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Choroid plexus papillomas are very rarely reported neoplasms in both the surgical and radiological literature. The authors present their series of 7 papillomas and 1 carcinoma. They review the recent and former literature with the aim of demonstrating the role and usefulness of radiotherapy.
PURPOSE: Choroid plexus tumors (CPT) are rare childhood neoplasms. The relatively small number of reported cases and the controversies surrounding the clinical and pathological classification of these tumors have made it difficult to define a standard of care for these patients. Our intention is to contribute to the body of knowledge of these tumors and further define the role of adjuvant therapy. METHODS AND MATERIALS: We performed a retrospective review of 14 children with choroid plexus neoplasms referred to St. Jude Children's Research Hospital between October 1985 and December 1987. Ten patients had choroid plexus carcinoma (CPC) based on pathologic criteria and evidence of brain invasion at surgery or leptomeningeal disease (M+); 4 patients had choroid plexus papilloma (CPP). Patients with CPP were initially treated with surgery alone whereas patients with CPC were generally treated with postoperative therapy that included chemotherapy (CT) and/or craniospinal irradiation (CSI) with a focal boost to the primary site. For most patients CT consisted of combinations of cyclophosphamide, etoposide, vincristine, and a platinum agent. The median CSI dose was 35.2 Gy (range 24-46.2 Gy). The median primary site dose was 55.2 Gy (range 49.6-64 Gy). RESULTS: Seven of the 10 CPC cases presented with leptomeningeal dissemination; two of these patients have succumbed to disease. Of the 3 patients with M0 status, all are alive with no evidence of disease (NED). The medial time to relapse from the time of surgery was 5.3 mo (range 3-25 mo). Seven CPC patients were treated with gross total resection (GTR). Three of these patients (2 M0, 1 M+) received CT without CSI and are currently NED (27, 69, and 60 mo respectively). One M+ patient progressed on CT and has stable disease after CSI (6 mo), one (M0) received CT and CSI and is NED (120 mo), one (M+) is currently on CT with objective response (3 mo) and one (M+) died of progressive disease (24.5 mo) despite CT and CSI. Three patients with CPC had subtotal resection (STR). One of these patients (M+) received CT and CSI and is NED (23 mo), one (M0) had an elective second resection GTR alone and is currently NED (153 mo), and one (M+) developed progressive disease (13.5 mo) while on CT and died despite CSI. Among the 4 CPP patients, GTR was performed in two; both were NED at 54 and 81 mo. Two patients with CPP (one with focal atypia) were treated with STR initially; both transformed to CPC at 7 and 27 mo, respectively. Both were currently NED following salvage with (1) GTR and CSI alone (98 mo) or (2) STR, CT, and CSI (62 mo). Six of the 12 survivors in this series had significant neuropsychological sequelae. CONCLUSION: The prognosis of CPP is good for patients treated with GTR. Malignant transformation occurred in 2 CPP patients with less than GTR. Patients with localized CPC who undergo GTR have had a favorable outcome with the addition of chemotherapy or irradiation. CSI may not be routinely indicated in M0 children following GTR. There is evidence that salvage with radiation therapy may be successful following progression on chemotherapy. For patients treated with STR, the use of CT and CSI appears to be necessary.
Primary choroid plexus (CP) tumors are rare neoplasms that present in childhood or, less frequently, in adult life. The majority are benign and amenable to complete surgical excision, but occasionally more invasive variants are encountered. Although generally pathologically distinct, occasionally primary CP neoplasms may be difficult to distinguish from metastatic papillary carcinomas or papillary ependymomas. Conventional cytologic markers are not sufficiently specific to permit accurate diagnosis of primary CP tumors. The authors have reported that the CP is the unique site of synthesis within the brain of transthyretin (TTR, prealbumin), a transport protein for thyroxine and retinol. They therefore investigated the utility of TTR as a biochemical marker for CP tumors. They detected intense immunoreactivity for TTR at high dilutions of primary antiserum in the neoplastic epithelium of all of nine primary CP tumors (six papillomas and three carcinomas), but not in eight cellular or three papillary intracerebral ependymomas, meningiomas, oligodendrogliomas, astrocytomas, primary extracerebral papillary carcinomas (three thyroid, two breast) or five of six cerebral metastases from systemic papillary carcinomas. In one case of cerebral metastasis from papillary thyroid carcinoma, rare isolated immunoreactive cells were observed. Faint staining of the stromal-ependymal junction was seen in myxopapillary ependymomas of the filum terminale, which were otherwise nonreactive. By in situ hybridization, TTR mRNA was abundant in neoplastic CP epithelium, confirming local TTR synthesis. The authors conclude that TTR is synthesized by neoplastic CP epithelium and is an excellent marker for primary CP neoplasms.
