PMC8572639_01
Clinical and imaging features. (a) Extraoral examination showed exophytic and expansive lesion around orbital region. (b) MRI axial and sagittal slices demonstrated tumor invasiveness into the periorbital region, sinonasal tract, left maxilla, and orbit roof.
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A 20-year-old Afro-descendant man, complained of a painful, exophytic, and expansive lesion, with rapid growth in the left hemimaxilla region, affecting the orbital region, with ipsilateral ocular proptosis and partial loss of visual acuity (Figure 1(a)). The Magnetic Resonance Imaging (MRI) exam revealed an expansive and infiltrative lesion centered in the maxillary sinus/left osteomeatal complex, extending to the periorbital and orbital region with left proptosis and extra-axial intracranial invasion that measured 91 x 75 x 57 mm (Figure 1(b)).
Histological and IHC analysis of alveolar RMS. Small cells with scarce cytoplasm and rounded and hyperchromatic nucleus grouping into nests with pseudoalveolar spaces under optical microscopy at 20x (a) and 40x (b). Strong and diffuse nuclear marking (higher than 50%) of myogeny (c) and MyoD1 (d) in neoplastic cells that corroborated initial diagnosis.
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Histopathological analysis was performed by incisional biopsy of the lesion in the supraorbital region, in the submental lymph node, and in the nasal region (Figure 2), the results of which confirmed its undifferentiated mesenchymal origin.
Clinical examination after neoadjuvant QT. (a) Partial response to treatment was observed with increased tumor volume. (b) MRI in axial and sagittal slices confirmed. (c) CT for RT planning. (d) PTV contouring included submental lymph nodes. (e) CTV for tumor irradiation.
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Three initial cycles of QT+ were performed, based on ifosfamide (1800 mg) and etoposide (100 mg) that showed a partial response observed clinically (Figure 3(a)) and in the imaging examination (Figure 3(b)). Tomography (CT) was performed for initial RT planning (Figure 3(c)), and no metastasizing lesions were observed. The Intensity Modulated Radiotherapy (IMRT) technique was used to deliver a dose of 2 Gy/day of 50 Gy in the area of the prechemotherapy tumor and positive submental lymph node (Figure 3(d)); and 60 Gy was delivered in the area of the postchemotherapy tumor (Figure 3(e)).
Oral manifestations resulting from radiotherapy and therapeutic evolution of complications. Herpes simplex and Grade 2 mucositis lesions shown in region of the hard palate (a) and in the oropharynx and left lateral border of the tongue (b), respectively. Intraoral clinical appearance after local drug therapy and photobiomodulation (PBM), with regression of lesions in the hard palate (c), oropharynx, and left lateral border of the tongue (d).
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During RT, painful erythematous areas were observed in the hard and soft palate associated with herpes simplex lesions confirmed by exfoliative cytology, mucositis lesions on the lateral edge of the tongue (Figures 4(a) and 4(b)), and decreased salivary flow. Plus 5 ml for 15 days, 03 times a day; Nystatin (100,000 IU) 5 ml, 3 times daily, remaining in the mouth for 4 minutes every time it was used, for 07 days; and acyclovir 200 mg every 4 hours for 07 days. Photobiomodulation (laser therapy) was performed for improving the mucositis lesions, by using diode laser (Therapy EC, DMC, Sao Carlos, Sao Paulo, Brazil) with a beam output power of 100 mW, 4 J per point across the affected region (04 points per region), and a satisfactory clinical response was observed (Figures 4(c) and 4(d)).
Extraoral manifestations after RT. Dermatological changes induced by radiation (a) in the periorbital region (b) and local alopecia (b, c). (c) Clinical appearance after local use of ointments and moisturizing creams.
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In addition, areas of radiation-induced dermatitis were visualized in the left neck (Figure 5(a)) and in the ipsilateral periorbital region (Figure 5(b)); and alopecia (Figures 5(b) and 5(c)) was observed. The daily use of ointments and moisturizing creams was indicated for improvement of local condition (Figure 5(c)).
Clinical and imaging analyses after final RT session. (a) Reduced tumor volume was observed and by MRI in sagittal (b) and axial (c, d) slices that showed complete response to treatment with LCR obtained.
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At the final RT session, local tumor control was observed (Figure 6(a)). On MRI examination at 60 days after RT, decreased tumor volume was obtained (Figures 6(b)-6(d)); however, two months later, during a new cycle of adjuvant QT performed in January 2021 and based on vincristine (2 mg/IV), a metastasized lesion to the brain caused the death of the patient.