Objective: To study the clinicopathologic characteristics of diffuse myxoid malignant fibrous histiocytoma (MFH) of pleura.
Methods: The clinical and pathological features of a patient with diffuse myxoid MFH of pleura from this hospital were analyzed, and the reported literature of 7 patients with MFH of pleura were reviewed.
Results: The patient was a 65-year-old male. The primary manifestations were cough, chest pain, breathlessness, and a great quantity of bloody pleural effusion. Chest CT scan showed diffuse masses on the visceral and parietal pleura. Exploratory thoracotomy exposed diffuse translucent gray-white masses on the surface of visceral and parietal pleura, with diameters of 1 - 8 cm. Microscopic findings showed that diffuse spindle-shaped and pleomorphic tumour cells were within the myxoid stroma. Tumor cells were positive for vimentin, CD(68), lysozyme, and negative for CK, EMA, and desmin. The patient died of obstruction of superior vena cava, and circulatory failure at 24th postoperative day. Different from cases with single or multiple MFH of pleura reported in the literature, this case of diffuse myxoid MFH had an abrupt onset, and progressed aggressively.
Conclusion: Diffuse myxoid MFH of pleura is a very rare high-grade malignant tumor with very poor prognosis.
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SAGE Open Med Case Rep
December 2024
Department of Pathology, University of California San Francisco, UCSF, San Francisco, CA, USA.
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Department of Pathology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
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Department of Pathology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, PR China. Electronic address:
Superficial anaplastic lymphoma kinase (ALK)-rearranged myxoid spindle cell neoplasms are a recently identified subtype of cutaneous soft tissue tumors, distinct for their co-expression of CD34 and S100 and characterized by ALK gene rearrangements. Although 72 cases have been reported primarily as isolated case reports, this tumor subtype has yet to be included in the WHO classification of soft tissue tumors, underscoring the need for further study. In this study, we diagnosed two additional cases, both arising in the dermis and subcutaneous tissue.
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