A Wolf in the Jaw—“Pediatric Myofibroblastic Sarcoma Hanging from the Maxilla”: Low Grade, High Impact
Year: 2026
Type: article
Study design: Case Report
Abstract: with indentations and small nucleoli.Focal mild-to-moderate nuclear atypia and occasional mitotic activity was noted.On immunohistochemical expression, tumor cells were immunopositive for Vimentin, SMA (focal) while myogenin, desmin, S100, ALK, CD34, EMA, and Pan-TRK were negative, favoring the diagnosis of LGMS (Fig. 3).The patient was then administered three doses of ifosfamide and doxorubicin, again with no appreciable tumor regression.Given the lack of response to chemotherapy, the case was referred IntroductIonLow-grade myofibroblastic sarcoma (LGMS) is a rare malignant neoplasm of mesenchymal origin, showing a distinct predilection for the head and neck region, particularly the jaws and maxillofacial skeleton.1 Although primarily seen in adults, LGMS has been reported in children, where it may present as a painless, slowly progressive swelling, often mimicking benign lesions and complicating early diagnosis.2 Histopathological overlap with other spindle cell tumors, such as inflammatory myofibroblastic tumors (IMTs) and synovial sarcoma, further adds to diagnostic difficulty.Despite its low-grade designation, LGMS has a significant tendency for local recurrence and delayed metastasis.Cases involving the maxillary sinus and buccal mucosa are extremely rare, with fewer than ten reported worldwide.3 case descrIptIon A 7-year-old male child presented to the Pediatrics Outpatient Department (OPD), Postgraduate Institute of Medical Education and Research, with a rapidly enlarging, painless pinkish mass arising intraorally from the left upper jaw, with a duration of 2 months (Fig. 1).Contrast-enhanced computed tomography (CECT) revealed a hypodense soft tissue mass arising from the left upper alveolus, with erosion of the maxilla (Fig. 2).Initial evaluation with fine-needle aspiration cytology (FNAC) suggested a low-grade sarcomatous lesion.A subsequent biopsy revealed a spindled cells tumor arranged in short fascicles and focal storiform pattern in a collagenous and myxoid stroma, along with numerous thin-walled capillaries.Tumor cells exhibited ill-defined pale eosinophilic cytoplasm with fusiform, elongated, or wavy nuclei, as well as occasional round vesicular nuclei 1-
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W7128307666