OBJECTIVE: To evaluate choroid plexus neoplasms for p53 expression. CASE MATERIAL: We studied 10 choroid plexus tumors (four papillomas and six carcinomas) by immunohistochemistry using the DO7 anti-p53 monoclonal antibody. RESULTS: Three of four choroid plexus papillomas demonstrated no staining. Scattered nuclear and rare cytoplasmic positivity was present in one papilloma, which showed foci of increased mitotic activity (labeling index 2.5%). Six of six carcinomas were immunoreactive for p53, and three cases had labeling indexes of over 70%. All immunopositive choroid plexus tumors (7/7) exhibited nuclear staining. Punctate cytoplasmic positivity was identified in 5 of 7 cases. CONCLUSION: Our study suggests that altered p53 expression is detectable by immunohistochemistry in choroid plexus neoplasms and is consistently present in choroid plexus carcinomas.
Choroid plexus neoplasms account for less than 1% of all intracranial tumors, with papillomas (CPPs) more frequent than carcinomas (CPCs). Immunocytochemical characterization of these neoplasms has been limited. Glial fibrillary acidic protein (GFAP), S100 protein, and keratin have been variably demonstrated by others. Ten cases were identified at two hospitals over a 25-year period; six were children and four were adults. There were seven cases of CPP and three of CPC. Extracranial metastases occurred in one case of CPC and multiple local recurrences were common. Immunohistochemical examination was performed with polyclonal antibodies to keratin, alpha-fetoprotein (AFP), desmin, neurofilament, glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE), and S100 protein, and with monoclonal antibodies to vimentin, 45- to 54-kd cytokeratin (CKER), and carcinoembryonic antigen (CEA). Among the seven cases of CPP, five were positive for CKER, three for keratin, two for CEA, two for NSE, and five for S100. Three cases of CPC were positive for CEA, three for CKER, and two for keratin. With one exception, when a neoplasm was positive for CEA and S100 it was also positive for CKER. Positivity for CEA in this group was associated with a more aggressive histologic pattern and heralded a worse prognosis. S100 immunoreactivity appeared to predominate in well-differentiated neoplasms. Keratin and CKER were found in both CPP and CPC, but may be useful in the distinction from ependymomas. Statistical analysis resulted in the following classification rule: If the CEA stain is positive and the S100 stain is negative, then the tumor is malignant; otherwise, the tumor is benign.
Although choroid plexus papillomas (CPP) and primary choroid plexus carcinomas (CPC) are rare neoplasms of the central nervous system, they have been the subject of a number of immunohistochemical studies. To date, no unique or specific marker for these neoplasms has been found, however. Normal choroid plexus is a major site of transthyretin (TTR) synthesis, and recently this protein has been proposed as a possible specific marker of choroid plexus differentiation in tumors. In this study, we performed immunohistochemistry for TTR on 13 choroid plexus tumors (six CPP and seven CPC) and on 23 carcinomas metastatic to the brain, four of which had a papillary architecture. We also included four ovarian teratomas that contained choroid plexus elements. Two of the CPP had diffuse staining for TTR, while the four others stained only focally. Five of the CPC stained only focally and less intensely than the control, while one case was negative. Only one CPC stained as strongly and diffusely as normal choroid plexus. Two of the papillary and six of the nonpapillary metastases had focal staining similar to that seen in the five focally positive CPC. The choroid plexus elements of the ovarian teratomas stained as strongly as the positive control. These findings indicate that TTR immunoreactivity is not restricted to primary choroid plexus tumors. Furthermore, most choroid plexus carcinomas stain only weakly or not at all. This limits the usefulness of TTR immunohistochemistry in the diagnosis of primary choroid plexus neoplasms and in the distinction of CPC from metastatic carcinoma.
Choroid plexus neoplasms (CPN) are rare tumors occurring with a relative incidence of 0.5% of intracranial neoplasms in all age groups and 1.5-6.4% of all pediatric brain tumors. In children, the most common site of origin is the atria of the lateral ventricles where the CPN may represent either a carcinoma or a benign papilloma. CPN arising in the fourth ventricle are more common in adults, and are more likely to represent a benign papilloma. We present 2 children with CPN arising from the inferior fourth ventricle with similar features on MRI, one of which represented a benign papilloma and the other a malignant carcinoma. CPN should be included, even in young children, in the differential diagnosis of any infratentorial, intraventricular tumor.
