Ectomesenchymal chondromyxoid tumor: a comprehensive updated review of the literature and case report

Abstract

Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1). At present, ectomesenchymal cells that migrate from the neural crest are considered to play a pivotal role in tumor origin. All cases had a benign course, although there were three recurrences. Because of the rarity of this tumor and the need for differential diagnostic differentiation from myoepithelioma and pleomorphic adenoma, both oral surgeons and pathologists should be aware of this entity.

Ectomesenchymal chondromyxoid tumor with a significant proliferative index: A case report

“…Conversely, a low proliferative index of this tumor has been reported in the literature. [2][3][4]14,19,26,30 Considering the variable immunohistochemical profile of the ECT, the high proliferative index suggests the need for constant monitoring of these cases, with special attention to areas containing populations of small cells.…”

“…The main differential diagnosis of the ECT includes myoepithelioma 2,3,[5][6][7][8]11,12,15,17,19,20,23,24,31,32 and pleomorphic adenoma. 2,3,5,7,8,12,17,20,31 However, despite the presence of chondroid and myxoid differentiation and spindle-shaped cells, we found no plasmacytoid cells, glandular ducts, or salivary gland tissue. Other differential diagnoses are myxoma, mucocele, oral focal mucinosis, neurofibroma, and schwannoma.…”

“…1 Approximately 75 cases of ECT were published in English in the literature. [2][3][4] The tumor manifests as a submucosal nodule on the back of the tongue or, less frequently, at the base of the tongue and palate. 2,5 The ECT is usually small, ranging from 4 to 50 mm, 2 has no gender predilection, and affects patients between the first and eighth decades of life.…”

Mesenchymal tumours with RREB1–MRTFB fusion involving the mediastinum: extra‐glossal ectomesenchymal chondromyxoid tumours?

“…in 1995 1,2 . Affecting patients with a wide age range, ECT almost exclusively presents as a small (0.3–3.0 cm), painless, slow‐growing, discrete mass localised most commonly to the anterior dorsal surface of the tongue, with only a few studies reporting cases in the hard palate 3,4 and tonsil 5 . The lobular tumour consists of round to spindled cells arranged in cords, sheets or a reticulated pattern within myxoid, chondroid or hyalinised stroma.…”

Ectomesenchymal chondromyxoid tumour: an uncommon characteristic tumour of the anterior tongue

“…WHO had named this lesion as ‘ECMT of the anterior tongue’ due to the peculiar location of this tumour 2. It has a slight male predilection and the incidence is more commonly reported in patients around the fourth decade of life 3…”