scholarly journals Incidence of NUT carcinoma in Western Australia from 1989 to 2014: a review of pediatric and adolescent cases from Perth Children’s Hospital

BMC Cancer ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Tina Carter ◽  
Maxine Crook ◽  
Ashleigh Murch ◽  
Alex H. Beesley ◽  
Nick de Klerk ◽  
...  
Keyword(s):  
Western Australia ◽  
Malignant Tumors ◽  
Upper Airway ◽  
Initial Response ◽  
Spinal Tumor ◽  
Geographic Area ◽  
Response To Therapy ◽  
Catchment Population ◽  
Nut Carcinoma ◽  
Geographically Isolated

Abstract Background NUT carcinoma (NC), previously known as NUT midline carcinoma, is a rare and very aggressive cancer that occurs in both children and adults. NC is largely chemoresistant, with an overall survival of less than 7 months. Because the carcinoma is not restricted to a particular organ, diagnosis is often a challenge. In the absence of a clearly determined incidence for NC, we sought to study the diagnosis of patients in a well-defined population. Methods We systematically reviewed records of all patients that presented to the Oncology Department of the Princess Margaret Hospital for Children from 1989 to 2014. This institution in the geographically isolated state of Western Australia has a catchment population of around 2 million. We then identified all high grade undifferentiated sarcomas or carcinomas in the 0–16 year age group. Results Over 26 years, we found 14 patients of 16 years or younger with undifferentiated malignant tumors. Of these, five tumors were positive by immunohistochemistry for the NUT/NUTM1 (Nuclear Protein in Testis) protein and/or the translocation t(15;19). Three patients presented with thoracic tumors, one with a para-spinal tumor, and one had an upper airway nasopharyngeal carcinoma. In all five cases, there was an initial response to therapy and then progression. This 26-year survey was conducted in a geographically isolated state with a well-defined population, and we determined an estimated incidence of NC of around 0.41 per million child years (0–16 yrs. of age) at risk. From three patients it was feasible to derive cell lines for further genetic analyses and drug screening. Conclusions For the first time, the incidence of NC could be determined in a well-defined geographic area. The calculated rate of NC incidence is consistent with a history of under-recognition for this malignancy. These findings indicate that improved diagnostic detection of NC would enable better management and counselling of patients. Our findings emphasize the heterogeneity of NC, and they highlight the need to develop personalised therapy options, and to consider a diagnosis of NC in undifferentiated malignant tumors.

THE INFLUENCE OF ADRENAL STEROIDS AND CORTICOTROPIN ON MASSIVE MYOCLONIC SEIZURES OF INFANCY

PEDIATRICS ◽  
1963 ◽  
Vol 32 (2) ◽  
pp. 169-174
Author(s):  
Patrick F. Bray
Keyword(s):  
Neurological Deficit ◽  
Prognostic Value ◽  
Rational Expectation ◽  
Initial Response ◽  
Response To Therapy ◽  
Adrenal Steroids ◽  
Focal Neurological Deficit ◽  
Rational Treatment ◽  
Follow Up Study

A 4-year follow-up study is reported on 10 infants whose minor motor seizures were treated intensively with cortisone and/or corticotropin. No correlation was found between the infants' initial clinical and electroencephalographic response to therapy and their follow-up intelligence quotients. The similar initial electroencephalographic findings, contrasted with the marked followup differences in levels of intellectual functioning, illustrate the limited prognostic value of the electroencephalogram in this syndrome. Similarly, no correlation was noted in the patients' initial response to therapy, and the presence or absence of microcephaly or focal neurological deficit. In the absence of any other rational treatment, and despite the dismal prospect suggested by this report and those of others, renewed efforts to treat patients earlier and more intensively with cortisone and corticotropin could be undertaken. However, in the light of 4 years' experience, such an approach might be a reflection more of therapeutic desperation than of rational expectation of good results.

