The symptomatology of aneurysmal bone cyst : the value of diagnostic imaging

Background: An aneurysmal bone cyst (ABC) is a benign lesion occurring mainly in children. Generally it is found in the metaphyses of long bones and in flat bones. Osteolysis, bone expansion, thinning of the cortex, osseous septa and fluid levels belong to the typical imaging findings in ABC cases. The purpose of the study was to determine the value of imaging methods: plain x-ray films, US, CT and MRI for the diagnosis of aneurysmal bone cyst and to work out the diagnostic algorithm for an ABC suspected on plain radiograms. Material/Methods: The material consists of 72 patients (44 boys, 28 girls) aged 2-23 years (mean age 12 yrs) with bone lesions diagnosed as aneurysmal bone cysts on pathologic examination. In all patients, plain radiographs were performed, in 26 - sonography (US), in 41 - computed tomography (CT), in 8 - magnetic resonance imaging (MRI). All the examinations were analyzed according to own system of evaluation of typical presentation in the particular methods. The histopathological diagnosis was established after biopsy or surgery. Results: The aneurysmal bone cysts were most frequently located in the long bones of the lower limbs (43%), in the long bones of the upper limbs (26.4%) and in flat bones (25%). The localization was mainly in the bone methaphyses (56.6%). On pathological examination, primary ABC was diagnosed in 65.3% of cases, secondary ABC in 23.6%, ABC and concomitant osteitis in 7%, ABC and giant cell tumor in 2.7%, ABC and chondrosarcoma in 1.4%. The application of four methods resulted in 72.2% consistency between diagnostic imaging and pathological examination. In case of only one method used the consistency was 75% for MRI, 63% for CT, 55.4% for plain films and 50% for US. In 100% of misdiagnosed cases there were no septa within osteolytic lesions on plain films, whereas in 43.8% features of malignancy were found. In 53% of misdiagnosed cases no fluid levels were observed on CT and in 69% on US. Conclusions: 1. The diagnosis of aneurismal bone cyst based on plain radiography is possible in cases with typical radiological signs. 2. Application of other methods significantly increases the percentage of correct diagnoses. 3. Lack of fluid levels does not exclude the diagnosis of ABC. 4. Imaging features of malignancy do not exclude the diagnosis of ABC

Tumor expression of survivin, p53, cyclin D1, osteopontin and fibronectin in predicting the response to neo-adjuvant chemotherapy in children with advanced malignant peripheral nerve sheath tumor

Purpose Selected cell-cycle regulators and extracellular matrix proteins were found to play roles in malignant peripheral nerve sheath tumor (MPNST) biology. We aimed to analyze whether initial tumor tissue expressions of survivin, p53, cyclin D1, osteopontin (OPN) and fibronectin (FN) correlate with the response to neo-adjuvant CHT (naCHT) in children with advanced inoperable MPNST. Methods The study included 26 children with MPNST (M/F 14/12, median age 130 months) treated in Polish centers of pediatric oncology between 1992 and 2013. Tissue expression of markers was studied immunohistochemically in the manually performed tissue microarrays and assessed semi-quantitatively as low and high, based on the rate of positive cells and staining intensity. Results Good response to naCHT was noted in 47.6%, while poor-in 52.4% of patients. The response to naCHT was influenced negatively by the presence of neurofibromatosis NF1 and high initial tumor tissue expression of OPN, survivin, p53 and cyclin D1. Patients with high tumor expression of either OPN, survivin or p53 and those with simultaneous high expression of ≥ 3 of the markers, responded significantly worse to naCHT, than patients, in whom expression of ≤ 2 markers were detected at diagnosis. Nearly, 85% of patients expressing ≥ 3 markers, responded poor to CHT; while 87.5% of children, expressing ≤ 2 markers, were good responders. Conclusion The initial tumor tissue expression of OPN, survivin, p53 and cyclin D1 may serve as markers to predict response to naCHT in pediatric advanced MPNST. Future studies in more numerous group of patients are needed to confirm these preliminary results

Recommendations for diagnostics and therapy of adult patients with malignant primary bone tumors

