Paediatric choroid plexus carcinoma: a retrospective case series from Karachi.

The objective of this study is to report clinical, radiological, and histopathological characteristics of three paediatric patients diagnosed as Choroid plexus carcinoma seen at our hospital, between 2015 and 2020. Three patients were diagnosed with choroid plexus carcinomas between 2015 and 2018. The mean age at diagnosis was 1.3 years (range 8 months to 1.5 years). All the three patients had subtotal resection and received adjuvant chemotherapy. One patient also received adjuvant radiotherapy. Despite these treatment measures, residual disease was noted in all three patients and two patients were subsequently treated on palliative care grounds. The average duration of follow-up after the first surgery for all three patients was approximately 33 months. Attaining satisfactory outcome in patients with CPC is challenging. Our case series reflects the difficulty in achieving gross total resection and ensuring that the disease does not recur.

Extracerebral choroid plexus papilloma in the pharynx with airway obstruction in a newborn: a case report.

BACKGROUND: Choroid plexus papillomas (CPPs) are rare, usually benign, neoplasms originating in the central nervous system. In this study, we present the first case of a giant airway-obstructing CPP in the pharynx of a newborn. CASE PRESENTATION: A cystic mass located in the pharynx was noted in a fetus at the 29th week of gestation. Elective cesarean section was performed at the 38th week of gestation with successful intubation and ex utero intrapartum treatment. On computed tomography, there was a huge airway-obstructing cystic mass in the choana and pharynx. Elective surgery with total excision was performed, and histological examination confirmed the diagnosis of CPP. CONCLUSION: We report the first case of an extracerebral airway-obstructing CPP in the pharynx of a newborn. Radiologic examinations are not enough for the diagnosis of CPPs, and complete excision of the tumor with histological confirmation is indispensable for accurate diagnosis and treatment.

Histogram Analysis Parameters ADC for Distinguishing Ventricular Neoplasms of Ependymoma, Choroid Plexus Papilloma, and Central Neurocytoma.

BACKGROUND To determine if histograms of ADC can be used to differentiate ventricular ependymomas, choroid plexus papillomas (CPPs), and central neurocytomas (CNCs). MATERIAL AND METHODS We retrospectively reviewed records from 185 patients from 1 January 2014 to 1 November 2018. We finally included a total of 60 patients: 36 (60.00%) had histologically confirmed ependymomas, 10 (16.67%) had CPPs, and 14 (23.33%) had CNCs, as determined by routine MRI scanning at 3.0T. The ADC histogram features were derived and then compared by Kruskal-Wallis test (they were not normally distributed). Bonferroni test was used to compare the 2 groups and then we determined the ROC. RESULTS Ependymomas had significantly higher mean, perc.01%, perc.10%, perc.50%, perc.90%, and perc.99% than CNCs. Ependymomas had significantly lower skewness than CNCs. Histogram metrics derived from mean, perc.01%, perc.10%, perc.50%, and perc.90% were significantly lower in the CNCs group than in the CPPs group. CPPs showed significantly lower skewness than CNCs. A threshold value of 86.50 for perc.50% to predict ependymomas from CNCs was estimated (AUC=0.97, sensitivity=97.20%, specificity=85.70%). Optimal diagnostic performance to predict CPPs from CNCs (AUC=0.96, sensitivity=100.00%, specificity=85.70%) was obtained when setting Perc.50%=84.00 as the threshold value. CONCLUSIONS The ADC histogram analysis may help to discriminate ependymomas, CPPs, and CNCs.

Metachronous Type I pleuropulmonary blastoma and atypical choroid plexus papilloma in a young child.

Pleuropulmonary blastoma (PPB) is a rare childhood tumor, often associated with germline DICER1 mutations and a risk for development of other benign and malignant tumors, a constellation termed DICER1 syndrome. A 1-year-old male was diagnosed with Type I PPB and screened regularly thereafter for detection of intrathoracic and intraabdominal disease. Ten months after diagnosis of PPB, he presented with headaches and vomiting. He was diagnosed with atypical choroid plexus papilloma, a lesion not previously reported with PPB. The presence of central nervous system symptoms in patients with PPB or a phenotype suggestive of DICER1 syndrome should prompt early intracranial imaging.

[Choroid plexus tumours in childhood: Experience in Sant Joan de Déu hospital]. / Tumores de plexo coroideo en la infancia: experiencia en el hospital Sant Joan de Déu.