A new Altivasum Hedley, 1914 (Turbinellidae, Vasinae) from the coast of southern Western Australia

The Festivus ◽  
2020 ◽  
Vol 52 (3) ◽  
pp. 212-217
Author(s):  
Merv Cooper ◽  
Stephen Maxwell
Keyword(s):  
New Species ◽  
Western Australia ◽  
The South ◽  
South West ◽  
Geographically Isolated

This paper presents a new Altivasum found off Jurien Bay, Western Australia at 60 m. This new species expands our understanding of the distribution, and in particular extends the northern range of Altivasum, in the South-west Marine Region. Altivasum pauladellaboscae n. sp. is more rhomboidal than A. hedleyi Maxwell and Dekkers, 2019, which is elongated, and has the formation of tubular spines on the shoulder of axial fold on the later whorls of the spire; these spines are not formed in A. pauladellaboscae n sp. Altivasum pauladellaboscae n sp. differs from A. profundum Dekkers and Maxwell, 2018 in having acute shoulder nodules. The South Australian, A. flindersi Verco, 1914 lacks the fibriated subsutural band found in A. pauladellaboscae n. sp. Altivasum clarksoni Maxwell and Dekkers, 2019 is geographically isolated and morphologically distinct, being more elongated and fibriated. This paper brings the number of described Altivasum species to five

Systemic Therapy for Osteosarcoma and Ewing Sarcoma

2015 ◽  
pp. e644-e647 ◽  
Author(s):  
Paul A. Meyers
Keyword(s):  
Ewing Sarcoma ◽  
Systemic Therapy ◽  
Dose Intensity ◽  
Initial Response ◽  
Response To Therapy ◽  
High Dose ◽  
Improve Outcome ◽  
Curative Therapy ◽  
Dose Methotrexate ◽  
Multiagent Chemotherapy

Curative therapy for both osteosarcoma and Ewing sarcoma requires the combination of effective systemic therapy and local control of all macroscopic tumors. Systemic therapy for osteosarcoma consists of multiagent chemotherapy. The most common regimen uses cisplatin, doxorubicin, and high-dose methotrexate. Addition of ifosfamide and etoposide to treatment for patients with poor initial response to therapy does not improve outcome. Addition of interferon to treatment for patients with favorable initial response does not improve outcome. Addition of liposomal muramyl tripeptide to chemotherapy may improve overall survival. Systemic therapy for Ewing sarcoma consists of multiagent chemotherapy including doxorubicin, vincristine, etoposide, and cyclophosphamide and/or ifosfamide. Increased dose intensity of therapy, either by shortening the intervals between cycles of chemotherapy or by increasing doses of chemotherapy, improves outcome. Regimens such as irinotecan/temozolomide or cyclophosphamide/topotecan have shown activity in metastatic recurrent Ewing sarcoma. Trials are ongoing to evaluate the addition of these drugs to existing multiagent regimens in order to test their ability to improve outcome. High-dose systemic therapy with autologous stem cell reconstitution is being tested for patients at high risk for recurrence; definitive results await completion of a prospective randomized trial.

scholarly journals Effects of COVID-19 Pandemic on Otolaryngology Surgery in Italy: The Experience of Our University Hospital

2020 ◽  
Vol 163 (1) ◽  
pp. 86-88 ◽  
Author(s):  
Massimo Ralli ◽  
Antonio Minni ◽  
Francesca Candelori ◽  
Fabrizio Cialente ◽  
Antonio Greco ◽  
...  
Keyword(s):  
Head And Neck ◽  
Bone Fractures ◽  
Malignant Tumors ◽  
Nasal Bone ◽  
Upper Airway ◽  
Neck Surgery ◽  
University Hospital ◽  
Outpatient Procedures ◽  
Therapeutic Procedures ◽  
Adults And Children

Otolaryngology and head and neck surgery underwent drastic changes during the COVID-19 pandemic. Since March 10, the first day of the lockdown in Italy, diagnostic and therapeutic procedures were limited to emergency and oncology cases, while outpatient procedures and clinical examinations were temporarily suspended to limit virus diffusion and to reallocate personnel into wards dedicated to COVID-19. In our otolaryngology unit, between March 10 and April 28, 2020, we performed 96 surgical procedures; they mainly consisted in diagnosis and treatment of malignant tumors of the head and neck (77%), management of acute upper airway obstruction in adults and children (14.7%), drainage of abscesses of the head and neck (6.2%), and treatment of nasal bone fractures (2.1%). When comparing these data with those of the same period in 2019 for emergency and oncology procedures, we noticed a drastic reduction of head and neck abscesses and nasal bone fractures, while oncology surgery remained stable.

