Low-grade fibromyxoid sarcoma, also known as Evans tumor, is a low-grade sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site, with only a few cases reported in the literature. Here, we present a case of Evans tumor occurring in an unusual and rarely reported location; an intrathoracic mass arising from the diaphragmatic pleura.

No abstract

From this joint analysis emerges the fact that margin and disease status influences local and central control, but DFS remains modest, given the high risk of distant metastases. Intensified local treatment needs to be tested in the context of more efficient concurrent, neo-, and adjuvant systemic therapy.

Introduction Low-grade fibromyxoid sarcoma, also known as Evans tumor, is a low-grade sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site with only a few cases reported in the literature. Here we present a case of Evans tumor occurring in an unusual and rarely reported location: the mediastinum. Method A 32-year-old male presented with history of persistent cough, and subsequent imaging revealed a left diaphragmatic tumor measuring around 11 cm with associated chest wall implants. Grossly, the tumor was well circumscribed, arising from the parietal pleura with attachment to both lung and diaphragm and focally involving visceral pleura with no extension into the underlying lung parenchyma. Histologically, the tumor was composed of uniform and bland-appearing spindle cells interspersed among large areas of hyalinized rosettes, so-called giant rosettes. By immunohistochemistry, the tumor was positive for EMA, bcl-2, TLE-1, and MUC-4 and negative for STAT-6, ruling out solitary fibrous tumor. FISH for SYT gene rearrangement was performed and was negative, ruling out synovial sarcoma. Given the overall morphology and the phenotype, the final diagnosis was low-grade fibromyxoid sarcoma with giant collagen rosettes (also known as hyalinizing spindle cell tumor with giant rosettes). Conclusion Mediastinal low-grade fibromyxoid sarcoma is a rare entity that should be considered in the differential diagnosis of visceral soft tissue tumors. Pathologic examination and a thorough immunohistochemical and molecular workup play a vital role in establishing this rare and challenging diagnosis.

Objectives: To evaluate histologic changes in middle ear and eustachian tube (ET) mucosa of mice after exposure to tobacco or electronic cigarette (e-cigarette) smoke. To determine whether there were any mitigating effects of middle ear application of anti-IL-13 or the epidermal growth factor receptor antagonist AG1478 on noted changes within ET mucosa.Study Design: Controlled animal study. Methods: Fifty BALB/cJ mice were randomly assigned to one of five groups: A control group with no smoke exposure, two groups exposed to tobacco smoke, and two groups exposed to e-cigarette vapor. Within the exposed groups after 4 weeks of exposure, one ear was infiltrated with a saline hydrogel and the other ear with hydrogel of either Anti-IL-13 or AG1478. After four more weeks of exposure, the animals were euthanized and the ETs were evaluated for mucosal changes.Results: Compared to control animals with no smoke exposure, there were significant decreases in the numbers of goblet cells within the ET mucosa of mice exposed to tobacco smoke and e-cigarette vapor. No significant differences in cilia, mucin, or squamous metaplasia were noted. Neither anti-IL-13 nor AG178 significantly altered goblet cell count in the ET mucosa of mice exposed to tobacco smoke; however, both agents significantly increased goblet cells within the ET mucosa of mice exposed to e-cigarette vapor.Conclusion: Short-term tobacco smoke and e-cigarette vapor significantly decrease goblet cell count in mouse ET mucosa. Middle ear application of both anti-IL-13 and AG1478 resulted in an increase in goblet cell count among mice exposed to e-cigarette vapor, but not to tobacco smoke.