Publicação
Endolymphatic sac tumor : a report of 2 cases with long term follow-up
| Resumo: | Background: The endolymphatic sac tumor (ELST) is an extremely rare petrous bone neoplasm that can happen sporadically or in association with Von Hippel Lindau (VHL) disease. While it has a very small risk of metastization, large tumors are often difficult to completely remove and prone to local recurrence. Method: Retrospective analysis of two ELST cases with long term follow up. Results: Two ELST cases are described in detail and compared to the current literature. In one of the cases there was a long period of 20 years from the first symptoms until diagnosis. Seven years after surgery, the patient presented with tumor recurrence at the surgical scar, left submandibular and internal jugular lymph node area of the neck, operatory cavity and also with suspicious nodules in the left temporal fossa. Bibliographic research only revealed another case of endolymphatic sac tumor with an extracranial component. Conclusion: Late diagnosis and treatment are linked to difficult disease control with higher rates of tumor recurrence, incomplete excision and post-surgical morbidity. For all these reasons, early intervention should be promoted and clinicians should be aware of the presenting symptoms as well as the importance of screening in VHL patients. |
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| Autores principais: | Oliveira, Guilherme |
| Assunto: | Tumor do saco endolinfático Anatomia patológica |
| Ano: | 2014 |
| País: | Portugal |
| Tipo de documento: | dissertação de mestrado |
| Tipo de acesso: | acesso restrito |
| Instituição associada: | Universidade de Lisboa |
| Idioma: | inglês |
| Origem: | Repositório da Universidade de Lisboa |
| Resumo: | Background: The endolymphatic sac tumor (ELST) is an extremely rare petrous bone neoplasm that can happen sporadically or in association with Von Hippel Lindau (VHL) disease. While it has a very small risk of metastization, large tumors are often difficult to completely remove and prone to local recurrence. Method: Retrospective analysis of two ELST cases with long term follow up. Results: Two ELST cases are described in detail and compared to the current literature. In one of the cases there was a long period of 20 years from the first symptoms until diagnosis. Seven years after surgery, the patient presented with tumor recurrence at the surgical scar, left submandibular and internal jugular lymph node area of the neck, operatory cavity and also with suspicious nodules in the left temporal fossa. Bibliographic research only revealed another case of endolymphatic sac tumor with an extracranial component. Conclusion: Late diagnosis and treatment are linked to difficult disease control with higher rates of tumor recurrence, incomplete excision and post-surgical morbidity. For all these reasons, early intervention should be promoted and clinicians should be aware of the presenting symptoms as well as the importance of screening in VHL patients. |
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