Neurologische Störungen

Gastrointestinal Leiomyosarcoma with Disease Progression to the Central Nervous System: Case Report

Abstract

Joao Gustavo Rocha Peixoto dos Santos, Iuri Santana Neville,Luiz Guilherme Cernaglia Aureliano Lima, Guilherme Alves Lepski and Manoel Jacobsen Teixeira

Introduction: Leiomyosarcomas (LMS) are rare malignant neoplasms with smooth muscle differentiation, which occur more commonly in the uterus, digestive tract or retroperitoneum. Before 2000, the mesenchymal gastric tumors were divided in leiomyomas, leiomyoblastomas and leiomyosarcomas. After discovery of the role of KIT oncogene, a distinct entity in the mesenchymal gastrointestinal tumors line has surfaced: GIST (Gastrointestinal Stomal Tumors). The true gastric leiomyosarcoma has become a rare entity. The presentation with brain metastasis had only been reported in the pre-GIST era. Case report: A female, 57 years old, admitted in October 2013, for investigation of abdominal pain, finding a lesion in the left illiac fossa continuing with the terminal ileum. She was submitted to the resection of the lesion with histopathological result showing leiomyosarcoma (6 mitotic figures/10 high power field; IHQ: vimentin positive, S-100 negative, desmin positive). Started chemotherapy. However, presented progression of the disease for subcutaneous tissue, liver (submitted to radiotherapy) and nervous system (only one lesion in the posterior fossa). The patient was submitted in March 7th 2016 to suboccipital craniotomy and en-block resection of the lesion. The microscopic examination revealed a fasicular spindle cell malignant neoplasm, with necrotic areas, with intense and diffuse desmin and smooth muscle actin-positivity in immunohistochemical study, which confirmed the leiomyosarcoma metastatic lesion hypothesis. The patient evolved well during the postoperative period, it was peformed the postoperative MRI, that showed a satisfactory resection. She remained hospitalized until the fourth postoperative day, with no complications, and was then discharged for outpatient follow-up. Conclusion: We have described a case of gastric leiomyosarcoma, emphasizing that even in front of the rarity of leiomyosarcoma itself as a primary lesion (especially after the discovery of GIST as an independent mesenchymal line tumor), this lesion may progress and present central nervous system metastasis, a fact not before reported in literature.