Sinonasal Teratocarcinosarcoma

Abstract

The goal of this study was to assess the pathological and differential diagnoses of sinonasal teratocarcinosarcoma (SNTCS) in order to ultimately improve the diagnosis and treatment of this rare disease. Data from 2 cases of sinonasal teratocarcinosarcoma from the Wuxi People's Hospital (China) were analyzed. The clinical presentation for these patients consisted of nasal obstruction, epistaxis, and headache. On further physical examination, the presence of a polypoid mass was identified and, despite surgery and radiotherapy, both cases experienced recurrence. Histologically, the tumors showed a heterogeneous mixture of components from the 3 germ layers, primitive neuroepithelial elements, diagnostic immature squamous cell nests (clear cell nests), and various epithelial and mesenchymal components. Staining of the different germ layers corresponded with the appropriate immune markers. In case 1, the postradiotherapy resection specimen was completely dominated by a mature teratoma, with a complete absence of the corresponding adenocarcinoma and fibrosarcoma components. To date, this is the first study describing this composition within an SNTCS recurrent tumor. In summary, SNTCS is a rare tumor characterized by the presence of benign and malignant epithelial, mesenchymal, and dysembryomal components. Owing to its heterogeneous histologic appearance, adequate sampling and recognition of all SNTCS components are needed for future diagnosis. Currently, surgical removal, postoperative radiotherapy, and a histology-specific multidrug chemotherapy appear to be the best therapeutic approach. Future individualized therapy may also hold promise.

Sinonasal teratocarcinosarcoma: a case report and literature review

“…6,7 The incidence is relatively low, with fewer than 150 cases reported worldwide and only ∼10 cases in China. We reviewed the literature from 1968 to 2020, and 107 SNTCS cases were identified during this period (Table 1) 5,8–22 .…”

“…The nasal cavity and sinuses are the most commonly involved sites, followed by the nasopharynx, pharyngeal sidewall, and anterior skull base. 8 Nasal endoscopy detects most nasal lesions, and endoscopic examination of an SNTCS typically shows a solid tumor that is grayish-white to dark red in color, irregularly shaped, brittle, bleeds easily, and exhibits localized surface necrosis. 24 SNTCS usually lacks specific CT and MRI features; the images are similar to those of common malignant sinus tumors.…”

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors

“…As such, many authors believe these tumors are not of germ cell origin but instead originate from totipotent cells in the olfactory fossa, although several other postulated theories exist regarding underlying histogenesis. Treatment is with multimodality therapy, most often reported through case reports and case series 1434,2014–2051 . However, despite the rarity of this tumor there have been two systematic reviews published, the most recent of which was published in January 2021 by Chapurin et al 2052 .…”

Teratocarcinosarcoma of the head and neck: Clinicopathologic review of a rare entity

“…The latter is characterized by a biphasic appearance with a variable combination of malignant epithelial and sarcomatous elements, in contrast to the triphasic appearance of TCS. 4,6,18,36 On the other hand, metastatic melanoma of the head and neck rarely shows TCS-like dedifferentiation with complete loss of melanocytic markers. Recognition of this phenomenon is important to keep in mind since the management plan for malignant melanoma is totally different from the management plan for TCS.…”