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Vol. 69. Núm. 5.
Páginas 304-305 (Septiembre - Octubre 2018)
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Vol. 69. Núm. 5.
Páginas 304-305 (Septiembre - Octubre 2018)
Case study
DOI: 10.1016/j.otorri.2017.06.010
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Myopericytoma of the tongue base: A case report
Miopericitoma de la base lingual: presentación de un caso clínico
Stefano Rubinoa,
Autor para correspondencia

Corresponding author.
, Rita De Berardinisa, Daniele Colombob, Alessandro De Padovaa
a Department of Otolaryngology and Head and Neck surgery, University of “Tor Vergata”, Rome, Italy
b Department of Pathology, University of “Tor Vergata”, Rome, Italy
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Case report

A 48-year old man, with no history of cancer, presented to our Department of Otolaryngology and Head and Neck Surgery complaining of stomatolalia by 7 months and then dysphagia for solid and liquid foods for 3 months. He had no paresthesia or motor impairment of tongue. Clinical examination revealed a not ulcerated nodular lesion of the tongue base. On cervical MRI with gadolinium-based contrast media, a 3cm nodule occupying hypopharynx lumen was located between tongue base and epiglottis with base implant on valleculae. The lesion showed more contrast enhancement in correspondence of infero-posterior pole due to the presence of ectatic vascular structures. There were not enlarged lymphnodes (Fig. 1).

Figure 1.

(A) Sagittal T2-weighted MRI scan. (B) Axial T2-weighted fat-suppressed MRI scan showing a round, hyperintense lesion with circumscribed margins which occupies the entire oropharyngeal space. (C) Coronal T2-weighted MRI scan showing a round, hyperintense lesion with circumscribed margins arising from the base of tongue and dislocating the epiglottis.


Excision of the lesion by median pharyngotomy and temporary tracheotomy were performed in March 2014. It was placed a nasogastric tube to perform enteral feeding for the first two days, and in VII post-operative day the endotracheal tube was removed. There were no postoperative complications. Histological assessment of the excised lesion demonstrated a 3.2cm nodule corresponding to a proliferative and vascular lesion with immunohistochemical characters of MPC, with monomorphic oval to spindle shaped myoid appearing cells showing a perivascular growth pattern on Hematoxylin–Eosin staining and reactivity to Caldesmon immunostaining, without aspects of malignancy and a proliferative index lesser than 5% (Fig. 2). The patient is subject to follow up and currently there is no evidence of recurrence.

Figure 2.

(A) Monomorphic oval to spindle shaped myoid appearing cells showing a perivascular growth pattern (Hematoxylin–Eosin staining, magnification 100×). (B) Caldesmon immunostaining highlights tumoral cells (Caldesmon immunostaining, magnification 100×).


Myopericytoma (MPC) is a rare mesenchymal tumor derived from perivascular myoid cells and characterized by a tendency for concentric perivascular growth and a generally benign behavior. It is commonly affecting the skin and soft tissues of extremities. In sporadic cases it can locate in the oral and facial region. We present a rare case of large myopericytoma of the tongue base.1,2

MPC is a very rare tumor, generally considered as a slow-growing benign neoplasm, although few cases of low-grade malignant behavior are described.3

It was often confused with angioleiomyoma and myofibroma4 until 2006 when Mentzel et al.5 demonstrated that MPC is a perivascular-myoid neoplasm of the skin and soft tissue, with numerous thin-walled blood vessels and concentric periluminal proliferation of ovoid, plump, spindled and/or round myoid tumor cells. The immunohistochemical findings are very useful for the diagnosis as MPC is immunoreactive to smooth muscle actin (SMA), muscle-specific actin (MSA), vimentin and h-Caldesmon beside a characteristic negativity to desmin and CD34.3,6

It arises mostly in the skin and soft tissues of upper and lower extremities, head and neck region and trunk.7 The usual diameter is less than 2cm. CT and MRI contrast-enhanced imaging may be helpful in the differential diagnosis and pre-operative evaluation of MPC, despite a low specificity. It can be generally observed a peripheral enhancement and reactive lymphnodes.7,8 The usual treatment is a complete and wide surgical excision, as the tumor may recur locally, and may need ancillary procedures (e.g. the temporary tracheotomy in this case) or complex approaches in neoplasms involving other functions (upper airways patency, swallowing, facial nerve preservation, etc.). In conclusion, this report describes one of the first cases, to our knowledge, of a MPC implanting on vallecular region and involving the tongue base and epiglottidis, that needed a complex preoperative planning, in which radiological imaging became a vital helping hand, and a difficult surgical approach because of its large dimension and involvement of upper airways. Finally, immunohistochemical findings represent the key to the differential diagnosis of MPC from other perivascular myoid cell-based neoplasms of the same regions.

Conflict of interest

The authors declare no conflict of interest or funding received for this article.

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Copyright © 2017. Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello
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