scholarly journals The Emerging Role of Microbiota and Microbiome in Pancreatic Ductal Adenocarcinoma

Biomedicines ◽  
2020 ◽  
Vol 8 (12) ◽  
pp. 565
Author(s):  
Sona Ciernikova ◽  
Maria Novisedlakova ◽  
Danka Cholujova ◽  
Viola Stevurkova ◽  
Michal Mego
Keyword(s):  
Pancreatic Ductal Adenocarcinoma ◽  
Malignant Tumors ◽  
Current Knowledge ◽  
Early Stage ◽  
Poor Response ◽  
Response To Therapy ◽  
Oral Microbiome ◽  
Ductal Adenocarcinoma ◽  
The Impact

Pancreatic ductal adenocarcinoma (PDAC) is one of the most aggressive malignant tumors due to the absence of biomarkers for early-stage detection and poor response to therapy. Since mounting evidence supports the role of microbiota composition in tumorigenesis and cancer treatment, the link between microbiome and PDAC has been described. In this review, we summarize the current knowledge regarding the impact of the gut and oral microbiome on the risk of PDAC development. Microenvironment-driven therapy and immune system interactions are also discussed. More importantly, we provide an overview of the clinical trials evaluating the microbiota role in the risk, prognosis, and treatment of patients suffering from PDAC and solid tumors. According to the research findings, immune tolerance might result from the microbiota-derived remodeling of pancreatic tumor microenvironment. Thus, microbiome profiling and targeting represent the potential trend to enhance antitumor immunity and improve the efficacy of PDAC treatment.

Efficacy of Megestrol Acetate (Megace) in the Treatment of Patients With Early Endometrial Adenocarcinoma: Our Experiences With 21 Patients

2009 ◽  
Vol 19 (2) ◽  
pp. 249-252 ◽  
Author(s):  
Zahra Eftekhar ◽  
Narges Izadi-Mood ◽  
Fariba Yarandi ◽  
Hadi Shojaei ◽  
Zahra Rezaei ◽  
...  
Keyword(s):  
Therapeutic Response ◽  
Endometrial Adenocarcinoma ◽  
Initial Response ◽  
Response To Therapy ◽  
Megestrol Acetate ◽  
Dilatation And Curettage ◽  
Stage Ia ◽  
The Mean ◽  
Well Differentiated ◽  
A Prospective Study

Background:There are therapeutic dilemmas regarding fertility-preserving treatment among young women with well-differentiated endometrial carcinoma.Materials and Methods:Twenty-one patients with stage IA well-differentiated endometrial adenocarcinoma were enrolled in a prospective study. The treatment initiated with 160 mg/d of megestrol acetate. The patients underwent dilatation and curettage and hysteroscopy after 3 months, and in cases of normal pathology, the therapy continued for another 3-month period. In patients who did not respond to treatment, the dosage of the drug was doubled (320 mg/d), and the therapy continued for another 3 months. At the second time, patients who did not respond to treatment were recommended for hysterectomy, and in patients who responded to treatment, an additional 3 months of treatment with megestrol acetate (320 mg/d) was administered.Results:Our results showed a response rate of 85.71% (18 patients), and 3 patients underwent hysterectomy. The mean (SD) treatment duration was 8.85 (2.00) months (range, 6-12 months). The response to therapy was observed in 5 patients (27.78%) with a dosage of 160 mg/d, and the remaining patients with 320 mg/d. Pregnancy occurred in 5 patients (27.78%). Recurrence happened in 3 (16.67%) of 18 patients who responded to treatment who did not give a permit to undergo hysterectomy and received medication again. Two (66.67%) of these patients experienced remission again, whereas the other one was candidate for hysterectomy.Conclusions:The results of this study show that, when an initial response is not achieved or when disease recurs, use of 320 mg/d seems to be associated with a better therapeutic response. Furthermore, serious complications were not observed with this dosage.

