Kane Shubhada V, Karpate Arti A, Bal Munita, Juvekar S L, Pai Prathmesh S
Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India.
Head Neck Pathol. 2009 Mar;3(1):31-6. doi: 10.1007/s12105-008-0094-x. Epub 2008 Dec 4.
Sinonasal teratocarcinosarcoma (SNTCS) is a rare and highly malignant tumour with combined features of a teratoma and carcinosarcoma. We report the first case of a SNTCS in 23 year old male treated with neo-adjuvant chemotherapy followed by cranio-facial resection. The resection specimen displayed cellular maturation in the neuroectodermal component. The patient presented with a short history of nasal obstruction, epistaxis and headache. On imaging, a bone destroying lesion of left paranasal sinuses and nasal cavity was identified. The diagnosis of SNTCS could be offered only on the third biopsy which showed heterogeneous admixture of primitive neuroectodermal, epithelial and mesenchymal elements. An adequate sampling with high index of suspicion is needed to catch hold this rare tumor. Tumor was excised after 4 cycles of neo-adjuvant chemotherapy. On microscopic examination, it showed similar epithelial and mesenchymal components as the pretreatment biopsies. However, the primitive neuroectodermal component displayed extensive neuronal maturation. The undifferentiated neuroectodermal cells were completely absent in the post chemotherapy specimen. This case throws light on the morphologic evidence of chemotherapy induced maturation in the neuroectodermal component within SNTCS, an event hitherto not reported in the literature in case of SNTCS.
鼻窦畸胎癌肉瘤（SNTCS）是一种罕见的高度恶性肿瘤，具有畸胎瘤和癌肉瘤的综合特征。我们报告了首例23岁男性SNTCS患者，先接受新辅助化疗，随后进行颅面切除术。切除标本的神经外胚层成分显示出细胞成熟。该患者有鼻塞、鼻出血和头痛的短暂病史。影像学检查发现左侧鼻窦和鼻腔有骨质破坏病变。仅在第三次活检时才确诊为SNTCS，活检显示原始神经外胚层、上皮和间充质成分混合不均。需要进行足够的采样并保持高度怀疑指数才能确诊这种罕见肿瘤。新辅助化疗4个周期后切除肿瘤。显微镜检查显示，其上皮和间充质成分与术前活检相似。然而，原始神经外胚层成分显示出广泛的神经元成熟。化疗后标本中未分化的神经外胚层细胞完全消失。该病例揭示了SNTCS中神经外胚层成分化疗诱导成熟的形态学证据，这一事件在SNTCS的文献中迄今尚未报道。
