Prolonged survival in a patient with sinonasal teratocarcinosarcoma with cranial extension

Abstract

Sinonasal teratocarcinosarcoma is a rare malignant neoplasm characterized by the combined histological features of carcinosarcoma and teratoma. The primary symptoms of this tumor are usually nasal obstruction and epistaxis, and a nasal cavity mass is the most common clinical finding. The authors describe an exceptionally rare case in which the patient presented with massive intracranial extension and exhibited confusion as an initial symptom. He subsequently underwent combined radical surgery and radiation therapy and has remained free of disease for 31 months. The surgical approach to the lesion, histological features, and clinical course are detailed.

Sinonasal Teratocarcinosarcoma: A Rare Clinical Entity managed by Medial Maxillectomy and Adjuvant Chemoradiation

“…13,14 Usually, squamous cell or adenocarcinoma is seen and the mesenchymal component shows spindle, smooth, skeletal muscle, cartilage and bone features. 6 Common neoplasm consisting of nests.…”

“…6,7 As the tumor is highly aggressive and rare no adequate radiotherapy doses are described and individualization of therapy should be considered. 13,14 The complex tumor shape and close proximity to critical structures has made 3D CRT and IMRT better in delivering higher radiation doses to the target volumes. 3 Cervical node metastasis are seen in 17% and no elective neck dissection is indicated.…”

“…3 Most of the literature reported a 89% incidence in males and 11% in females. [2][3][4][5][6][7][8][9][10][11][12][13][14][15] The rapid growth and extensive local destruction hinders early diagnosis. Diagnosis is made by the presence of immature neural tissue and fetal epithelial structure admixed with various malignant and mesenchymal elements.…”

“…Diagnosis is made by the presence of immature neural tissue and fetal epithelial structure admixed with various malignant and mesenchymal elements. 3,[11][12][13][14][21][22][23][24] An origin from a primitive neural crest or stem or progenitor cell with multidirectional differentiation is currently favored. 14,20,24 Local recurrence after surgery was seen in 30 to 43%, hence, adjuvant treatment is recommended in the form of radiation and chemotherapy.…”

Teratocarcinosarcoma rinosinusal: Reporte de un caso

“…Los hallazgos clínicos más característicos son un tumor en la cavidad nasal, que compromete el seno maxilar ipsilateral o el seno etmoidal. Suele ser un hallazgo de las cirugías transesfenoidales por neurocirujanos 10 . Heffner y cols 11 en 1984 describió por primera vez esta neoplasia, y en una serie de 20 pacientes reportó una mortalidad de 60% a los 3 años, con un promedio de sobrevida de 1,7 años.…”