Primary extraosseous dural chondrosarcoma: a case report

Background Dural chondrosarcoma is a very rare intracranial tumor, given that meninges do not normally contain cartilaginous tissue from which it can originate. We present a case of primary extraosseous dural chondrosarcoma. Case presentation A 48-year-old woman presented to our tertiary center neurosurgery consultation with progressive headache, vomiting, vertigo, and gait instability of 5 months’ duration. An initial brain CT revealed a large parietal mass with gross calcifications and subtle hyperostosis of the inner table. Subsequent brain MRI showed a heterogeneous expansive lesion with a honey-comb enhancement. Discussion of intra- or extra-axial location was warranted, and finally, initial presurgical suspicion of meningioma arose although some atypical imaging features were detected. The differential diagnosis included solitary fibrous tumor–hemangiopericytoma and dural metastasis. Total resection of the lesion was performed, extra-axial origin was confirmed, and pathology resulted in a primary dural chondrosarcoma. Conclusion The importance of this case presentation lies in the unusual nature of the final diagnosis, the brief literature review and differential diagnosis with emphasis on imaging pearls, as well as the useful reminder for physicians to consider less frequent diseases when key findings do not unambiguously lead to the usual suspects.

Large multicystic spinal lesion in a young African migrant: a problem of differential diagnosis

We describe a rare case of large, fully cystic spinal schwannoma in a young adult from The Gambia. The initial clinical suspicion was spinal cystic echinococcosis. He came to our attention reporting progressive walking impairment and neurological symptoms in the lower limbs. An expansive lesion extending from L2 to S1 was shown by imaging (ie, CT scan and MRI). Differential diagnoses included aneurysmal bone cyst and spinal tuberculosis and abscess; the initial suggested diagnosis of spinal cystic echinococcosis was discarded based on contrast enhancement results. The final diagnosis of cystic schwannoma was obtained by histopathology of the excised mass. Cystic spinal lesions are rare and their differential diagnosis is challenging. Awareness of autochthonous and tropical infectious diseases is important, especially in countries experiencing consistent migration flow; however, it must be kept in mind that migrants may also present with ‘non-tropical’ pathologies.

ACOUSTIC SCHWANNOMA WITH INTRATUMORAL HEMORRHAGE –REPORT OF AN UNUSUAL COMPLICATION

Acoustic Schwannoma is a benign neoplasm of the 8th cranial nerve pair, and accounts for 8-10% of all intracranialtumors. Intratumoral haemorrhage is unusual in these tumors and can lead to hydrocephalus or aggravate a preexisting condition. This paper reports a case of acoustic Schwannoma in a child with complication with intratumoral hemorrhage that lead patient to death. Female patient, 9 years old, presented anacusis on the right, sudden, progressing with vomiting, intense headache and vertigo. Magnetic resonance imaging of the skull showed expansive lesion in the cistern of the right cerebellar angle, with content inside the internalauditory canal, with compression of the adjacent brain stem. The diagnostic hypothesis was acoustic Schwannoma. It progressed with worsening of headache, initially controlled with medication and later loss of consciousness. Computed tomography of the skull revealed an expansive lesion in the cistern of the right cerebellar angle with signs of bleeding with extension to the ventricular system. External ventricular shunt was performed, but the patient evolved with several complications, septic shock and death. Intratumoral hemorrhage in the acoustic Schwannoma is a rare event, but its occurrence worsens the clinical picture and may lead to death of the patients due to intracranial hypertension.

