Pediatric Chordoid Glioma withChondroid Metaplasia

Chordoid gliomas are uncommon primary brain tumors that arise in the region of the third ventricle. Reports of this entity to date have been limited to adults. We present a case of a chordoid glioma arising in the hypothalamic/third ventricle region of a 12-year-old male who presented with visual symptoms. The neoplasm consisted of cords and clusters of well-differentiated, spindled-to-rounded cells containing abundant eosinophilic cytoplasm within a prominent mucinous matrix. Unlike other chordoid gliomas, this lesion contained islands and sheets showing cartilaginous differentiation intermixed with the glial component. A graded transition between neoplastic glial and chondroid regions was evident, and cells in both regions were strongly immunoreactive for GFAP and S-100. Cartilaginous metaplasia is infrequent in gliomas, but occurs most often in pediatric neoplasms of the midline such as this chordoid glioma. Thus, chondroid metaplasia represents an unusual histopathologic feature of chordoid glioma—in this case, presenting in a child. Received March 30, 2001; accepted May 31, 2001.     

Third ventricular ependymal cyst presenting with acute hydrocephalus

Ependymal cysts are generally located in the cerebral parenchyma but rarely found in the third ventricle. A 4-year-old boy presented with headache, vomiting, and upward gaze palsy. His consciousness gradually deteriorated in the course of 6 h. A magnetic resonance imaging study disclosed dilation of the lateral ventricle and a cystic mass in the third ventricle. We performed an endoscopic resection of the cyst wall. The cyst originated on the lateral wall of the third ventricle and obstructed the aqueduct. Histological examination confirmed a diagnosis of ependymal cyst. The patient recovered quickly and his headache and nausea disappeared. Third ventricular ependymal cysts are a rare cause of acute hydrocephalus but an important differential diagnosis. Their neuroendoscopic resection can resolve disturbances in cerebrospinal fluid circulation, is useful for cyst wall removal, and appears to be superior to shunt placement.     

Pituitary apoplexy due to prolactinoma in a Taiwanese boy: patient report and review of the literature

We report a Taiwanese boy who presented with apoplexy of a prolactinoma. A 12 9/12 year-old boy presented to our clinic with headache and visual deficit of bitemporal hemianopsia. Skull X-ray showed an enlarged sella. Magnetic resonance imaging (MRI) of the sella turcica showed a 4 x 2.5 x 2.5 cm mass, located at the sella turcica and extending upward to compress the optic chiasm. Preoperative laboratory data showed hyperprolactinemia, hypothyroidism and hypocortisonism. After a stress dose of i.v. hydrocortisone was given, he underwent transsphenoid surgery to remove the tumor. Immunohistochemical stains were positive for PRL in the tumor cells. After surgery, he suffered from neurogenic diabetes insipidus, hypopituitarism and hyperprolactinemia, with serum PRL level of 491 ng/ml. Visual field examination was normal 4 months later. In conclusion, pituitary apoplexy is rare in children but should be considered if a patient suffers from headache, vomiting, and visual deficit. Brain MRI is preferred for diagnosis. Dopaminergic agonists should be given if residual tumor or recurrence of prolactinoma is found after transsphenoidal surgery.     

Pseudotumoral acute hemicerebellitis in a child

Acute cerebellitis is an inflammatory disorder usually involving both sides of the cerebellum and presenting with localized signs such as ataxia. Hemicerebellitis is extremely rare in children and may clinically and radiologically resemble a tumor. There are very few reports of hemicerebellitis needing decompressive surgery. We report a case of hemicerebellitis in a 15 year old child presenting with severe headache but no cerebellar symptoms whose brain CT revealed an ill-defined mass compressing the fourth ventricle (pseudotumoral). MRI of the posterior fossa revealed a swollen left cerebellar hemisphere, supra-tentorial hydrocephalus and tonsil herniation, without any cerebral or brain stem lesions. Due to worsening symptoms she required a decompressive craniectomy, and the biopsy revealed an inflammatory process, possibly viral. At follow-up she was asymptomatic and the MRI revealed only slight cerebellar atrophy. This case had an atypical clinical presentation and illustrates that though a self-limited evolution is expected, surgical intervention may be needed in acute cerebellitis.     

