Misdiagnosing recurrent medulloblastoma: the danger of examination and imaging without histological confirmation

Weintraub, Lauren; Miller, Todd S.; Friedman, Ilana; Abbott, Rick; Levy, Adam

doi:10.3171/2013.10.peds13231

Cited by 9 publications

(10 citation statements)

References 30 publications

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“…Indeed, recent evidence has suggested that a number of late medulloblastoma recurrences diagnosed radiologically may be in fact secondary malignant gliomas histologically. 25,26 Finally, the increasing number of 5-year survivors across treatment eras reflects improved 5-year survival from medulloblastoma as a result of improvement in treatment. Although the sample size differs across the decades, comparisons of treatment changes with outcomes across these decades is valid because our cohort represents each era's 5-year survivor population in an unbiased way.…”

Section: Discussionmentioning

confidence: 99%

Late Morbidity and Mortality Among Medulloblastoma Survivors Diagnosed Across Three Decades: A Report From the Childhood Cancer Survivor Study

Salloum

Chen

Yasui

et al. 2019

JCO

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PURPOSE Treatment of medulloblastoma has evolved from surgery and radiotherapy to contemporary multimodal regimens. However, the impact on long-term health outcomes remains unknown. METHODS Cumulative incidence of late mortality (5 or more years from diagnosis), subsequent neoplasms (SNs), and chronic health conditions were evaluated in the Childhood Cancer Survivor Study among 5-year survivors of medulloblastoma diagnosed between 1970 and 1999. Outcomes were evaluated by treatment exposure, including historical therapy (craniospinal irradiation [CSI] ≥ 30 Gy, no chemotherapy), high risk (CSI ≥ 30 Gy + chemotherapy), standard risk (CSI < 30 Gy + chemotherapy), and by treatment decade (1970s, 1980s, 1990s). Rate ratios (RRs) and 95% CIs estimated long-term outcomes using multivariable piecewise exponential models. RESULTS Among 1,311 eligible survivors (median age, 29 years [range, 6 to 60 years]; median time from diagnosis, 21 years [range, 5 to 44 years]), the 15-year cumulative incidence rate of all-cause late mortality was 23.2% (diagnosed 1970s) versus 12.8% (1990s; P = .002), with a recurrence-related mortality rate of 17.7% versus 9.6% ( P = .008). Lower late mortality rates as a result of other health-related causes were not observed. Among 997 survivors who completed a baseline survey, the 15-year cumulative incidence of SNs was higher among survivors with multimodal therapy (standard risk, 9.5%; historical, 2.8%; P = .03). Survivors treated in the 1990s had a higher cumulative incidence of severe, disabling, life-threatening, and fatal chronic health conditions (56.5% in 1990s v 39.9% in 1970s; P < .001) and were more likely to develop multiple conditions (RR, 2.89; 95% CI, 1.31 to 6.38). However, survivors of standard-risk therapy were less likely to use special education services than high-risk therapy survivors (RR, 0.84; 95% CI, 0.75 to 0.93). CONCLUSION Historical changes in medulloblastoma therapy that improved 5-year survival have increased the risk for SNs and debilitating health conditions for survivors yet reduced the need for special education services.

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Section: Discussionmentioning

confidence: 99%

Late Morbidity and Mortality Among Medulloblastoma Survivors Diagnosed Across Three Decades: A Report From the Childhood Cancer Survivor Study

Salloum

Chen

Yasui

et al. 2019

JCO

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“…Weintraub et al, have also described a 10-year old girl with suspected relapsed medulloblastoma when she presented with new clinical deficits and radiological evidence consistent with recurrence. 19 In this case as well, both clinical and imaging findings completely resolved after treatment with steroids. A definite diagnosis was not established.…”

Section: Introductionmentioning

confidence: 51%

“…A definite diagnosis was not established. 19 The case highlights the risks of assuming recurrence based on clinical and imaging changes alone. In our patient, after a short-lived initial improvement both symptoms and radiological findings worsened when the steroids were weaned.…”

