Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2015 Jun;9(2):300-4.
doi: 10.1007/s12105-014-0569-x. Epub 2014 Sep 7.

Sclerosing paraganglioma of the carotid body: a potential pitfall of malignancy

Raffaella Santi  1 , Alessandro Franchi, Valeria Saladino, Massimo Trovati, Giovanna Cenacchi, Massimo Squadrelli-Saraceno, Gabriella Nesi
Affiliations
Case Reports

Sclerosing paraganglioma of the carotid body: a potential pitfall of malignancy

Raffaella Santi et al. Head Neck Pathol. 2015 Jun.

Abstract

Paragangliomas (PGs) of the head and neck region are typically benign, slow-growing neuroendocrine tumours. At times, they may exhibit unusual histological features, such as prominent stromal sclerosis (sclerosing PG), which may raise concerns of malignancy. We describe a case of sclerosing PG of the carotid body, emphasizing the value of immunohistochemical stains for differential diagnosis. A 43-year-old woman presented with a painless lump on the neck. A magnetic resonance imaging scan demonstrated a hypervascular lesion of the carotid body, which was surgically excised. Grossly, the lesion measured 1.8 cm at maximum diameter. On microscopic examination, irregular nests and tiny bundles of neoplastic cells were found between thick bands of fibrous tissue. Focal nuclear cytomegaly and marked pleomorphism were noted. Neoplastic cells proved to be immunoreactive for chromogranin, synaptophysin and neuron specific enolase, but negative for cytokeratins, smooth muscle actin and CD34. Ultrastructurally, numerous mitochondria, rough endoplasmic reticulum structures and endocrine granules were seen in the cytoplasm of the tumour cells. On consideration of the above-mentioned clinico-pathological and ultrastructural findings a diagnosis of sclerosing PG was established. Sclerosing PG is a rare entity which may mimic a malignant neoplasm. The recognition of this unusual morphological variant of PG, together with appropriate immunostains, leads to the correct diagnosis.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Sclerosing PG showing irregular nests and tiny bundles of epithelioid to spindle cells embedded in a large amount of sclerotic stroma (a) marked nuclear pleomorphism and enlargement was occasionally seen (b)
Fig. 2
Fig. 2
Neoplastic cells were immunoreactive for chromogranin (a) and synaptophysin (b) and negative for cytokeratins (c)
Fig. 3
Fig. 3
Electron microscopy revealing numerous mitochondria as well as dense core (neuroendocrine) granules (inset) in the cytoplasm of sclerosing PG cells
Fig. 4
Fig. 4
Tumour cells displaying cytoplasmic and distinctly granular reactivity for SDHA (a) and SDHB (b)
See this image and copyright information in PMC

References

    1. Lack EE. Atlas of tumor pathology, 4rd Series, fascicle 8. Washington DC: Armed Forces Institute of Pathology; 2007.
    1. DeLellis RA, Lloyd RV, Heitz PU, et al., editors. World Health Organization classification of tumours. Pathology and genetics of tumours of endocrine organs. Lyon: IARC Press; 2004.
    1. Bikhazi PH, Roeder E, Attaie A, Lalwani AK. Familial paragangliomas: the emerging impact of molecular genetics on evaluation and management. Am J Otol. 1999;20:639–643. - PubMed
    1. Pellitteri PK, Rinaldo A, Myssiorek D, et al. Paragangliomas of the head and neck. Oral Oncol. 2004;40:563–575. doi: 10.1016/j.oraloncology.2003.09.004. - DOI - PubMed
    1. Manolidis S, Shohet JA, Jackson CG, Glasscock ME., 3rd Malignant glomus tumors. Laryngoscope. 1999;109:30–34. doi: 10.1097/00005537-199901000-00007. - DOI - PubMed

Publication types

MeSH terms

LinkOut - more resources