Multicentric osteosarcoma presenting as retrobulbar mass: a diagnostic enigma

Osteosarcoma is the most common primary malignant bone tumor of children and adolescents. It often presents as a solitary lesion; multicentric osteosarcoma with synchronous lesions occurring at multiple skeletal sites is very rare. We report a 9-year-old boy with multicentric osteosarcoma who presented with a left retrobulbar non-sclerotic mass. The multiple lesions in bone were mostly non-sclerotic on radiological examination except for a single lesion in the left tibia. Biopsy of the retrobulbar mass showed an unclassifiable poorly differentiated malignant tumor. Marrow aspiration smears showed many large, often segregated, round cells that expressed NB84a. However, trephine biopsy showed the formation of tumoral osteoid by the malignant cells, finally permitting the definitive diagnosis of osteosarcoma to be made. A hypertetraploid clone with complex structural abnormalities was demonstrated by cytogenetic study.     

Intracranial metastasis of Wilms' tumor involving the tectal plate without pulmonary involvement. Case report.

Intracranial metastasis without pulmonary involvement of Wilms' tumor is very rare, and most previously reported metastatic sites have been in the cerebral parenchyma. We experienced a rare case of metastasis of Wilms' tumor in the tectal plate without pulmonary involvement. A 3-month-old boy was admitted to our hospital due to hydrocephalus. After insertion of a ventriculoperitoneal shunt, there were no neurological deficits. Five months after the operation, a mass 5 cm in diameter extending from the tectal plate into the third ventricle was found. A mass 10 cm in diameter within the retroperitoneal space, which occupied the right kidney, was also found. Both tumors were resected and diagnosed histologically as Wilms' tumor. Despite every effort including chemotherapy and radiotherapy, the patient died at 14 months old. The majority of children with hydrocephalus showing thickening of the tectal plate would most likely have tectal glioma; however, this unusual case report reminds us that metastasis of Wilms' tumor can occur in the tectal plate causing hydrocephalus.     

Inflammatory myofibroblastic tumor of the lung: a rare cause of atelectasis in children

Although rare, inflammatory myofibroblastic tumor is the most common primary lung mass in children. We report the case of an 11-year-old boy investigated for persistent cough and dyspnea with complete left lung atelectasis mimicking pneumonia. CT and MRI showed an endobronchial mass of the left main bronchus. The boy underwent endoscopic resection of the tumor and histology was in favor of an inflammatory myofibroblastic tumor of the lung. This diagnosis should be suspected in children with recurrent pneumonia. The prognosis is good after complete resection.     

Collision tumor of meningioma and malignant astrocytoma

The authors report a 12-year-old boy who was presented with headache, nausea, vomiting and seizure. His magnetic resonance imaging showed 2 adjacent tumors in the region of the left ventricular trigone. The pathology of tumors reported collision tumors composed of meningioma and malignant astrocytoma.     

Anaplastic astroblastoma presenting as massive, sudden-onset, intraparenchymal hemorrhage

Astroblastoma is a rare primary glial tumor of children and young adults, typically located in the cerebral hemispheres and presenting as a well-circumscribed, nodular, often cystic enhancing mass. The existence of astroblastoma as a distinct clinicopathologic entity has long been debated but is recognized in the 2007 WHO classification of CNS tumors. The grading of these tumors remains unsettled. Currently, no grade has been established and there are no clearly defined diagnostic criteria for low- or high-grade tumors. Astroblastomas in general are thought to have a favorable outcome with low rates of recurrence following gross total resection. We report a case of astroblastoma with 'high-grade/anaplastic' histology in a 12-year-old, previously healthy girl. The patient experienced severe, sudden-onset headache, and quickly became comatose. Head CT showed a massive intraparenchymal hemorrhage in the right frontal lobe with surrounding edema. She underwent emergent posterior frontal craniotomy for decompression and hematoma evacuation. Pathologic examination revealed a sharply demarcated hemorrhagic heterogeneous glial tumor with focal papillary architecture, densely hyalinized blood vessels, and intensely GFAP-positive perivascular cells. The tumor showed unequivocal high-grade features including an elevated proliferative index. The diagnosis of anaplastic astroblastoma was rendered. The patient expired on postoperative day 30. This case illustrates the potential poor outcome of high-grade astroblastoma and highlights the morphologic heterogeneity of this rare neoplasm.     