The role of image in the diagnosis of pseudotumor lesions: a pictorial essay

Introduction: The clinical-radiological differentiation between neoplastic and pseudotumoral lesions has an important role in therapeutic approach, with repercussions on the conduct to be taken. When facing an expansive lesion, in addition to neoplasms, a list of differential diagnoses must be considered, including inflammatory, infectious, hemorrhagic lesions, among others. In this context, the role of imaging methods is highlighted in order to anticipate surgical management in the presumption of an aggressive neoplasm or, on the other hand, avoid an unnecessary invasive procedure. Objectives: To identify the warning signs of pseudotumor lesions in imaging studies. Methodology: Retrospective pictorial review of the archives of Diagnostic Imaging service of Santa Casa de São Paulo, between 2010 and 2020. Results: The warning signs that indicate a probable pseudotumoral lesion include spontaneous hypersignal in T1, marked hyposignal in T2, absence of significant expansive effect, cavitated lesion with high signal in diffusion, incomplete ring impregnation, restricted peripheral ring diffusion and spectral relations (choline / creatine) lower than 2. Each of these distinguishing features is discussed and exemplified. Conclusions: Although the distinction between expansive neoplastic and pseudotumoral lesions is not always direct, the systematic use of the exposed criteria allows greater assertiveness in the differential diagnosis. If the diagnostic doubt persists, therapeutic proof tests have a fundamental role in this context and, unfortunately, in some cases the final diagnosis will only be possible with biopsy.

Isolated neuroparacoccidioidomycosis as a pseudotumoral lesion in the absence of systemic disease

Background: Paracoccidioidomycosis (PCM) is a systemic, progressive, noncontagious, and often chronic disease caused by the fungus Paracoccidioides brasiliensis that rarely affects the central nervous system (CNS). The condition is usually treated using antifungal drugs, and some cases may require surgery. Case Description: A 55-year-old man, a smoker, without known comorbidities, was referred to the neurosurgery team with a history of a single epileptic seizure a week before hospital admission followed by progressive right- sided hemiparesis. Head computed tomography and brain magnetic resonance imaging showed an intra-axial expansive lesion affecting the left parietal lobe, associated with extensive edema and a regional compressive effect producing slight subfalcine herniation that was initially managed as an abscess. After the failure of antibiotic treatment, the patient underwent a neurosurgical procedure for excision of the lesion. Histopathological analysis revealed that it was PCM and there was no evidence of impairment of other systems due to the disease. Conclusion: PCM can be a serious, debilitating disease and is potentially fatal. Although isolated CNS involvement is rare, it must be considered, especially in endemic areas, as late diagnosis and treatment severely decreases good outcome rates.

MON-919 Primary Neuroendocrine Tumor of the Central Nervous Sistem, a Case Report and Literature Review

Introduction Neuroendocrine Neoplasms are rare, with an incidence of 5 to 100,000 inhabitants, constituting 1% of all malignancies, presenting high survival rates in general, even in metastatic diseases. However, in those poorly differentiated, as in the following case, survival is around 4% in 5 years. We will describe a case of primary neuroendocrine tumor in the brain, of which is uncommon in the literature. Clinical case A 26 years women was referred to the ER of Santa Casa de São Paulo, in January 2019, to be evaluated by neurosurgery, due to progressive left hemiparesis and headache for 3 months, which got worse in 4 days. On CT scan, there was a 6 x 6 cm solid-cystic, expansive, lesion in the right frontal lobe, with perilesional edema and contralateral midline 1.3cm deviation and subfalcine herniation. Thus, the tumor was resected soon, with anatomopathological analysis showing poorly differentiated tumor of cells with scarce cytoplasm, hyperchromatic nuclei and high mitotic activity. Immunohistochemical analysis finds 50% Ki67, with focal p53, TTF1, CD99, CD 56 and synaptophysin positivity. The main hypotheses, then, consisted of Neuroendocrine Carcinoma. Four months after surgery, the patient reported worsening deficit, headache, pain, weight loss, being referred to the Emergency Room, once more. In RM an expansive lesion was found 6.6 x 4.4 cm, in the right frontoparietal surgical cavity, edema, compression and 0,4 cm midline deviation. The patient was once again submitted to emergency neurosurgery, with microsurgical resection. The pathology was identical to the previous one. We proceed with hormonal evaluation, regarding to Medular Thyroid carcinoma, Gastrinoma, Insulinoma, Pheochromocytoma, Carcinoid tumor and others. Imaging exams were also performed to investigate other primary sites: no changes in CT scan of the chest and abdomen and PET CT FDG. However, this one showed recurrence of the intracranial lesion, with three sites of involvement, all hypermetabolic: one of 4.1 x 2.9 cm (SUV 4.9) and another of 3.9 x 3.3 cm (SUV 8, 4) in the right frontoparietal region and medial nodule to the right thalamus of 1.2 cm (SUV 6.1). Patient currently maintain left hemiparesis, frequent pain, taking carbamazepine due to epileptic seizures, and considerable weight loss. She has an important limitation of daily activities and basic self-care, with 50% Karnofsky scale. Due to relapse, palliative radiotherapy was initiated in the region of the tumors. Conclusion The patient had a poor outcome in relation to cancer, with little possibility of treatment due to poor tumor differentiation and poor performance status.

