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Malignant granular cell tumor at the retrotracheal space
1Department of Pathology, Yonsei University Wonju College of Medicine, Korea.
Yonsei Medical Journal
|April 13, 1999
Summary
Malignant granular cell tumor (MGCT) is extremely rare. Diagnosis requires considering aggressive clinical findings and specific histologic features like necrosis and nuclear atypism, even if not all classic signs are present.
Area of Science:
- Oncology
- Pathology
Background:
- Malignant granular cell tumor (MGCT) is an exceptionally rare neoplasm.
- Diagnosis of MGCT typically relies on identifying metastasis and an aggressive clinical course.
Observation:
- A case of MGCT in a 21-year-old pregnant woman is presented.
- The tumor was located in the retrotracheal space and extended into the thoracic inlet.
- Chest CT revealed multiple tumor nodules in both lung fields.
Findings:
- Histological examination showed sheets of large ovoid cells with eosinophilic cytoplasm, eccentric nuclei, and prominent nucleoli, with slight nuclear atypism and focal necrosis.
- Immunohistochemistry was positive for S-100 protein, neuron-specific enolase (NSE), and CD68.
- Electron microscopy revealed autophagic vacuoles within tumor cells.
Implications:
- The diagnosis of MGCT should be considered even with subtle histologic findings if aggressive clinical behavior is observed.
- Histologic features such as nuclear atypism, necrosis, and mitotic activity can suggest malignancy in MGCT.
- This case highlights the diagnostic challenges and the importance of integrating clinical and histological data for rare neoplasms.