Choroid plexus papillomas are rare, constituting approximately 0.5% of all intracranial neoplasms. Four benign choroid plexus papillomas and one choroid plexus carcinoma were retrospectively reviewed in patients aged 4-20 months who had been examined with magnetic resonance (MR) imaging with a field strength of 0.5 T or 1.0 T and with computed tomography (CT) before and after the administration of contrast material. In general, the tumors were of intermediate signal intensity on T1-weighted images and of either intermediate or increased signal intensity (T2 lengthening) with T2 weighting. All demonstrated variable areas of internal signal void interpreted as signifying regional blood flow, calcification, or old hemorrhage. CT findings included relatively uniform contrast enhancement. Microscopic pathologic changes of the benign lesions mimicked the appearance of normal choroid plexus and confirmed the highly vascular nature of these tumors. MR imaging, with its high-resolution multiplanar techniques, offers direct visualization of these lesions in relation to normal anatomy and better discrimination and confirmation of their intraventricular location, facilitating surgery and postoperative follow-up.
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Sixty-seven tumor specimens of epithelial choroid plexus neoplasms obtained by 60 biopsies and 7 autopsies from 52 patients were investigated. Diagnoses of the first operations were choroid plexus papilloma (PP; 32 cases), choroid plexus papilloma with histological atypies (atypical PP; 6 cases), and choroid plexus carcinoma (PC; 14 cases). Carcinoembryonic antigen was expressed by 2 of the 3 biopsies autoptically recognized as metastatic carcinomas and by 2 autopsy cases of PC, while it was absent in all biopsies of true choroid plexus tumors. Tumor cells positive for transthyretin (TTR, prealbumin), S-100 protein (S100), and glial fibrillary acidic protein (GFAP) were detected in 39, 46 and 13, respectively, of the 49 cases of true choroid plexus tumors. Fourth ventricle tumors expressed more S100 (number of positive tumor cells) than lateral ventricle tumors, PP more S100 and TTR than atypical PP/PC. Tumors from patients 20 years of age and older expressed more GFAP and TTR than tumors from younger patients. Of the 30 patients with complete follow-up 19 were alive 2 to 11 years after surgery, including 7 recurrencies. Eleven died from the tumor 4 months to 7 years after surgery. The following histopathologic features (in order of decreasing significance) were correlated with poor prognosis (recurrency or fatal outcome): less than 50% of the tumor cells heavily positive for S100, presence of mitoses, absence of TTR-positive cells, brain invasion by cell nests, absence of marked stromal edema, and presence of necrotic areas. Our results indicate that some histologic features correlate significantly with poor prognosis and that immunohistochemical results correlate with tumor localization, age, and malignancy.
The pattern of basement membrane deposition was investigated in benign and malignant choroid plexus tumours using an immunocytochemical method to detect laminin, an intrinsic basement membrane component. The results were compared with intracranial ependymomas and myxopapillary ependymomas of the filum terminale. Expression of the intermediate filaments cytokeratin and glial fibrillar acidic protein (GFAP) and the epithelial marker carcino-embroyonic antigen (CEA) was also assessed. Laminin was detected in a subepithelial location in all the choroid plexus tumours but in none of the ependymomas, although basement membrane fragmentation was seen in the choroid plexus carcinomas. In contrast, GFAP was present in all the types of tumour examined. Cytokeratin (as detected by CAM 5.2) was present in all the choroid plexus lesions and also in the ependymomas arising in the spinal canal. CEA was not detected. We conclude that immunostaining for laminin assists distinction between choroid plexus tumors and papillary ependymomas and between benign and malignant choroid plexus tumours.
Fourteen children less than 2 years old with choroid plexus papilloma (CPP) were studied. Of these patients nine had the tumor in the lateral ventricles (LV), three in the fourth ventricle (4th V), one in the third ventricle (3rd V) and one in the third and left lateral ventricle (LLV). Ten cases showed benign CPP, three cases malignant CPP, and one case had a venous malformation of the choroid plexus (VMCP). Computed tomography (CT) was the best method for neuroradiological study, although a very small CPP in the 3rd V was not detected in an early study. Malignant and benign CPP can be differentiated with CT, especially if the study is performed with contrast media. Pneumoencephalography must be rejected because it is very dangerous and may be the cause of death in some cases. The follow-up of patients with CPP showed a poor prognosis, even after correctly removing the tumor and inserting a shunt.