Bone sarcomas comprise a heterogenous group of rare mesenchymal tumors (less than 0.5% of malignant neoplasms in adults). From clinical point of view they can be divided into two main groups: spindle-cell sarcomas (osteosarcomas, majority of chondrosarcomas and less common subtypes) and small-cell sarcomas (mainly Ewing family of tumors). Correct diagnosis and effective therapy is performed by cooperation of radiologists, oncological and orthopedics surgeons, clinical oncologists, radiotherapists, rehabilitants, pathologists, nuclear medicine specialists and molecular biologists. The most important principle in diagnostics and therapy of primary malignant bone tumors is multidisciplinary work in experienced centers. Improvement of diagnostics, implementation of combined therapy and technological developments caused the increase of limb-sparing surgery indications and better long-term results of their treatment. Onkol. Prak. Klin. 2010; 6, 6: 355–369Mięsaki kości u dorosłych stanowią heterogenną grupę bardzo rzadkich nowotworów pochodzenia mezenchymalnego (poniżej 0,5% nowotworów złośliwych u dorosłych). Pod względem klinicznym mięsaki kości można podzielić na mięsaki wrzecionowatokomórkowe (obejmujące mięsaki kościopochodne, większość chrzęstniakomięsaków i inne rzadsze podtypy) oraz drobnokomórkowe (głównie rodzina mięsaków Ewinga). Prawidłowe rozpoznanie i skuteczne leczenie skojarzone pierwotnych nowotworów kości są sumą współpracy radiologów, chirurgów onkologów i chirurgów ortopedów, onkologów klinicznych, radioterapeutów, rehabilitantów, patologów, specjalistów medycyny nuklearnej i biologów molekularnych. Bezwzględnym warunkiem w diagnostyce i leczeniu pierwotnych nowotworów złośliwych kości jest wielodyscyplinarna współpraca wielospecjalistyczna w doświadczonych ośrodkach. Polepszenie diagnostyki mięsaków kości, wprowadzenie zasad terapii skojarzonej i postęp technologiczny spowodowały rozszerzenie wskazań do stosowania operacji oszczędzających kończynę oraz poprawiły odległe wyniki leczenia. Onkol. Prak. Klin. 2010; 6, 6: 355–36

Zalecenia dotyczące postępowania diagnostyczno-terapeutycznego u chorych na pierwotne nowotwory złośliwe kości

Mięsaki kości u dorosłych stanowią heterogenną grupę bardzo rzadkich nowotworów pochodzenia mezenchymalnego (poniżej 0,5% nowotworów złośliwych u dorosłych). Prawidłowe rozpoznanie i skuteczne leczenie skojarzone pierwotnych nowotworów kości są sumą współpracy radiologów, chirurgów onkologów i chirurgów ortopedów, onkologów klinicznych, radioterapeutów, rehabilitantów, patologów, specjalistów medycyny nuklearnej i biologów molekularnych. Bezwzględnym warunkiem w diagnostyce i leczeniu pierwotnych nowotworów złośliwych kości jest wielodyscyplinarna współpraca wielospecjalistyczna w doświadczonych ośrodkach. Polepszenie diagnostyki mięsaków kości, wprowadzenie zasad terapii skojarzonej i postęp technologiczny spowodowały rozszerzenie wskazań do stosowania operacji oszczędzających kończynę oraz poprawiły odległe wyniki leczenia

Oxidative and Antioxidative Status Expressed as OSI Index and GSH/GSSG Ratio in Children with Bone Tumors after Anticancer Therapy Completion

Aims. There are no data on the redox status of children with bone tumors in complete disease remission. Therefore, the presented study examined the reduced/oxidized glutathione (GSH/GSSG) ratio, total oxidant capacity (TOC) and total antioxidant capacity (TAC) values as well as the oxidative stress index (OSI) for assessing alterations in the oxidant/antioxidant balance in 35 children with osteosarcoma or Ewing’s sarcoma after anticancer therapy completion (median 14 months) compared with a control group. Methods. GSH, GSSG, TOC, TAC concentrations and bone alkaline phosphatase (BALP) activity were evaluated by immunoenzymatic (ELISA) and enzymatic methods. Results. We found no differences in serum BALP activity between all survivors with bone tumors and the control group. Patients with osteosarcoma after anticancer therapy completion had significantly higher values of TAC, GSH and the GSH/GSSG ratio as well as GSSG than healthy subjects. In patients with Ewing’s sarcoma, we found significantly higher values of TOC concentration compared with healthy children. In addition, survivors with Ewing’s sarcoma had higher TOC concentrations and OSI index values (p < 0.01), but a lower GSH/GSSG ratio (p < 0.05) than survivors with osteosarcoma. A positive correlation between TOC and the post-therapy period was observed in survivors. Conclusions. We found that in survivors with bone tumors, a disturbed balance between prooxidants and antioxidants persists after the completion of anticancer treatment. Moreover, an increased TOC value together with the post-therapy period may suggest increasing oxidative processes in survivors with bone tumors after treatment. Further observations will allow assessment of the relationship between the oxidant/antioxidant status and the predisposition of survivors to bone neoplastic disease recurrence

