Choroid plexus tumors are uncommon brain tumors that primarily occur in children. Most of these tumors originate from the intraventricular area, and the most common clinicalpresentation is increased intracranial pressure. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. Here, we present 2 rare cases of adult choroid plexus carcinoma (CPC), each with distinct clinical presentation and progression. The first case was a 40-year-old male who presented with multiple intraventricular masses. After surgical biopsy, radiation and intrathecal chemotherapy failed to elicit any response. The patient progressed with spinal cord dissemination and expired 1 year later. The second case presented with visual disturbance, and brain MRI revealed a large ovoid juxtaventricular mass with peritumoral edema. This 49-year-old female patient underwent craniotomy for what was thought to be a high-grade glioma; however, the mass was connected to the choroid plexus at the operative field. Her pathology specimen was diagnosed as CPC, and adjuvant systemic chemotherapy was administered. She has now been free of recurrence for 10 months. The description of the presentation and progression of these rare adult-onset CPC provides insight for the diagnosis and treatment of other rare instances of choroid plexus tumors.
Adult ; Biopsy ; Brain ; Brain Neoplasms ; Cerebrospinal Fluid ; Child ; Choroid Plexus Neoplasms ; Choroid Plexus ; Choroid ; Craniotomy ; Diagnosis ; Drug Therapy ; Edema ; Female ; Fourth Ventricle ; Glioma ; Humans ; Intracranial Pressure ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pathology ; Recurrence ; Spinal Cord

Background: Decision-making concerning the treatment of choroid plexus tumor (CPT) in pediatric patients remains a topic of considerable debate. The aim of this work was to describe clinical features and prognostic risk factors of CPT in the pediatric population and to provide theoretical opinions regarding clinical decisions for CPT. Methods: The data of 96 patients with CPT and younger than 14 years were retrospectively analyzed. Clinical characteristics such as pathological type of CPTs, rate and severity of hydrocephalus, treatment and outcome, and recurrence were investigated. For categorical variables, the Pearson's Chi-square test was performed. The Mann-Whitney U-test was used for comparisons between nonnormally distributed parameters. Log-rank test was used for progression-free survival (PFS). Results: The study included 70 choroid plexus papilloma (CPP) cases, 17 atypical choroid plexus papilloma (aCPP) cases, and 9 choroid plexus carcinoma (CPC) cases. Compared with patients with CPP or aCPP, patients with CPC had a shorter disease course (median: CPP, 4 months; aCPP, 2 months; CPC, 1 month; H: 23.5, P < 0.001), higher rate of acute hydrocephalus (CPP, 27.1%; aCPP, 52.9%; CPC, 77.8%; χ = 10.9, P < 0.05), and lower incidence of cure rate (CPP, 85.7%; aCPP, 70.5%; CPC, 33.3%; χ = 13.5, P < 0.05). The severity of hydrocephalus with tumor in the lateral or third ventricle was significantly higher than that with tumors in the fourth ventricle (severe hydrocephalus: lateral ventricle, 51.7%; third ventricle, 47.0%; fourth ventricle, 11.1%; χ = 26.0, P < 0.001). Patients with gross total surgical resection had no better PFS than those with partial resection because of the use of adjuvant therapy in the latter (χ = 4.0, P > 0.05). Patients with CPC experienced shorter time for recurrence than those with CPP or aCPP (χ = 40.1, P < 0.0001). Conclusions Our results indicated that CPP in the fourth ventricle could trigger serious clinical symptoms at an early stage, requiring early intervention. Adjuvant treatment might be necessary for patients with partially resected CPP, aCPP, and CPC to achieve a favorable outcome.
Child ; Child, Preschool ; Choroid Plexus Neoplasms ; mortality ; pathology ; surgery ; Female ; Humans ; Hydrocephalus ; etiology ; Infant ; Male ; Neoplasm Metastasis ; Neoplasm Recurrence, Local ; Prognosis ; Risk Factors

Down syndrome (DS, trisomy 21) is associated with neuroanatomical abnormalities, including choroid plexus cysts and various types of brain tumors. Trisomy 21 is associated with oncogenic factor, especially in brain tumor. The brain of DS patients had a smaller volume of gray and white matter and an unbalanced cerebellum volume, indicating a smaller volume overall than normal. We report a case of a DS male patient who had an incidentally discovered neuroglial cyst in left cerebellar vermis. He visited our hospital with gait disturbance and fatigue. But, the neurologic exam was normal. To the best of our knowledge, this is the first reported case of a neuroglial cyst in a trisomy 21 patient. As the developmental mechanisms of a cyst and the choroid plexus are related, more research is needed.
Brain ; Brain Neoplasms ; Central Nervous System Cysts ; Cerebellar Vermis ; Cerebellum ; Choroid Plexus ; Down Syndrome* ; Fatigue ; Gait ; Humans ; Male ; Neuroglia ; Trisomy ; White Matter

