Pulmonary involvement in children with Langerhans cell histiocytosis.

BACKGROUND: Pulmonary Langerhans cell histiocytosis (pLCH) is a rare disease, mostly a component of multisystemic LCH. We aimed to investigate the clinical features and treatment results in children with pLCH. METHODS: We retrospectively reviewed the clinical, radiological, and treatment data of 37 patients with pLCH, diagnosed from 1974 to 2022. RESULTS: 10% (n=37) of 367 patients with LCH had lung involvement. The median age was 1.8 years (range: 0.4 & 17.7) with a male-to-female ratio of 2.3. At admission 29.7% (n=11) presented with respiratory symptoms. Imaging showed a spectrum from nodular opacities to multiple cysts. All but one patient had multisystem disease. Twenty-nine received vinblastine-containing therapy. Ten-year event-free (EFS) and overall survival (OS) rates were 47.8% and 63.3%, respectively. In children younger and older than two years of age, the 10-year EFS was 53.3% vs. 40.2% and the 10-year OS was 58.7% vs. 68.8%, respectively. In children with and without risk organ involvement, 10-year EFS was 51.9% vs. 46.3% and 10-year OS was 51.9% vs. 73.7%. CONCLUSIONS: Lung and multisystem involvement are significant concerns in LCH, highlighting the need for careful management to reduce morbidity and mortality.

Ifosfamide-induced Encephalopathy With Rapid Response to Thiamine: A Pediatric Case.

Ifosfamide is an important chemotherapeutic agent used in the therapeutic protocols of many malignant tumors. Central nervous system toxicity of ifosfamide manifests with encephalopathy in 10% to 30% of patients treated with ifosfamide. Thiamine and methylene blue have been reported beneficial in the treatment and prevention of ifosfamide-induced encephalopathy (IIE). We describe an episode of encephalopathy developed at the third cycle of ifosfamide treatment in a child with Ewing sarcoma. With the administration of thiamin, the encephalopathy resolved and no episode was noted during subsequent courses of ifosfamide. Previous use of cisplatin, concomitant use of opioids, low levels of serum albumin and hemoglobin, and elevated levels of serum creatinine are potential risk factors for IIE. The current case illustrates the possibility of IIE even in the absence of such additional risk factors, treated successfully with thiamin and draws attention to the need for close neurological monitorization of patients treated with ifosfamide.

Long-term clinical course and prognosis of hot water epilepsy: 15-Year follow-up.

OBJECTIVE: Hot water epilepsy (HWE) is a type of reflex epilepsy triggered by bathing with hot water. Hot water epilepsy is generally considered as a self-limiting benign disease although its long-term course and prognosis remains unknown. In this study, we aimed to determine the long-term clinical course and prognosis of hot water epilepsy and possible factors affecting them. METHODS: The diagnosis of HWE was made based on the clinical history obtained from patients and their first degree relatives witnessing to the seizures and video recordings of seizures if available; then, the type of seizure was identified. Good prognosis was defined as patients whose seizures were controlled with or without preventive measures and who did not require antiepileptic treatment. The poor prognosis was defined as patients whose seizures continued despite preventive measures and required antiepileptic treatment. RESULTS: The study included 50 (31 male and 19 female) patients with a mean follow-up of 17.63 ±â€¯10.46 (median, 15.0) years. The age at onset of seizure was 14.52 ±â€¯12.71 (median: 10.0) years. There were 38 (76%) patients in the good prognosis group. 18 (36%) of them achieved complete remission, who did not require preventive measures. In the remaining 20 (40%) patients, seizures could be controlled with only preventive measures. Seizures could be controlled with antiepileptic treatment in only 1 (2%) of 12 (24%) patients in the poor prognosis group. A significant relationship was found between the frequency of hot water seizures (HWSs) and poor prognosis (p = 0.019), as well as the presence of spontaneous seizures outside of bathing and poor prognosis (p = 0.000). SIGNIFICANCE: Hot water epilepsy, as previously known, is not a self-limiting benign disease. Approximately ¾ of the cases have a good prognosis, but the rest are in the case of chronic epilepsy. The low response rate to antiepileptics' treatment suggests that the pathogenesis of the HWE may differ from other epilepsies.