Effective treatment of severe hypertensive crisis in 2-year-old child with neuroblastoma

We describe a case of a 2-year-old boy with neuroblastoma and high catecholamine levels which developed a severe hypertensive crisis. An oral propranolol and oral angiotensin-converting enzyme inhibitor were used with a mild short transient benefit. However, an intravenous labetalol and oral doxazosin used for over 3 weeks resulted in successful blood pressure control. This report highlights the prolonged use of α and β-adrenergic antagonist therapy in children with neuroblastoma with refractory catecholamine-induced hypertension

Solid Cancers in the Premature and the Newborn: Report of Three National Referral Centers

Advances in multidisciplinary care for pediatric cancer have resulted in significant improvement in cure rates over the last decades; however, these advances have not been uniform across all age groups. Cancer is an important cause of perinatal mortality, yet the full spectrum of malignant neoplasms in newborns is not well defined. Methods: The authors have reviewed the clinical features and outcomes of 37 newborns with congenital malignant tumors treated at three referral centers in North, Central, and South Poland between 1980 and 2014. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan–Meier methods and compared using long-rank test and Cox models. Results: Twenty-two patients were diagnosed prenatally. The most common diagnoses were neuroblastoma (48.7%), followed by malignant germ-cell tumor (16.2%), and Wilms' tumor (8.1%). Neuroblastoma was the most common malignancy among full-term infants, and malignant sacrococcygeal teratoma was the most common malignancy in premature infants. Thirty patients (81%) are alive with a median follow-up of 4.8 years from diagnosis. Patients with Wilms' tumor and malignant germ-cell tumors had the best outcomes (5-year OS 100% for both), whereas the worst prognosis was observed for sarcoma patients (5-year OS 72.92%). Premature infants had better outcome than full-term infants (5-year OS 92.8% vs. 72.58%, respectively). Conclusion: Although rare, neonatal cancers can present with an aggressive clinical behavior, but they have a generally good outcome. Early diagnosis and management by expert multidisciplinary teams that integrate perinatal medicine experts with pediatric and surgical oncologists are critical. Centralized care with clear referral pathways that facilitate early initiation of specialized treatment should be prioritized

Cancer Stem Cell Markers in Rhabdomyosarcoma in Children

(1) Background: The aim of the present study was to assess the cancer stem cell (CSC) markers CD24, CD44, CD133, and ALDH1A1 in rhabdomyosarcoma (RMS) in children and to define their prognostic role in this group of patients. (2) Methods: The study material was archival tissue specimens collected from 49 patients under 18 years of age and who had been diagnosed with RMS. Immunohistochemistry (IHC) was used to evaluate the expression of the selected CSC markers in the tumor tissue. Expression was evaluated using a semiquantitative IRS scale based on the one developed by Remmele and Stenger and was correlated with the clinical and pathomorphological parameters of prognostic importance in RMS. (3) Results: Expression of the selected CSC markers CD24, CD44, CD133, and ALDH1A1 was demonstrated in 83.7%, 55.1%, 81.6%, and 100% of the RMS patients, respectively. The expression of all of the assessed CSC markers was statistically significantly higher in the study group versus the control group. No significant correlation was found between the expression of the selected CSC markers and clinical and pathological prognostic factors that were analyzed. The expression of the CSC markers did not have a significant influence on RMS survival rates. (4) Conclusions: The results of the conducted study confirm the expression of selected CSC markers in rhabdomyosarcoma tissue in children. The study did not support the prognostic relevance of the expression of any of the assessed CSC markers. However, further studies are needed to fully understand the relevance of the selected CSC markers in RMS carcinogenesis