Choroid plexus tumours are rare, with a peak incidence in the first two years of life. The most common location is the lateral ventricle in children, while in adults it is the fourth ventricle. The most common clinical manifestation is the signs and symptoms of intracranial hypertension. They are histologically classified as plexus papilloma, atypical plexus papilloma, and plexus carcinoma. A review is presented on choroid plexus tumours treated in the Hospital Sant Joan de Déu between 1980 and 2014. A total of 18 patients have been treated. An analysis was made of the demographic, clinical, histological data, treatment, and recurrences. The treatment of choice is complete resection, accompanied by adjuvant therapy in carcinomas. In atypical papillomas, the use of adjuvant therapies is controversial, reserving radiation therapy for recurrences. Papillomas have a good outcome, whereas atypical papillomas and carcinomas outcome is poor.

The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas.

INTRODUCTION: We performed a retrospective study on clinical assessment, tumor location, radiological imaging, histopathological characteristics, and therapeutic management of 7 patients affected by choroid plexus carcinoma (CPC) or atypical choroid plexus papilloma (ACPP) who have been observed in the last 12 years. METHODS: Four patients fulfilled the criteria for classification as ACPP and three cases as CPC. The median age of the patients at the diagnosis was 42 months (range 3-190 months). Except one older patient (15 years old), all patients were younger than 3 years of age. In all patients affected by ACPP, a total surgical resection was achieved. Two children relapsed 12 and 8 months following radical removal. Both of them underwent adjuvant chemotherapy (carboplatin, cyclophosphamide, etoposide, doxorubicin, and methotrexate); a complete remission was maintained in all cases. In all three patients with CPC, it was impossible to achieve complete resection at first surgery. The response to chemotherapy was variable: in one case, it was complete with complete remission following 6 months; in one case, it was partial with reduction on volume (the patient underwent second-look surgery with complete resection); in the third case, there was no response and the patient progressed and finally died with metastatic disease, 8 months after chemotherapy was started. For children with CPC, the OS was 75% at 6 years. RESULTS: In our series, surgery associated with chemotherapy led to long-term survival in 4/4 patients affected by ACPP and 2/3 patients affected by CPC. Clinical results achieved in our series confirm that our therapeutic regimen is feasible and efficient as a possible adjuvant treatment for both CPC and ACPP. It also suggests that surgery has a pivotal role in the management of most children affected by CPTs.

[Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades]. / Tratamiento de los tumores de plexos coroideos en el Hospital Infantil Niño Jesús de Madrid: nuestra experiencia en las últimas tres décadas.

OBJECTIVE: To review childhood patients with choroid plexus tumors (CPT) who underwent surgery at Hospital Infantil Niño Jesús of Madrid since January 1981 to September 2014. MATERIAL AND METHODS: Registered charts were analyzed based on the epidemiology, tumor grade, clinical profile, location, dissemination characteristics, therapy, prognosis and complications. RESULTS: Seventeen childhood patients were recorded with CPT. Cases were distributed so that 9 cases were choroid plexus-papilloma (CPP) (52.9%), 2 cases atypical CPP (11.7%) and 6 cases choroid plexus-carcinoma (CPC) (35.2%). Age at diagnosis was less than 2 years in 14 of the 17 patients (82.3%) and the incidence was higher in males (82.3% of the cases). Gross total resection was performed in 16 patients (94.1%). Adjuvant treatment was used in 6 patients (all this cases with CPC) (35.2%). Two of the 17 patients died (11.7%), showing an incidence density of 0.01 deaths/year. CONCLUSIONS: Our case series is consistent with previous published in scientific literature regarding epidemiology, tumor grade, clinical presentation, radiological features and therapeutic approach. Gross total resection is considered the therapeutic gold standard for choroid plexus tumors. Chemotherapy and radiotherapy should be used as adjuvant treatment in CPC and recurrent or remaining atypical CPP.

Retrosigmoid approach for resection of an extraventricular choroid plexus papilloma in the cerebellopontine angle.

Choroid plexus papillomas (CPP) are uncommon benign brain tumors that usually arise in the fourth ventricle in adults and lateral ventricles in children. Extraventricular CPPs are rare and can be found primarily in the cerebellopontine angle (CPA). We present a case of primary extraventricular CPP in the right CPA successfully resected with retrosigmoid approach. Detailed surgical techniques of retrosigmoid craniotomy and tumor dissection are presented in high definition video with narration. The video can be found here: http://youtu.be/6591en3nWlY .

