Miscellaneous Tumors Research Articles

Predicting the survival of patients with painful tumours treated with palliative radiotherapy: a secondary analysis using the 3-variable number-of-risk-factors model

BackgroundThe 3-variable number-of-risk-factors (NRF) model is a prognostic tool for patients undergoing palliative radiotherapy (PRT). However, there is little research on the NRF model for patients with painful non-bone-metastasis tumours treated with PRT, and the efficacy of the NRF model in predicting survival is unclear to date. Therefore, we aimed to assess the prognostic accuracy of a 3-variable NRF model in patients undergoing PRT for bone and non- bone-metastasis tumours.MethodsThis was a secondary analysis of studies on PRT for bone-metastasis (BM) and PRT for miscellaneous painful tumours (MPTs), including non-BM tumours. Patients were grouped in the NRF model and survival was compared between groups. Discrimination was evaluated using a time-independent C-index and a time-dependent area under the receiver operating characteristic curve (AUROC). A calibration curve was used to assess the agreement between predicted and observed survival.ResultsWe analysed 485 patients in the BM group and 302 patients in the MPT group. The median survival times in the BM group for groups I, II, and III were 35.1, 10.1, and 3.3 months, respectively (P < 0.001), while in the MPT group, they were 22.1, 9.5, and 4.6 months, respectively (P < 0.001). The C-index was 0.689 in the BM group and 0.625 in the MPT group. In the BM group, time-dependent AUROCs over 2 to 24 months ranged from 0.738 to 0.765, while in the MPT group, they ranged from 0.650 to 0.689, with both groups showing consistent accuracy over time. The calibration curve showed a reasonable agreement between the predicted and observed survival.ConclusionsThe NRF model predicted survival moderately well in both the BM and MPT groups.

Predictive Factors of Long-Term Neurologic Outcome and Progression-Free Survival in Intramedullary Spinal Cord Tumors: A 10-year Single-Center Cohort Study and Review of the Literature

Intramedullary spinal cord tumors (IMSCTs) are a rare subgroup of neoplasms, encompassing both benign, slow-growing masses, and malignant lesions; radical surgical excision represents the cornerstone of treatment for such pathologies regardless of histopathology, which, on the other hand, is a known predictor of survival and neurologic outcome postsurgery. The present study aims to investigate the relevance of other factors in predicting survival and long-term functional outcomes. We conducted a review of current literature on functional outcomes of IMSCTs, as well as a 10-years prospective analysis of a wide cohort of patients with diagnosis of IMSCTs who underwent surgical resection at our institution. Our series encompasses 60 patients with IMSCTS, among which 36 ependymomas, 6 cavernous angiomas, 5 hemangioblastomas, 6 WHO Grade I-IV astrocytomas, 3 intramedullary spinal metastases and 4 miscellaneous tumors. GTR was achieved in 76,67% of patients, with high preoperative McCormick grade, syringomyelia and changes at neurophysiologic monitoring being the strongest predictors at multivariate analysis (P= 0.0027, P= 0.0017 and P= 0.001 respectively). Consistently with literature, preoperative neurologic function is the most important factor predicting long-term functional outcome (0.17, CI 0.069-0.57 with P= 0.0018), advocating for early surgery in the management of IMSCTs, whereas late complications such as myelopathy and neuropathic pain were present regardless of preoperative function.

Molecular Pathology of Thyroid Tumors: Essential Points to Comprehend Regarding the Latest WHO Classification.

