Dados do Trabalho

Título

CHONDROMYXOID ECTOMESENCHYMAL TUMOR OF THE OROPHARYNX

Apresentação do caso

Female patient, 36 years old, without comorbidities, denies smoking and alcoholism, with a positive family history of malignant neoplasm, mother who died of squamous cell carcinoma of the larynx and grandfather adenocarcinoma of the prostate. Approximately 1 year ago, she noticed the appearance of a “lump” in the retroauricular region and was diagnosed and treated for mumps. Due to the progressive enlargement of the nodule, a contrast-enhanced computed tomography of the neck showed a voluminous, solid, infiltrative, expansive lesion with soft tissue attenuation, centered on the right oropharynx, compromising deep suprahyoid cervical spaces. The mass invades the parapharyngeal, carotid, parotid and right masticatory spaces. In the carotid space, it deviates posteriorly the cervical segment of the internal carotid artery and the internal jugular vein. In the parotid space, the lesion appears to infiltrate the entire deep lobe of the parotid. The mass measures 7.0x3.5x6.5. Lymph node enlargement in chain Ib and IIa on the right. The patient developed dyspnea and required tracheostomy and exploratory cervicotomy with level IIa tumor biopsy. Having a histopathological diagnosis of chondromyxoid ectomesenchymal tumor and immunohistochemistry that showed positive cytokeratin 7, positive S100, positive GFAP and 1-2% Ki67 favoring the diagnosis of myxoid mesenchymal spindle cell neoplasia.

Discussão

The chondromyxoid ectomesenchymal tumor (ECT) is a benign neoplasm involving exclusively the oral cavity, mainly in the anterior region of the tongue, and with extremely rare histogenesis. The first report in the literature was in 1995. It is a slow and painless clinical entity. The age group found is quite variable from 9 to 78 years old and without sex predilection. The main differential diagnoses to be considered are myoepithelioma and myxoid neurofibroma. The immunohistochemistry profiles of the published cases show consistent positivity for S100, GFAP, desmin and AE1/AE3. The present report presents histology similar to others described in the literature with positivity for S-100 and GFAP. Treatment basically consists of conservative surgical excision and follow-up. Recurrence has been seen in less than 10% of cases and may occur within 19 months after resection.

Comentários finais

The patient was evaluated by the IHBASE head and neck surgery team and considered an inoperable case performed as exclusive radiotherapy treatment.

Palavras-chave

chondromyxoid ectomesenchymal tumor, oropharynx and radiotherapy.

Área

Oncologia - Tumores de cabeça e pescoço