Chemotherapy-Induced Neuronal Maturation in Sinonasal Teratocarcinosarcoma—a Unique Observation

Abstract

Sinonasal teratocarcinosarcoma (SNTCS) is a rare and highly malignant tumour with combined features of a teratoma and carcinosarcoma. We report the first case of a SNTCS in 23 year old male treated with neo-adjuvant chemotherapy followed by cranio-facial resection. The resection specimen displayed cellular maturation in the neuroectodermal component. The patient presented with a short history of nasal obstruction, epistaxis and headache. On imaging, a bone destroying lesion of left paranasal sinuses and nasal cavity was identified. The diagnosis of SNTCS could be offered only on the third biopsy which showed heterogeneous admixture of primitive neuroectodermal, epithelial and mesenchymal elements. An adequate sampling with high index of suspicion is needed to catch hold this rare tumor. Tumor was excised after 4 cycles of neo-adjuvant chemotherapy. On microscopic examination, it showed similar epithelial and mesenchymal components as the pretreatment biopsies. However, the primitive neuroectodermal component displayed extensive neuronal maturation. The undifferentiated neuroectodermal cells were completely absent in the post chemotherapy specimen. This case throws light on the morphologic evidence of chemotherapy induced maturation in the neuroectodermal component within SNTCS, an event hitherto not reported in the literature in case of SNTCS.

A Case of a Beta-Human Chorionic Gonadotropin Secreting Sinonasal Teratocarcinosarcoma

“…Malignant sarcomatous transformation is also documented. 1,[5][6][7] In this case, smooth muscle actin highlighted an anaplastic smooth muscle component in this heterogeneous tumor. There are rare reports of pure leiomyosarcomas secreting β-hCG in the literature.…”

Sinonasal teratocarcinosarcoma: is minimally invasive resection followed by adjuvant histology directed chemo-radiation a better alternative to radical excision? Case report

“…Other symptoms raising suspicions of malignancy, such as dysphagia, odynophagia, epiphora, vision loss, exophthalmos, anosmia, headache and altered sensorium appears when the tumour spreads into the the orbit and intracranially. 3 Our patient presented with complaints of progressive nasal obstruction, anosmia, intermittent epistaxis and fullness of right cheek. The progressive nature of symptoms in our patient may be attributed to the aggressive rapidly growing behaviour of SNTCS.…”

Sinonasal Teratocarcinosarcoma- A Rare Tumour not so Rarely Misdiagnosed

“…The average survival as reported by Heffner and Hyams is 1.7 years (2). Analysis of the available literature on STCS showed a strong male predominance with an average age of presentation at about 51.75 years (7,8).…”