Germ cell tumours

A. Cardesa M.A. Luna

Malignant germ cell tumours and terato-carcinosarcoma exhibiting histologic features similar to germ cell tumours of the gonads arise on rare occasions in the sinonasal tract. Immature teratomas and teratomas with malignant transformation are tumours of infancy and early childhood, whereas sinonasal yolk sac tumour and sinonasal teratocarcinosarcoma have only been documented in adults.

Immature teratoma

ICD-O code 9080/3

Immature teratomas are rare in the sinonasal tract and nasopharynx, and are composed of variable quantities of immature tissue elements, mostly neu-roepithelial, that are interspersed with mature and immature tissues derived from the three embryonic germ layers. They are tumours of infancy and childhood {2317}.

Immature teratomas tend to be either solid-nodular or solid-cystic, while mature teratomas are usually cystic. The tumour may contain cystic spaces lined by ciliated pseudostratified epithelium as well as primitive neuroepithelium with rosettes. Mitotic figures are frequently present in the immature areas; however, cellular atypia is not found. In infants and children, a teratoma with malignant transformation has to be excluded. In adults, thorough sampling of the specimen is mandatory to rule out teratocarci-nosarcoma. Immature teratomas rarely behave in a malignant fashion {570}.

Teratoma with malignant transformation

ICD-O code 9084/3

Teratoma with malignant transformation is a neoplasm containing benign tissue elements of all three germinal layers and, in addition, a somatic malignancy. There is only a single reported case involving the sinonasal tract of a 13-month-old boy. The malignant component was a squamous cell carcinoma {1379}. The tumour was locally aggressive and recurred after surgery. There was no further recurrence 2 years after chemotherapy {1379}.

Sinonasal yolk sac tumour (endodermal sinus tumour)

ICD-O code 9071/3

This is a tumour that has the histological features of embryonic yolk sac, indistinguishable from yolk sac tumour (endodermal sinus tumour) of the gonads. Only two cases have been reported to arise in the sinonasal tract {1623}. Both patients were adults (aged 34 and 43 years). In one case, there was an admixed component of sinonasal nonkeratinizing carcinoma. The behaviour has been aggressive.

Sinonasal teratocarcinosarcoma

Definition

A complex malignant sinonasal neoplasm combining features of teratoma and carcinosarcoma. Benign and malignant epithelial, mesenchymal, and neural elements are typically present, including immature tissue with blastomatous features, while embryonal carcinoma, chori-ocarcinoma or seminoma is absent.

Synonyms

Malignant teratoma, blastoma, teratocar-cinoma, teratoid carcinosarcoma

Epidemiology

Sinonasal teratocarcinosarcoma is very rare {755}. Approximately 60 cases have been published {755,1042,1619,1970, 2339,2578,2749}. Patients are exclusively adults, with age ranging from 18-79 years (mean 60 years). There is a marked male predominance {1042}.

Localization

It almost exclusively arises in the ethmoid sinus and maxillary antrum. One tumour has been reported to arise in the roof of the nasopharynx and another from the dorsum of the tongue {1042}.

Clinical features

Patients present with a short history of nasal obstruction and epistaxis {1042}. Imaging studies reveal a nasal mass, occasionally accompanied by opacifica-tion of the paranasal sinuses. Bone destruction may be seen.

Macroscopy

Tumours are usually bulky, soft to rubbery, and red-tan to purple.

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