Wei Shi, Carroll William, Lazenby Audrey, Bell Walter, Lopez Robert, Said-Al-Naief Nasser
Department of Pathology, University of Alabama at Birmingham, Birmingham, AL 35242, USA.
Ann Diagn Pathol. 2008 Dec;12(6):415-25. doi: 10.1016/j.anndiagpath.2007.05.003. Epub 2007 Oct 24.
Sinonasal teratocarcinosarcoma is a highly malignant, polymorphous neoplasm that combines features of carcinosarcoma and teratoma. We describe the clinicopathologic features and management of a well-documented example of this unique entity that involved a 41-year-old Hispanic man. The patient presented with a history of multiple episodes of epistaxis, nasal obstruction and frontal headaches. Computerized tomography scans and magnetic resonance imaging revealed a large mass filling the left nasal cavity and extending to the cribriform plate with involvement of the ethmoid sinuses, lamina papyracea, and orbit. The patient underwent a complex procedure for a T3N0 tumor. Histologic examination revealed a heterogeneous admixture of epithelial, mesenchymal, and neuroepithelial elements. The mesenchymal components consist of fibrous stroma and myxomatous areas, labeled with calponin and smooth muscle actin. The epithelial components vary from clear cells, nonkeratinizing epithelium to glandular pattern, and keratin containing cysts. Immature neuroepithelium and olfactory neuroblastomalike tissue are highlighted with neuroendocrine markers. Postoperatively, the patient had a rapid local recurrence of the tumor and underwent reexcision, and was treated with radiotherapy and chemotherapy. Twelve months after his primary resection, computerized tomography scans revealed an intrathoracic tumor with dominant mass in the left hilum and metastases to the mediastinum, left pleural space, and both lungs. The histologic nature of his chest mass remains undetermined. Among 54 cases of reported sinonasal teratocarcinosarcoma, 67% of patients with initial single surgical resection and 80% of patients primarily treated with radiotherapy had recurrence, or metastatsis, or unresponsiveness to treatment. The high rate of local recurrence and metastasis is indicative of its highly aggressive biologic behavior. Almost half of the patients died of tumor within 3 years of diagnosis, despite aggressive therapy. Seventy percent of the patients who survived more than 1 year had the initial therapeutic regiments of combined surgery and adjuvant therapies, suggesting that aggressive therapeutic approaches may improve the treatment outcome.
鼻窦畸胎癌肉瘤是一种高度恶性的多形性肿瘤，兼具癌肉瘤和畸胎瘤的特征。我们描述了一个记录详尽的该独特实体病例的临床病理特征及治疗情况，该病例涉及一名41岁的西班牙裔男性。患者有多次鼻出血、鼻塞和前额头痛病史。计算机断层扫描和磁共振成像显示一个大肿块充满左侧鼻腔并延伸至筛板，累及筛窦、纸样板和眼眶。该患者因T3N0肿瘤接受了复杂手术。组织学检查显示上皮、间充质和神经上皮成分的异质混合。间充质成分由纤维性基质和黏液样区域组成，用钙调蛋白和平滑肌肌动蛋白标记。上皮成分从透明细胞、非角化上皮到腺管样结构以及含角蛋白的囊肿不等。未成熟神经上皮和嗅神经母细胞瘤样组织用神经内分泌标记物突出显示。术后，患者肿瘤局部迅速复发并接受了再次切除，随后接受放疗和化疗。初次切除12个月后，计算机断层扫描显示胸腔内有肿瘤，以左肺门为主肿块并转移至纵隔、左侧胸腔和双肺。其胸部肿块的组织学性质仍未确定。在54例报告的鼻窦畸胎癌肉瘤病例中，67%初次接受单一手术切除的患者以及80%主要接受放疗的患者出现复发、转移或对治疗无反应。局部复发和转移的高发生率表明其具有高度侵袭性的生物学行为。尽管进行了积极治疗，近一半患者在诊断后3年内死于肿瘤。存活超过1年的患者中有70%最初接受了手术联合辅助治疗方案，这表明积极的治疗方法可能改善治疗结果。