Double Discordance in the Hypoplastic Left Heart

A neonate was transferred for an intracardiac mass. Initially, the mass was mistaken for a hypoplastic right ventricle, a thrombus, or a tumor. Only a thorough segmental and sequential analysis showed double discordance with a hypoplastic left ventricle. No such entity has yet been described. Electronic Supplementary Material The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

A large planum sphenoidale meningioma with sinonasal extension in a child. Case report and review of the literature

Anterior cranial base meningiomas are rare tumors in children. Due to the extensive involvement of orbit, paranasal sinuses, midface, and anterior skull base, a multidisciplinary approach is warranted. We present a case of a child with a large planum sphenoidale meningioma extending into subfrontal region, ethmoid and maxillary sinuses inferiorly, and orbits laterally. The patient, a 4-year-old girl, presented with long-standing nasal stuffiness and swelling of the midface. An extended frontobasal approach through a bifrontal craniotomy was used to resect the intracranial portion of this mass. The tumor had eroded through nasal septum, medial orbital walls, and left maxilla, structures which were not readily accessible from a cranial approach alone. A modified Weber-Ferguson incision was used for a transfacial approach to resect the residual mass below the skull base. The advantages of combining the bifrontal craniotomy with a transfacial split provided the added exposure to maximize the extent of resection.     

Intracranial endodermal sinus tumors associated with growth hormone replacement therapy in a girl

The primary intracranial endodermal sinus tumor (EST) is regarded as a rare histological subtype that is often associated with components of other germ cell tumors, and there are no reports on the onset of intracranial ESTs after growth hormone (GH) replacement therapy. The authors report an extremely rare case of pure primary EST associated with GH replacement therapy. A 15-year-old girl with GH deficiency experienced headache, nausea, and vomiting after GH replacement therapy for a 17-month period. Magnetic resonance imaging showed 2 tumor masses located in the pineal region and frontal horn of the right lateral ventricle, respectively. Before surgery, the authors administered 1 cycle of neoadjuvant chemotherapy, which shrank the tumor and facilitated surgical intervention. The larger mass located in the pineal region was removed via a right occipital transtentorial approach, and postoperative histopathological analysis revealed a pure EST. While there is a clear association between the initiation of GH replacement therapy and the development of the EST in this case, the causal effect cannot be specified. Nevertheless, this case demonstrates that GH replacement therapy must be used cautiously.     

Intradiploic orbital roof meningioma with pneumosinus dilatans in a child: a case report and review of the literature

Intradiploic meningioma of the orbital roof is a type of intraosseous meningioma. It is very rare in children, and only 7 cases have been reported in the pediatric age group. We report a case of a 14-year-old female who presented with progressive exophthalmos, diplopia and pain involving the right eye. Computed tomography (CT) scan and magnetic resonance imaging (MRI) revealed an intradiploic orbital roof tumor with diploe expansion and pneumosinus dilatans. The tumor was completely removed surgically. The histopathological diagnosis was transitional meningioma. The clinical and radiological findings of intadiploic meningioma are discussed with a review of the relevant literature.     

Neonatal pulmonary blastoma

A thoracic mass was identified on a routine ultrasound (US) examination in the 30th week of gestation. A postnatal chest radiograph, US, and computed tomography demonstrated a well-delineated, solid, cystic tumor in the left lower lobe. The mass was excised and a diagnosis of pulmonary blastoma (PB) was made on pathologic examination. The patient is alive and free of disease 8 years after the operation. PB is a rare primary neoplasm of the lung, adults being more frequently affected than children. This case appears to be the third neonatal PB reported and one of the few long-term survivors.     

Fronto-orbitonasal intradiploic meningioma in a child

Intradiploic meningioma, which may be classified as a subgroup of intraosseous meningioma, is a rarely encountered disorder. To date, less than 10 cases have been reported. Here, we report a case of fronto-orbitonasal intradiploic meningioma. A 12-year-old female with exophthalmos and diplopia was operated on for a cranial intradiploic mass lesion. Histopathological evaluation of the specimen confirmed the diagnosis of intradiploic psammomatous meningioma. Her exophthalmos did not change, but the diplopia disappeared. This case is unique in that it is an extensive case of intradiploic meningioma of the orbital roof and frontal base in a child. Intradiploic meningiomas generally are of psammomatous type. Especially tumors adjacent to the orbita cause exophthalmos; cases located on the other side of the calvarium may not cause any symptom or sign other than headache or sometimes a mass on the scalp. Treatment, as with meningiomas located in the intracranial cavity, is total resection of the lesion.     

