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D Ashley Hill

Publications and source records attributed to D Ashley Hill.

At least 19 recordsLinked to original sources
PubMed2007

Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma.

BACKGROUND: Pleuropulmonary blastoma (PPB) is a rare tumor of pleura and lung in young children. Central nervous system (CNS) complications, particularly cerebral parenchymal metastases, occur in aggressive forms of PPB: Types II and III PPB. This article evaluates cerebral and meningeal metastases, cerebrovascular events (CVA) caused by tumor emboli, spinal cord complications, and intracranial second malignancies in PPB. PROCEDURE: International PPB Registry and literature cases were evaluated for CNS events. Cerebral metastasis patients were evaluated for gender, side of origin of PPB, PPB Type, interval from diagnosis to metastasis, status of chest disease, treatment, and outcome. Standard statistical methods were used to calculate the cumulative probability of cerebral metastasis and survival following metastasis. RESULTS: Thirty-nine cases of cerebral metastasis were identified in 5/53 Registry Type II cases, 15/44 Registry Type III cases, and 19/143 literature Type II/III cases. Metastases occurred 1-60, median 11.5 months after diagnosis. Chest disease was controlled in 50% of children at time of metastasis. The cumulative probability of cerebral metastasis by 5 years from diagnosis was 11% for Type II patients (95%CI (confidence interval): 2-20%) and 54% for Type III patients (95%CI: 31-76%). Seven children survive cerebral metastasis. Other CNS complications were post-operative CVA (five cases), spinal cord invasion or compression (six), leptomeningeal disease (three), and second intracranial malignancies (two). CONCLUSIONS: Cerebral metastasis is more frequent in PPB than in other childhood sarcomas. Clinicians should screen for this complication. Diverse other CNS complications are less common and require careful diagnosis.

Brain Neoplasms↗
PubMed2006

Type I pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry.

PURPOSE: Type I pleuropulmonary blastoma (PPB) is a rare, cystic lung neoplasm in infants characterized by subtle malignant changes and a good prognosis. Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. This article describes the first collection of type I PPB cases, analyzes outcome based on treatments of surgery or surgery plus chemotherapy, and presents type I PPB management recommendations. PATIENTS AND METHODS: Type I PPB cases from the International PPB Registry and literature were evaluated using standard statistical methods for outcomes based on age at diagnosis, sex, thoracic side, surgical extent, length of follow-up, constitutional/familial disease, pre-existing lung cysts, intrathoracic findings, and treatments (surgery or surgery and chemotherapy). RESULTS: Thirty-eight type I PPB cases were identified: Registry (n = 30) and literature (n = 8). Twenty children had surgery alone; eight (40%) experienced recurrence; and four died. Eighteen children had surgery and adjuvant chemotherapy; one experienced recurrence and died. All recurrences were type II or III PPB. Recurrence-free survival was higher in the surgery + chemotherapy group (P = .01); overall survival did not differ (P = .18). The improved recurrence-free survival was found only in males. Four of nine children with recurrence survived. CONCLUSION: Adjuvant chemotherapy appears to benefit type I PPB patients. Benefit limited to males requires broader substantiation. Salvage after types II and III recurrence is poor (four of nine; 44%). A rigorous surveillance schedule after type I PPB diagnosis might detect early recurrence and be an acceptable alternative to adjuvant chemotherapy.

Adolescent↗
PubMed2006

Preterm premature rupture of membranes: diagnosis and management.

Preterm premature rupture of membranes is the rupture of membranes during pregnancy before 37 weeks' gestation. It occurs in 3 percent of pregnancies and is the cause of approximately one third of preterm deliveries. It can lead to significant perinatal morbidity, including respiratory distress syndrome, neonatal sepsis, umbilical cord prolapse, placental abruption, and fetal death. Appropriate evaluation and management are important for improving neonatal outcomes. Speculum examination to determine cervical dilation is preferred because digital examination is associated with a decreased latent period and with the potential for adverse sequelae. Treatment varies depending on gestational age and includes consideration of delivery when rupture of membranes occurs at or after 34 weeks' gestation. Corticosteroids can reduce many neonatal complications, particularly intraventricular hemorrhage and respiratory distress syndrome, and antibiotics are effective for increasing the latency period.

