xRead - Nasal Obstruction (September 2024) Full Articles

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KUANetal.

1 Presentation SPs are rare and represent less than 5% of all blood cell dyscrasias. 1859 EMPs make up about one third of these disorders, 1860 with an incidence of 0.04–3 per 100,000. 1861 EMPs can occur anywhere in the body, but most frequently arise in the head and neck region, accounting for approx imately 1% of all head and neck tumors. 1861 They have a predilection for the nasal cavity, paranasal sinuses, and nasopharynx. 1862 Other sites of involvement include the oropharynx, salivary glands, and larynx. SPs of the skull base are most commonly found at the sphenoclival region; other common skull base sites include the petrous apex and orbital roof. 1863 EMPs most frequently affect middle-aged patients, and the diagnosis is usually made in the fifth decade of life. 1861 Patients typically present between 30 and 60 years of age, although EMP has been diagnosed in children as young as 5. Males are two to three times more likely to be affected. 1861 EMPs have been reported in association with EBV and HIV infection, although a causal relationship has not been definitively established. 1864,1865 EMPs have been found to have a lower rate of con version to MM (10%–30%), 1866 as compared to SBPs, which have a progression rate reported between 48% and 85%. 1861 Conversion to MM appears to be most likely within the first 2 years of diagnosis, but it has been described up to 15 years later. 1867 Once MM develops, the 10-year OS has been reported to be under 10%. 1861 That said, there are observations that the prognosis for those who develop MM from an initial EMP could be more favorable than those who directly developed MM. 1867 The clinical presentation of sinonasal EMPs varies with the anatomical location of the tumor. 1861 Nasal obstruction and epistaxis are the most frequently reported symptoms. Patients with maxillary sinus involvement may present with facial swelling or pain, while those with tumor exten sion to the orbit may have diplopia, visual changes, or proptosis. Headache can also be a feature but is less common and nonspecific. Patients with skull base EMPs may present with cranial nerve palsies resulting in pto sis, diplopia, hearing loss, or imbalance. In many of these patients, the diagnosis may only be made in retrospect after initial surgical resection. 1863 On nasal endoscopy, the tumors present as a fleshy, hyperemic, or hemorrhagic 1868 mass with contact bleeding. 1869 There may be associ ated superficial ulceration 1870 and necrosis. Larger lesions may cause a visible mass of the maxilla, palate, or orbit. 1871

Cost

There is significant cost to the intervention including institutional costs for equipment and patient time and morbidity from treatment. Preponderance of benefits over harms. Patients who have received previous head and neck radiation deserve careful consideration of the morbidity of reirradiation given increased side effects.

Benefits–harm assessment

Value

judgments

Policy level Recommendation. Intervention RT should be offered to all patients

undergoing treatment for ENKTL for LRC benefits.

G Extramedullary plasmacytoma Extramedullary plasmacytomas (EMPs) are rare malig nancies composed of monoclonal plasma cells. EMPs are a subset of solitary plasmacytomas (SPs) and lie on the disease spectrum of plasma cell dyscrasias. EMPs are positioned between monoclonal gammopathy of undeter mined significance (MGUS), which is premalignant and often clinically silent, and multiple myeloma (MM). 1857 SPs are characterized by their isolated, localized nature, com pared to MM, which is disseminated. SPs can be further subdivided into EMPs, which arise from extraosseous soft tissue, and solitary plasmacytoma of bone (SPB), which has a bony origin. The chief clinical impact of EMPs and SBPs, other than symptoms arising from the mass, is the risk posed by their possible progression to MM. In 2014, the International Myeloma Working Group (IMWG) has proposed diagnostic criteria to separate these clinicopathological entities in light of differences in treatment options and prognostic implications. It rec ommended that SPs be diagnosed on the basis of (1) biopsy-proven solitary bone (SPB) or soft tissue (EMP) lesion showing clonal plasma cells, (2) normal bone mar row studies, (3) normal skeletal survey and MRI (or CT) of the spine and pelvis, and (4) an absence of end-organ dam age such as hypercalcemia, renal insufficiency, anemia, or bone lesions that can be attributed to a lymphoplasmacytic cell proliferative disorder. 1858 If marrow studies demonstrate infiltration of clonal plasma cells, but these remain < 10% of all nucleated cells, these lesions are classified as “solitary plasmacytoma with minimal marrow involvement” and carry a poorer progno sis than EMPs with no marrow involvement. The presence of over 10% of clonal plasma cells in marrow studies raises the suspicion of MM or smoldering MM.