Rare case: Langerhans cell histiocytosis # medical imaging #  classic cases diagnosed.2017-08-13 00:53:58 25 ℃
A, patient information and image
Patient：Male, 39 years old.
Chief complaintNo obvious cause of neck pain: 4 months ago, after aggravating, rest after remission, accompanied by pain at night.
History of Present IllnessNo.
Two, Q & A Case Challenge
The permeability of bone destruction of the lesion edge, clear boundary, so the E answer is wrong.
In the MRI image on the performance of MRI, a disease description, the following is not true? (Dan Xuan)
A lesions showed slightly long T1 and long T2 signal.
B C4-5 disc signal
C soft tissue mass with clear edge
D soft tissue mass on a wide range of spread
E enhanced scanning lesions were homogeneous enhancement
Answer: Interpretation of C
Paravertebral soft tissue mass, wide spread, edge blur, back into the spinal canal, the dural sac compression, enhanced scanning lesions significantly enhanced uniformity. The C error.
According to the above clinical data with CT and MRI features, the most likely diagnosis in this case for which of the following? (Dan Xuan)
B eosinophilic granuloma
D invasive vascular
Answer: Interpretation of B
Three, diagnosis analysis
The characteristics of the cases for young and middle-aged men, chronic course, C4 vertebral body, right lateral block and C5 vertebral osteolytic bone destruction, considered from the bone lesions. The border area of bone destruction of cortical bone is not clear, complete, paravertebral soft tissue invasion, part intraspinal, blur; lesions showed slightly long T1 and long T2 signal intensity, signal uniformity, homogeneous enhancement; should take into account the inflammatory granuloma may.
Four, diagnosis and differential diagnosis
The characteristics of this case for young women, no specific clinical symptoms. Solid nodules of right lower neck isolated, first consider the source of lymph lesions and neurogenic lesions. In this case, lesion density, and obvious homogeneous enhancement, CT enhanced scanning lesions around multiple tortuous dilated blood vessels, suggesting that Castleman disease.
1, eosinophilic granuloma
Vertebral eosinophilic granuloma showed osteolytic bone destruction, lobulated, clear boundary and no sclerosis, vertebral compression, cortical bone is not complete, paravertebral soft tissue invasion range, no internal necrosis, enhanced scanning lesions significantly enhanced uniformity. Although this case does not meet a good age, but still need to consider the diagnosis of eosinophilic granuloma.
In this case, osteolytic bone destruction area with clear boundary and paravertebral wide range of lesions, on the spread of these signs; prompt us to think of tuberculosis may at the time of diagnosis. But the lesions involving the adjacent vertebral body and intervertebral disc not involved; bone destruction sequestrum; paraspinal lesions is widespread but no abscess formation, does not support tuberculosis.
In 3, lymphoma
The case of paravertebral soft tissue invasion and spread to a wide range of spinal canal, without internal necrosis, homogeneous enhancement, consideration should be given to the lymphoma. But the intraspinal involvement is limited to the anterior portion of the spinal canal, not around the dural sac growth; bone destruction and the boundary is clear, does not meet the diagnosis of lymphoma.
4, aggressive hemangioma
The median age of spinal aggressive hemangiomas was 40 years old, the boundary area of bone destruction of cortical bone is not clear, complete, local soft tissue mass invasion of the spinal canal, was significantly enhanced, consistent these characteristics and the case of image. But in this case no bony destruction of honeycomb and palisade zone, the T2 signal is slightly high, soft tissue invasion, we do not consider the diagnosis of invasive hemangioma.
Operation and pathology
Surgical findings:C4 vertebral body soft tissue showed fatty.
Pathology:With Langerhans cell histiocytosis. CD163 (+), CD1a (+), CD68 (-), Ki-67 (10%+), Langerin (+), S-100 (+).
The 2013 WHO classification of bone tumor in Langerhans cell histiocytosis is classified as not clear the nature of the tumor tumor can be divided into single and multiple skeletal bone. Langerhans cell histiocytosis contains three types of lesions: Le Xue Han - Ke Xue - disease, disease and eosinophilic granuloma.
Eosinophilic granuloma occurred in the skull, occur in the spine are relatively rare, the peak incidence of 5-10 years. Eosinophilic granuloma is a self limiting disease, according to clinical manifestations and pathology can be divided into cells, proliferation of granuloma and resolution, imaging characteristics are different in different periods. In the proliferation of tissue cells, osteolytic bone destruction was eroded or swelling, the cortical bone is not complete, vertebral compression, such as flat vertebral; paravertebral soft tissue invasion and edge blur, and intraspinal involvement spread. To the development of granuloma period, bone destruction, paraspinal soft tissue mass boundary tends to be clear, surrounding the lesions showed different degrees of bone sclerosis and other reparative changes. To subsidise period, bone destruction, narrowing the scope of lesions and surrounding bone sclerosis, vertebral height recovered to different degrees; soft tissue tumors to shrink or disappear. The internal lesions without cystic necrosis, uniform density or signal; involvement of adjacent vertebrae, but not involving the intervertebral disc. Eosinophilic granuloma occurred flat vertebra, especially in children; adult vertebral flat rare, only 10% probability.
In this case, bone destruction, clear border of vertebral compression, vertebral and spinal soft tissue invasion, with inflammatory granuloma; lesions without involvement of the intervertebral disc, no dead bone, paravertebral extensive lesions but no abscess formation, these findings do not support tuberculosis in favour of eosinophilic granuloma diagnosis. In this case, although the age is big, but the combination of imaging findings, still should take into account the eosinophilic granuloma may.
Expert comment:Department of Radiology, No.3 Hospital of Peking University professor Yuan Huishu
1. Jiang L, Liu XG, Zhong WQ et al. Langerhans cell histiocytosis with multiple spinal involvement. Eur Spine J 2011; 20: 1961-1969.
2. Montalti M, Amendola L. Solitary eosinophilic granuloma of the adult lumbar spine. Eur Spine J 2012; 21 Suppl 4: S441-444.
3. Plasschaert F, Craig C, Bell R et al. Eosinophilic granuloma. A different behaviour in children than in adults. J Bone Joint Surg Br 2002; 84: 870-872.
4. Pang Chaonan, Liu Xiaoguang, Yuan Huishu. Analysis of CT features of cervical Langerhans cell histiocytosis. Chinese Medical Imaging Technology 2009; 25: 1072-1074.
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