RESUMEN
Ameloblastic fibrosarcoma is a rare malignant odontogenic tumour characterized by a benign epithelial component within a malignant fibrous stroma. Its behaviour is relatively benign, with absence of metastatic disease, and the prognosis is reported to be good. It is a paradoxical neoplasm with "sarcomatous" morphological and immunohistochemical patterns but with a favourable clinical course. We report a new case of this tumour in a mandibular ramus of a 31-years-old male patient, that was surgically excised and treated with adjuvant chemotherapy and radiotherapy. Five years later the patient is free of disease. The growth potential of ameloblastic fibrosarcoma is evaluated and compared with a related lesion, the ameloblastic fibroma. The sarcomatous mesenchymal component of ameloblastic fibrosarcoma is positive to Ki67, PCNA and p53, in front of the negativity of ameloblastic fibroma.
Asunto(s)
Neoplasias Mandibulares/patología , Tumores Odontogénicos/patología , Adulto , Quimioterapia Adyuvante , Células Epiteliales/patología , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Antígeno Ki-67/análisis , Masculino , Neoplasias Mandibulares/cirugía , Mesodermo/patología , Tumores Odontogénicos/cirugía , Pronóstico , Antígeno Nuclear de Célula en Proliferación/análisis , Radioterapia Adyuvante , Proteína p53 Supresora de Tumor/análisisRESUMEN
BACKGROUND: Cervical cystic lymph node metastases as first and sole manifestation of occult papillary thyroid carcinoma are observed exceptionally rarely. In the seven patients here reported, a cystic, ovoid mass in the lateral aspect of the neck was the initial symptom of the papillary microcarcinoma. METHODS: There were six men and one woman, aged between 17 and 54 years (mean 31.7 years), who complained of round, movable, painless masses in the lateral aspect of the neck. Two patients were first seen with two cervical tumors. Tumors had been present from a few days to 12 months (mean 5.1 months). The cystic nature of tumors was demonstrated by echographic studies. In all cases, thyroid tumors were not palpable on physical examination and no abnormalities of the thyroid gland were shown by other diagnostic procedures. RESULTS: The diagnosis was made preoperatively by fineneedle aspiration cytology of the nodes in five of the seven cases. All patients underwent thyroidectomy with conservative neck dissection followed by radioactive iodine therapy. After a follow-up period from 1 to 7 years, all patients are alive with no apparent signs of recurrence or metastasis. CONCLUSIONS: In a young patient with solitary lateral cervical cyst, the diagnosis of lymph node metastasis from occult papillary thyroid carcinoma should be considered. Any lateral mass requires tissue diagnosis, and fine-needle aspiration is usually adequate for clarification of the histology. Ipsilateral modified neck dissection and total thyroidectomy followed by radioactive iodine therapy offers a favorable prognosis.