Large choroid plexus teratoma: A rare cause of congenital hydrocephalus.

Teratomas form the most common type of congenital brain tumors, frequently presenting as stillbirth. The largest neonatal series of intracranial teratomas reported a 12% survival rate. Although the first teratoma of the lateral ventricle was reported in 1961 by Maier, neonatal intracranial teratoma of the lateral ventricle is an extremely rare entity. We report here a large intracranial poorly differentiated teratoma arising from choroid plexus of lateral ventricle. This typically presented at birth with a large congenital hydrocephalus.

Extraneural metastases in anaplastic ependymoma.

Ependymoma are rare glial neoplasm, it rarely metastasize outside the central nervous system. We present a case of anaplastic ependymoma with extraneural metastases with review of literature. A ten-year-old male child presented with anaplastic ependymoma of choroid plexus and treated with craniospinal radiotherapy in 1998. He had intracranial recurrence in 2004, confirmed by biopsy. He was given adjuvant chemotherapy in form of PCV. At 10 months after completion of chemotherapy, he developed extracranial scalp metastasis and so was treated with palliative local radiation therapy to the scalp metastasis and systemic chemotherapy with oral Etoposide. Scalp metastasis completely disappeared and ataxia improved. After five cycles of chemotherapy, the patient had progression of disease in form of scalp and cervical lymph node metastasis confirmed by fine needle aspiration cytology, biopsy and immunohistochemistry. He was given salvage chemotherapy (carboplatin + ifosfamide + etoposide) at 3-weekly. He had partial response and was still on chemotherapy till May 2007.

Papillomas and carcinomas of the choroid plexus: histological and immunohistochemical studies and comparison with normal fetal choroid plexus

CONTEXTO: Os tumores do plexo coróide são raros. Os resultados de dados imuno-histoquímicos são escassos e controversos, o mesmo valendo para o plexo coróide normal. MÉTODO: Treze casos de tumores do plexo coróide e cinco exemplares de plexo coróide fetal normal foram submetidos a estudo imuno-histoquímico, utilizando-se marcadores para antígenos epiteliais, neurais e estromais. RESULTADOS/CONCLUSAO: Os achados histológicos mais relevantes foram células claras em 3/5 papilomas (PP) e 7/8 carcinomas (CA) e em todos os 5 plexos fetais; células rabdóides, desmoplasia e proliferação vascular foram encontradas, respectivamente, em 3, 4 e 5 casos de 6 CA pouco diferenciados, mas não nos PP e CA bem diferenciados. A pancitoqueratina AE1/AE3 foi fortemente positiva em todos os 13 casos, mesmo no componente indiferenciado do CA pouco diferenciado, em que a reatividade foi focal em 3 casos e difusa em outros 3. A citoqueratina de baixo peso molecular (35bH11) não foi expressa em nenhum dos 8 CA, mas estava presente em todos os 5 PP. Em 4/6 CA pouco diferenciados houve reatividade para actina de músculo liso (1A4) em 10-30% das células. Este achado ocorreu também em um caso sem células rabdóides. Laminina não foi detectada em nenhum dos 6 CA pouco diferenciados, mas estava presente em 4 PP e em 2 CA bem diferenciados. Todos os 5 plexos fetais expressaram GFAP.

Choroid plexus papillomas of the cerebellopontine angle.

The cerebellopontine angle (CPA) is a rare site for the growth of choroid plexus papilloma (CPP). The clinicoradiological diagnosis of this tumor in the CPA is difficult because of the nonspecific clinical presentation and radiological features. Five cases of choriod plexus papilloma (3 males, 2 females) operated upon at this center are reviewed. All the cases were operated upon by retromastoid suboccipital craniectomy. As they all presented with a typical CPA syndrome without any distinctive radiological feature, a clinicoradiological diagnosis of CPP could not be reached in any of these. The diagnosis of CPP could only be suspected at the operation table and established on histopathological examination. Two patients developed recurrences at the primary site following surgery. One patient developed recurrence twice despite gross total excision of tumor in each sitting. Subsequently, this patient remained symptom free for a follow-up period of 1 year. Another patient developed recurrence 2 years following surgery, but he died due to septicemia and aspiration pneumonitis. Therefore definitive surgery could not be performed. Radiotherapy was offered to one of the patients having residual mass post operatively, to render her symptom free for a 4 year follow-up. The remaining two patients have also showed progressive improvement in their symptoms following surgery for 4 years on follow-up. Hydrocephalus was a common feature in all the cases preoperatively, but only one required shunt CSF diversion, because of rapid deterioration in visual equity. In all other cases, hydrocephalus was managed conservatively. Surgery remains the main modality of treatment for CPP, both for primary and recurrent tumors, but radiotherapy may have a role in cases of recurrence, which are quite frequent.

Choroid plexus papilloma of the fourth ventricle.

A 14 year old girl presented with a 6 months' history of headache with vomiting, ataxia and cerebellar signs. CT and MRI revealed obstructive hydrocephalus secondary to a large fourth ventricular mass which enhanced markedly on contrast administration. Histopathology of the resected mass revealed papillary structures lined by cuboidal cells confirming choroid plexus papilloma.

Two Primary Intracranial Tumors of Different Histology: Report of a Case with a Choroid Plexus Papilloma and a Concurrent Vestibular Schwannoma in the Cerebellopontine Angle

A very rare case of multiple primary intracranial tumors is reported. A 41-year-old female patient was referred for surgery with a cerebellopontine angle (CPA) tumor. Medical history and MRI study showed typical findings of a right acoustic neuroma with a hydrocephalus. Neurological, dermatological, and ocular examinations revealed no evidence of neurofibromatosis. During surgery, a red-colored cauliflower like mass was found in the right CPA. The roof of the fourth ventricle could be seen through the lateral recess after removal of the tumor. Another mass, a 1.5-cm sized schwannoma protruding through the right internal auditory meatus, was removed by the transmeatal approach. Although the tumor masses were in contact and compressed against each other, there was a clear demarcation between them. Histological examination confirmed that the first mass was a typical choroid plexus papilloma with fibrovascular core, and that the second was a schwannoma. The patient recovered without any new neurological deficit. Result of a Medline search indicated that this rare combination of multiple primary tumors has not been reported previously.

Carcinoma de plexo coróide: relato de quinze casos / Choroid plexus carcinoma: report of 15 cases

O carcinoma de plexo coróide (CPC) é um raro tumor do sistema nervoso central (SNC), derivado do epitélio de revestimento dos plexos coróides, que acomete pacientes abaixo dos 3 anos de idade. Apresentamos um estudo clínico, epidemiológico e histopatológico de 15 casos de CPC. Destes, 10 eram do gênero masculino. As idades variaram de 4 meses a 21 anos. Quanto a localização, acometeram predominante o ventrículo lateral (73,3 por cento). Os sinais e sintomas predominantes foram hidrocefalia (62,5 por cento), hipertensão intracrania (25 por cento) e crise convulsiva (12, por cento). Os pacientes foram submetidos a tratamento cirúrgico com ressecção parcial em 75 por cento dos casos e completa em 25 por ento. Houve um óbito per-operatório. Em 85,7 por cento dos pacientes houve recidiva tumoral, com evolução a óbito em média 13,6 meses após o diagnóstico. Apenas um paciente encontra-se vivo num seguimento de 5 anos. Esses dados suportam o prognóstico sombrio e a alta mortalidade do CPC.