xRead - Nasal Obstruction (September 2024) Full Articles

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ICAR SINONASAL TUMORS

behavior of the primary tumor, the extent of metastases (oligometastatic or extensively disseminated disease), and the availability of effective systemic therapy. The prognosis in the majority of cases is poor. Improving patient QOL is usually the goal of treatment with pain relief, bleeding pre vention, and alleviation of other symptoms. Most patients should be treated with palliative intention. 1910,1912,1921 Historically, management of sinonasal metastatic dis ease focused on palliative RT, and the role of surgery was typically limited to biopsy and tumor debulking. Given the rarity of sinonasal metastatic disease, most reported infor mation is limited to case reports and case series. RT has been reported to be effective in symptom control and, occa sionally, improved PFS even for tumors like RCC, which tend to be radioresistant. 1922,1923 While there is an unclear relationship between the response rate and the radiation dose for sinonasal metastasis of any origin, various radio therapy regimens were reported, with no apparent benefit to specific regimen. 1912,1922–1924 A recent clinical trial sug gested that patients with a limited number of metastases of diverse primary tumor origins and nonsinonasal metas tases sites could be cured if all lesions are eradicated when treated with stereotactic ablative RT. 1925 This con cept might eventually be extrapolated to the sinonasal region but requires further investigation. In addition, RT may be given as an urgent treatment to alleviate pain, reduce bleeding, and shrink the lesion. 1922,1926,1927 In recent years, with the evolution of EEA, a paradigm shift toward including the option for surgical treatment, while maintaining the oncologic principles of exposure, adequate margins, and reconstruction, may be suitable for selected patients with metastatic disease. 1910,1912 EEAhas been reported to provide symptom relief and improved QOL while minimizing morbidity and might be considered for select patients. 1928,1929 For instance, surgical resection for a synchronous oligometastatic focus of disease may prolong survival. 1923,1930,1931 For disseminated metastatic disease, surgical resection may provide symptomatic pal liation. Depending on the primary tumor, concomitant systemic chemotherapy, hormonal therapy, immunother apy, or radioactive iodine ablation may be appropriate adjuvant treatment. 1910,1912,1922,1930 Given the relative rarity of sinonasal metastatic disease, each case should be care fully assessed in a multidisciplinary fashion to determine the best mode of treatment. OTHER MALIGNANT LESIONS A variety of other malignant neoplasms may originate within the nasal cavity or paranasal sinuses, includ ing malignant germ cell tumors, carcinosarcoma, and teratocarcinosarcoma. 1932–1937 These are extremely rare XXVIII

tumors and can be confused with other pathologic enti ties before detailed histologic analysis has been completed. Presenting signs and symptoms are often nonspecific, mostly encompassing symptoms of nasal obstruction and recurrent epistaxis, among other symptoms such as facial pain and pressure. 1932,1938 As with other sinonasal neo plasms, both CT and MRI work in concordance, delineat ing involvement of surrounding neurovascular and orbital structures. PET/CT imaging may provide some added ben efit to rule out the possibility of metastatic disease to the nasal cavity and paranasal sinuses, as opposed to primary tumor originating within the sinonasal tract. 1933,1934 Inthis particular group of tumors, demographics and immuno histochemical stains may provide value toward diagnosis and optimal treatment selection. A Germ cell tumors Malignant germ cell tumors are most often found within the testes and ovaries, as implied by the germ cell ori gin. Extragonadal germ cell tumors may occur in up to 20% of cases and are often found within midline structures along the axial skeleton including the retroperitoneum, the anterior mediastinum, and the suprasellar space, to name but a few. 1935–1938 The head and neck subsite as a whole only accounts for roughly 1% of all extrago nadal malignant germ cell tumors; even less common are primary sinonasal germ cell tumors. 1939 Germ cell tumors are classically divided into seminomatous germ cell tumors (SGCTs) and nonseminomatous germ cell tumors (NSGCTs), with the vast majority of extragonadal tumors being the nonseminomatous type. 1940 SGCTs encompass seminomas, which may also be referred to as germinomas or dysgerminomas. It is quite unusual to find these outside of the gonads as noted by very few case reports in the literature. 1941,1942 When seminoma has been reported within the sinonasal cavity, it has been in the setting of distant metastasis from a primary gonadal site. Extrapolating from primary gonadal seminomatous tumors, however, they are extremely sensitive to RT and/or chemotherapy and should be treated accordingly. 1942 NSGCTs are far more common than seminomas out side of the gonads and may be found within the sinonasal cavity with no clear primary gonadal source. The most common type of malignant germ cell tumor encountered within the sinonasal cavity is the yolk sac tumor (YST), also commonly referred to as endodermal sinus tumor. 1943,1944 Often found in younger patients and in females, these tumors can be pure or mixed, with most of them being the mixed type. 1945–1947 Mixed YSTs are often found in con junction with other germ cell tumor components, such as embryonal cell carcinoma or teratoma. A few diagnostic