Langerhans cell histiocytosis of skull: a retrospective study of 18 cases

Zhang, Xiang Heng; Zhang, Ji; Chen, Zheng He; Sai, Ke; Chen, Yin Sheng; Wang, Jian; Ke, Chao; Guo, Cheng; Chen, Zhong Ping; Mou, Yong

doi:10.21037/apm.2016.11.04

Cited by 13 publications

(7 citation statements)

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“…Long-term follow-up is necesssary as recurrence may occur. 10 In conclusion, rapid expansion and invasion may occur in calvarial unifocal LCH with a low Ki-67 nuclear antigen expression. Even though the clinical and MRI findings may resemble an infection, rapidly expanding calvarial localised LCH should also be kept in mind in the differential diagnosis in an adult when a destructive skull lesion and peripheral and/or septal enhancing extra-axial soft tissue component is present.…”

Section: Discussionmentioning

confidence: 82%

A rapidly expanding calvarial Langerhans cell histiocytosis with low Ki-67 in an adult: a challenging diagnosis on magnetic resonance imaging

et al. 2017

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Langerhans cell histiocytosis may have a wide variety of clinical presentations. The growth of localised form is usually slow and it mainly involves bones such as the skull, femur, spine, ribs, mandible and pelvis in children. The spectrum of clinical manifestations and magnetic resonance imaging findings of the disease may sometimes mimic infections as well as benign and malignant tumours. In this report, we describe an unusual case of rapidly progressive calvarial Langerhans cell histiocytosis with a painful and tender mass in a 27-year-old man that created diagnostic and therapeutic challenges. We also discuss the relationship between Ki-67 proliferative index and the expansion of the disease. Magnetic resonance imaging studies revealed a bone defect on the left frontal bone with soft tissue components, showing a prominent expansion within a month. The clinical manifestation with the enhancement pattern of epidural soft tissue component on contrast-enhanced magnetic resonance imaging suggested an infection besides Langerhans cell histiocytosis. Immunohistochemical expression of Ki-67 antigen was 5%. The presence of a relatively low Ki-67 value indicates that the inflammatory response may have a more important role than local Langerhans cell proliferation in the aggressive clinical course and rapid expansion.

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Section: Discussionmentioning

confidence: 82%

A rapidly expanding calvarial Langerhans cell histiocytosis with low Ki-67 in an adult: a challenging diagnosis on magnetic resonance imaging

et al. 2017

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“…In general, such LCH is anticipated to have a good prognosis without the need for systemic therapy after successful initial treatment. [2,6,16,17] However, LCH in adults is rare and not well understood. Furthermore, most of our knowledge about its diagnosis and treatment comes from pediatric studies.…”

Section: Discussionmentioning

confidence: 99%

“…Most present as osteolytic lesions or develop in association with the previous surgeries. [5,9,[11][12][13]17] Here, we present an isolated calvarial LCH in an adult patient that developed within a short period of time.…”

Section: Introductionmentioning

confidence: 99%

Calvarial Langerhans cell histiocytosis in an adult presenting rapid growth

Sugiyama

Tsutsumi

Hashizume

et al. 2022

Surgical Neurology International

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Background: Langerhans cell histiocytosis (LCH) arising in the skull is rare in adulthood. Case Description: A 58-year-old woman experienced a durable headache. Cerebral magnetic resonance imaging (MRI) at presentation showed no abnormalities; however, MRI and computed tomography (CT) performed 6 weeks later revealed the emergence of a well-demarcated, heterogeneously enhancing calvarial tumor accompanied by irregular-shaped bone erosion. On MRI, the temporalis muscle and subcutaneous tissue adjacent to the tumor were extensively swollen and enhanced. The patient underwent en bloc resection. The microscopic appearance of the tumor was consistent with that of LCH. Postoperative systemic 18F-fluorodeoxyglucose positron emission tomography/CT did not detect any abnormal accumulation. Conclusion: LCH may develop within a short period. It should be considered as a differential diagnosis when a rapidly growing calvarial tumor is encountered, even when the patient is an adult. Prompt histological verification is recommended in such cases.

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“…The lesion typically does not cross the sutures. CT may also allow to visualize the soft tissue component (3) .…”

Section: Discussionmentioning

confidence: 99%

“…The differential diagnosis of EG of the skull in children includes a congenital skull defect (10) , an epidermoid or dermoid cyst (3) , a growing fracture (8) , a hemangioma and metastatic neuroblastoma (4) .…”

Section: Discussionmentioning

confidence: 99%

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

Vanhoenacker¹,

Verlooy

Praeter

2018

J Ultrason

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Langerhans cell histiocytosis is a tumor-like condition characterized by idiopathic proliferation of Langerhans cells. The disease may involve the skeleton as well as other organs systems. Bone involvement may be solitary or multifocal. Unifocal osseous Langerhans cell histiocytosis may involve virtually any bone, with the calvarium being most frequently involved. Plain radiography, computed tomography and magnetic resonance imaging are the most used techniques for detection and characterization of the lesion. The use of ultrasound is less known, although it may be a valuable technique in detection and follow-up of superficially located lesions such as calvarial lesions. This case report describes an 8-year-old girl, in whom the lesion was initially detected by ultrasound. Furthermore, ultrasound was used to evaluate spontaneous resolution of the lesion. The knowledge of ultrasound characteristics may be important to avoid unnecessary radiation and gadolinium administration, particularly in a pediatric population.

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Langerhans cell histiocytosis of skull: a retrospective study of 18 cases

Cited by 13 publications

References 0 publications

A rapidly expanding calvarial Langerhans cell histiocytosis with low Ki-67 in an adult: a challenging diagnosis on magnetic resonance imaging

A rapidly expanding calvarial Langerhans cell histiocytosis with low Ki-67 in an adult: a challenging diagnosis on magnetic resonance imaging

Calvarial Langerhans cell histiocytosis in an adult presenting rapid growth

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

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