Oral ectomesenchymal chondromyxoid tumor: case report and literature review

Portnof, Jason E.; Friedman, Joel M.; Reich, R.; Freedman, Paul D.; Behrman, David

doi:10.1016/j.tripleo.2009.05.040

Cited by 25 publications

(48 citation statements)

References 18 publications

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“…Given the benign nature of ECMT, surgical excision is the preferred treatment. Recurrences have been reported in only 4 cases to date, at intervals varying from 3 months to 20 years post-surgery [5,10]. The rate of recurrence thus appears to be low with complete excision.…”

Section: Discussionmentioning

confidence: 99%

Ectomesenchymal Chondromyxoid Tumour of the Posterior Tongue

Cardin

Fiset

Zeitouni

et al. 2013

Head and Neck Pathol

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Ectomesenchymal chondromyxoid tumor (ECMT) is a rare benign neoplasm arising in the tongue. With only 45 cases reported in the literature, there are several unique features defining this lesion. Firstly, almost all patients present with an asymptomatic slow growing mass on the anterior dorsum of the tongue. At the microscopic level, it is recognizable as a well-circumscribed unencapsulated proliferation of uniform round to fusiform cells embedded in a chondromyxoid matrix. Lastly, the immunohistochemistry profile is characterised by positivity for glial fibrillary acidic protein and frequent positivity for S-100 and cytokeratins. We report a case of a mass located on the posterior dorsum of the tongue and meeting the aforementioned morphological and immunohistochemical criteria of ECMT.The patient is a 43 year old male who presented to the Ears Nose and Throat clinic at the McGill University Health Center with odynophagia and mild dysphagia that had been present for 3 months. He did not have any significant prior medical or family medical history, did not take medications, and did not smoke or consume alcohol. The patient felt a slowly growing mass at the back of his throat that would occasionally trigger a gag reflex and the sensation of narrowing of his throat. He had mild dysphagia but retained normal tongue strength, range of motion and sensation. On examination, an exophytic mass was visible on the back of the tongue that crossed the midline and was soft on palpation. The mass obliterated the space between the uvula and the palatoglossal arch.A CT scan without contrast was performed which showed a large cystic appearing mass at the tongue base (Fig. 1). A fine needle aspiration of the mass produced 15 mL of red fluid. A cell block of the fluid was prepared and showed whorls of spindled cells with alternating hypercellular and hypocellular areas with scattered thinwalled vessels. Nuclear atypia was minimal and no mitotic figures were identified. Immunohistochemistry of the cell block showed positive staining for vimentin, GFAP, cyclin D1, synaptophysin and CD57. Desmin, factor VIII, myogenin and epithelial membrane antigen (EMA) staining were weak and/or patchy. Cytoplasmic beta-catenin staining was seen, but without nuclear stain. Immunohistochemistry was negative for cytokeratin (CK) AE1/AE3, CK5/6, p63, p16, HMB-45, CD34, CD31, c-kit, smooth muscle actin (SMA), bcl-2, factor XIIIa, calponin, neurofilament, S-100 and chromogranin. A low grade mesenchymal neoplasm was favored over a salivary gland neoplasm, and it was recommended that the patient have local resection for definitive diagnosis.For the surgery, a mouth gag was introduced and the oral cavity was visualized. The tongue was grasped and a tonsillectomy gag was placed in the oral cavity to expose the base of the tongue. With the tongue retracted, the tumor

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Section: Discussionmentioning

confidence: 99%

Ectomesenchymal Chondromyxoid Tumour of the Posterior Tongue

Cardin

Fiset

Zeitouni

et al. 2013

Head and Neck Pathol

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“…It usually occurs almost exclusively in the anterior portion of the tongue as a dome-shaped or sessile protrusion,1-12 except for one case in the posterior tongue 13. Morphologically, these tumors also display characteristic tumor cells embedded in myxoid to chondroid stroma.…”

Section: Discussionmentioning

confidence: 99%

Ectomesenchymal Chondromyxoid Tumor in the Anterior Tongue: Case Report of a Unique Tumor

et al. 2012

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Ectomesenchymal chondromyxoid tumor (ECMT) is a rare tumor, exclusively arising in the anterior tongue. Thirty-eight cases have been reported in the English literature. It usually presents as a sessile protrusion and shows round to spindle cells embedded in myxoid to chondroid stroma. Tumor cells are almost always positive for polyclonal glial fibrillary acidic protein (GFAP). We report our experience in the recent treatment of a case of ECMT, the third case in 3 years. The mass in the anterior tongue revealed characteristic morphologic features of ECMT and the expression of polyclonal GFAP. Although ECMT should be differentiated from other mesenchymal tumors including myoepithelioma, its clinical, morphological, and immunohistochemical features enable its diagnosis, especially when pathologists are aware of it.

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“…Man geht davon aus, dass sich diese Tumoren aus versprengtem undifferenziertem Gewebe der Neuralleiste ableiten, da sich der vordere Teil der Zunge aus dem Neuralleistenmesenchym des ersten Kiemenbogens entwickelt [5,8]. In der aktuellen WHO-Klassifikation der Kopf-/Halstumoren wird der ECMT als Weichteiltumor klassifiziert [2].…”

Section: Diskussionunclassified

“…4 7 Diffuse, kräf-tige zytoplasmatische Expression von "glial fibrillary acidic protein" durch die Tumorzellen (Vergr. 100:1) nente) sowie der Tatsache, dass sich in der anterioren dorsalen Zunge keine Speicheldrüsen finden, als unwahrscheinlich [7,8].…”

Section: Introductionunclassified

“…Woo et al [11] argumentieren, dass myoepitheliale Zellen die Expression von myogenen Markern verlieren können und daher die Negativität für SMA nicht gegen das Vorliegen eines Myoepithelioms spricht. Die Mehrheit der publizierten Arbeiten teilt jedoch die Ansicht, dass es sich beim ECMT um eine von den Myoepitheliomen abgegrenzte Entität handelt [4,7,8]. In der aktuellen WHO-Klassifikation der Kopf-/Halstumoren von 2005 wird der ECMT als eigene, von den Myoepitheliomen abgegrenzte Entität aufgeführt [2].…”

Section: Introductionunclassified

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