Intracranial extraskeletal myxoid chondrosarcoma is extremely rare, with only seven patients previously reported. We present a case report of a 21-year-old woman admitted for weakness in her right extremities and symptoms of increased intracranial pressure. Magnetic resonance imaging (MRI) revealed hydrocephalus and a well-enhanced large mass around her left thalamus. A left parietal craniotomy and a cortisectomy at the superior parietal lobule were performed. Total surgical resection was also performed, and pathology results confirmed an extraskeletal myxoid chondrosarcoma. Postoperative MRI showed no residual tumor, and the patient underwent radiotherapy. After six months of radiotherapy, the patient's headache and weakness had improved to grade IV. This malignant tumor showed high rates of recurrence in previous reports. We here report another occurrence of this highly malignant and rare tumor in a patient treated using total surgical excision and adjuvant radiotherapy.
Brain Neoplasms ; Chondrosarcoma ; Choroid Plexus ; Craniotomy ; Extremities ; Female ; Headache ; Humans ; Hydrocephalus ; Intracranial Pressure ; Magnetic Resonance Imaging ; Neoplasm, Residual ; Radiotherapy, Adjuvant ; Recurrence ; Thalamus ; Young Adult

OBJECTIVETo investigate the diagnostic significance of D2-40 and annexin-1 in the ependymal tumors.

METHODSTo analyses the expression of D2-40, annexin-1, EMA and Ki-67 by immunohistochemistry in 52 cases of ependymal tumors (48 cases of ependymomas, 4 cases of choroid plexus papilloma) from Xuanwu Hospital from 2005 to 2009. Ten cases of corresponding normal brain tissue were also obtained as control.

RESULTSThirty-two of forty-eight (66.7%) cases of ependymomas were positive for D2-40. "Dot-like" and "ring-like" structures were commonly observed in ependymomas (55.3%, 21 of 38 cases) and anaplastic ependymomas (5 of 6 cases) with D2-40 staining. There was no difference in the expression between D2-40 and Ki-67 (r(s) = -0.013, P = 0.931). For annexin-1, 87.5% (42 of 48 cases) of the ependymomas were positive. The specific "granular structures" and cilium were observed in ependymomas (1 of 4 cases of myxopapillary ependymomas and 11 of 38 cases of ependymomas respectively) for annexin-1. The difference in expression between annexin-1 and Ki-67 was statistically significant (r(s) = -0.405, P = 0.005). D2-40 in combination of EMA and annexin-1 increased the positive rate to 100% in ependymomas. Choroid plexus papillomas were all positive for D2-40 and annexin-1. The control tissue was negative for D2-40 but positive for annexin-1 in the capillaries.

CONCLUSIONSThe specific structures are valuable in diagnosing of ependymal-genetic tumors, and are highlighted by D2-40 and annexin-1. D2-40 in combination of EMA and annexin-1 is a useful diagnostic marker for ependymal tumors.

Adolescent ; Adult ; Annexin A1 ; metabolism ; Biomarkers, Tumor ; metabolism ; Brain Neoplasms ; diagnosis ; metabolism ; pathology ; Child ; Child, Preschool ; Ependymoma ; diagnosis ; metabolism ; pathology ; Female ; Humans ; Immunohistochemistry ; Ki-67 Antigen ; metabolism ; Male ; Membrane Glycoproteins ; metabolism ; Middle Aged ; Mucin-1 ; metabolism ; Papilloma, Choroid Plexus ; diagnosis ; metabolism ; pathology ; Young Adult

OBJECTIVETo study the clinicopathologic features and immunophenotype of endolymphatic sac tumor (ELST) and normal endolymphatic sac.

METHODSThe clinical and histologic features were evaluated in 5 cases of ELST. Eight cases of choroid plexus papilloma at cerebellopontine angle and 2 cases of normal endolymphatic sac were used as controls. Immunohistochemical study for vimentin, AE1/AE3, CK8/18, CK5/6, EMA, GFAP, synaptophysin, S-100 protein, CEA, TTF-1, VEGF, D2-40, calponin, calretinin and Ki-67 was carried out.