In 2022, the new WHO Classification of Endocrine and Neuroendocrine Tumors, Fifth Edition (beta version) (WHO 5th), was published. Large-scale genomic analyses such as The Cancer Genome Atlas (TCGA) have revealed the importance of understanding the molecular genetics of thyroid tumors. Consequently, the WHO 5th was fundamentally revised, resulting in a systematic classification based on the cell of origin of tumors and their clinical risk. This paper outlines the following critical points of the WHO 5th. 1. Genetic mutations in follicular cell-derived neoplasms (FDNs) highlight the role of mutations in the MAP kinase pathway, including RET, RAS, and BRAF, as drivers of carcinogenesis. Differentiated thyroid cancers such as follicular thyroid carcinoma (FTC) and papillary thyroid carcinoma (PTC) have specific genetic alterations that correlate with morphological classifications: RAS-like tumors (RLTs) and BRAF p.V600E-like tumors (BLTs), respectively. 2. The framework for benign lesions has been revised. The WHO 5th introduces a new category: "developmental abnormalities". Benign FDNs comprise "thyroid follicular nodular disease", follicular thyroid adenoma (FTA), FTA with papillary architecture, and oncocytic adenoma (OA). "Hürthle cell adenoma/carcinoma" is renamed oncocytic adenoma/carcinoma of the thyroid (OA/OCA), which can be distinguished from FTA/FTC by its unique genetic background. 3. Low-risk tumors include NIFTP, TT-UMP, and HTT, and they have an extremely low malignant potential or an uncertain malignant potential. 4. PTC histological variants are reclassified as "subtypes" in the WHO 5th. 5. The concept of high-grade carcinomas is introduced, encompassing poorly differentiated thyroid carcinoma (PDTC), differentiated high-grade thyroid carcinoma (DHGTC), and high-grade medullary thyroid carcinoma (MTC). 6. Squamous cell carcinoma is included in anaplastic thyroid carcinoma (ATC) in the WHO 5th due to their shared genetic and prognostic features. 7. Other miscellaneous tumors are categorized as salivary-gland-type carcinomas of the thyroid, thyroid tumors of uncertain histogenesis, thymic tumors within the thyroid, and embryonal thyroid neoplasms. The WHO 5th thus emphasizes the importance of classifying tumors based on both genetic abnormalities and histomorphology. This approach aids in achieving accurate pathological diagnosis and facilitates the early selection of appropriate treatment options, including molecular targeted therapies.

CIA-II is associated with lower-grade glioma survival and cell proliferation.

The role of CIA-II has been clarified in several types of tumors; however, whether dysregulated CIA-II expression is also involved in the pathophysiology of lower-grade glioma (LGG) remains undisclosed. A comprehensive pan-cancer analysis of the expression patterns and prognostic significance of CIA-II in miscellaneous tumors was undertaken. Subsequently, a detailed bioinformatics analysis was executed to identify putative correlations between CIA-II expression and clinical features, prognosis, biological functions, immunological characteristics, genomic alterations, and chemotherapeutics in LGG. In vitro studies were implemented to examine the potential roles of CIA-II in LGG. CIA-II expression was found to be abnormally elevated in a variety of tumors, including LGG. Additionally, patients with LGG with higher CIA-II expression owned worse prognosis. Importantly, the results declared that CIA-II expression was an independent prognostic indicator for LGG. Moreover, the expression of CIA-II was tightly interrelated with immune cell infiltration, gene mutations, and chemotherapeutics in LGG. In vitro studies revealed that CIA-II was increased and strongly related to the cell proliferation in LGG. CIA-II may be an independent prognostic factor and a serviceable therapeutic target in LGG.

Ovarian Masses in Children and Adolescents: A Review of the Literature with Emphasis on the Diagnostic Approach.

Most abdominal masses in the pediatric population derive from the ovaries. Ovarian masses can occur in all ages, although their incidence, clinical presentation and histological distribution vary among different age groups. Children and adolescents may develop non-neoplastic ovarian lesions, such as functional cysts, endometrioma, torsion, abscess and lymphangioma as well as neoplasms, which are divided into germ cell, epithelial, sex-cord stromal and miscellaneous tumors. Germ cell tumors account for the majority of ovarian neoplasms in the pediatric population, while adults most frequently present with epithelial tumors. Mature teratoma is the most common ovarian neoplasm in children and adolescents, whereas dysgerminoma constitutes the most frequent ovarian malignancy. Clinical manifestations generally include abdominal pain, palpable mass, nausea/vomiting and endocrine alterations, such as menstrual abnormalities, precocious puberty and virilization. During the investigation of pediatric ovarian masses, the most important objective is to evaluate the likelihood of malignancy since the management of benign and malignant lesions is fundamentally different. The presence of solid components, large size and heterogenous appearance on transabdominal ultrasonography, magnetic resonance imaging and computed tomography indicate an increased risk of malignancy. Useful tumor markers that raise concern for ovarian cancer in children and adolescents include alpha-fetoprotein, lactate dehydrogenase, beta subunit of human chorionic gonadotropin, cancer antigen 125 and inhibin. However, their serum levels can neither confirm nor exclude malignancy. Management of pediatric ovarian masses needs to be curative and, when feasible, function-preserving and minimally invasive. Children and adolescents with an ovarian mass should be treated in specialized centers to avoid unnecessary oophorectomies and ensure the best possible outcome.