scholarly journals Identification of plasma microRNA-22 as a marker for the diagnosis, prognosis, and chemosensitivity prediction of osteosarcoma

2020 ◽  
Vol 48 (12) ◽  
pp. 030006052096781
Author(s):  
Zhen-bin Diao ◽  
Tian-xiao Sun ◽  
Yi Zong ◽  
Bo-chuan Lin ◽  
Yuan-sheng Xia
Keyword(s):  
Malignant Tumors ◽  
Poor Response ◽  
Response To Therapy ◽  
Healthy Controls ◽  
Significant Independent Predictor ◽  
Large Tumor ◽  
Altered Expression ◽  
Reverse Transcription Pcr ◽  
Noninvasive Biomarker ◽  
Plasma Microrna

Objective MicroRNA (miR)-22 plays crucial roles in malignant tumors and is involved in regulation of chemosensitivity. Additionally, altered expression of circulating miR-22 has been reported in various cancers. This study was designed to investigate plasma miR-22 expression in patients with osteosarcoma (OS) and determine its diagnostic, prognostic, and chemosensitivity prediction value. Methods Plasma miR-22 levels in 120 patients with OS and 120 healthy controls were detected by real-time quantitative reverse transcription PCR. Associations of plasma miR-22 expression with the patients’ clinicopathological features and prognosis were then assessed. Results Plasma miR-22 levels in patients with OS were significantly lower than those in healthy controls. Low plasma miR-22 levels were correlated with large tumor size, advanced clinical stages, positive distant metastasis, and poor tumor response to preoperative chemotherapy. Plasma miR-22 could discriminate OS patients from controls and distinguish patients with a good response to therapy from those with a poor response to therapy. Multivariate analysis revealed that low plasma miR-22 expression was a significant independent predictor of unfavorable prognosis. Conclusions Altered plasma levels of miR-22 might serve as a novel, noninvasive biomarker for OS diagnosis, prognosis, and chemosensitivity prediction.

scholarly journals Current Virulence of Pyrenophora teres on Barley in Western Australia

Plant Disease ◽  
2001 ◽  
Vol 85 (9) ◽  
pp. 960-966 ◽  
Author(s):  
Sanjiv Gupta ◽  
Robert Loughman
Keyword(s):  
Western Australia ◽  
Error Variance ◽  
Geographic Area ◽  
Australian Isolate ◽  
Pyrenophora Teres ◽  
Sources Of Resistance ◽  
Pathogenic Variation ◽  
Resistant Varieties ◽  
Significant Line ◽  
Spot Type

Studies on variation, occurrence, and distribution of virulence in Pyrenophora teres are helpful to identify effective sources of resistance that can be used for barley breeding in Western Australia. Seventy-nine isolates of Pyrenophora teres were collected from different barley fields of Western Australia in 1995-96. Seventy-four induced net type symptoms (P. teres f. teres) and five induced spot type symptoms (P. teres f. maculata). Net type isolate responses on 47 barley lines were similar to the range of responses induced by nine historical isolates collected in the region between 1975 and 1985. These net type isolates were classified into two distinct groups based on virulence to the cultivar Beecher. Isolates were further classified into eight groups based on minor pathogenic variation among the population. The virulence phenotype present in an eastern Australian isolate was not observed in any isolates collected from Western Australia. An analysis of variance on a subset of 12 net type isolates indicated a significant line × isolate interaction (P < 0.001), with the interaction term variance component four times larger than the error variance. Based on these studies, the virulence among net type isolates has remained stable in Western Australia for the last 19 years. Spot type isolates were collected from a wider geographic area than previously reported and varied in virulence based on response to barley line Herta. Variation in spot-type isolates is reported for the first time from the region. The results from this study are being used in the development of resistant varieties.

