xRead - Nasal Obstruction (September 2024) Full Articles

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resection when feasible, followed by adjuvant RT. 1979–1981 Local recurrence remains considerably high, with frequent reports of distant metastatic disease. 1982–1985

features seem to be fairly consistent with diagnosis of YST, including the classic histologic appearance of Schiller– Duval bodies. 1706,1948–1951 These are described as sheets of cuboidal cells with variable atypia that can be seen in sin gle lines surrounding blood vessels. IHC may also elucidate some key features, including identification of alpha feto protein (AFP) and SALL-4 expression. 1952,1953 SerumAFP is often elevated, which can become particularly impor tant as a serum biomarker for cancer surveillance. 1954 Given the rarity of these tumors, treatment modalities are widely variable. They often include multimodal treat ment, including surgery, RT, and chemotherapy. Many of these are quite sensitive to chemotherapy and a variety of agents have been previously used. 1955–1958 This includes chemotherapeutics such as bleomycin, etoposide, cis platin, carboplatin, ifosfamide, vincristine, 5-fluorouracil, and others. 1706,1943–1961 Although tumors may be respon sive to multiple forms of therapy, recurrence is commonly local, and early metastases may be present in up to 50% of cases. 1961 Choriocarcinoma represents another form of NSGCT that may be found within the nasal cavity or paranasal sinuses. It can occur primarily within the sinonasal cav ity or be metastatic from a primary gonadal site. 1962,1963 These may resemble many other tumors radiographically and histologically, but unique to these NSGCTs is the staining pattern and elevated serum levels of beta human chorionic gonadotropin (ß-hCG). 1946,1964 Again, this is a particular tumor marker that can help with diagnosis but can also aid in prognostication and cancer surveil lance. These are extremely rare as primary tumors in the sinonasal cavity, but when encountered they have been treated with maximal surgical resection followed by systemic therapy. 1964–1967 This often mimics treatment of primary gonadal choriocarcinoma, starting with cisplatin, etoposide, and ifosfamide (often referred to as VIP). 1962–1968 B Carcinosarcoma Carcinosarcoma represents another rare malignancy that may occur in the sinonasal cavity. 1935,1969–1974 It is consid ered a biphasic subtype of sarcomatoid carcinoma, with biphasic growth patterns of both epithelial and mesenchy mal components (rather than a collision tumor). 1975–1977 Demographically, there seems to be a slight preponder ance in males and elderly patients. 1977 It is more often encountered elsewhere in the head and neck, including the laryngopharynx and salivary glands. Commonly affected sinonasal subsites include the nasal cavity, followed by the maxillary sinus. 1975–1978 Again, as these tumors are exceed ingly rare, there is no standard for definitive management. Treatment is often multimodal, including radical surgical

C Teratocarcinosarcoma Initially referred to as nasal blastoma, teratocarcinosar coma is now an increasingly recognized malignant sinonasal neoplasm that encompasses features of epithe lial, mesenchymal, and malignant teratoma. 1986–1993 It is often encountered in the nasal cavity and ethmoid sinus, with 20% of tumors demonstrating intracranial exten sion at initial presentation. 1994–2001 A triphasic growth pattern is noted, including the benign and malignant epithelial and mesenchymal components along with primitive neuroectodermal (teratoid) elements. 2002–2008 Microscopically, these tumors often demonstrate “fetal appearing” squamous epithelium and clear cytoplasm with large nucleoli, arranged in a teratoid or organoid appearance. 2009–2014 Despite the implication of the pre cursor “terato,” no germ cell features are encountered in this tumor histologically. 2013,2014 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 multimodal ity 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 A total of 127 patients were identi fied after review, with outcome data only available for 58 patients. The median age was 50 years and there is a strong male predilection (83%). The vast majority of patients were treated with surgery plus adjuvant RT or CRT. With a mean follow-up of 21 months, the recurrence rate was 38% and 2-year OS was 55%. Kaplan–Meier analysis shows an OS advantage for patients who are treated with multimodal therapy, but no differences in disease recurrence. These findings are similar to a prior systematic review published in2014. 2053 To conclude, other rare malignant neoplasms can be encountered within the nasal cavity or paranasal sinuses. They often lack distinguishing clinical or radiographic fea tures compared to more common SNM. Demographics may lend some clues, but ultimately the final diagnosis is predicated on tissue biopsy. IHC and serum markers, such as ß-hCG and AFP, can be important not only for diagno sis but also for prognosis and tumor surveillance. There is a paucity of data, and many treatment options are extrapo lated from case reports, case series, and systematic reviews when available. Accordingly, these patients warrant dis-