Small round cell desmoplastic tumour. Atypical morphology in the sub-maxillary gland
| Autor: | Ángel Batuecas Caletrío, María Teresa Flores Corral, Pablo Santos Gorjón, Juan Luis Gómez González, Fernando Sánchez González |
|---|---|
| Rok vydání: | 2009 |
| Předmět: |
Adult
Male Pathology medicine.medical_specialty business.industry medicine.medical_treatment Submandibular Gland Mesenchymal stem cell Chromosomal translocation General Medicine Salivary Gland Neoplasms Malignancy medicine.disease Submandibular gland Radiation therapy medicine.anatomical_structure Stroma Humans Medicine Immunohistochemistry business Rare disease |
| Zdroj: | Acta Otorrinolaringologica (English Edition). 60:141-143 |
| ISSN: | 2173-5735 |
| Popis: | Desmoplastic small round cell tumour (DSRCT) is a rare disease usually affecting young males. There are no other articles with a sub-maxillary location. The tumour consists of nests and masses of undifferentiated small round cells embedded in a desmoplastic stroma. The co-expression of epithelial, muscular and neuronal antigens distinguishes this entity from other small round cell tumours. The t(11;22)(p13;q12) translocation is a recurrent characteristic of this type of tumour. We report a case of desmoplastic small round cell tumour of the sub-maxillary gland, with an evolution of 8 months, affecting a 36 year old male. He suffered chronic lymphatic leukaemia five years ago and needed a bone marrow transplant. There was a 4x3 cm tumour. There were no signs of malignancy on the CT scan. A right sub-maxillectomy was performed. The pathology analysis gave a diagnosis of DSRCT. Post-surgical radiotherapy was given. The definitive diagnosis was reached using immunohistochemical techniques, such as polyphenotypical differentiation (epithelial, mesenchymal and neural), and by demonstration of translocation (11;22)(p13;q12). Sub-maxillary location is very rare. |
| Databáze: | OpenAIRE |
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