Primary papillary epithelial tumor of the sella: a case report of an emerging tumor type.

Primary papillary epithelial tumor of the sella (PPETS) is a newly described tumor entity with prototypical location in the sella. Only two case series with ten cases have been described so far. These tumours have a typical papillary architecture with bland nuclear features, TTF-1 immunopositivity and low MIB-1-labelling index. Many of these tumours were previously assigned under the category of 'ectopic choroid plexus papilloma'. PPETS expands the group of TTF-1 positive tumours of the central nervous system. Histomorphology plays an essential role in making this diagnosis. We report a case of 44-year-old female with a sellar mass lesion, who presented with progressive loss of vision and diagnosed as primary papillary epithelial tumor.

Chordoid glioma of the third ventricle: four cases including one case with papillary features.

Chordoid glioma is a rare, slowly growing tumor of the CNS, which is always located in the third ventricle of adults. Chordoid glioma has classic histological features consisting of clusters and cords of epithelioid tumor cells embedded within a mucinous stroma with rich lymphoplasmacytic infiltrate. The important distinctive immunohistochemical feature of this neoplasm is strong and diffuse reactivity for GFAP. Here, we report four cases of chordoid glioma that occupied the anterior portion of the third ventricle or suprasellar region. These four cases were all adult females with almost typical clinical, radiological, histologic and immunohistochemical characteristics of chordoid glioma. However, in one case there was an unusual histologic finding with regard to the papillary region. In this region, elongated tumor cells were observed radiating toward a central vessel to form characteristic papillary structures. Immunohistochemically, three cases showed strong reactivity for GFAP, and one exhibited weak reactivity. All cases were focally positive for epithelial membrane antigen, CD34 and D2-40, but negative for neurofilament protein (NFP). Several ultrastructural investigations have supported the ependymal origin of chordoid glioma. In some cases of immunoreactivity for NFP, some authors have supposed that chordoid glioma originates from a multipotential stem cell with glial and neuronal cell differentiation. With regard to the present four cases with immunoreactivity for D2-40 (an ependymal marker) and CD34 (undifferentiated neural precursors) and based on previously published data, we considered that the majority of chordoid gliomas had an ependymal origin, and that a small minority might have originated from a multipotential stem cell having ependymal and neuronal cell differentiation.

Choroid plexus papilloma in a mature cystic teratoma of ovary: A rare case report.

Mature cystic teratoma of the ovary is the most common type of germ cell tumor. It constitutes approximately 20% of all ovarian neoplasms. As a rare occurrence, however, several types of benign and malignant tumours developing secondarily in dermoid cysts have been reported. Those of central nervous origin are almost exclusively gliomas of astrocytic, ependymal or oligodendroglial lineage. Choroid plexus tumors are unusual intracranial tumors which comprised of only 0.4-0.6% of all brain tumors. These are neuroectodermal in origin and similar in structure to a normal choroid plexus in the form of multiple papillary fronds mounted on a well vascularized connective tissue stroma. The present case report highlights the presence of a choroid plexus tumor in a mature cystic teratoma of the ovary in a 27-year-old woman who came for safe confinement and cesarean section.

Discrimination of paediatric brain tumours using apparent diffusion coefficient histograms.

OBJECTIVE: To determine if histograms of apparent diffusion coefficients (ADC) can be used to differentiate paediatric brain tumours. METHODS: Imaging of histologically confirmed tumours with pre-operative ADC maps were reviewed (54 cases, 32 male, mean age 6.1 years; range 0.1-15.8 years) comprising 6 groups. Whole tumour ADC histograms were calculated; normalised for volume. Stepwise logistic regression analysis was used to differentiate tumour types using histogram metrics, initially for all groups and then for specific subsets. RESULTS: All 6 groups (5 dysembryoplastic neuroectodermal tumours, 22 primitive neuroectodermal tumours (PNET), 5 ependymomas, 7 choroid plexus papillomas, 4 atypical teratoid rhabdoid tumours (ATRT) and 9 juvenile pilocytic astrocytomas (JPA)) were compared. 74% (40/54) were correctly classified using logistic regression of ADC histogram parameters. In the analysis of posterior fossa tumours, 80% of ependymomas, 100% of astrocytomas and 94% of PNET-medulloblastoma were classified correctly. All PNETs were discriminated from ATRTs (22 PNET and 4 supratentorial ATRTs) (100%). CONCLUSIONS: ADC histograms are useful in differentiating paediatric brain tumours, in particular, the common posterior fossa tumours of childhood. PNETs were differentiated from supratentorial ATRTs, in all cases, which has important implications in terms of clinical management. Key Points • MR based apparent diffusion coefficient histograms can help differentiate paediatric brain tumours • ADC histogram parameters correctly classified the great majority of posterior fossa tumours.

