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Objectives: The prognosis in infants with brain tumors has hitherto been very poor. The purpose of the study was to collect and analyze information regarding the clinical presentation, diagnosis, and management of these patients and to assess the eventual prognosis regarding survival and response to treatment. Materials and methods: This study retrospectively reviewed the records of 22 infants with brain tumors at our institution between November 1995 and October 2009. Their medical records were retrieved for age at diagnosis, presenting features, location, histology, surgical procedures, adjuvant treatment, recurrence, and survival. Results: The patients included 18 boys and four girls. The median age at diagnosis was 3 months with a range of antenatal diagnosis at 36 weeks of gestation up to 11.9 months. The group included four patients with definite congenital tumors presented in the perinatal period. The common presenting signs included increased head circumference, seizure, and vomiting. Over half of the tumors were histologically benign; however, medulloblastoma/primitive neuroectodermal tumor is the most frequent tumor type, accounting for six patients. Surgical resection was attempted in 18 patients, and three of them died in early postoperative period. Cerebrospinal fluid diversion was required in 11 patients, and seven of these patients needed VP shunting. Four patients received adjuvant chemotherapy, but one of them subsequently received salvage radiotherapy. Conclusion: Because of the expandability of the skull, brain tumors in infants may have protean manifestations. Although pathology categorization was quite a variable in our study, three quarters have tangibly survived after current therapeutic modalities. [ABSTRACT FROM AUTHOR]

We investigated whether the intensity of thymidylate synthase (TS) staining in tissue samples obtained from gastric cancer (GC) patients undergoing gastrectomy could predict response to 5-FU-based adjuvant chemotherapy after gastrectomy. Clinicopathological features of 124 patients with histologically proven GC who underwent radical gastrectomy were retrospectively reviewed. Tissue samples obtained from these patients were immunohistochemically stained for assessing TS expression. We arbitrarily classified the TS staining results as low (<20% cytoplasmic immunostaining) and high (≥20% cytoplasmic immunostaining) TS expression. The clinicopathological features of the low TS expression group patients were typically similar to those of the high TS expression group patients. However, multivariate forward stepwise logistic regression analysis revealed that low TS expression was independently associated with females and responders to 5-FU-based adjuvant chemotherapy. The median follow-up duration for the 124 GC patients who had undergone curative resection was 41.3 months. The GC patients who showed poor tumor differentiation and high TS expression had short disease-free survival (DFS) and overall survival (OS). Low TS expression is significantly associated with female GC patients and responders to 5-FU-based adjuvant chemotherapy. It predicts longer DFS and OS in selected GC patients treated with 5-FU-based adjuvant chemotherapy after curative resection. The results suggest that prospective assessment of TS staining intensity in tissue samples obtained from GC patients undergoing gastrectomy would be useful to predict the patients who would be benefited from 5-FU-based adjuvant chemotherapy after gastrectomy. [ABSTRACT FROM AUTHOR]

Among patients with medulloblastoma, an unrare aggressive central nervous system (CNS) tumor found mostly in the posterior fossa, drop metastasis from primary location to anatomically lower sites including whole spinal cord is known as a potential way for tumor to spread. There were already lots of extensive discussions regarding diagnosis and treatment for such common neurologic complication in systemic cancer; however, fewer were found about medulloblastoma. This study aimed to reexamine the possible known prognostic factors, especially drop metastasis, and defined the influence of them. We retrospectively review a series of 36 patients who suffered from posterior fossa medulloblastoma and found leptomeningeal metastasis in 12 of them. Incidence, diagnosis, prognostic factors, and treatments for this common complication are discussed. The magnetic resonance images (MRI) of these patients were reviewed. The time sequence between primary surgical procedure and development of MRI evidences of drop metastasis was used for subgrouping. Log-rank test was used for survival analysis. By survival analysis, patients with late drop metastasis, defined as new radiologic evidence of leptomeningeal involvement after primary CNS procedures, were found to have significant survival difference to those with early metastasis defined as drop metastasis found at the time of diagnosis (log-rank test, p = 0.0047). Further stratification of patients within this group may help in evaluation of prognosis and development of different treatment strategies. [ABSTRACT FROM AUTHOR]