Algorithms↗
PubMed2006

Familial association of pleuropulmonary blastoma with cystic nephroma and other renal tumors: a report from the International Pleuropulmonary Blastoma Registry.

OBJECTIVE: To characterize the association of pleuropulmonary blastoma (PPB) with cystic nephroma (CN) and other renal tumors. STUDY DESIGN: Complete clinicopathologic review of cases from the International PPB Registry and literature. RESULTS: We identified 18 patients with PPB associated with 20 renal tumors (15 CN), either in themselves or family members. All patients with PPB were <5 years of age. All but one of the renal diagnoses were made before 4 years of age. Eleven children had both PPB and renal tumor, one of whom also had a sibling with CN. Six children with PPB alone had one or more family members with CN. The mother of one child with PPB had Wilms' tumor. Pulmonary disease was bilateral in four patients. Renal disease was bilateral in three patients. Two children with PPB and bilateral renal cystic tumors also had intussusceptions because of small bowel juvenile polyps. In six families, dysplasia/neoplasia affected organs other than lung and kidney. CONCLUSIONS: CN or related tumors were found in 9.2% of 152 Registry-reviewed PPB cases. The occurrence of rare pulmonary and renal tumors together in patients and/or family members, the early age of onset, and the multiplicity of tumors is compatible with a constitutional genetic predisposition.

Child, Preschool↗
PubMed2005

The impact of early resection of primary neuroblastoma on the survival of children older than 1 year of age with stage 4 disease: the St. Jude Children's Research Hospital Experience.

BACKGROUND: It remains unclear whether primary tumor resection benefits patients with metastatic neuroblastoma. The authors assessed the impact of extent and timing of resection on outcome in these patients. METHODS: The authors reviewed the records of 124 patients > 1 year of age at diagnosis of International Neuroblastoma Staging System Stage 4 neuroblastoma. The survival estimates of those who did and did not have a gross total resection (GTR) and of those who had initial versus delayed GTR were compared. Surgical complications were reviewed. RESULTS: The 5-year survival estimates were comparable for the 90 patients who had a GTR and the 17 who underwent surgery but did not have a GTR (29.9% +/- 5.1% [standard error] vs. 29.4% +/- 10.1%). The 7 patients who underwent GTR at the time of diagnosis had a higher 5-year survival estimate than the 83 patients who had a GTR after induction chemotherapy (83.3% +/- 13.9% vs. 25.2% +/- 5.0%) (P = 0.001). Five-year event-free survival estimates were similarly higher in the initial-GTR group (57.1% +/- 18.7% vs. 14.5% +/- 4.2%) (P = 0.002). These two groups did not differ significantly in age at diagnosis (P = 0.118), site of primary tumor (P = 0.34), MYCN amplification status (P = 1), serum lactate dehydrogenase activity at diagnosis (P = 0.34), or treatment protocol (P = 0.22). Twenty-two (21%) patients had a surgical complication. CONCLUSIONS: In this small cohort of patients with metastatic neuroblastoma, GTR at the time of diagnosis offered a survival benefit. Further prospective studies are warranted before this approach can be applied to all patients with metastatic neuroblastoma.

Adolescent↗
PubMed2005

High telomerase RNA expression level is an adverse prognostic factor for favorable-histology Wilms' tumor.

