Ewing's sarcoma of bone: Oncologic and functional results

This paper describes 29 patients with Ewing's sarcoma of bone treated between 1975 and 1990 at the University of Nijmegen Hospital, Nijmegen, The Netherlands. Osteomyelitis was the primary diagnosis in 24%. Treatment consisted of chemotherapy in combination with surgery and/or radiotherapy. Nine patients received radiotherapy only; five of them died of disease. Five patients underwent an intralesional excision; four of them died of disease. Twelve patients underwent a wide excision; there is no evidence of disease in any of them. Three patients underwent a radical disarticulation; all died of disease. The disease-free survival at 1.5 years was 66%. This figure at 5 years was 55%. After wide excision and reconstruction in tumors of expendable, femoral or radial bones good functional results were obtained in all cases. © 1995 Wiley-Liss, Inc.     

The incidence and natural course of occult inguinal hernias during TAPP repair

Background

One of the proposed advantages of laparoscopic inguinal hernia repair is complimentary inspection of the contralateral side and possible detection of occult hernias. Incidence of occult contralateral hernias is as high as 50 %. The natural course of such occult defects is unknown and therefore operative rationale is lacking. This study was designed to analyze the incidence of occult contralateral inguinal hernias and its natural course.

Methods

A total of 1,681 patients were diagnosed preoperatively with unilateral inguinal hernia. None of these patients had complaints of the contralateral side preoperatively. All patients underwent laparoscopic inguinal hernia transabdominal preperitoneal (TAPP) repair. Operative details were analyzed retrospectively. Patients with occult contralateral defects were identified and tracked. Patients with an evident occult hernia received immediate repair. Patients with a smaller beginning or incipient hernia were followed.

Results

In 218 (13 %) patients, an occult hernia was found at the contralateral side during preoperative exploration. In 129 (8 %) patients, an occult true hernia was found. In 89 (5 %) patients, an occult incipient hernia was found. An incipient hernia was defined as a beginning hernia. All patients with an incipient hernia were followed. The mean follow-up was 112 (range 16–218) months. Twenty-eight (32 %) patients were lost to follow-up. In the 61 remaining patients, 13 (21 %) occult incipient hernias became symptomatic requiring repair. The mean time between primary repair and development of a symptomatic hernia on the contralateral side was 88 (range 24–210) months.

Conclusions

This study shows that the incidence of occult contralateral hernias is 13 % during TAPP repair of unilateral diagnosed inguinal hernias. In 5 % of the cases, the occult hernia consisted of a beginning hernia. Eventually, one of five will become symptomatic and require repair. These outcomes support immediate repair of occult defects, no matter its size.     

Differentiation between rebound thymic hyperplasia and thymic relapse after chemotherapy in pediatric Hodgkin lymphoma

Background

Rebound thymic hyperplasia (RTH) is a common phenomenon caused by stress factors such as chemotherapy (CTX) or radiotherapy, with an incidence between 44% and 67.7% in pediatric lymphoma. Misinterpretation of RTH and thymic lymphoma relapse (LR) may lead to unnecessary diagnostic procedures including invasive biopsies or treatment intensification. The aim of this study was to identify parameters that differentiate between RTH and thymic LR in the anterior mediastinum.

Methods

After completion of CTX, we analyzed computed tomographies (CTs) and magnetic resonance images (MRIs) of 291 patients with classical Hodgkin lymphoma (CHL) and adequate imaging available from the European Network for Pediatric Hodgkin lymphoma C1 trial. In all patients with biopsy-proven LR, an additional fluorodeoxyglucose (FDG)-positron emission tomography (PET)-CT was assessed. Structure and morphologic configuration in addition to calcifications and presence of multiple masses in the thymic region and signs of extrathymic LR were evaluated.

Results

After CTX, a significant volume increase of new or growing masses in the thymic space occurred in 133 of 291 patients. Without biopsy, only 98 patients could be identified as RTH or LR. No single finding related to thymic regrowth allowed differentiation between RTH and LR. However, the vast majority of cases with thymic LR presented with additional increasing tumor masses (33/34). All RTH patients (64/64) presented with isolated thymic growth.

