xRead - Nasal Obstruction (September 2024) Full Articles

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The addition of RT for incomplete resection was associ ated with decreased recurrence rate and trended toward an increased time to recurrence; however, in patients receiv ing RT following complete surgical resection, there was no improvement in recurrence rate compared to those with complete surgical resection alone. 1008 Analysis of the SEER database showed that surgery plus chemotherapy improved survival to a greater extent than chemother apy alone, although the outcomes of surgery alone were more favorable. 1014 Skull base origin conferred worse prognosis as compared to sinonasal primary site. 1015 Mul tiple different chemotherapeutic agents have been used with overall poor response rates, regardless of whether they were first-, second-, or third-line treatments. 1016,1017 The only two surviving cases in a series of 21 locally advanced and unresectable HPCs had undergone neoad juvant chemotherapy. 1016 Alternative agents that inhibit VEGF or tyrosine kinase have shown more favorable responses or partial responses ( n = 14). 1018–1020 Reported recurrence rate of sinonasal HPC is 20.3%–29.9% with an average mean follow-up of 47 months. 1007,1008 Incomplete resection led to recur rence or tumor-related death in 71% (15/21) undergoing an endoscopic approach and 100% (3/3) undergoing an open approach. 1008 The rate of metastasis has been reported at 1.9%, with no increased risk from high-grade or large tumors. 1008 The majority of recurrences happen within 5 years, though late recurrence up to 210 months has been described and thus long-term follow-up is indicated. 1007 F Glomangiopericytoma Glomangiopericytoma (GPC), also known as sinonasal type HPC, is a rare mesenchymal neoplasm with low malignant potential. Symptoms are typically nonspecific, with epistaxis being the most common, followed by nasal obstruction and headache. 1021 GPC has a slight female predominance, an average age of presentation of 58 years (ranging from 3 months to 87 years), and an average duration of symptoms prior to presentation of 19months. 1022,1023 Similar to HPC, GPC may present with associated osteomalacia. 1022 Although there are no clear risk factors, increased vascularity from trauma, pregnancy, hypertension, or use of corticosteroids has been associated withGPC. 1024 GPC is in part characterized by CTNNB1 mutations and positive immunohistochemical staining for nuclear β -catenin. 1025 Lasota et al. demonstrated that onco genic CTNNB1 mutations activate β -catenin signaling and upregulate Wnt -signaling and cyclin D1 expression, which dysregulate the cell cycle leading to neoplastic transformation. 1026 This pathway is thought to play a cen

tral role in the pathogenesis of GPC. GPCs are typically well-defined round or lobulated soft-tissue masses, often with erosive bone remodeling commonly located in the ethmoid or sphenoid sinuses. 1027,1028 MRI characteristics may include avid enhancement, moderate to high T2 signal intensity with signal voids, and rapid wash-in and wash out on dynamic contrast-enhanced MRI and high ADC values indicating low malignant potential. 1027 T2 signal voids indicate high vascularity, and embolization may be considered prior to resection. 1029,1030 The term GPC was first used to describe a tumor that exhibits both the branching vessels of HPCs and rounded cells with pale or basophilic cytoplasm of glo mus tumors. 1031 It is a submucosal tumor with pericytic myoid differentiation. Macroscopically, it is beefy, red, soft, and hemorrhagic. 1032 Microscopically, GPC is a nonencap sulated tumor with spindle shaped cells in a storiform pattern, branching vessels in a staghorn configuration and perivascular hyalinization. 1023,1024,1032 Immunohistochem ical staining is commonly positive for smooth muscle actin, vimentin, and nuclear β -catenin while lacking expression of CD34, AE1/AE3, Bcl-2, CD99, CD117, Factor VIIIR Ag, S100, and the NAB2-STAT6 fusion complex seen in SFT. Surgical resection is the primary treatment for GPC. 1024,1028,1033 In one systematic review, clean sur gical margins resulted in 100% 5-year survival with no evidence of recurrence or metastasis (10/10). 1022 For large and highly vascularized tumors, preoperative emboliza tion has been shown to decrease tumor size, resection area, and intraoperative bleeding, increasing the likeli hood of total resection. RT and chemotherapy alone have a high rate of recurrence and insufficient data exist to support adjuvant RT, particularly in cases of complete resection. 1029,1034 The use of RT and chemotherapy was shown in one study to be significantly correlated to recur rence/metastasis ( p = 0.03). 1022 This association could be because RT and/or chemotherapy was used in cases of larger and more extensive tumors or ones not amendable to complete resection, but this was not discussed in detail. Other studies have shown that in the rare case of unresectable or metastatic disease, RT and chemotherapy can be useful as adjuvant or palliative treatment. 1028,1031 Overall prognosis is favorable with a 5-year survival rate of 88% and a recurrence rate of 17%, which change based on complete versus partial resection. 1021,1023 Recur rence has been described to occur up to 12 years later, but most recur within 5 years. 1035 Recurrence is associ ated with incomplete resection, bony invasion, bilateral involvement, adjuvant chemo/RT, severe nuclear pleomor phism, tumor > 5 cm, and high mitotic rate. 1022,1023 One systematic review found that actin immunonegativity and CD34 immunopositivity correlated with poor prognosis in patients with GPC. 1022 Metastasis is rare and typically pre-