PURPOSE: A primary objective of the fifth National Wilms Tumor Study (NWTS-5) was to identify prognostic indicators for patients with favorable-histology Wilms' tumor. The prognostic significance of telomerase expression level in primary tumor samples was assessed. PATIENTS AND METHODS: A case-cohort study was conducted involving 291 NWTS-5 registrants. Telomerase activity was measured using the telomeric repeat amplification protocol (TRAP). Expression levels of TERT mRNA (encoding the telomerase catalytic component) and TERC/hTR (the telomerase RNA template) were measured using quantitative real-time polymerase chain reaction. RESULTS: After excluding samples because of lack of viable tumor, RNA degradation, or insufficient clinical information, 244 patients remained for the final analysis (96 with relapse and 148 without relapse). Univariate analysis revealed a positive correlation between relative risk (RR) of relapse and levels of TERT mRNA and TERC expression. For each doubling in TERT mRNA and TERC level, the RR increased by a factor of 1.16 (95% CI, 1.04 to 1.29; P = .01) and 1.35 (95% CI, 1.11 to 1.64; P = .003), respectively. The one third of patients whose tumors had the highest TERC expression level had an RR of 2.06 (95% CI, 1.14 to 3.70; P = .02) compared with patients with the lowest level. TERC expression level remained a significant prognostic indicator in a multivariate analysis adjusting for TERT mRNA, tumor stage, and patient age. TRAP level did not correlate with RR of relapse. Telomerase expression levels were not predictive of overall survival. CONCLUSION: Telomerase RNA expression level may provide a clinically useful adjunct to the current risk classification schema for favorable-histology Wilms' tumor.

Biomarkers, Tumor↗
PubMed2005

USCAP Specialty Conference: case 1-type I pleuropulmonary blastoma.

Pleuropulmonary blastoma (PPB) was defined in 1988 by Manivel et al. in a series describing 11 intrathoracic pulmonary neoplasms in young children. The PPB is a unique peripheral pulmonary or pleural-based tumor of childhood that is characterized in its earliest form as a bland-appearing multiloculated cyst with small foci of tumor cells and in later forms as mixed and predominantly primitive, overtly malignant neoplasms. Prior to the introduction of the PPB as a distinct entity, this tumor had been reported in the literature as pulmonary blastoma, sarcoma arising in mesenchymal cystic hamartoma, embryonal sarcoma, malignant mesenchymoma, primary pulmonary rhabdomyosarcoma and rhabdomyosarcoma arising in congenital adenomatoid malformation or bronchogenic cyst. Over the past 15 years, PPB has come to be recognized in centers around the world. With the establishment of the Pleuropulmonary Blastoma Registry by Jack Priest, MD, and colleagues, there has been improved understanding of this rare pediatric neoplasm.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2005

Desmoplastic nested spindle cell tumor of liver: report of four cases of a proposed new entity.

We describe a distinctive tumor of the liver in four children composed of nested spindled and epithelioid cells with extensive desmoplasia that we have termed "desmoplastic nested spindle cell tumor of the liver." All four patients were previously healthy. One patient had a presumptive diagnosis of hepatic hemangioma 11 years prior to presentation. Grossly, the tumors were well circumscribed, lobular white masses, ranging from 2.8 to 15 cm in diameter. These tumors were characterized by the presence of cohesive nests of plump, bland spindle cells arranged in short fascicles with an accompanying desmoplastic stroma. Epithelioid areas ranging from palisading epithelioid cells at the periphery of some nests to pseudoglandular and polygonal cells with intercellular bridges were invariably present. Mitotic activity was low. Calcification and ossification were present. Non-neoplastic bile ducts and hepatic elements were seen both within and surrounding the tumor cell nests. Each tumor displayed cytoplasmic reactivity for vimentin, pan-cytokeratin, CD57, and nuclear staining for WT1. Neuroendocrine markers were negative. Ultrastructurally, the tumor cells showed focally well-developed cell junctions, basal lamina, and few cytoplasmic organelles. All tumors were confined to the liver and were resected without complication. Two patients received postoperative adjuvant therapy for presumed hepatoblastoma. The patients are doing well without recurrence at 7.5 years, 7 years, 5 years, and 8 months post-surgery. The morphologic appearance and immunohistochemical profile of these lesions are unique in our experience and represent a new category of pediatric liver tumor.

Adolescent↗
PubMed2005

IUD removal with colonoscopy: a case report.

