Abstract
Purpose
Sinonasal teratocarcinosarcoma (SNTCS) is a rare and highly malignant neoplasm that often involves the anterior skull base. This study reviews the published literature related to SNTCS. Clinical presentation, demographics, radiographic diagnosis, pathology, treatment, and management outcomes of this uncommon disease are reported.
Methods
A systematic review in the published English literature was conducted. A MEDLINE/PubMed search and bibliographic examination of articles pertaining to SNTCS were performed. Each case was analyzed for patient demographics, clinical presentation, tumor location, diagnosis, treatment, and survival outcome.
Results
A total of 49 journal articles were included. Individual patient data were reported in 86 cases. The average age of the patients was 54.5 years (range, 0.1 to 85 years), with a strong male predilection (7:1). Average follow-up was found to be 38.9 months (range, 2 to 372 months). The most common treatment method was surgery with radiation therapy, utilized in 59.3% of patients. Out of 71 cases with reported outcome and follow-up, there were 21 cases of recurrence, 8 cases with metastasis, and 6 cases reporting both recurrence and metastasis. Forty-two out of 71 (59.2%) patients survived at the time of follow-up.
Conclusions
This study describes the largest pool of SNTCS patients to date. SNTCS is a rare and aggressive malignant skull base tumor with a poor prognosis with frequent recurrence and metastasis. Although a variety of treatment paradigms have been reported in the literature, radical surgical resection followed by radiation therapy appears to be the most commonly used treatment option.
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Introduction
Sinonasal teratocarcinosarcoma (SNTCS) is a rare, highly aggressive neoplasm that usually arises in the paranasal sinuses and has a predilection for males. The tumor can also extend intracranially, further complicating the treatment plan for this disease. The diagnosis is based on the presence of malignant epithelial elements and two or more malignant mesenchymal components, such as fibroblasts, cartilage, bone, and smooth muscle. An important aspect for recognizing a teratocarcinosarcoma is the presence of “fetal-appearing” clear-cell squamous epithelium and organoid structures such as tubular or glandular formations . Germ cell components are absent in teratocarcinosarcoma .
The term SNTCS was initially described by Heffner and Hyams in 1984 in their analysis of 20 cases, however its histological description had been previously published . Diagnosis can be challenging because of its infrequency and phenotypic diversity. Histological studies have demonstrated a variety of tissues including epithelial, mesenchymal, and neural elements as well as teratoid elements, suggested by the distinct squamous epithelium .
Treatment of this malignant neoplasm presents a challenge due to aggressive and rapid growth along with the likelihood of recurrence and potential intracranial extension. The most commonly recommended treatment is radical surgical resection followed by radiation therapy. Reports of adjuvant chemotherapy are limited and therefore its role is not well defined. Because of the rarity of this tumor, the clinical characteristics and optimal treatment strategy are not well characterized. In this study, we performed a systematic analysis of SNTCS cases reported in the literature, and evaluated the clinical presentation, treatment modality, and outcomes.
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