Multifocal choroid plexus papillomas: case report.

Choroid plexus papillomas (CPP) are rare tumors of the central nervous system, usually occurring in the ventricular system. Apart from spinal drop metastases, CPPs are generally unique tumors. In this report, the authors present two exceptional adult cases involving multiple benign CPPs located in both intra- and extraventricular locations. The tumors were located in the fourth ventricle and Meckel's cave in one case and the cerebellopontine angle, the frontotemporal region and the fourth ventricle in the other. A review of the literature revealed that these two cases represent the first reported occurrences of multiple choroid plexus papillomas involving Meckel's cave and frontotemporal intraparenchymal locations.

Malignant congenital orbital teratoma: Glioblastoma multiforme with primitive neuronal pattern and choroid plexus papilloma: A rare diagnosis at an unusual age.

Childhood orbital teratomas are usually congenital lesions that are most often present at birth with progressive, massive unilateral proptosis. During the routine controls of 27-year-old woman between 26-27th weeks of pregnancy her fetal ultrasonography (USG) revealed a mass in the eye of the fetus, and termination was recommended. The family refused the termination option and in the 37th week of pregnancy, vaginal delivery is performed in an external medical center. The mass in the baby's eye was surgically removed. Microscopic examination revealed disorganized ocular tissues, adipose tissue, microcalcification, nerve plexuses as well as areas of neuronal nodules including hypercellular areas, palisatic necrosis, and microvascular proliferation. The immunprofile--patchy GFAP staining in the areas of cellular epithelioid and spindled cells that also show an focal and sparse expression p53 staining and a high proliferation rate in Ki67 staining--confirmed the hematoxylin-eosin (HE) impression of a teratoma with a component of glioblastoma (GBM). In this unique presentation of a malignant orbital teratoma with a GBM, we have identified three tumor components: (1) GBM component, (2) nodules of neuroglial tissue with mature neurons and BRAF mutation, and (3) papillary proliferation possibly representing a choroid plexus papilloma.

Fetal tumors of the choroid plexus: is differential diagnosis between papilloma and carcinoma possible?

Fetal choroid plexus tumors are uncommon. The prognosis is widely variable and depends on the histological findings: papilloma or carcinoma. We report a case of prenatal diagnosis of choroid plexus mass detected by ultrasound at 33 weeks of gestation. Prenatal (T1, T2, T2* and diffusion weighted sequences) magnetic resonance imaging (MRI) was used to rule out a hematoma. Follow-up examination by ultrasound and MRI revealed a significant increase in the volume of the mass, suggesting a diagnosis of malignant tumor. A healthy neonate was delivered by Cesarean section at 38 weeks of gestation. Full surgical excision of the tumor was performed at 20 days after delivery and histological analysis revealed a papilloma.

Primary choroid plexus papilloma in the pituitary fossa: case report and literature review.

Choroid plexus papillomas are rare tumors of the central nervous system and are usually confined to the ventricular system. We illustrated a primary choroid plexus papilloma in the pituitary fossa. A 31-year-old female presented with amenorrhea and intermittent galactorrhoea, with no visual complaints in the last 2 years. Endocrine testing showed no hormone excess or deficiency of the pituitary and target glands, except for a higher prolactin level (56 ng/ml). A sharply circumscribed regular mass in the sellar region occupying the entire sella turcica and extending into the suprasellar cistern was demonstrated on MR imaging with gadolinium diethylenetriamine pentaacetic acid. The patient underwent an endonasal trans-sphenoidal approach. Complete microsurgical excision and complete preservation of the normal pituitary gland was achieved, with normal prolactin level. The histopathology showed that the lesion was a choroid plexus papilloma. Theories of the origin, the differential diagnosis, and treatment of the rare tumor are discussed.

[Diagnostic use of D2-40 and annexin-1 in ependymal tumors].

OBJECTIVE: To investigate the diagnostic significance of D2-40 and annexin-1 in the ependymal tumors. METHODS: To 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. RESULTS: Thirty-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. CONCLUSIONS: The 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.