BACKGROUND: Intrauterine device (IUD) perforation of the bowel is uncommon. Although IUD perforation may be asymptomatic, the most common complaint is unexplained abdominal pain. CASE: A case of IUD perforation of the large bowel was diagnosed 7 years after insertion. The patient presented with unexplained lower abdominal pain diagnosed initially as pelvic inflammatory disease. Laparoscopy revealed that the IUD was embedded deeply in the rectum. Bowel preparation and intravenous antibiotics followed by colonoscopy using a grasping snare resulted in successful IUD removal. CONCLUSION: Patients presenting with IUDs embedded in the large bowel may benefit from attempted removal using colonoscopy rather than laparotomy. Bowel preparation, intravenous antibiotics and pos-textraction evaluation to rule out perforation may be prudent.

Adult↗
PubMed2004

Expression of WT-1, Bcl-2, and CD34 by primary renal spindle cell tumors in children.

The confident diagnosis of renal spindle cell tumors in children is often difficult. An immunohistochemical study of WT-1, Bcl-2, and CD34 was performed to determine their expression profiles and to assess the potential utility of these immunohistochemical markers in the differential diagnosis of 36 cases of renal spindle cell tumors of childhood. The cases included 11 stromal predominant Wilms tumors, 12 cellular mesoblastic nephromas, 9 clear cell sarcomas of the kidney (CCSK), and 4 monophasic synovial sarcomas. WT-1 was uniformly positive in primitive undifferentiated stromal Wilms tumors (6 of 6) and negative in the differentiating and differentiated stromal elements of Wilms tumors (0 of 5). WT-1 was also negative in cellular mesoblastic nephromas (0 of 12), CCSKs (0 of 12), and synovial sarcomas (0 of 4). Bcl-2 was expressed in all stromal Wilms tumors (11 of 11), all synovial sarcomas (4 of 4), some CCSKs (4 of 9), and none of the cellular mesoblastic nephromas (0 of 12). Although CD34 was absent in the tumor cells of all the tumors studied (0 of 36), CD34 immunohistochemistry nicely demonstrated the evenly distributed septal capillaries characteristic of CCSK in all 9 cases of this tumor. We conclude that a combination of WT-1 and Bcl-2 immunohistochemistry may aid in the distinction of stromal Wilms tumor, monophasic synovial sarcoma, cellular mesoblastic nephroma, and CCSK.

Antigens, CD34↗
PubMed2004

Neutropenic enterocolitis as a presenting complication of acute lymphoblastic leukemia: an unusual case marked by delayed perforation of the descending colon.

Neutropenic enterocolitis (NE) is a life-threatening complication most commonly seen in patients receiving intensive chemotherapy for acute leukemia. The condition usually affects the terminal ileum, cecum, or ascending colon. In rare instances, NE may occur before the initiation of chemotherapy or involve more distal bowel. The authors report the case of a 2-year-old girl who had NE affecting the descending colon as a presenting complication of acute lymphoblastic leukemia. Despite aggressive medical interventions, including granulocyte infusions, she had a delayed bowel perforation that was managed successfully with surgery. This case highlights the challenges of treating patients who have NE as an initial manifestation of acute leukemia.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2004

Pleomorphic rhabdomyosarcoma of the anterior abdominal wall following multimodality treatment for carcinoma of the rectum.

Postradiation sarcomas are rare, and the most commonly reported ones are malignant fibrous histiocytoma, osteosarcoma, angiosarcoma, fibrosarcoma, malignant peripheral nerve sheath tumor, and high-grade pleomorphic sarcoma, not otherwise specified. There are a few case reports of postradiation rhabdomyosarcomas following treatment of retinoblastoma, breast cancer, endometrial adenocarcinoma, and Hodgkin's disease. Secondary neoplasms following radiation and surgical treatment of rectal adenocarcinomas have not been reported in the English literature. We report a case of pleomorphic rhabdomyosarcoma of the anterior abdominal wall following treatment of rectal carcinoma, and we review the literature.