Section: Introductionmentioning

confidence: 99%

Challenges in the Diagnosis of Medulloblastoma Recurrence at an Unusual Site in a Patient With Prader-Willi Syndrome

Panagopoulou¹,

Sattar²,

Aquilina³

et al. 2019

Journal of Pediatric Hematology/Oncology

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Medulloblastoma is the most common malignant paediatric brain tumour. Survival rates range between 50-80% depending on histology and other biological features, metastases and treatment approach. Prader-Willi syndrome (PWS) is a genetically inherited disorder characterized by dysmorphic features, mental retardation, obesity and hypogonadism among other features. We describe a 10.5-year-old girl with PWS and previous standard-risk medulloblastoma that relapsed in the pons three years after the end of treatment. Diagnosis of relapse was delayed by a preceding varicella infection, an initial clinical/radiological response to steroids and the unusual location and was confirmed with a stereotactic biopsy. The patient did not respond to secondline treatment. This is the first report of a medulloblastoma in a patient with PWS

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“…However, while the majority of primary CNS ET typically show contrast enhancement, the degree of enhancement can be variable, and considering only MB, approximately 11% to 25% do not enhance [ 7 – 10 ]. In addition, although MRI findings of pia-arachnoidal or focal nodular brain enhancement were previously described as highly specific in the diagnosis of recurrent disease [ 11 ], contrast enhancement may occur as a result of radiation therapy [ 12 , 13 ], thus posing a major diagnostic challenge.…”

Section: Introductionmentioning

confidence: 99%

Added value of diffusion weighted imaging in pediatric central nervous system embryonal tumors surveillance

et al. 2017

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Diffusion weighted imaging (DWI) has an established role in primary CNS embryonal tumor (ET) characterization; however, its diagnostic utility in detecting relapse has never been determined. We aimed to compare DWI and conventional MRI sensitivity in CNS ET recurrence detection, and to evaluate the DWI properties of contrast-enhancing radiation induced lesions (RIL).Fifty-six patients with CNS ET (25 with disease relapse, 6 with RIL and 25 with neither disease relapse nor RIL) were retrospectively evaluated with DWI, conventional MRI (including both T2/FLAIR and post-contrast images), or contrast-enhanced MR imaging (CE-MRI) alone. MRI studies were independently reviewed by two neuroradiologists for detection and localization of potential brain relapses. Sensitivity for focal relapse detection was calculated for each image set on a lesion-by-lesion basis. A descriptive per subject analysis was also performed. Evaluation of follow-up MRI studies served as standard of reference.Focal recurrence detection sensitivity of DWI (96%) was significantly higher than conventional MRI (77%) and CE-MRI alone (51%) (p=0.0003 and p<0.0001). On per subject analysis there were not missed diagnoses for DWI. At the time of DWI relapse detection, conventional MRI missed 2 diagnoses, and CE-MRI 8. Analysis of medulloblastoma relapses revealed that DWI identified a higher number of focal lesions than CE-MRI in subjects with classic variant. All but one RIL did not show restricted diffusion.In conclusion, DWI is a valuable complementary technique allowing for improved detection of focal relapse in CNS ET patients, particularly in children with classic medulloblastoma, and may assist in differentiating recurrence from RIL.

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Misdiagnosing recurrent medulloblastoma: the danger of examination and imaging without histological confirmation

Cited by 9 publications

References 30 publications

Late Morbidity and Mortality Among Medulloblastoma Survivors Diagnosed Across Three Decades: A Report From the Childhood Cancer Survivor Study

Late Morbidity and Mortality Among Medulloblastoma Survivors Diagnosed Across Three Decades: A Report From the Childhood Cancer Survivor Study

Challenges in the Diagnosis of Medulloblastoma Recurrence at an Unusual Site in a Patient With Prader-Willi Syndrome

Added value of diffusion weighted imaging in pediatric central nervous system embryonal tumors surveillance

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