Spontaneous resolution of unifocal Langerhans cell histiocytosis of the skull: potential role of ultrasound in detection and imaging follow-up

Vanhoenacker, Filip; Verlooy, Joris; Praeter, Mania De

doi:10.15557/jou.2018.0038

Cited by 6 publications

(3 citation statements)

References 9 publications

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“…- The accessibility of the lesions for monitoring with both clinical examination and, potentially, easily available diagnostic modalities, such as ultrasound [ 11 , 13 ] which has been proposed as a valid follow-up imaging modality, with the advantage of sparing young patients from radiation [ 11 ].…”

Section: Discussionmentioning

confidence: 99%

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

Benato¹,

Riva²,

Raneri³

2023

Childs Nerv Syst

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Introduction Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited. Methods Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG. Results In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination. Conclusion Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.

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Section: Discussionmentioning

confidence: 99%

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

Benato¹,

Riva²,

Raneri³

2023

Childs Nerv Syst

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“…Nevertheless, it is interesting to note the subset of patients who had isolated bony lesions that underwent spontaneous resolution—even without any additional local therapy, such as curettage. Future studies in radiological features of quiescent SS-LCH ( 18 ) may reduce need for any surgery in this otherwise benign subtype of LCH.…”

Section: Discussionmentioning

confidence: 99%

A “Wait-and-See” Approach to Quiescent Single-System Langerhans Cell Histiocytosis to Spare Children From Chemotherapy

Lee

et al. 2020

Front. Pediatr.

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Background: Langerhans Cell Histiocytosis (LCH) is a childhood disorder of histiocytes that is generally treated with systemic chemotherapy. Spontaneous resolution has been previously reported in Single System LCH (SS-LCH), which is less aggressive than multisystem disease. However, there are no clear guidelines on which patients can be safely spared from systemic chemotherapy. Here, we propose a risk stratification framework based on disease quiescence as determined by clinical and biochemical features of inflammation, to identify low risk patients who may be potentially spared from chemotherapy through a conservative "wait-and-see" approach. Methods: Retrospective analysis in a single institution was conducted in children with SS-LCH, comparing features of inflammation and outcomes of those who received chemotherapy vs. those with quiescent disease, who were managed conservatively. Results: Of 44 children with SS-LCH, only patients without risk-organ involvement were considered for conservative management. A "wait-and-see" approach was adopted for patients with quiescent disease as defined by clinical and biochemical evidence of disease activity. Following 2 weeks of watchful observation, decisions were made to either start treatment or continue conservative management. Based on data collected at diagnosis, patients with quiescent disease had a lower mean platelet count 339 × 10 9 /L (95%C.I: 285-393) vs. 482 × 10 9 /L (95% C.I: 420-544) p < 0.01, a lower mean white cell count 9.3 × 10 9 /L (95%C.I: 7.5-11.1) vs. 13.1 × 10 9 /L (95%C.I: 11-15.2) p < 0.01 and lower Erythrocyte-Sedimentation-Rate (ESR) 8.2 mm/h (95%C.I: 5.4-11) vs. 53.7 mm/h (95%C.I: 11-96.3) p = 0.04, suggesting that these are potential biochemical markers of disease activity. Other features of disease quiescence noted were rapid progression, functional disability, presence of a skull depression rather a lump and the lack of fever. Conclusions: Further studies are required to validate our proposed framework to determine disease activity in SS-LCH. Within the limits of this current analysis, it appears that low-risk patients with clinically and biochemically quiescent SS-LCH, may potentially be spared from chemotherapy with good long-term outcomes.

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“…Интраскопически представляет собой мягкотканное объемное образование в участке литического поражения кости свода черепа, края костного дефекта ровные или скошенные. УС-признаки эозинофильной гранулемы: дефект кости (чаще теменной); костный дефект заполнен гипо эхогенной массой, распространяющейся в пределах мягких тканей и интракраниально; по ЦДК чаще имеются признаки кровотока; из-за отсутствия кости визуализируются внутричерепные структуры под очагом поражения [43,45,53,54]. W. Kosiak и соавт.…”

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Skull and scalp ultrasound in children: a review

Kriukova,

Iova,

Kryukov

et al. 2023

Lučevaâ diagn. ter.

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INTRODUCTION: An important task of modern pediatrics is to ensure radiation safety of diagnostic examinations, especially in young children. One of the options for reducing radiation exposure at the stages of screening diagnostics and dynamic monitoring is a wider use of ultrasound.OBJECTIVE: To analyze the data of domestic and foreign literature on the possibilities of ultrasound examination of the cranial vault bones, cranial sutures and scalp in children.MATERIALS AND METHODS: The literature search was performed in open Russian and English databases Medline, PubMed, Web of Science, RSCI, eLIBRARY using keywords and phrases: «skull ultrasound», «scalp ultrasound», «cranial sutures ultrasound», «point of care ultrasound», «pediatric POCUS» without limitation of retrospective depth.RESULTS: Based on the literature data and our own long-term experience in the use of cranial ultrasonography in clinical practice, the indications and examination technique, as well as the key ultrasound signs of the most frequent types of pathology are described. Prospects of scalp and skull ultrasonography within PoCUS, FAST, including the use of portable sonoscopes based on smartphones and tablets are outlined.CONCLUSION: Ultrasound of the skull and scalp is a quick, simple, affordable, harmless method of screening and monitoring the most frequent types of pathologies of the cranial vault bones, cranial sutures, and soft tissues of the scalp in children (for example, fractures, synostoses, neoplasms).

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Spontaneous resolution of unifocal Langerhans cell histiocytosis of the skull: potential role of ultrasound in detection and imaging follow-up

Cited by 6 publications

References 9 publications

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature

A “Wait-and-See” Approach to Quiescent Single-System Langerhans Cell Histiocytosis to Spare Children From Chemotherapy

Skull and scalp ultrasound in children: a review

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