437. A Single-Center Experience of Solid versus Hematologic Malignancies in People Living with HIV

Abstract Background People living with HIV (PLWH) have an increased risk of cancer, including AIDS-defining and non-AIDS-defining malignancies. Some of the contributing factors are related to the high-risk lifestyle choices, but the introduction of HAART has reduced the incidence of AIDS-defining cancers. Methods We conducted a retrospective study of PLWH who were diagnosed with cancer between November 2019 and March 2022. We identified 60 patients who had HIV infection prior to the diagnosis (dg) of cancer or concurrently diagnosed with cancer. We evaluated patient’s characteristics, CD4 T cell counts, HIV viral load, adherence to HAART, solid and hematologic (HM) malignancies. Results Sixty pts were included in the analysis. The median age was 49.50 (range, 21-76 yrs) and 48 pts (80%) were male. The majority were African Americans (78.3%). Around 75% of pts were smokers, 35% of pts use alcohol, and 33.3% use illicit drugs. Cancer types were non-Hodgkin lymphoma (NHL) (11 pts, 18.3%), skin cancer (8 pts, 13.3%), Kaposi sarcoma (KS) (7 pts, 11.7%), prostate cancer (7 pts, 11.7%), head and neck cancer (5 pts, 8.3%), breast cancer (4 pts, 6.7%), Hodgkin lymphoma, leukemia, and lung cancer with same percentage each (3 pts, 5%), colorectal cancer (2 pts, 3.3%), and miscellaneous solid tumors (7 pts, 11.7%). AIDS-defining cancers were identified in 28.3% of pts. The median time between HIV and cancer dg was 8 years (range, 0-31 yrs). Eight pts (13.3%) were diagnosed with HIV and cancer at the same time [NHL (3 pts), myeloid leukemia (1 pt), and solid tumors different than cervical cancer (4 pts)]. A group of 43 pts (71.7%) had solid tumors were compared with 17 patients (28.3%) had HM. There were no differences between the two groups regarding gender, sexual orientation, smoking history, medical comorbidities, time between HIV and cancer dg. Pts with HM were younger (P-value of 0.05), and the rate of alcohol use was higher in pts with solid tumors (P-value, 0.003). HIV viral load &amp;gt; 200 copies/ml was higher in HM (75%) than solid tumors (44.1%) with P-value of 0.06 and the adherence rate to HAART was higher in pts with solid tumors (67.6%) compared to HM (38.4%) with a P-value of 0.1, prior to cancer dg. Conclusion PLWH with HM are more likely to be younger, non-adherent to HAART, and have higher HIV viremia compared to pts with solid tumors. Disclosures All Authors: No reported disclosures.

Histopathological study of pediatric solid malignant tumors in a tertiary care hospital

Background: Pediatric malignancies are extremely heterogeneous yet account for only approximately 1% of all tumor diagnosis. These entities are important to recognize because their clinical features are non-specific and may mimic common disorders of childhood .Pediatric solid malignant tumors are clinico-biologically distinct. They are more aggressive but usually respond satisfactorily to chemotherapy. The advanced multidisciplinary approach results in improved overall survival and a better quality of life for majority of the patients [1].Aims & Objectives: To study the incidence, age and sex distribution of Pediatric solid malignant tumors. To study the histopathological appearances of various Pediatric solid malignant tumors.Materials and Methods: A retrospective observational cohort study over a period of 5 years (January 2017 to September 2021) in the age group of 0-14 years was conducted in civil hospital, BJ Medical College, Ahmedabad. The data was retrieved from LIS (laboratory information system).The data consisted of information about the surgical pathology specimens received in histopathology laboratory from surgical departments. All specimen were examined macroscopically, processed by routine paraffin processing method and haematoxylin and eosin stained sections were studied microscopically.Results: In our study of 170 malignant cases, patients were stratified in 3 age groups; 0-5 years, 6-9 years, 10-14 years. Total of 110 cases were observed among males and 60 cases among females. Most of the malignancies 67(39.4%) were observed among 0-5 years age group. Central Nervous System (CNS) malignancies 123(72.35%) was most common followed by soft tissue sarcoma, lymphoma, germ cell tumors, renal malignant tumors, bone tumors and miscellaneous malignant tumors.