Conclusion

Isolated thymic LR is very uncommon. CHL relapse should be suspected when increasing tumor masses are present in distant sites outside of the thymic area. Conversely, if regrowth of lymphoma in other sites can be excluded, isolated thymic mass after CTX likely represents RTH.     

Improved survival for adolescents and young adults with Hodgkin lymphoma and continued high survival for children in the Netherlands: a population-based study during 1990–2015

Population-based studies that assess long-term patterns of incidence, major aspects of treatment and survival are virtually lacking for Hodgkin lymphoma (HL) at a younger age. This study assessed the progress made for young patients with HL (<25 years at diagnosis) in the Netherlands during 1990–2015. Patient and tumour characteristics were extracted from the population-based Netherlands Cancer Registry. Time trends in incidence and mortality rates were evaluated with average annual percentage change (AAPC) analyses. Stage at diagnosis, initial treatments and site of treatment were studied in relation to observed overall survival (OS). A total of 2619 patients with HL were diagnosed between 1990 and 2015. Incidence rates increased for 18–24-year-old patients (AAPC + 1%, P = 0·01) only. Treatment regimens changed into less radiotherapy and more ‘chemotherapy only’, different for age group and stage. Patients aged 15–17 years were increasingly treated at a paediatric oncology centre. The 5-year OS for children was already high in the early 1990s (93%). For patients aged 15–17 and 18–24 years the 5-year OS improved from 84% and 90% in 1990–1994 to 96% and 97% in 2010–2015, respectively. Survival for patients aged 15–17 years was not affected by site of treatment. Our present data demonstrate that significant progress in HL treatment has been made in the Netherlands since 1990.     

Development of a LC method for pharmaceutical quality control of the antimetastatic ruthenium complex NAMI-A

Imidazolium trans-tetrachloro(dimethylsulfoxide)imidazoleruthenium(III) (NAMI-A) is a novel ruthenium complex with selective activity against metastases currently in Phase I clinical trials in the Netherlands. Pharmaceutical quality control of NAMI-A drug substance and lyophilized product warranted the development of an assay for determination and quantification of NAMI-A and degradation products. A high performance liquid chromatography (HPLC) method was developed, consisting of a C18 column with 0.50 mM sodium dodecylsulfate in 3% methanol at pH 2.5 (acidified using trifluoromethanesulfonic acid) as the mobile phase and UV-detection at 358 nm. The HPLC method was proven to be linear, accurate and precise. Stress testing showed that degradation products were separated from the parent compound. By combining results of nuclear magnetic resonance (NMR) and HPLC experiments, one degradation product was identified as the mono-hydroxy species of NAMI-A. HPLC analysis with off-line detection of the eluate with flameless atomic absorption spectrometry (F-AAS) showed that under most conditions, all ruthenium-containing compounds show a peak in the HPLC chromatogram and that all ruthenium applied to the column is recovered quantitatively. For completely degraded solutions of NAMI-A some ruthenium is retained on the column. Suitability of the HPLC method for the pharmaceutical quality control of NAMI-A lyophilized product was demonstrated.     

Post-error adaptation in adults with high functioning autism

Deficits in executive function (EF), i.e. function of the prefrontal cortex, may be central in the etiology of autism. One of the various aspects of EF is error detection and adjusting behavior after an error. In cognitive tests, adults normally slow down their responding on the next trial after making an error, a compensatory mechanism geared toward improving performance on subsequent trials, and a faculty critically associated with activity in the anterior cingulate cortex (ACC). The current study evaluated post-error slowing in people with high functioning autism (HFA) (n=36), taking symptom severity into account, compared to the performance of a normal control group (n=32). Symptom severity in the HFA group was defined in terms of level of adaptation: living independently (outpatients; n=12) and living residentially (inpatients; n=24). Half the group of inpatients was on medication; the results of their performance were analyzed separately. A computerized version of a memory search task was used with two response probability conditions. The subjects in the control group adjusted their reaction time (RT) substantially after an error, while the group of participants with HFA appeared to be overall slow, with no significant adjustment of RT after an error. This finding remained significant if the medication factor was taken into account, and was independent of the degree of severity of the autistic disorder, as defined by the dichotomy 'inpatient versus outpatient'. Possible causes and implications of the finding are discussed.