Abstract Object  The coexistence of spinal arteriovenous malformation (AVM) and a familial cerebral cavernous hemangioma (CCH) is extremely rare. Methods  A 9-year-old boy suddenly developed severe paraplegia and urinary dysfunction. Spinal magnetic resonance imaging (MRI) scan revealed a cervical and upper thoracic intramedullary lesion. Due to acute neurological dysfunction, the patient underwent emergency surgical exploration. An intramedullary vascular lesion was found and excised. Pathologically, AVM was noted. After the surgery, the boy was ambulatory with left lower limb stiffness. MRI scan of the brain revealed multiple cerebral cavernous hemangioma. Symptomatic multiple CCH in his mother and grandmother were also noted. Conclusions  We concluded that the presence of spinal AVM should be suspected if the patient with familial CCH develops the signs of space-occupying lesion of the spinal cord, facilitating early diagnosis of the spinal AVM. [ABSTRACT FROM AUTHOR]

Squamous cell carcinoma (SCC), the most common primary malignant tumor of the conjunctiva, has a variable clinical presentation and immunohistochemical profile. Abundant cell cycles exist, including MIB-1 (Ki67 antigen), p16, p53, and p63, within the conjunctiva SCC. This investigation first reports the expressions of cell cycle markers in SCC. A retrospective study was conducted between December 1976 and June 2004, comprising 13 consecutive patients with conjunctiva SCC who were treated with surgical excision. Detailed clinical parameters were also reviewed. Overexpression of MIB-1, p16, p53, and p63 genes were studied by immunohistochemistry. Genechip containing 39 subtypes was used to elucidate human papillomavirus (HPV). The study group contained 13 (100%) men, with a mean age of 68±18 years and follow-up period of 20±17 months. The sample included four (33%) SCC located in the left eye and two (17%) recurrent SCC. Overexpression of the p53 and p63 was considerably higher than that of the p16 (P<0.01). HPV DNA was not detected in any of the 13 cases. This work first examined the immunohistochemical overexpression of cell cycle (MIB-1, p16, p53, and p63) in SCC. This investigation then showed that the expression of cell cycles in SCC was associated with key tumor clinicopathological features. This approach can help distinguish the potential roles of cell cycle in the development of SCC. [ABSTRACT FROM AUTHOR]

Purpose: We conducted a pilot trial to evaluate the value of 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) in gestational trophoblastic tumours (GTTs). Methods: Patients with placental site trophoblastic tumour (PSTT), high-risk GTT (World Health Organisation score ≥8, disease onset at postpartum or greater than 6 months after antecedent pregnancy), metastatic GTT, recurrent/resistant GTT after chemotherapy, or post-molar GTT with unexplained abnormal β-hCG regression and patients undergoing re-evaluation after salvage treatment were enrolled. PET was undertaken within 1 week after computed tomography (CT). Clinical impacts of additional PET were determined on a scan basis. Results: A total of 14 patients were recruited. Sixteen PET scans were performed, with one patient having three serial studies. Benefits of additional PET were seen in 7 of 16 (43.8%) scans; these benefits included disclosure of chemotherapy-resistant lesions (n=2), exclusion of false-positive CT lesions (n=1), detection of an additional lesion not found by conventional imaging (n=1) in high-risk GTT at the start of primary chemotherapy, and confirmation of complete response to treatment for PSTT or to salvage therapy for recurrent/resistant GTT (n=3). On the other hand, in two instances there were false-negative PET findings, six scans yielded no benefit, and one showed an indeterminate lesion. Conclusion: Our preliminary results suggest that 18F-FDG PET is potentially useful in selected patients with GTT by providing precise mapping of metastases and tumour extent upfront, by monitoring treatment response and by localising viable tumours after chemotherapy. A larger study is necessary to further define the role of 18F-FDG PET in GTT. [ABSTRACT FROM AUTHOR]

Lipoblastoma/lipoblastomatosis is an uncommon benign lipomatous tumor affecting mainly infants and children. It presents as a painless mass located frequently in upper and lower extremities. The present study was conducted to characterize the clinicopathologic features of lipoblastoma/lipoblastomatosis in a Chinese population. A retrospective survey of 16 cases of pathologically confirmed lipoblastoma/lipoblastomatosis from 1988 to 2002 was performed. The available clinical information and histological sections were reviewed. The study involved 10 males and 6 females ranging in age from 5 to 49 months (median, 11.5 months). The patients presented with mass involving neck ( n=5), inguina ( n=3), feet ( n=2), arm ( n=1), leg ( n=1), thorax ( n=1), mesentery ( n=1), buttock ( n=1), and presacral region( n=1). The lesions were removed surgically. Histologically, the majority (11/16) of primary tumors were of diffuse type. Follow-up ranging from 5 to 125 months was available in 13 patients. Four patients experienced a recurrence of tumors in the neck ( n=2) and lower extremities ( n=2) 11–84 months after operation. The most common site of involvement of lipoblastoma/lipoblastomatosis in our series was the neck. A follow-up period of more than 3 years is recommended for patients with this condition. [ABSTRACT FROM AUTHOR]

