Teratocarcinosarcoma

ICD-O:8980/3

The term "teratocarcinosarcoma" is designated to an unusual entity in which a malignant sinonasal tera-tomatous tumour also shows features of carcinosarcoma

[112]. Patients suffering from this malignancy are exclusively adults (age range 18-79 years; mean 60 years) [64, 75, 112, 156, 195, 227]. There is a male predominance and symptoms are non-specific with nasal obstruction and epistaxis produced by a nasal cavity mass (Fig. 2.19a) [112].

Histologically, sinonasal teratocarcinosarcoma (SNTCS) comprises a multiplicity of cell types of varying maturity (Fig. 2.19b). The epithelial component includes keratinising squamous epithelium, pseu-dostratified columnar ciliated epithelium and glandular structures lined by cuboidal and columnar cells including mucus cells. Masses of immature epithelial cells, some containing glycogen or mucin, are frequently found. "Foetal-appearing" clear cell squamous epithelium is a common finding and a very important diagnostic clue for some authors [112]. Neuroepitheli-al elements with rosettes and neuroblastoma-like areas are in most instances present. These epithelial and neuroepithelial elements occur in close relationship with each other and with mesenchymal elements, the most prominent of which are immature cells with oval or elongated nuclei. The mesenchymal cells may exhibit skeletal muscle differentiation with cross striations (Fig. 2.19c). Foci of cartilage, smooth muscle, adipose tissue and fibrovascular tissues may also be present. In spite of the common occurrence of areas having a variety of mature tissues, mitotic activity and cytological features of malignancy are demonstrable in the undifferentiated areas of both the epithelial and mesenchymal elements [75].

In order to achieve the correct diagnosis of SNTCS a thorough sampling of the specimen is required. Inadequate sampling may lead to mistaken diagnoses of esthesioneuroblastoma, squamous cell carcinoma, un-differentiated carcinoma, adenocarcinoma, malignant craniopharyngioma, malignant mixed tumour of salivary gland type, mucoepidermoid carcinoma, adeno-

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Fig. 2.19. Teratocarcinosarcoma. a CT scan showing massive de- immature blastematous tissue. c Immature glands surrounded by structive infiltration of the nasal cavity and maxillary sinus on the blastematous elements at the lower left; presence of immature stri-

left. With permission [75] b Cystic spaces filled with mucin are ated muscle, upper right and centre partly covered by benign columnar epithelium and surrounded by squamous carcinoma and others [105]. In contradistinction to malignant gonadal teratomas, which are frequently found in patients at a younger age, sinonasal teratocarcinosarcoma does not contain areas of embryonal carcinoma, choriocarcinoma or germinoma (sem-inoma), as seen in many germ cell tumours. This makes a germ cell origin of SNTCS unlikely [112]. The histogenesis of sinonasal teratocarcinosarcoma is debatable. The presence of neural tissue in these neoplasms raises the possibility that the origin could be in some way related to the olfactory membrane, or alternatively to the sinonasal membrane as a whole, since the olfactory membrane also develops from it [112].

Sinonasal teratocarcinosarcomas are locally aggressive tumours, with rapid invasion of soft tissues and bone, and metastasise to regional lymph nodes and sites, such as the lung. The average survival of a patient with SNTCS is 1.7 years, with 60% of the patients not surviving beyond 3 years. The treatment of SNTCS is controversial, but an aggressive initial therapeutical approach with a combination of surgical resection, radiotherapy and chemotherapy is usually recommended [112].

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