Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells with inflammatory cells, characterized by the activation of histiocytic cells or their proliferation that affects structures of the human body, including the temporal bone which localization is rare in children. The objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare our case with previously reported series. We report the case of a young 4-year-old patient with a history of otorrhea and recurrent otalgia, with left axillary non-blowing exophthalmia progressive since 5 months associated with indurated left temporal swelling and also an indurated swelling of posterior face of the left shoulder on pathological fracture. A cerebro-orbital MRI performed at home showed an extra-conical process with extension at the temporal fossa. The patient, then, underwent an open-air biopsy of the temporal mass and the pathological analysis returned to favor Langerhans cell histiocytosis. The diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The child was treated by chemotherapy with a good outcome. Our results concord with literature. The prognosis is different and better in children with unifocal bone disease than those with a multifocal disease with a survival rate of 65%-100%.
Background: Thoracic complications of ventriculoperitoneal (VP) cerebrospinal fluid shunting are rare and the diagnosis is difficult without neurological impairment. Case Description: We report a case of a 36-year-old woman who had a VP shunt in the right side when she was 13 years for a posterior fossa ependymoma and hydrocephalus. 23 years after surgery, she developed acute yellowfish cough and sputum, and the computed tomography scan found an intrathoracic cyst. She had a thoracotomy for the cyst and during surgery, we found the peritoneal catheter of the VP shunt, with a collected abscess in the left side. The patient was treated for the abscess and the VP shunt was removed. We also review the literature cases of thoracic complications after VP shunts. Conclusion: Thoracic abscess due to VP shunt migration is extremely rare and could happen after a long time delay VP shunt surgery.
Primary intracranial ependymomas originate from ependymal cells. They may migrate mainly in the spinal cord but rarely metastasize outside the central nervous system. Metastases outside the central nervous system are rare. Metastatic diffusion from the central nervous system is low due to the unique interaction of the brain and the tumor with the blood-brain barrier. Nevertheless, three main hypotheses have been mentioned in the literature, the tumor growth, the surgical manipulation (which may be considered to be the case in our patient), and the aggressiveness of the tumor according to the Ki67 index. We report the case of a 16-year-old female, who underwent complete surgical removal of a left occipital 2007 WHO grade II ependymoma. 3 years later, the patient presented multiple cervical and occipital indurated masses. MRI showed a left hemispheric meningeal infiltration, with multiple nodules located on the neck, occiput and mastoid. Histopathological study of a left temporal surgical biopsy and resection of an occipital subcutaneous nodule turned to be metastases of an anaplastic ependymoma. The ependymoma considered as a benign tumor could very quickly turn into malignancy by its metastatic potential. Early diagnosis and longer follow-up of patients would be recommended for a rapid management.
Background: Chordomas are rare benign, but locally aggressive tumors that are regularly encountered in the clivus and sacrum. Although they are rarely found in the thoracic spine, they can contribute not only to local bone destruction and spinal instability, lead to cord compression, and major neurological deficits. Case Description: A 56 year-old-male originally underwent a T12 laminectomy with debulking for a thoracic chordoma. Two years later, the lesion recurred contributing to a significant paraparesis. The new MR showed a T2 hyper intense lesion with huge epidural extension that warranted a 360° surgical decompression and fusion. The histopathology from both surgeries confirmed the diagnosis of a chordoma. Conclusion: Combined 360° decompression and fusion was warranted to resect a T12 chordoma that recurred 2 years following an original laminectomy with debulking procedure.
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