RESULTSThe age of onset of ELST ranged from 23 to 35 years (median = 24 years). The male-to-female ratio was 2:3. The clinical presentation was tinnitus, otalgia, hearing loss, otorrhagia with effusion and headache. The duration of symptoms ranged from 6 months to 10 years. Local recurrences were noted in 3 cases. Radiologically, the tumors were located at cerebellopontine angle and demonstrated petrous bone destruction. Histologic examination showed that the tumors had a papillary-glandular pattern. The papillae were covered by a single layer of low cuboidal cells. The tumor cells had distinct cell borders and contained eosinophilic to clear cytoplasm. The nuclei were slightly atypical and sometimes apically located. Focal dilated glandular structures with colloid-like material were also identified. The surrounding stroma was vascularized. All of the 5 cases had dural or petrous bone infiltration. Immunohistochemical study showed that all of the 5 cases were positive for AE1/AE3, CK8/18, CK5/6 and VEGF, 4 cases for EMA, 3 cases for calponin (focal), 2 cases for vimentin, 2 cases for S-100 protein, 1 case for GFAP and 1 case for synaptophysin (focal and weak). The Ki-67 index measured less than 1%. The staining for D2-40, calretinin, CEA and TTF-1 was negative. The 2 cases of the normal endolymphatic sac were positive for AE1/AE3 and CK8/18, and negative for CK5/6, EMA, S-100 protein, GFAP and synaptophysin. The 8 cases of choroid plexus papilloma were positive for synaptophysin. Seven cases were also positive for S-100 protein, 2 cases for GFAP and 1 case for D2-40. All of the 8 cases were negative for EMA, CK5/6 and calponin.

CONCLUSIONSELST is a rare slow-growing and potentially malignant tumor with a tendency of bone invasion and local recurrence. Distant metastasis is not observed. It must be distinguished from choroid plexus papilloma occurring at cerebellopontine angle. Correlation with clinical, radiologic and immunohistochemical findings would also be helpful.

Adenocarcinoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Adult ; Calcium-Binding Proteins ; metabolism ; Cerebellar Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Cerebellopontine Angle ; pathology ; Diagnosis, Differential ; Endolymphatic Sac ; pathology ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Keratin-5 ; metabolism ; Keratin-6 ; metabolism ; Male ; Microfilament Proteins ; metabolism ; Mucin-1 ; metabolism ; Neoplasm Recurrence, Local ; Papilloma, Choroid Plexus ; metabolism ; pathology ; Tomography, X-Ray Computed ; Young Adult

BACKGROUND: The pigmented or melanotic variant of choroid plexus carcinoma is very rarely encountered. CASE REPORT: We report herein a case of melanotic or pigmented choroid plexus carcinoma in the posterior fossa of an 11-year-old female. CONCLUSIONS: A histopathological study supported by immunohistochemistry helped the authors to diagnose this rare entity. How to differentiate this tumor from pigmented papillary medulloblastoma, cerebellar papillary ependymoma, and metastatic malignant melanoma is discussed.
Carcinoma ; Child ; Choroid ; Choroid Plexus ; Choroid Plexus Neoplasms ; Ependymoma ; Humans ; Immunohistochemistry ; Medulloblastoma ; Melanins ; Melanoma

Adult ; Choroid Plexus Neoplasms ; pathology ; Diagnosis, Differential ; Ear Neoplasms ; pathology ; radiotherapy ; surgery ; Endolymphatic Sac ; pathology ; Glomus Jugulare Tumor ; pathology ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; Male ; Meningioma ; pathology ; Skull Neoplasms ; pathology ; radiotherapy ; surgery ; Temporal Bone ; pathology ; Tomography, X-Ray Computed

Choroid Plexus Neoplasms ; pathology ; Female ; Humans ; Infant ; Lateral Ventricles ; pathology

Intracranial calcification is known to be a physiologic phenomenon and is often seen in brain CTs of patients visiting the emergency department. The pattern of a calcified lesion may be related to the pathologic condition, and calcified lesion itself may cause neurologic symptoms. The causes of pathologic intracranial calcification are infection, brain tumor, vascular disorder, endocrinologic disorders, and genetic disorder associated with calcium metabolic defects. The most common sites of intracranial calcification are the basal ganglia, subcortical tissue of the cerebrum, the thalamus, the choroid plexus, and the dentate nucleus of the cerebellum. The diagnosis of pathologic calcification can be done by using brain CT or MRI, and pathologic calcification should be differentiated from other causes of calcification by using laboratory data. We report and discuss a case of extensive intracranial calcification with idiopathic hypoparathyroidism.
Basal Ganglia ; Brain ; Brain Neoplasms ; Calcinosis ; Calcium ; Cerebellar Nuclei ; Cerebellum ; Cerebrum ; Choroid Plexus ; Diagnosis ; Emergency Service, Hospital ; Humans ; Hypoparathyroidism* ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Thalamus