The effect of the COVID-19 pandemic on paediatric cancer care: lessons learnt from a major paediatric oncology department in Turkey.

The COVID-19 pandemic has disrupted cancer care. An audit at a major Paediatric Oncology Department in Turkey was performed to determine its impact on paediatric cancer care. A comparison was made among the number of daily paediatric cancer patients, diagnostic and treatment procedures. The data for the 'COVID-19 period' (10 March to 31 October 2020) were compared with the corresponding 'prior year control period' (10 March to 31 October 2019). Moreover, presentation delay (duration between first symptoms to healthcare visit) was calculated for new cases. The findings indicate that the mean 34.7 outpatients per day during 'COVID-19 period' was significantly lower than the 'prior year control period' (52.2). There were 17.7 inpatients per day during the 'COVID-19 period' which was significantly lower than 23.8 inpatients per day during the 'prior year control period'. Significant reduction in the daily mean number of patients undergoing chemotherapy, radiotherapy, surgery and imaging studies during the 'COVID-19 period' was also evident. A negative trend in the diagnosis of new paediatric cancers was evident with 128 new cancer cases during the 'COVID-19 period', whereas the corresponding number was 212 for the 'prior year control period'. The presentation delay (median 31 days) remain unchanged during the 'COVID-19 period'. The findings suggest significant damage to paediatric cancer care during the COVID-19 pandemic. Appropriate obligatory actions by oncology societies and policymakers can minimise longer term negative impacts.

Extraovarian juvenile granulosa cell tumor in a prepubertal child: novel location of a rare tumor.

Juvenile granulosa cell tumor (JGCT) is the most common type of sex cord stromal tumor arising from gonadal structures of children and young adults. We present a 3.5-year-old girl with JGCT located in retroperitoneum without ovarian involvement. Extragonadal occurrences of other sex cord stromal tumors have been rarely reported, but this is the first case of JGCT in an extragonadal location. We speculate the possible underlying mechanism of sex cord stromal tumor formation in extragonadal locations. Furthermore, clinical presentation, differential diagnosis and management of this tumor in childhood are discussed.

Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature.

BACKGROUND: Primary spinal, intradural, extramedullary Ewing sarcoma (PSIEES) is exceptionally uncommon. Here, we present an interesting pediatric case with a PSIEES diagnosis confirmed by the presence of a specific fusion protein in the tumor tissue and who then developed a cerebellar recurrence. We also reviewed the PSIEES cases in childhood reported in the literature. CASE: An 8.5-year-old boy was admitted to a local hospital with a one-month history of severe back and limb pain, and inability to move his lower limbs. Physical examination revealed paraparesis in the lower extremities. Spinal MRI revealed multiple intradural extramedullary masses at the L2-L3, L4-5 and L5-S1 levels. He underwent surgery and near total excision of all three masses were performed. Histopathological diagnosis of Ewing Sarcoma was confirmed with EWS-ERG gene rearrangement. The patient was treated according to EuroEwing chemotherapy protocol. A total dose of 4500 cGy radiotherapy was applied to the tumor location at L2-S1 paravertebral region. Eighteen months after the end of treatment, a mass in the left cerebellar hemisphere was determined. Gross total excision was performed. Histopathological examination of the tumor showed Ewing sarcoma. Radiological screening revealed isolated central nervous system recurrence. A total of 4500 cGy radiotherapy was applied. He is on a second-line treatment consisting of gemcitabine and docetaxel without any evidence of disease. CONCLUSIONS: Ewing Sarcoma with spinal intradural region in childhood is very rare. We could only find 17 pediatric cases reported in the literature. Neurological findings occur earlier in tumors of this region. The prognosis is worse than other extraosseous Ewing sarcoma.