Intracranial lipoma manifesting with change in preexisting headache characteristics

Intracranial lipomas represent 0.1% to 0.5% of all intracranial tumors. Approximately half of the patients are asymptomatic. In symptomatic patients, headache is the most common symptom. We describe the case of a 71-year-old woman with history of generalized seizures and episodic migraine for about 30 years. In recent months, there has been a change in the characteristics of headache. She was admitted to the emergency room with muscle weakness in left hemibody and intense headache onset approximately four hours ago. Neuroimaging exams revealed a median frontal expansive lesion suggestive of intracranial lipoma.

Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare, usually benign, slow-growing tumor, that commonly affects patients aged 30 to 50 years-old. The manifestations of dysplastic cerebellar gangliocytoma are nonspecific and are related both to the mass effect produced by its growth and to the location of the lesion. Cerebellar symptoms such as ataxia are often present. In 40% of cases, the tumor is associated with Cowden syndrome, which is part of a group of genetic disorders called polypoid hamartoma complex. In this case report, the patient presented expansive lesion in the posterior fossa, compatible with LDD, associated with macrocephaly. These findings are considered major criteria for Cowden syndrome. When together, they confirm the diagnoses. To our knowledge, this is the first report of the association of LDD and Cowden syndrome in Brazil.

Brain Tumor in Differential Diagnosis of Seizures in Puerperium: Case Report

Convulsive crisis is a transient disturbance of cerebral function, and the etiology of which may be manifold. Its clarification is essential for establishing adequate therapy and seizure control. In the pregnancy-puerperal cycle, eclampsia is the most common cause of it, but brain tumor, epilepsy and other disorders are part of the differential diagnosis. We report a case of seizure triggered by tumor in a primigest, 22 years old, at 38 weeks’ gestation with premature rupture of membranes. Cesarean section was performed due to non-reassuring fetal condition. On the second day of puerperium, she presented recurrent episodes of generalized tonic-clonic seizures and elevated blood pressure. She received magnesium sulfate for 24 hours and persisted with severe headache. Magnetic resonance imaging of the brain showed an expansive lesion in the left frontoparietal region. The patient underwent intracranial microsurgery on the eighth day after cesarean section, with complete resection of the lesion and anatomopathological diagnosis of schwannoma. She went through good postoperative evolution, without neurological deficits, and stayed on phenobarbital, with no seizures until 6 months of follow-up. From the case we conclude that although the first diagnosis in pregnant or puerperal women with seizures should be eclampsia, there are other causes, such as brain tumor and Posterior Reversible Encephalopathy Syndrome (PRES). Careful evaluation is required, especially in refractory cases and not responding to usual treatment.

Neuroparacoccidiomicosis Simulating Posterior Fossa Tumor

Paracoccidioidomycosis (PCM) is a systemic granulomatous disease caused by a fungus known as Paracoccidioides brasiliensis. Central nervous system (CNS) is affected in 12% of the cases, but posterior fossa involvement is rarer. We report a case of a patient who presented an expansive lesion at the posterior fossa causing a right hemispheric cerebellar syndrome. After lesion excision and analysis, the diagnosis of Neuroparacoccidiomicosis was confirmed. Our case highlights this rare presentation of this pathology and its capacity to simulate neoplastic lesions.