Blood-Derived Liquid Biopsies Using Foundation One® Liquid CDx for Children and Adolescents with High-Risk Malignancies: A Monocentric Experience.

Simple SummaryPrecision oncology requires tumor molecular profiling to identify actionable targets. Blood-derived liquid biopsy (LB) is a potential alternative that is not yet documented in real-world settings, especially in pediatric oncology. Analyzing, retrospectively, the use of LB in children with refractory relapsing diseases, we were able to show that this is a feasible alternative to tissue biopsy, resulting in successful analysis in a subset of patients.Precision oncology requires tumor molecular profiling to identify actionable targets. Tumor biopsies are considered as the gold standard, but their indications are limited by the burden of procedures in children. Blood-derived liquid biopsy (LB) is a potential alternative that is not yet documented in real-world settings, especially in pediatric oncology. We performed a retrospective analysis of children and teenagers with a relapsing or refractory disease, upon whom LB was performed using the Foundation One® liquid CDx from 1 January 2020 to 31 December 2021 in a single center. Forty-five patients (27 boys) were included, with a median age of 9 years of age (range: 1.5–17 years old). Underlying malignancies were neuroblastoma (12 patients), bone sarcoma (12), soft tissue sarcoma (9), brain tumors (7), and miscellaneous tumors (5). Forty-three patients had metastatic disease. Six patients had more than one biopsy because of a failure in first LB. Median time to obtain results was 13 days. Overall, analysis was successful for 33/45 patients. Eight patients did not present any molecular abnormalities. Molecular alterations were identified in 25 samples with a mean of 2.1 alterations per sample. The most common alterations concerned TP53 (7 pts), EWS-FLI1 (5), ALK (3), MYC (3), and CREBBP (2). TMB was low in all cases. Six patients received treatment based on the results from LB analysis and all were treated off-trial. Three additional patients were included in early phase clinical trials. Mean duration of treatment was 85 days, with one patient with stable disease after eight months. Molecular profiling using Foundation One® Liquid CDx was feasible in pediatric patients with high-risk solid tumors and lead to identification of targetable mutations in a subset of patients.

Pan‑cancer analyses reveal the regulation and clinical outcome association of PCLAF in human tumors.

Studies have shown that PCNA clamp associated factor (PCLAF) plays a paramount role in a variety of cancers; however, the expression profile and the specific molecular mechanism of PCLAF in cancer remains unclear, as is its value in the human pan-cancer analysis. Based on the publicly available datasets of The Cancer Genome Atlas (TCGA) and Gene Expression Omnibus (GEO), a comprehensive analysis of the probable carcinogenic effects of the PCLAF gene was performed in 33 human cancers. It was found that PCLAF is highly expressed in cancer tissues compared with normal tissues, and is significantly correlated with poor prognosis. We found that the eight tumors with significantly high PCLAF expression presented with decreased DNA methylation levels of PCLAF, including cholangiocarcinoma (CHOL), cervical squamous cell carcinoma and endocervical adenocarcinoma (CESC), glioblastoma multiforme (GBM), pheochromocytoma and paraganglioma (PCPG), sarcoma (SARC), testicular germ cell tumor (TGCT), stomach adenocarcinoma (STAD), and uterine corpus endometrial carcinoma (UCEC). The expression of PCLAF was found to be positively correlated with activated CD4 T cells (Act CD4) and type 2 T helper (Th2) cells, suggesting that PCLAF may play a particular role in tumor immune infiltration. In addition, the functional mechanism of PCLAF also involves the mitotic cell cycle process, cell division, and DNA replication. Our first pan-cancer study provides a relatively extensive understanding of the carcinogenic effects of PCLAF in miscellaneous tumors.

