Diagnosis and treatment of Cushing's disease (CD) is a demanding endeavor. We evaluated the effectiveness of inferior petrosal sinus sampling (IPSS) in preoperative tumor lateralization and surgical outcomes. Fifty-two consecutive patients with CD and normal magnetic resonance imaging (MRI) were enrolled between 2009 and 2015. Selective catheterization of petrosal sinuses for IPSS was performed. All patients underwent microsurgical dissection, and if a lesion was found, underwent lesion resection. Demographic, biochemical, and intraoperative findings were prospectively collected and reviewed 6 and 12 months postoperatively. Forty-five patients (86.4%) had clear lateralization while seven patients had bilateral elevation. Twenty-two patients (42%) were found to have a macroscopic lesion, defined as an abnormal tissue during microsurgical dissection, which was thus resected. No patient had a lesion larger than 4 mm. Eighty-one percent of patients had the lesion where IPSS had been predicted. Hemi-hypophysectomy was performed on twenty-eight patients based on IPSS findings, since no lesion was found. Eighty-six percent of patients with lesion resection had biochemical remission by 12 months. Biochemical remission was documented in 78 and 71% of patients who underwent hemi-hypophysectomy by 6 and 12 months, respectively. With careful microsurgical dissection, a macroscopic lesion could be found in a significant number of patients with normal MRI. When no lesion was found, we recommend performing a hemi-hypophysectomy based on IPSS findings, since prediction accuracy was high and remission rates were significant.
A 50-year-old woman presented to the emergency room complaining of severe headache. A non-contrasted head CT was obtained, which demonstrated a hyperdense image compatible with an intracerebral hemorrhage in the posterior region of the left temporal lobe. The patient displayed no neurological deficit during the consultation and a subsequent MRI showed a temporoinsular bleeding lesion that was suggestive of an atypic meningioma or a metastatic lesion. Afterwards, neck, chest and abdomen CT scans were performed, and the imaging ruled out a secondary neoplasm. The patient underwent surgical resection of the lesion, and a solid tumor was found with no bleeding associated. The pathology reported a WHO I fibroblastic meningioma.
Papillary glioneural tumors are infrequent neoplasms of the central nervous system, classically presenting with an indolent clinical course, rarely being related to an aggressive presentation, and not being associated with ischemic or another type of paraneoplastic phenomena. We describe the first case of this type of presentation with a literature review of the current knowledge of this entity. A 16-year-old female presented with a hemorrhagic intra-axial lesion, confirmed to be a papillary glioneural tumor in the histopathological analysis, being associated with multiple ischemic cerebral and posterior fossa strokes without another discernable cause than the presence of a paraneoplastic syndrome, not previously reported with this type of neoplasms. A literature review is presented detailing the current knowledge about this entity and emphasizing the need for greater knowledge about its natural history, immunobiology and treatment alternatives, opening a new window for the study of this pathology and establishing the need for a strict follow-up of patients who have this kind of tumors in order to learn more about their evolution. The papillary glioneural tumor, a rare entity having generally with a benign course, can have different presentations. Greater knowledge is needed to understand this behavior in order to optimize patient’s outcomes.
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