Concomitant intraventricular colloid cyst and low-grade astrocytoma of the brainstem in a 16-year-old boy

Multiple primary brain tumors are commonly observed in patients with a history of brain radiation therapy or neurofibromatosis. The concomitant presence of 2 different types of brain tumors in a single location or chamber is a very rare clinical presentation in the absence of such a predisposing factor. The authors report on the case of a 16-year-old boy presenting with different types of brain tumors in 2 ventricular chambers concomitantly. This boy had a medium-sized colloid cyst of the third ventricle and a large fibrillary astrocytoma fungating from the brainstem into the floor of the fourth ventricle. The lesions were successfully excised in 2 separate surgeries. Radiotherapy was used as the adjuvant mode of therapy. There has been no sign of tumor recurrence after 16 months of follow-up. Clinical awareness and recognition of such a combination of tumors is important because they will dictate special treatment strategies depending on the individual biological aggressiveness of each tumor.     

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.     

Cyclophosphamide for the treatment of progressive low-grade astrocytoma: a Pediatric Oncology Group phase II Study.

PURPOSE: Results of a phase II trial of cyclophosphamide (CPM) for children with progressive low-grade astrocytoma are reported. PATIENTS AND METHODS: Fifteen patients with a median age of 39 months (range, 2 to 71) were included in this study. The tumors of 11 children were located in the optic pathway, hypothalamus, or thalamus. Four courses of intravenous CPM 1.2 g/m2 were administered every 3 weeks during the upfront window portion of this protocol. Subsequently, chemotherapy was to continue with CPM, vincristine, and carboplatin for 2 years. RESULTS: By study design, the first 14 patients were centrally reviewed after completion of the initial 4 CPM courses. Toxicity was primarily hematologic. One patients had a complete response, 8 had stable disease, and 5 had progressive disease (PD). The excessive number of children with PD prompted study closure. CONCLUSION: CPM as used in this protocol showed insufficient activity against astrocytoma to justify further patient accrual.     

Wilms' tumor detection in patients with sporadic aniridia. Successful use of ultrasound

Eleven patients with sporadic aniridia have been followed up for periods of time ranging from eight months to ten years. The initial renal evaluation included an intravenous pyelogram and ultrasound. Ultrasound evaluation was then performed every four to six months until 8 years of age. After 8 years of age, evaluation was performed every six to 12 months. Two of 11 patients were found to have Wilms' tumors. Ultrasound was sensitive enough to find small lesions prior to extension from the kidney. Ultrasound evaluation permits early detection without repeated radiation, sedation, or contrast and offers an easy technique for follow-up of patients with sporadic aniridia who have a high likelihood of developing a Wilms' tumor.     

Desmoplastic infantile astrocytoma and characteristics of the accompanying cyst. Case report

A desmoplastic infantile astrocytoma (DIA) is an extremely rare tumor that comprises a solid astrocytic tumor accompanied by a large cyst and involves the superficial cerebral cortex and leptomeninges in infants. The solid part of this type of tumor has been well described in various reports and books, but characteristics of the cystic portion have remained unclear. Because adequate resection is required to ensure a favorable prognosis, information about the cyst is very important for diagnostic purposes and surgical planning. The authors report on the clinical and histological features of the cyst in a case of a DIA. A 12-month-old boy presented with vomiting. Contrast-enhanced magnetic resonance imaging revealed a strongly enhancing single-lobed large cyst located in the deep white matter, under the solid part of the tumor attached to the dura mater of the left frontal lobe. Both the solid and cystic portions of the tumor were surgically removed. The border between the cyst wall and surrounding white matter was unclear. Histologically, the cyst wall was composed of gliosis representing a rough accumulation of reactive astrocytes, lymphocytes, and small capillary vessels in edematous parenchyma, but no tumor cells. The present case and previous reports suggest that the cyst does not contain tumor cells, even if strongly depicted on contrast-enhanced neuroimaging, and that a thickly enhancing cyst wall indicates gliosis with accumulation of numerous small vessels.     

Does chemotherapy have a role in low-grade astrocytoma management? a report of 13 cases

Surgery is the treatment of choice for low-grade astrocytoma while radiotherapy is carried out only when total resection is not possible. This study assessed the effectiveness of chemotherapy in nonresectable cases. Thirteen children with nonresectable astrocytoma were treated with carboplatin and etoposide and after four cycles the response to treatment was evaluated according to radiologic criteria. The results were: one with complete response (CR), three with minor response (MR), six with stable disease (SD), and three with progressive disease (PD). Moreover, in 77% there was an improvement in the neurologic picture. In particular, two cases with hypothalamic astrocytoma showed a regression of the diencephalic syndrome following chemotherapy. In six cases chemotherapy was carried out, at reduced dosage, after the first four cycles either because there was clinical improvement or because it was necessary to postpone radiotherapy in very young patients. After a follow-up period ranging between 11 and 63 months (average: 30 months), nine of the 13 patients are alive (69%) while four died of disease progression. Further studies would be useful to evaluate the role of chemotherapy in the management of low-grade astrocytoma. © 1995 Wiley-Liss, Inc.     

Successful use of danazol for hereditary angio-oedema.

A 10-year-old boy with severe hereditary angio-oedema was given danazol, 200 mg each day. There was a pronounced reduction in the frequency and severity of oedema.     

