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[A palate tumor revealed by pain]
Marc Polivka1, Michel Wassef, Guillaume Lot
1Service d'Anatomie Pathologique, Hôpital Lariboisière, 2 rue Ambroise, Cedex, France.
Annales De Pathologie
|November 1, 2002
Summary
This case study highlights a rare malignant granular-cell tumor of the palate, initially misdiagnosed. Recurrence and invasion of the trigeminal nerve (V2) confirmed its aggressive nature.
Area of Science:
- Oncology
- Pathology
- Surgical Oncology
Background:
- Granular-cell tumors (GCTs) are rare, typically benign neoplasms of neural crest origin.
- Malignant variants of GCTs are exceptional and pose diagnostic challenges, often relying on metastasis for definitive diagnosis.
- Distinguishing between benign and malignant GCTs can be difficult based solely on histology and immunohistochemistry.
Observation:
- A 41-year-old female presented with hard palate pain, initially suspected as adenoid cystic carcinoma.
- Histological examination revealed a granular-cell tumor infiltrating palatine bone.
- Recurrent pain and imaging confirmed tumor extension along the maxillary nerve (V2) through the foramen rotundum.
Findings:
- Two radical surgical interventions confirmed an infiltrating malignant granular-cell tumor of the V2 nerve and palatine mucosa.
- The tumor showed characteristic granular cells, PAS positivity, and S100 protein expression.
- Immunohistochemistry revealed no p53 expression and low Ki67 proliferation index (<5%), with controversial prognostic value.
Implications:
- This case underscores the rarity and diagnostic difficulty of malignant granular-cell tumors.
- Tumor size >5 cm, recurrence, and infiltrative growth are critical indicators of malignancy.
- Further research is needed to clarify the prognostic significance of p53 and Ki67 in these rare neoplasms.