Olfactory groove monophasic sinovial sarcoma and von Recklinghausen’s disease: A case report and literature review

Background: Soft-tissue sarcomas are a rare and diverse group of neoplastic lesions. They represent only 1% of malignant tumors in adults and 15% in children. Synovial sarcoma (SS) is a type of soft-tissue sarcoma, accounting for 5–10% of cases, and commonly affecting extremities. Diagnosis, treatme...

Full description

Saved in:
Bibliographic Details
Published in:Surgical neurology international 2023-07, Vol.14, p.231, Article 231
Main Authors: Nery, Breno, de Alencar Neto, Joaquim Fechine, Melo, Layssa Rhossana dos Santos, Costa, Rodrigo Antônio Fernandes, Quaggio, Eduardo, de Medeiros, Luísa Segato, de Sousa Segundo, José Alencar, de Lima, Nicolle Fortuny, Rivero, Renan Lopez
Format: Article
Language:English
Subjects:
Case Report
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Background: Soft-tissue sarcomas are a rare and diverse group of neoplastic lesions. They represent only 1% of malignant tumors in adults and 15% in children. Synovial sarcoma (SS) is a type of soft-tissue sarcoma, accounting for 5–10% of cases, and commonly affecting extremities. Diagnosis, treatment, and prognosis remain challenging especially when localized in uncommon areas, such as intracranial lesions. Case Description: A 13-year-old male patient with a clinical history of neurofibromatosis Type I (NF1) presenting holocranial headache with jet vomiting and apathy 2 days before admission, without neurological deficits and/or focal findings. On magnetic resonance imaging: an extra-axial infiltrative lesion with contrast uptake at the base of the skull in the olfactory groove topography. After total tumor resection, the anatomopathological examination showed monophasic SS. The patient returned after 6 months with similar symptoms, and the lesion recurred and was reoperated. Unfortunately, 7 months after the second surgery, the patient died. Conclusion: SS can occur extraarticulously and with a variable clinical presentation and poor prognosis despite adjuvant therapies with radiotherapy and chemotherapy. In individuals with clinical history of NF1, there is still no direct correlation between the two manifestations, although current descriptions are suggestive of a possible interaction.
ISSN:2229-5097
2152-7806
DOI:10.25259/SNI_338_2023