The intrapleural administration with thymic peptides in malignant pleural effusion: A clustered systematic review and meta-analysis

BackgroundThymic peptides (TPs) are often used to control malignant pleural effusion (MPE). So, we performed a clustered systematic review and meta-analysis to clarify the treatment regimens of TPs for MPE, demonstrate their clinical effectiveness and safety, and reveal the indications and optimal usage for a desired effectiveness. Materials and methodsWe collected all trials of TPs for MPE from Chinese and English databases (from inception until May 2021). After evaluating their bias risk, we pooled the data from each regimen using the meta-analysis or descriptive analysis, and summarized the evidence quality using the Grading of Recommendation Assessment, Development and Evaluation approach (GRADE). ResultsThirty-four trials were clustered into TPs for MPE from lung cancer or miscellaneous tumors. The TPs combined with chemical agents were mainly used in MPE from lung cancer. All five regimens, only thymosin with oxaliplatin (L-OHP) significantly improved the complete response (CR) [2.40 (1.84 to 3.13)], quality of life [2.04 (1.20 to 3.48)], 0.5- and 1-year overall survival (OS) rate [5.75 (3.02 to 10.92) and 5.29, (1.71 to 16.36)]. It also up-regulated the T lymphocyte levels, and reduced the pleurodesis failure, disease progression and adverse events. In patients with moderate to large volume, Karnofsky Performance Status score ≥ 50 or anticipated survival time ≥ 3 months, the thymosin (300 mg/time, one time/week and lasting two to eight times) with oxaliplatin (100 mg/m2) achieved a desired response. Most results were moderate quality. ConclusionsThe current evidences indicate that the TPs are important pleurodesis agents, which combination with chemical agents are mainly used in MPE from lung cancer. The thymosin with L-OHP is a main regimen, which shows a significant improvement in clinical responses, antitumor immunity, and with a reasonable security. The evidence also provides indications and optimal usage for achieving a desired effectiveness.

Rubidium Uptake in Chest Tumors on PET/CT.

Background Chest tumors are often found incidentally on cardiac scans; we aimed to describe the findings of rubidium (Rb) in incidentally discovered extracardiac tumors. Materials and Methods We reviewed a database of cardiac Rb scans performed over a period of 11 years and identified those with a previously unsuspected malignancy seen on the plane of section. We then measured maximum standard uptake value for each of the tumors, as well as background lung, liver, mediastinum, and body wall. In cases where fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) was available, we compared Rb results with FDG PET/CT. Results We identified 63 patients meeting criteria including full visualization of a tumor of at least 1.0 cm with no prior treatment. Of these patients, 17 had breast, 36 had lung, and 10 had miscellaneous other tumors. We selected patients with either breast or lung tumors for further analysis. Overall uptake was relatively stable between rest and stress but lower than FDG PET/CT; it was generally low and similar to blood pool. There was a small but statistically significant correlation between estrogen receptor positivity and Rb uptake in breast tumors. There was a stable pattern of uptake in background tissues, with liver being greater than mediastinal blood pool, which in turn was more avid than lung, which was more avid than subcutaneous body wall tissues. Lung showed a noticeable tendency toward increased uptake in dependent regions, likely reflecting low-level atelectasis. Conclusion Uptake was stable between rest and stress but low relative to FDG PET/CT; some correlations with receptors suggest it may be useful in molecular imaging.

Primary Diffuse Leptomeningeal Atypical Teratoid/Rhabdoid Tumor Initially Masquerading as Guillain–Barré Syndrome: A Case Report and Literature Review

AbstractA 10-year-old male child patient was admitted with a chief complaint of progression of lower limb weakness lasting for 5 weeks. An initial clinical picture was reminiscent of Guillain–Barré syndrome. Repeated cerebrospinal fluid (CSF) cytological examinations were negative for neoplastic cells, but leptomeningeal biopsy targeting positron emission tomography (PET) avid lesion confirmed the invasion of tumor cells which were negatively stained with Brahma-related gene 1. An extensive literature review identified five cases of primary diffuse leptomeningeal atypical teratoid/rhabdoid tumors, and the clinical characteristics, including ours, were characterized as follows: (1) there was a bimodal age distribution in young children and adolescents with a male predominance, (2) roughly half of the patients fulfilled the diagnostic criteria of albuminocytologic dissociation and CSF cytology was neither sensitive nor specific to establish a definitive diagnosis, and (3) cerebrospinal magnetic resonance imaging findings were mostly indistinguishable from those of infectious or inflammatory diseases. We would like to suggest that primary leptomeningeal tumor should be included in the differential diagnosis of progressive polyneuropathy even in the absence of CSF cytological findings and implementation of preoperative PET may enhance the diagnostic accuracy of such a miscellaneous central nervous system tumor.