Abdominal Wall↗
PubMed2003

ERBB1 is amplified and overexpressed in high-grade diffusely infiltrative pediatric brain stem glioma.

PURPOSE: This study was conducted to investigate the incidence of ERBB1 amplification and overexpression in samples of diffusely infiltrative (WHO grades II-IV) pediatric brain stem glioma (BSG) and determine the relationship of these abnormalities to expression and mutation of TP53 and tumor grade. EXPERIMENTAL DESIGN: After central pathology review, the incidence of ERBB1 amplification and overexpression was determined in 28 samples (18 surgical biopsy and 10 postmortem specimens) of BSG using quantitative PCR and immunohistochemistry, respectively. Mutation and expression of TP53 were also determined in these same samples by direct sequence analysis of microdissected tumor material and immunohistochemistry, respectively. All experimental procedures were performed blind to tumor grade. RESULTS: Twelve, 9, and 7 tumors were classified as WHO grades II, III, and IV, respectively. A significant increase in ERBB1 expression was observed with increasing tumor grade (P < 0.001). Two grade IV tumors displayed intense membranous ERBB1 expression in 90% of tumor cells in association with high-level ERBB1 gene amplification. One grade III tumor also contained low-level amplification of ERBB1. Six tumors demonstrated TP53 nuclear immunoreactivity, and six contained a mutation in TP53. No correlation was observed between abnormalities in TP53 and either tumor grade or amplification and overexpression of ERBB1. CONCLUSIONS: These data suggest that ERBB1 signaling is important for the development of childhood BSG and is worthy of study as a therapeutic target in this disease. Our data also indicate that the genetics of childhood BSG are complex and include both grade-dependent amplification and overexpression of ERBB1 and grade-independent expression and mutation of TP53.

Adolescent↗
PubMed2003

Nausea and vomiting of pregnancy.

Nausea and vomiting of pregnancy, commonly known as "morning sickness," affects approximately 80 percent of pregnant women. Although several theories have been proposed, the exact cause remains unclear. Recent research has implicated Helicobacter pylori as one possible cause. Nausea and vomiting of pregnancy is generally a mild, self-limited condition that may be controlled with conservative measures. A small percentage of pregnant women have a more profound course, with the most severe form being hyperemesis gravidarum. Unlike morning sickness, hyperemesis gravidarum may have negative implications for maternal and fetal health. Physicians should carefully evaluate patients with nonresolving or worsening symptoms to rule out the most common pregnancy-related and nonpregnancy-related causes of severe vomiting. Once pathologic causes have been ruled out, treatment is individualized. Initial treatment should be conservative and should involve dietary changes, emotional support, and perhaps alternative therapy such as ginger or acupressure. Women with more complicated nausea and vomiting of pregnancy also may need pharmacologic therapy. Several medications, including pyridoxine and doxylamine, have been shown to be safe and effective treatments. Pregnant women who have severe vomiting may require hospitalization, orally or intravenously administered corticosteroid therapy, and total parenteral nutrition.

Acupressure↗
PubMed2003

Over-the-counter medications in pregnancy.

Pregnant women commonly use over-the-counter medications. Although most over-the-counter drugs have an excellent safety profile, some have unproven safety or are known to adversely affect the fetus. The safety profile of some medications may change according to the gestational age of the fetus. Because an estimated 10 percent or more of birth defects result from maternal drug exposure, the U.S. Food and Drug Administration has assigned a risk category to each drug. Many drugs have not been evaluated in controlled trials and probably will not be because of ethical considerations. Of the commonly used over-the-counter medications, acetaminophen, chlorpheniramine, kaolin and pectin preparations, and most antacids have a good safety record. Other drugs, such as histamine H2-receptor blockers, pseudoephedrine, and atropine/diphenoxylate should be used with caution. If use of smoking cessation products is desired, the intermediate-release preparations minimize the amount of nicotine while maintaining efficacy. With all over-the-counter medications used during pregnancy, the benefit of the drug should outweigh the risk to the fetus.

Female↗