Sinonasal teratocarcinosarcoma treated with surgery and proton beam therapy: clinical, histological aspects and differential diagnosis of a new case

Abstract

Summary Sinonasal teratocarcinosarcoma is a rare aggressive malignant tumor with a primary setting involving the nasal cavity followed by the ethmoid sinus and maxillary sinus. It accounts for approximately 3% of all head and neck cancers and less than 1% of all tumors. Nasal obstruction, recurrent epistaxis and headache represent the typical clinical presentation. Imaging shows the presence of a mass in the nasal cavity. The treatment usually consists of surgery and adjuvant intensity modulated radiotherapy. The rarity and the variability of the histological features make its diagnosis particularly difficult. In this paper, we report a case of sinonasal teratocarcinosarcoma in a 62-year-old male treated with a multidisciplinary approach. As an alternative to intensity modulated radiotherapy, we proposed proton beam therapy for the first time. The patient benefited from the new and personalized protocol that provided excellent results and few adverse effects. At 45 months follow-up there is no evidence of relapse and the patient is in good health.

Sinonasal Teratocarcinosarcoma: A Rare and Highly Aggressive Neoplasm

“…In this case, the patient was referred by an otolaryngologist after inadequate tissue sample biopsy and a wrongful initial diagnosis. They have histologic characteristics of malignant teratomas as well as carcinosarcomas with no germ cell parts and a growth pattern that includes epithelial, mesenchymal, and primitive neuroectodermal components with neural rosettes [1][2][3]10]. The epithelial components are highly variable that can include both benign and malignant squamous and glandular components which include columnar and cuboidal cells with or without cilia as shown in Figures 4, 5.…”

“…In about 20% of cases, intracranial extension is also found [2]. SNTCSs comprise about 3% of all head and neck tumors and about less than 1% of all tumors [3,4]. TCS was termed "teratoid carcinosarcoma", "teratoma", "tetracarcinosarcoma", or "malignant teratomas" in the past, and then in 2005, the World Health Organization approved the proper term for it as SNTCS [5].…”

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

“…8,45 Alternatively, proton beam therapy can provide satisfactory results in some patients. 46 Neoadjuvant chemotherapy with a "cisplatin and etoposide protocol" may be considered in patients with unresectable TCSs. 44 In patients with p.S45F β-catenin mutations, targeted inhibitors of β-catenin may be applied following adequate future trials of these medications.…”