August 25, 2016
A 34-year-old man with no past medical history presents with 8 months of dysphonia.
Malignant Fibrous Histiocytoma (pleomorphic undifferentiated sarcoma) of the Larynx
- Background:
- Malignant fibrous histiocytoma (or pleomorphic undifferentiated sarcoma according the the 2002 WHO classification) of the larynx is a rare tumor, representing < 1% of all airway tumors. This type of tumor is more common in the retroperitoneum and limbs.
- Clinical Presentation:
- Persistent dysphonia, cough, and hemoptysis (seen in ulcerated lesions)
- Key Diagnostic Features:
- NECT: A well-defined oval exophytic polypoid, usually non-infiltrative, lesion with homogeneous enhancement
- Histology: Hematoxylin and eosin stain show a proliferation of spindle cells arranged on a fibrous stroma and multinucleated and xantomatous cells.
- Immunohistochemistry: Shows tumor cells positive for CD68 and vimentin
- Endoscopic Exam: Shows an exophytic polypoid mass arising usually from one of the vocal cords
- Differential Diagnoses:
- Non-aggresive lesions are well-defined, without infiltration of the neighboring structures.
- The differential diagnoses include neurogenic, smooth muscle (leiomyoma), or vascular (hemangioma) tumors, and non-squamous neoplasias.
- All these lesions are rare in this region; leiomyoma and hemangioma are the most common.
- Neurogenic tumors are most commonly located in the supraglottic region.
- Hemangiomas usually show intense enhancement that washes out within 1–2 min after injection.
- Leiomyoma and non-squamous malignancies show similar non-specific imaging features. Squamous cell carcinoma appears as an exophytic infiltrative mass that may invade adjacent structures.
- Treatment:
- Surgery is the treatment of choice. Prognosis depends on the degree of resection and the presence of metastasis.