Clinicopathological study of pineal gland lesions

Background: Pineal parenchymal tumors are the second most common form of pineal tumor.Aims: To study the incidence, age distribution, gender distribution of pineal region tumors and post-operative outcome.Materials and Methods: The present study is a prospective study comprising 20 cases of Pineal Region tumors, studied over a two and half year period. Data related to all cases were retrieved from archives. Available clinical data including, patient age, sex, clinical features, Imaging and surgical findings, tumor histology, outcome were also received from the Department of Neurosurgery Medical record Department. All pineal region tumors received at Neurosurgery department with adequate pre-operative; intra-operative and postoperative information were included in the study. Pineal region tumors comprising the Pineal parenchymal cell tumors, Germ cell tumors, Glial cell tumors, and other miscellaneous tumors and cysts.Results: Most common age group for the pineal region tumors is second and third decade and the Male to Female ratio is 1:1. Malignant nature of pineal region tumors is 17 cases incidence being 85%. Predominant grade of the pineal region tumors are grade I tumors commonest presenting symptoms being headache and vomiting. Commonest clinical sign is papilledema of features suggestive raised intracranial pressure. Most of patients presented at delayed stage with large tumor and morbid state with hydrocephalus, majority of the patients underwent shunt surgery before definitive surgery, some patients underwent external ventricular drainage simultaneously after surgery. No surgical mortality, one patient detoriated after third post operative day did not recovered and died. Inpatient had developed meningitis, sepsis didn't recover and died. Mortality 2 cases. Medium follow up period is one year. Three cases followed recurrent symptoms advised radiotherapy. Two cases came with shunt malfunction. Conclusion: Many of the approaches are interchangeable and they are depending on anatomical location and surgeon s choice and experience.

Rubidium uptake in chest tumors on positron emission tomography/computed tomography

Chest tumors are often found incidentally on cardiac scans; we aimed to describe the findings of rubidium in incidentally discovered extracardiac tumors. We reviewed a database of cardiac rubidium scans performed over a period of 11 years and identified those with a previously unsuspected malignancy seen on the plane of section. We then measured maximum standardized uptake value for each of the tumors, as well as background lung, liver, mediastinum, and body wall. In cases where fluorodeoxyglucose positron emission tomography-computed tomography (FDG-PET/CT) was available, we compared rubidium results to FDG-PET/CT. We identified 63 patients meeting criteria including full visualization of a tumor of at least 1.0 cm with no prior treatment. Of these patients, 17 had breast, 36 had lung, and 10 had miscellaneous other tumors. We selected patients with either breast or lung tumors for further analysis. Overall uptake was relatively stable between rest and stress but lower than FDG-PET/CT; it was generally low and similar to blood pool. There was a small but statistically significant correlation between estrogen receptor positivity and rubidium uptake in breast tumors. There was a stable pattern of uptake in background tissues, with liver being greater than mediastinal blood pool, which in turn was more avid than lung, which was more avid than subcutaneous body wall tissues. The lung showed a noticeable tendency toward increased uptake in dependent regions, likely reflecting low-level atelectasis. Uptake was stable between rest and stress but low relative to FDG-PET/CT; some correlations with receptors suggest it may be useful in molecular imaging.

Rare Tumors in Children and Adolescents – the STEP Working Groupʼs Evolution to a Prospective Registry

AbstractBackground Very rare tumors (VRT) in children and adolescents have such a low incidence that until recently, they have not been integrated into the clinical and scientific network of pediatric oncology. Data is very limited and consistent treatment strategies are missing. Thus, VRTs are classic orphan diseases. To counteract this problem, the Arbeitsgemeinschaft für Seltene Tumorerkrankungen in der Pädiatrie (STEP) was founded. Here we report on patient recruitment during the first 10 years. Patients Patients aged up to 18 years and not included in any other clinical trial or GPOH registry were included in this analysis. Methods Data was collected from 2008 to 2018 by means of a standardized form. The recorded diagnoses were descriptively analyzed focusing on histology, localization, and year of report. Results A total of 623 patients with VRTs were registered. During 2008–2014, the annual number of registrations was around 40 and is around 90 since 2015. Most frequent diagnoses included tumors of the skin (n=150), tumors of the gastrointestinal tract (n=102), tumors of the gonads (n=77), the ENT region (n=68), and miscellaneous tumors (n=107). Discussion With the establishment of central structures for clinical consultation and documentation of VRTs, the number of registrations increased. Comprehensively, VRTs are as common as other classic pediatric oncology tumors, but extremely heterogeneous in terms of localization, histology, and prognosis. By a centralized and complete registration and analysis of VRTs, also in collaboration with international partners, it is possible to develop treatment strategies and thus greatly increase treatment quality.