Juvenile papillomatosis of the breast in a 9-year-old girl

 Juvenile papillomatosis (JP) is a rare benign, proliferative breast tumor in children. We observed a 9-year-old girl with a large soft-tissue mass at the lower outer quadrant of the left breast. Ultrasonography showed an ill-defined, inhomogeneous mass with numerous small, hypoechoic areas. The tumor was completely excised. Histopathology revealed JP of the breast. The case is presented and the literature is reviewed. Accepted: 31 January 2000     

Childhood choroid plexus neoplasms

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.     

ANAPLASTIC LARGE CELL LYMPHOMA WITH PRIMARY INVOLVEMENT OF SKELETAL MUSCLE: A Rare Case Report and Review of the Literature

Primary skeletal muscle ALCL is very rare. Here the authors report a case of skeletal muscle ALCL that was proven pathologically. A 14-year-old boy presented with a persistent fever, chills, night sweats, headache, and significant weight loss. A CT scan of the abdomen showed a hazy mass about 3.2 × 1.2 cm in his left sacrospinalis. Ultrasonography revealed a low-echo and irregular mass in the left lumbar muscle measuring 8 × 1.4 × 3.6 cm in size and a similar mass 8 × 3.5 × 3.7 cm in size in the femoral muscle of the left thigh. MRI demonstrated an abnormal mass signal 4 × 3 × 9 cm in size infiltrating the left sacrospinalis muscle. The biopsy specimen was taken from the femoral muscle of the left thigh at surgery. Histopathological examination revealed a diffuse infiltration of large and atypical cells with pleomorphic nuclei and abundant cytoplasm. Immunohistological staining showed these atypical cells were positive for CD30 (Ki-l), anaplastic lymphoma kinase (ALK), epithelial membrane antigen (EMA), CD3, CD45RO, and CD68. The morphology and immunophenotype were consistent with CD30-positive, ALK-positive, and ALCL of T-cell lineage. The patient's condition was diagnosed as CD30-positive primary skeletal muscle ALCL.     

Massive congenital intracranial immature teratoma of the lateral ventricle with retro-orbital extension: a case report and review of the literature

Germ cell tumors comprise 0.4-3.1% of all intracranial tumors, and teratoma constitutes 9-30% of them. Congenital intracranial tumors are very rare and only account for 0.5-1.5% of all childhood brain tumors. The most common type of these tumors present at birth is teratomas, which represent 0.5% of all intracranial tumors. Most teratomas are midline tumors located predominantly in the sellar and pineal regions. In this study, we report a neonatal intracranial immature teratoma at the lateral ventricle because of its rare location. A 5-day-old female neonate presented with a history of irritability and left eye protrusion since birth. A head computed tomographic (CT) scan and magnetic resonance imaging (MRI) disclosed a large tumor filling the left lateral ventricle and extending into the ipsilateral retro-orbital space. With left frontotemporoparietal craniotomy, a large grayish-white lobulated vascular mass was encountered, and total removal of tumor was performed. Histological examination revealed the diagnosis of immature teratoma. The prognosis of congenital intracranial immature teratoma is usually poor because the lesions are extensive when they are identified. Prenatal ultrasonography is necessary for the prenatal diagnosis. Fetal MRI should be made for the evaluation of intracranial tumor. If the tumor is detected before the 24th week of gestation, termination of the pregnancy should be considered.     

经小脑延髓裂入路显微手术治疗儿童第四脑室肿瘤

目的 探讨经小脑延髓裂(CMF)人路显微手术治疗儿童第四脑室肿瘤的方法,提高手术治疗效果.方法 对17例2～14岁原发第四脑室肿瘤患儿,采用枕下正中切口、横窦下颅骨开窗骨瓣成形及经小脑延髓裂入路显微切除肿瘤,其中广泛型CMF切开12例,外侧壁型切开3例,外侧隐窝切开2例;硬脑膜严密缝合或修补,骨瓣复位并固定.结果 肿瘤全切除15例,次全切除2例,病理结果髓母细胞瘤9例,室管膜瘤5例,星形细胞瘤3例,无围手术期死亡发生.术后15例患儿症状体征均明显改善或消失,无新发共济失调、震颤、肌张力降低及脑积水、小脑缄默综合征等并发症.复发2例,均为髓母细胞瘤,2年后死亡1例.结论 经小脑延髓裂人路能较好地显露儿童第四脑室肿瘤,可减少因切开小脑蚓部和向侧方牵拉损伤小脑所导致的并发症发生.