immunostaining, 400×). B. The tumor cells were positive to vimentin(immunostaining, 400×). C. The tumor cells exhibited CD56 expression(immunostaining, 400×).D. The tumor cells exhibited NSE expression (immunostaining, 400×).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Immunophenotype of endolymphatic sac tumor. A. The tumor cells showed positive reactivity with cytokeratin (Pan) (immunostaining, 400×). B. The tumor cells were positive to vimentin(immunostaining, 400×). C. The tumor cells exhibited CD56 expression(immunostaining, 400×).D. The tumor cells exhibited NSE expression (immunostaining, 400×).
Mentions: The tumor showed diffusely positive reactivity with cytokeratin (Pan) (Figure 3A), cytokeratin 19, cytokeratin 5/6, cytokeratin 7, EMA, vimentin (Figure 3B), CD56 (Figure 3C), and NSE (Figure 3D) and also showed variable reactivity with GFAP and VEGF. Thyroglobulin, TTF-1, S-100, and calretinin immunoreactivity was not observed. The Ki-67 immunostain showed a proliferation index of < 1%. The colloid-like material showed positive reactivity with PAS.

Bottom Line: Neither the symptoms nor a family history of VHL disease were found in the patient.The findings indicate that ELST is a rare neoplasm with benign histopathological appearance and clinically destructive behavior.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