Abstract
Sinonasal teratocarcinosarcoma (SNTCS) is a rare and aggressive neoplasm characterized by the combination of malignant teratoma and carcinosarcoma. Sinonasal teratocarcinosarcoma usually is found mainly among adults, and the common original sites of SNTCS were nasal cavities and paranasal sinuses. There are fewer than 50 reported SNTCS cases in the literature. The most common cause of treatment failure is local recurrence. Distant metastasis of SNTCS is seldom described because of the invasive character of the cancer. We report on a 52-year-old man with SNTCS involving the left sinonasal cavity. Recurrent cervical metastasis developed in the 18th, 23rd, and 28th month after initial sinonasal surgery. Despite multiple aggressive neck surgeries and postoperative adjuvant radiotherapy, the patient died of lung metastasis 43 months after the initial surgery. Our patient was younger than other reported patients with SNTCS; besides, good locoregional control and lung metastasis have not been reported previously. An aggressive elective neck dissection should be performed in the early disease stage, and more attention should be given to the soft tissue surrounding any possible lymphadenopathy. This may decrease the risk of lower cervical lymph node or distant metastasis in patients with SNTCS.
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Introduction
Sinonasal teratocarcinosarcoma (SNTCS) is a rare and aggressive neoplasm characterized by the combination of malignant teratoma and carcinosarcoma. There are fewer than 50 reported SNTCS cases in the literature. The 3- and 5-year survival rates are about 30% and 20%, respectively . The most common cause of treatment failure is local recurrence . Distant metastasis of SNTCS is seldom described because of the invasive character of the cancer. We report a 52-year-old man with SNTCS involving the left sinonasal cavity. Recurrent cervical metastasis developed in the 18th, 23rd, and 28th month after initial sinonasal surgery. Despite multiple aggressive neck surgeries and postoperative adjuvant radiotherapy, the patient died of lung metastasis 43 months after the initial surgery.
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Case report
A 52-year-old man presented with progressive left nasal obstruction and intermittent nasal bleeding of 1 month’s duration. Physical examination showed a reddish-purple, fleshy, polypoid friable mass in the left sinonasal cavity ( Fig. 1 ). There was no palpable lymphadenopathy. Computed tomography of the head and neck revealed a soft tissue mass filling the left upper sinonasal area and partial cloudiness of left maxillary sinus ( Fig. 2 ). There was no obvious lymphadenopathy. Biopsy of the tumor revealed a malignant neoplasm consisting of both epithelial and mesenchymal components.
We resected the tumor via a lateral rhinotomy approach. The resected areas included the left middle and superior turbinates, left anterior and posterior ethmoid sinuses, medial wall of the left maxillary sinus and anterior wall of left sphenoid sinus. There was no obvious invasion of anterior skull base and lamina papyracea, and this is confirmed by the frozen section study. Thus, we did not perform a further craniofacial resection. Microscopically, beneath the respiratory epithelium were miscellaneous components, including squamous epithelium with clear cytoplasm, primitive mesenchymal cells within a myxoid background, gland-like structures lined by columnar epithelium, reticular arrangement of polygonal cells, aggregates of primitive neuroblastoma-like cells, and bone-like islands ( Fig. 3 ). Abundant mitosis and individual cell necrosis were noted in the areas of primitive cells. Immunohistochemical studies showed diffuse and strong positive staining of vimentin in the primitive mesenchymal and neuroblastoma-like cells. High- and low-molecular-weight cytokeratin was found in the columnar epithelial cells and polygonal cells arranged in reticular patterns. A few spindle cells were positive for S100 and synaptophysin. Even fewer cells stained weakly with glial fibrillary acidic protein and sarcomeric actin. The results indicated that the tumor had components of teratoma and carcinosarcoma. Thus, the diagnosis of SNTCS was made.
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