The optimal postoperative management of pediatric intracranial ependymomas is controversial. We analyzed clinical prognostic factors for their influence on outcome in such children. Our retrospective series included 15 with supratentorial and 28 with infratentorial tumors. Twenty ependymomas were grade II, and 23 were anaplastic. Complete resection was performed in 18 patients, incomplete resection in 19, and stereotactic biopsy in 6. Radiotherapy was done in 31 patients and chemotherapy in 13. The surviving patients have been followed 8–232 months (median: 69 months). The median survival time was 30 months, and 5-year overall survival and progression-free survival rates were 53.9% and 45.9%, respectively. By tumor site: supratentorial, 56.6% and 50.9%; infratentorial, 52.3% and 42.5%. Multivariate analysis identified complete resection (5-year progression-free survival, 71.8%) and age <3 years old as significant favorable and adverse prognostic features (relative risk, 2.59; 95% CI, 1.05–6.38), respectively. Twenty-six children relapsed 1–107 months after diagnosis (median: 12 months). Relapses were local in 22 cases, and combined local and distant in three cases. Only one of 15 patients with supratentorial tumors developed isolated spinal metastasis. Failure at the primary site is the major obstacle to improve cure rates. The extent of surgical resection and age were the only statistically significant prognostic factors. [ABSTRACT FROM AUTHOR]

Cardiac hamartomas are very rare and are demarcated masses of enlarged, hypertrophied, mature myocytes and collagen tissue. Cardiac hamartomas are generally circumscribed in the right ventricle or atrium, but not reported in the crista terminalis (CRT). The CRT is crucial in electrophysiology, is related to arrhythmogenesis, and is targeted by radiofrequency catheter procedures. Previous works only described the benign natures of prominent CRT using non-invasive methods. This study describes an unusual cardiac hamartoma originating from the CRT and extending toward the tricuspid valve. Microscopically, this hamartoma comprised dense collagen and adipose tissue, mixed with hypertrophy, but with disarrayed cardiomyocytes. An irregular gap junction, connexin43, was demonstrated in this cardiac hamartoma. [ABSTRACT FROM AUTHOR]

We describe a case of primary intracranial medulla oblongata germinoma in a 16-year-old girl who presented with progressive headache and blurred vision. Magnetic resonance imaging demonstrated a heterogeneous exophytic mass arising from dorsal aspect of medulla oblongata with extension into fourth ventricle. The differential diagnosis for this patient had included ependymoma, exophytic glioma, medulloblastoma and choroid plexus papilloma. After surgical resection and radiation therapy, she remains alive and recurrence-free for 7 years. [ABSTRACT FROM AUTHOR]

Langerhans cell histiocytosis (LCH) involving the thyroid is very rare. It can be easily confused with far more common benign goiters or thyroid neoplasms. We report on a 5-month-old female patient presenting with progressive enlargement of an anterior neck mass. This patient underwent left subtotal thyroidectomy following which a diagnosis of isolated LCH involving the thyroid gland was confirmed. A course of chemotherapy was administered, and oral thyroxine replacement was initiated. Eighteen months after this treatment, the patient remained in complete remission. Following a thorough review of the literature, as best we are aware, this patient is the youngest individual suffering LCH who has been reported in the literature. In conclusion, isolated LCH of the thyroid is rare, and its diagnosis can be challenging for a clinician and typically requires appropriate awareness. Local excision is the treatment of choice, and prolonged follow-up is recommended. [ABSTRACT FROM AUTHOR]

Presents information on a case study regarding primitive neuroectodermal tumor of the brain in a girl with Turner syndrome diagnosed after 4 years of growth hormone therapy. Clinical condition of the patient; Histopathology of Turner syndrome; Effect of growth hormone therapy on serum concentrations of IGF-1.