Proteomic signatures of acute myeloid leukemia (AML) distinguishes different outcome groups across cytogenetics and identified potential therapy targets

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 6523-6523
Author(s):  
R. Tibes ◽  
Y. Qiu ◽  
K. Coombes ◽  
B. Hennessy ◽  
H. Kantarjian ◽  
...  
Keyword(s):  
Transcriptional Profiling ◽  
Initial Response ◽  
Response To Therapy ◽  
Specific Expression ◽  
Therapy Targets ◽  
Class 1 ◽  
Marker Distribution ◽  
Large Clusters ◽  
Subsequent Relapse ◽  
Risk Of Relapse

6523 Background: Cytogenetics (CG) guide AML treatment but reliable markers predicting response and relapse within CG groups are missing. We therefore determined whether functional proteomic signatures can classify AML into groups with different outcomes and risk of relapse. Method: Using Reverse Phase Protein Array, total and phospho-site specific expression of 37 proteins in 73 primary AML was measured. Outcomes in the set were comprised equally of primary refractory (PR), relapsed (Rel) and continuous complete remission (CCR) patients. Cell lysates were spotted on nitrocellulose coated slides, probed with validated antibodies, expression intensities were quantified, data was standardized and analyzed for correlations using different clustering approaches. Results: Unsupervised hierarchical clustering based on Pearsons’ correlation distance yielded 4 large clusters. Subsequent perturbation bootstrap re-sampling arranged samples into four classes that correlated with initial response to therapy and risk of relapse (see Table ). Protein profiles in each of he 4 classes differed. Cytogenetic marker distribution were similar across the 4 clusters. Class 1 and 4 demonstrated a similar predictive value of patient outcome as cytogenetics. In classes at highest risk of relapse (2, 3) different proteins were predictive of response. In class 2, the most discriminatory proteins predicting CCR were elevated AMPK, p27, 4-EBP1, BclXL. In class 3, relapsed patients had elevated PTEN, phospho-Stat3, total Stat3, and phospho-PKCα compared to CCR patients. Conclusion: Pretreatment protein expression signatures divide AML into classes that predict for initial achievement of CR and subsequent relapse independent of CG. Poteomic profiling may suggest potential therapy targets as opposed to CG or transcriptional profiling. These preliminary results need to be confirmed in formal training and test sets prior to changing patient management. [Table: see text] No significant financial relationships to disclose.

Successful Desensitization to Oxaliplatin

2005 ◽  
Vol 39 (5) ◽  
pp. 966-969 ◽  
Author(s):  
Lydia ◽  
Nishan H Fernando ◽  
Hurbert I Hurwitz ◽  
Michael A Morse
Keyword(s):  
White Woman ◽  
Initial Response ◽  
Response To Therapy ◽  
Controlled Environment ◽  
Metastatic Colon Cancer ◽  
Hypersensitivity Reactions ◽  
Platinum Compounds ◽  
Probability Scale ◽  
Case Summary ◽  
Life Threatening

OBJECTIVE: To report the successful desensitization of a patient to oxaliplatin utilizing an 8-hour desensitization regimen in a controlled environment. CASE SUMMARY: A 53-year-old white woman with metastatic colon cancer was receiving oxaliplatin, bevacizumab, and capecitabine every 2 weeks, with a partial response to therapy. On her fifth cycle of this regimen, she experienced diaphoresis, hypotension, nausea, abdominal cramping, and coryza. According to the Naranjo probability scale, oxaliplatin, and not bevacizumab, was the probable cause of the hypersensitivity reaction. The woman continued therapy with capecitabine and bevacizumab, resulting in stable disease. Due to her initial response to the oxaliplatin-based regimen, it was decided to attempt desensitization to oxaliplatin in a controlled, inpatient environment. An 8-hour desensitization schedule was employed, and the patient successfully completed an additional 3 cycles with full-dose oxaliplatin. DISCUSSION: Hypersensitivity reactions to platinum-containing compounds are well described and potentially life threatening. With expanded use of oxaliplatin in various malignancies, an increased number of hypersensitivity reactions will likely be reported. Patients with previous hypersensitivity reactions to carboplatin are at risk for similar reactions to oxaliplatin. We achieved successful desensitization for oxaliplatin using increased concentrations of the drug over an 8-hour period concomitant with oral and intravenous corticosteroids and histamine blockers. CONCLUSIONS: Hypersensitivity reactions to platinum compounds may result in discontinuation of active therapies in patients with metastatic disease. Desensitization to oxaliplatin is possible utilizing this approach.

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