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MIB-1 immunohistochemistry was carried out on a retrospective biopsy series of epithelial choroid plexus neoplasms in order to assess the proliferation rate of tumour cells. The material included 14 cases of papilloma (WHO grade I) and five cases of carcinoma (WHO grade III). There was one recurrent tumour in the papilloma group, while three patients with carcinoma experienced recurrence. Choroid plexus obtained at autopsy from paediatric and adult patients with unrelated diseases served as control. The average MIB-1 labelling index of choroid plexus papillomas was 3.7%, while that of carcinomas was 14%, and that of normal choroid plexus was 0.02% to 0.06%. The age and sex of the patients as well as the tumour localization were not found to influence MIB-1 reactivity. The labelling index of recurrent lesions was not significantly different from that of the corresponding primary tumours. High MIB-1 labelling indices were associated with less favourable post-operative outcome in choroid plexus carcinomas and in one papilloma with atypical histology. However, most tumours with atypical histological features did not exhibit distinctive MIB-1 labelling indices. Analysis of growth fraction by MIB-1 immunohistochemistry may prove a useful ancillary method for assessing the malignant potential of choroid plexus neoplasms.
The study of intracranial vascular tumors is of significant importance because these tumors may stimulate vascular malformations, infarctions, or infections. This article describes vascular tumors and discusses their appearance on MR imaging studies. Choroid plexus neoplasms, primitive neuroectodermal tumors, hemangioblastoma and other disorders are discussed.
Lectin histochemistry of the normal and neoplastic human choroid plexus cells [six choroid plexus papillomas (CPPs) and three choroid plexus carcinomas (CPCs)] was performed using eight representative lectins to study the development of sugar chain structures and also to determine whether lectins were useful for a histopathological diagnosis of choroid plexus neoplasms (CPNs). The normal choroid plexus cells reacted with Ricinus communis (RCA-I). Canavalia ensiformis (Con A), Limax flavus (LFA) and Triticum vulgaris (WGA), while Arachis hypoaea (PNA) stained them only after the removal of sialic acid. Human fetal choroid plexus cells at 8 weeks gestation already showed the same lectin-binding patterns as adult ones. All CPNs were stained by RCA-I and Con A in a similar manner as the normal choroid plexus cells. Although seven CPNs were positive for LFA, two CPCs were not stained by LFA, which bound to sialic acid. Two LFA-positive CPPs were stained by PNA before the removal of sialic acid. Moreover, unlike the normal choroid plexus cells, Ulex europaeus-, Glycine maximus- and Dolichos biflorus-binding sites often appeared, and WGA-binding sites of three CPNs remained even after sialic acid removal. In conclusion, the glycosialylation in normal choroid plexus cells was completed during the early embryonic stage. The lectin-binding patterns of CPNs were heterogenous in each case. The alternation of the glycosialylation and/or acquisition of binding sites for some lectins was sometimes observed through a neoplastic transformation.
A case of choroid plexus neoplasm histologically composed of tubular structures lined by a layer of cuboidal epithelium is reported. The neoplasm was located in the fourth ventricle of a 26-year-old man. The intense positivity for antisera anti-vimentin, anti-cytokeratin, anti-EMA and anti-S100 protein exhibited by these cells was consistent with choroid plexus origin. The patient is alive and in good health 5 years after surgery. The lesion represents a benign choroid plexus neoplasm not previously reported. The name "tubular adenoma of choroid plexus" is suggested for this variant.
Choroid plexus neoplasms are rare epithelial tumors of the central nervous system. A carcinoma of the choroid plexus occurred in a child from a family with the breast cancer-sarcoma syndrome (Li-Fraumeni or SBLA syndrome), an inherited condition characterized by the development of diverse neoplasms (sarcoma, breast cancer, brain tumors, leukemia, adrenal cortical carcinoma, and others). Choroid plexus carcinomas were identified in two kindreds previously reported with the syndrome. The literature contains reports of choroid plexus neoplasms occurring in families and in individuals with multiple primary tumors. Choroid plexus neoplasm may be a manifestation of the inherited proclivity to tumor development in the breast cancer-sarcoma syndrome.