» Articles » PMID: 37258051

Clinicopathological Analysis of Extraneural Sporadic Haemangioblastoma Occurring in the Tongue

Overview
Journal BMJ Case Rep
Specialty General Medicine
Date 2023 May 31
PMID 37258051
Authors
Affiliations
Soon will be listed here.
Abstract

Haemangioblastoma is a morphologically distinctive tumour with uncertain histogenesis, typically occurring in the cerebellum, brain stem or spinal cord and less commonly in extraneural locations. Here, we present a case of haemangioblastoma occurring in the tongue, which is the first reported case in terms of the pathogenic site. The tumour was morphologically indistinguishable from central nervous system haemangioblastoma, that is, neoplastic stromal cells with cytoplasmic vacuolisation and abundant small vessels. Immunohistochemical studies revealed that the tumour cells were positive for S100, NSE, CD56, Syn, EMA, vimentin and α-inhibin, while negative for CK, SMA, factor Ⅷ, D2-40 and GFAP. Immunostainings for CD34 and CD31 outlined the rich and delicate vascular channels. Ki-67 expression was presented in approximately 3% of tumour cells. Primary haemangioblastoma has not been previously described at this site, and this case emphasises the need to consider haemangioblastoma in the differential diagnoses of neoplasms occurring in the tongue.

References
1.
Lu L, Drew P, Yachnis A . Hemangioblastoma in the lung: metastatic or primary lesions?. Case Rep Pathol. 2015; 2014:468671. PMC: 4276681. DOI: 10.1155/2014/468671. View

2.
Maher E, Neumann H, Richard S . von Hippel-Lindau disease: a clinical and scientific review. Eur J Hum Genet. 2011; 19(6):617-23. PMC: 3110036. DOI: 10.1038/ejhg.2010.175. View

3.
Carney E, Banerjee P, Ellis C, Albadine R, Sharma R, Chaux A . PAX2(-)/PAX8(-)/inhibin A(+) immunoprofile in hemangioblastoma: A helpful combination in the differential diagnosis with metastatic clear cell renal cell carcinoma to the central nervous system. Am J Surg Pathol. 2011; 35(2):262-7. DOI: 10.1097/PAS.0b013e3182064d11. View

4.
Bisceglia M, Muscarella L, Galliani C, Zidar N, Ben-Dor D, Pasquinelli G . Extraneuraxial Hemangioblastoma: Clinicopathologic Features and Review of the Literature. Adv Anat Pathol. 2017; 25(3):197-215. DOI: 10.1097/PAP.0000000000000176. View

5.
Deb P, Pal S, Dutta V, Srivastava A, Bhargava A, Yadav K . Adrenal haemangioblastoma presenting as phaeochromocytoma: a rare manifestation of extraneural hemangioblastoma. Endocr Pathol. 2012; 23(3):187-90. DOI: 10.1007/s12022-012-9207-x. View