Palliative care for children with central nervous system tumors: results of a Spanish multicenter study

Background Brain tumors represent the most common cause of cancer-related death in children. Few studies concerning the palliative phase in children with brain tumors are available. Objectives (i) To describe the palliative phase in children with brain tumors; (ii) to determine whether the use of palliative sedation (PS) depends on the place of death, the age of the patient, or if they received specific palliative care (PC). Methods Retrospective multicenter study between 2010 and 2021, including children from one month to 18 years, who had died of a brain tumor. Results 228 patients (59.2% male) from 10 Spanish institutions were included. Median age at diagnosis was 5 years (IQR 2–9) and median age at death was 7 years (IQR 4–11). The most frequent tumors were medulloblastoma (25.4%) and diffuse intrinsic pontine glioma (DIPG) (24.1%). Median number of antineoplastic regimens were 2 (range 0–5 regimens). During palliative phase, 52.2% of the patients were attended by PC teams, while 47.8% were cared exclusively by pediatric oncology teams. Most common concerns included motor deficit (93.4%) and asthenia (87.5%) and communication disorders (89.8%). Most frequently prescribed supportive drugs were antiemetics (83.6%), opioids (81.6%), and dexamethasone (78.5%). PS was administered to 48.7% patients. Most of them died in the hospital (85.6%), while patients who died at home required PS less frequently (14.4%) (p = .01). Conclusion Children dying from CNS tumors have specific needs during palliative phase. The optimal indication of PS depended on the center experience although, in our series, it was also influenced by the place of death (AU)

Palliative care for children with central nervous system tumors: results of a Spanish multicenter study.

BACKGROUND: Brain tumors represent the most common cause of cancer-related death in children. Few studies concerning the palliative phase in children with brain tumors are available. OBJECTIVES: (i) To describe the palliative phase in children with brain tumors; (ii) to determine whether the use of palliative sedation (PS) depends on the place of death, the age of the patient, or if they received specific palliative care (PC). METHODS: Retrospective multicenter study between 2010 and 2021, including children from one month to 18 years, who had died of a brain tumor. RESULTS: 228 patients (59.2% male) from 10 Spanish institutions were included. Median age at diagnosis was 5 years (IQR 2-9) and median age at death was 7 years (IQR 4-11). The most frequent tumors were medulloblastoma (25.4%) and diffuse intrinsic pontine glioma (DIPG) (24.1%). Median number of antineoplastic regimens were 2 (range 0-5 regimens). During palliative phase, 52.2% of the patients were attended by PC teams, while 47.8% were cared exclusively by pediatric oncology teams. Most common concerns included motor deficit (93.4%) and asthenia (87.5%) and communication disorders (89.8%). Most frequently prescribed supportive drugs were antiemetics (83.6%), opioids (81.6%), and dexamethasone (78.5%). PS was administered to 48.7% patients. Most of them died in the hospital (85.6%), while patients who died at home required PS less frequently (14.4%) (p = .01). CONCLUSION: Children dying from CNS tumors have specific needs during palliative phase. The optimal indication of PS depended on the center experience although, in our series, it was also influenced by the place of death.

Ensayos clínicos y cuidados paliativos pediátricos / Clinical trials and pediatric palliative care

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Role of 18F-FDG-PET/CT in the management of Burkitt lymphoma.

UNLABELLED: Burkitt lymphoma (BL) is highly FDG-avid even though its usefulness in the management of these patients is still controversial. AIM: We analyzed the role of positron emission tomography/computerized tomography (PET/CT) in staging newly diagnosed patients with BL and evaluating disease after first-line chemotherapy. METHODS: Fifty-two PET/CTs were performed in 32 patients (20 at diagnosis, 27 after treatment, five to monitor residual disease). Involved areas were retrospectively compared with those observed in contrast-enhanced CT. RESULTS: Discrepancies were found in 64.7% of patients for whom results of both tests at diagnosis were available (n = 17), most of them involving extranodal sites. Regarding response assessment, discrepancies were observed in 38% of patients with both tests (5/13): residual masses detected by CT with negative PET/CT. Of 27 patients with post-treatment PET/CT, 22 were in complete remission whereas one true-positive and four false-positive lesions (two nodal and two extranodal) were detected. With a median follow-up of 27 months, 22 patients with negative PET/CT did not relapse. Thus, negative predictive value (NPV) was 100%. With respect to positive predictive value (PPV), one of five patients with positive assays after treatment died due to progression while the remaining four had false-positive lesions. Nevertheless, for these four patients, mean SUVmax at nodal sites was 4.1 vs. 14.9 at diagnosis, while mean SUVmax at extranodal sites was 3.8 vs. 12.1. Thus, with a cutoff value for SUVmax < 66% of that observed at diagnosis, PPV was also 100%. CONCLUSION: More accurate staging can be achieved using PET/CT. NPV reaches 100%, and using a ΔSUV < 66%, a high PPV is also observed.

Primary cerebral radiotherapy-induced rhabdomyosarcoma: treatment with intraoperative carmustine implants.

INTRODUCTION: Primary cerebral rhabdomyosarcomas (cRMS) are extremely rare, with only 41 cases reported in the literature. Survival of patients with localized cRMS is 70% after 5 years but not in the case of intracranial neoplasms, where survival rarely exceeds 10 months. CASE REPORT: A 10-year-old female patient with a history of acute lymphoblastic leukemia (ALL) and holocranial radiotherapy (RT) 6 years ago, referred after partial surgical resection of a left parietal lesion, diagnosed as an embryonal tumor with mixed neuronal-glial differentiation (WHO grade IV). A second operation was performed for complete resection and placement of intracavitary chemotherapy (carmustine). The pathology revealed a high-grade undifferentiated neoplasm positive for myogenin and desmin that was compatible with cRMS. In the immunohistochemistry study, the neoplasm was positive for vimentin, myogenin, and desmin, as is characteristic of cRMS, and negative for synaptophysin and enolase, ruling out primitive neuroectodermal embriogenic tumor (PNET). Given a diagnosis of cRMS, a combined thoracoabdominal PET-CT scan was performed without finding other primary lesions and a bone marrow study was also performed without observing abnormalities. Consequently, the diagnosis was established as primary cRMS. DISCUSSION: Among the long-term sequelae of radiotherapy, neurocognitive disorders, brain disorders such as leukomalacia, vascular diseases, or secondary tumors, ranging from benign lesions such as meningiomas to more aggressive lesions such as ependymomas, which are high-grade gliomas, are described. In the brain MRI, our patient showed a radiotherapy-induced periventricular leukomalacia and a malignant lesion: a cRMS. The use of carmustine in this disease may facilitate local control.

Initial experience involving treatment and retreatment with carmustine wafers in combination with oral temozolomide: long-term survival in a child with relapsed glioblastoma multiforme.

BACKGROUND: Glioblastomas occur infrequently in children, and the prognosis is better than for glioblastomas seen in adults. Aggressive treatment is justified in pediatric patients. OBSERVATIONS: We present the case of a 6-year-old child with malignant posterior temporal glioma treated with surgery, radiotherapy, local chemotherapy with carmustine wafers, and oral therapy with temozolomide, both at initial diagnosis and at relapse 18 months later. After 6 years, the patient seems healthy with no focal neurologic signs, and imaging studies show no evidence of disease. CONCLUSION: Multimodal therapy was found to have a very positive outcome for a child with malignant glioma.