Successful use of rib grafts for cranioplasty in children

Numerous materials are available for use in cranioplasty including bone, plastics and metals. Rib grafts as a construct for cranial reconstruction offer several advantages: autologous bone source, a formable platform, low infection, regeneration at the donor site and high fusion rates. Criticism of rib graft cranioplasty includes scarring and pain at the donor site, irregular contour at the graft site and graft reabsorption. Since 1988, we have performed rib autograft cranioplasty on 13 patients. Seven females and 6 males combined for an average age of 6 years (11 months to 20 years) at the time of surgery. The mean follow-up from surgery is 27 months (2-48 months), with 11 subjects having at least 12 months. The commonest reasons for cranial reconstruction were: post-traumatic calvarial defect (n = 4), defect from previous craniotomy (n = 3), "growing" skull fracture (n = 2), and defect from previous encephalocele closure (n = 2). Mean defect size was 41 cm(2) (8-144 cm(2)) and average number of ribs harvested was 1.75 (1-3). Simple rib cranioplasty had a mean time in the operating room of 6 h and 30 min. No donor site complications were noted (pneumothorax, significant post-operative pain) and no post-operative infections were encountered. Excellent cranial contour was achieved in each of the 11 patients followed for a minimum of 12 months. One subject required staged reconstructive procedures owing to the size of the defect.     

Successful management of rhabdoid tumor of the liver

A 3-year-old male was referred because of fever, abdominal pain, and enlarged abdomen. Magnetic resonance imaging showed a very large lobulated mass involving predominantly the right lobe of liver. Tumor histology was consistent with rhabdoid tumor of the liver. The patient received 3 cycles of chemotherapy consisting of ifosfamide, carboplatin, and etoposide alternating with vincristine, adriamycin, and cyclophosphamide, at 3-week intervals. Follow-up magnetic resonance imaging revealed approximately 84% decrease in size of tumor after 2 cycles of chemotherapy. Patient underwent liver transplantation, as the tumor was unresectable. Six weeks posttransplant, the patient received 4 more cycles of chemotherapy. The patient is free of disease at evaluation 3 years posttransplant.     

Successful sleeve lobectomy of pediatric inflammatory myofibroblastic tumor

Inflammatory myofibroblastic tumor (IMT) is an uncommon solid tumor that was originally described in the lung. A 4‐year‐old girl was admitted to hospital with urticarial rash. On chest radiographs, an opacity was seen in the inferior zone of the left lung, and computed tomography showed a mass in the left lower lobe. Left lower sleeve lobectomy was performed, and the diagnosis was confirmed as IMT. Sleeve resection is the best option in lesions located in the mainstem bronchus or secondary carina. Herein, we present a rare case of IMT of the lung that was successfully treated with sleeve lobectomy. There have been fewer than 15 childhood cases of IMT reported in the literature, and the present 4‐year‐old patient is one of the youngest.     

Successful radiation therapy for supratentorial primitive neuroectodermal tumor and epidermolysis bullosa simplex

Epidermolysis bullosa simplex (EBS) is a heritable skin disorder characterized by skin fragility and blistering. While its most severe variant, dystrophic epidermolysis bullosa (DEB) is associated with squamous cell carcinoma (SCC), the development of extracutaneous neoplasms in EBS is extremely rare. We report a novel case of supratentorial primitive neuroectodermal tumor (sPNET) in a 7‐year male with EBS. Experience of radiation therapy and its challenges in children with EBS has rarely been reported. Pediatr Blood Cancer 2010; 54:170–172. © 2009 Wiley‐Liss, Inc.     

Successful chemotherapeutic decompression of epidural malignant germ cell tumor

A patient with a sacrococcygeal malignant germ cell tumor developed epidural spinal cord compression syndrome 32 days after surgical resection of tumor. Rapid resolution of symptoms was observed after chemotherapy with vinblastine, bleomycin, and cisplatinum. There was no radiation therapy or laminectomy for spinal compression. The patient had an excellent neurological recovery without any sequelae from spinal compression. Chemotherapy alone is an excellent approach to the management of epidural compression by malignant germ tumor.     

Successful chemotherapeutic treatment of diencephalic syndrome with continued tumor presence

A 7-month-old infant with typical features of diencephalic syndrome (DES) associated with a hypothalamic mass, most probably a glioma, was treated with chemotherapy. The tumor showed clear shrinkage, but after more than 2 years regrowth was noted. During the treatment period the child regained normal growth and became free of symptoms. As radiation therapy, especially at a young age, has significant adverse effects and a neurosurgical approach to the diencephalic region also has the potential to cause significant sequelae, a chemolherapeulic option, when it exists, is preferred. Thus, in an infant in whom a glioma is suspected to be the cause of the DES, based on the clinical picture and the neuroimaging appearance, chemotherapy should be considered the primary therapeutic modality. Even if its effect is temporary, its use is well justified. The most appropriate treatment protocol still needs to be determined.     

Successful parental use of an automated external defibrillator for an infant with long-QT syndrome

Congenital long-QT syndrome with 2:1 atrioventricular block presenting in the perinatal period is rare, has a poor prognosis, and leads to high risk for lethal ventricular arrhythmic events. An implantable cardioverter-defibrillator seems to be the most effective treatment in the prevention of arrhythmic sudden cardiac death in patients with long-QT syndrome. Technical limitations and risks associated with implantable cardioverter-defibrillators in asymptomatic infants is considered too great to justify use for primary prophylaxis against sudden cardiac death. In this case report we describe the first successful parental use of an automated external defibrillator prescribed for primary prophylaxis against sudden cardiac death in an infant with long-QT syndrome.