To evaluate the sensitivity of cytological examination of endobronchial biopsy, BAL, bronchial brushing and sputum in diagnosing lung carcinoma

All the cases of suspected bronchial malignancy were included in present study in which broncho-alveolar lavage (BAL) and bronchial biopsy samples were received in pathology department for evaluation. Respective bronchial brushings &amp; sputum samples were also studied where ever available. Cross sectional study.In our study, correctly diagnosed adenocarcinoma of lung on BAL were 27.7%, sqaumous cell carcinoma were 26.6%, small cell carcinoma were 40% and BAL cytodiagnosis from miscellaneous tumor could only be made in 14.2% cases. Endobronchial biopsy and BAL, both were studied, sensitivity of cases increases and number of false negative cases decreases for diagnosis. Bronchial brushing has better sensitivity than BAL for diagnosis of lung carcinoma. Sputum is less sensitive than bronchial brushing for diagnosis of lung carcinoma.

Primary tumors of the ribs – A pictorial essay

Primary tumors of the ribs are rare. However, they are often seen due to the high number of bones of the ribs and also the frequency of chest films taken for various conditions. They may be benign or malignant. Tumor like lesions are more in number than primary tumors of the ribs. Chest film is the first one to detect the tumor. Benign tumors include tumors arising from the osseous tissue, cartilaginous tissue, fibrous tissue, vascular tissue and other miscellaneous tumors arising from the other part of tissues. Similarly, primary malignant lesions arise from the tissues as mentioned earlier. Radiologically, there are several signs to identify and diagnose these tumors. Some of the lesions need advanced imaging such as CT, ultrasound, MRI, nuclide scan and PET CT.

Marginal Benefit/Disadvantage of Granulocyte Colony-Stimulating Factor Therapy After Autologous Blood Stem Cell Transplantation in Children: Results of a Prospective Randomized Trial

AbstractIn this prospective trial, a total of 74 children who were scheduled to undergo high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (PBSCT) were prospectively randomized at diagnosis to evaluate the effectiveness of exogenous granulocyte colony-stimulating factor (G-CSF) treatment in accelerating hematopoietic recovery after PBSCT. The diagnosis included acute lymphoblastic leukemia (ALL) (n = 27), neuroblastoma (n = 29), and miscellaneous solid tumors (n = 18). Eligibility criteria included (1) primary PBSCT, (2) chemotherapy-responsive disease, and (3) collected cell number >1 × 105 colony-forming unit–granulocyte-macrophage (CFU-GM)/kg and >1 × 106CD34+ cells/kg patient's body weight. After applying the above criteria, 11 patients were excluded due to disease progression before PBSCT (n = 6) or a low number of harvested cells (n = 5), leaving 63 patients for analysis; 32 patients in the treatment group (300 μg/m2 of G-CSF intravenously over 1 hour from day 1 of PBSCT) and 31 in the control group without treatment. Two distinct disease-oriented high-dose regimens without total body irradiation consisted of the MCVAC regimen using ranimustine (MCNU, 450 mg/m2), cytosine arabinoside (16 g/m2), etoposide (1.6 g/m2), and cyclophosphamide (100 mg/kg) for patients with ALL, and the Hi-MEC regimen using melphalan (180 mg/m2), etoposide (1.6 g/m2), and carboplatinum (1.6 g/m2) for those with solid tumors. Five patients (two in the treatment group and three in the control group) were subsequently removed due to protocol violations. All patients survived PBSCT. The median numbers of transfused mononuclear cells (MNC), CD34+ cells, and CFU-GM were, respectively, 4.5 (range, 1 to 19) × 108/kg, 8.0 (1.1 to 25) × 106/kg, and 3.7 (1.2 to 23) × 105/kg in the treatment group (n = 30) and 2.9 (0.8 to 21) × 108/kg, 6.3 (1.1 to 34) × 106/kg, and 5.5 (1.3 to 37) × 105/kg, respectively, in the control group (n = 28), with no significant difference. After PBSCT, the time to achieve an absolute neutrophil count (ANC) of >0.5 × 109/L in the treatment group was less than that in the control group (median, 11 v 12 days; the log-rank test, P = .046), although the last day of red blood cell (RBC) transfusion (day 11 v day 10) and the duration of febrile days (>38°C) after PBSCT (4 v 4 days) were identical in both groups. However, platelet recovery to >20 × 109/L was significantly longer in treatment group than control group (26 v 16 days; P = .009) and >50 × 109/L tended to take longer in the treatment group (29v 26 days; P = .126), with significantly more platelet transfusion-dependent days (27 v 13 days;t-test, P = .037). When patients were divided into two different disease cohorts, ALL patients showed no difference in engraftment kinetics between the G-CSF treatment and control groups, while differences were seen in those with solid tumors. We concluded that the marginal clinical benefit of 1 day earlier recovery of granulocytes could be offset by the delayed recovery of platelets. We recommend that the routine application of costly G-CSF therapy in children undergoing PBSCT should be seriously reconsidered.

Serum levels of microRNA-371a-3p are not elevated in testicular tumours of non-germ cell origin

PurposeSerum levels of microRNA-371a-3p (M371) have been shown to be a highly sensitive and specific biomarker for testicular germ cell tumours (TGCT). Little information exists on the expression of this marker in testicular neoplasms deriving from the gonadal stroma or other structures of the gonad. This study presents an expression analysis of the novel TGCT-biomarker M371 in a large cohort of testicular non-germ cell tumours.MethodsThe M371 expression was measured by quantitative real time PCR in serum of 99 patients with testicular tumours of non-germ cell origin, thereof 30 patients with malignant testicular lymphomas and 61 patients with gonadal stroma tumours such as Leydig cell tumours, Sertoli cell tumours and 8 cases with miscellaneous benign testicular tumours. Their M371 levels were compared to those of 20 patients with TGCT and to 37 tumour-free male controls.ResultsThe median expression levels of benign testicular tumours and testicular lymphoma are close to zero, thus, identical with those of controls and significantly lower than those of TGCT. In summary, this study provides further evidence for the notion that M371 is exclusively expressed by germ cell tumours and not by testicular neoplasms of the non-germ cell subtypes.ConclusionClinically, the test might be of value in preoperative characterization of benign testicular tumours eligible for conservative surgery.

Metro-SMHOP 01: Metronomics combination with cyclophosphamide-etoposide and valproic acid for refractory and relapsing pediatric malignancies.

In low- and middle-income countries, therapeutic options for advanced, refractory, or relapsing malignancies are limited due to local constraints such as cost of drugs, distance from oncology centers, and lack of availability of new anticancer drugs. Metronomics, which combines metronomic chemotherapy (MC) and drug repositioning, allows for the provision of new therapeutic options for patients in this setting. To evaluate the activity and toxicity of a metronomic regimen in Moroccan pediatric patients with refractory or relapsing malignancies. From July 2014 to January 2018, patients with refractory/relapsing solid tumors treated in five pediatric oncology centers were consecutively enrolled. The metronomic regimen consisted of 28-day cycles with daily oral administration of cyclophosphamide (30mg/m2 ) from days 1 to 21, together with oral etoposide (25mg/m2 ) from days 1 to 21 followed by break of one week and daily valproic acid (20mg/kg) from days 1 to 28. Ninety-eight children (median age, 8 years) were included. Underlying malignancies were neuroblastoma (24 patients), Ewing sarcoma (18), osteosarcoma (14), rhabdomyosarcoma (14), and miscellaneous tumors (28). A total of 557 cycles were given (median: 6; range, 1-18 cycles). One-year progression-free survival of our patients was 19%, and one-year overall survival was 22%. Complete response was obtained in three cases (3%), partial response in 11 cases (11%), and tumor stabilization for more than six months in 28 cases (28%). This three-drug metronomic combination was well tolerated and associated with tumor response and disease stabilization in 42 patients even for a long period.