幸世龍,楊毅軍,王小瑋,張 勇,馬玉龍,宋得夫
(西安市兒童醫(yī)院,陜西 西安 710003)
21例兒童髂骨骨質(zhì)破壞性病變的臨床分析
幸世龍,楊毅軍,王小瑋,張 勇,馬玉龍,宋得夫
(西安市兒童醫(yī)院,陜西 西安 710003)
目的 總結(jié)兒童髂骨骨質(zhì)破壞性病變的臨床特點(diǎn)。方法 回顧性分析2013年1月至2015年12月,西安市兒童醫(yī)院收治的髂骨骨質(zhì)破壞患兒的臨床資料。對(duì)21例患兒術(shù)后通過切除的病灶標(biāo)本確定病變性質(zhì),分析其臨床特點(diǎn)。結(jié)果 患兒年齡為24天~11.5歲,中位年齡3.5歲;男︰女=2.5:1。朗格漢斯細(xì)胞增生癥11例(9例多發(fā),2例單發(fā)),骨髓炎3例,尤文肉瘤2例,多發(fā)性纖維結(jié)構(gòu)不良2例,骨軟骨瘤、臀部橫紋肌肉瘤和急性淋巴細(xì)胞白血病各1例;有12例患兒在髂骨病灶之外還有多處病灶。所有病例影像學(xué)均表現(xiàn)為髂骨不同形態(tài)的骨質(zhì)破壞,發(fā)生在髂骨翼19例,髖臼2例。8例伴有大小不等的軟組織腫塊,5例見骨膜反應(yīng)。以髖部疼痛為首發(fā)癥狀9例,以下肢跛行為首發(fā)癥狀6例。經(jīng)99锝全身骨掃描陽性率僅占57.1%,多病灶者準(zhǔn)確率為66.7%。結(jié)論 兒童髂骨骨質(zhì)破壞以朗格漢斯細(xì)胞增生癥最多見,常合并多處骨病灶。臨床表現(xiàn)特異性不高,確診仍需病理檢查。
髂骨;骨質(zhì)破壞;兒童;診斷
髂骨骨質(zhì)破壞性病變可能涉及多種疾病,文獻(xiàn)多為個(gè)案報(bào)道,發(fā)生于兒童者較少見,易出現(xiàn)漏診及誤診。本文收集了西安市兒童醫(yī)院2013年1月至2015年12月診斷為兒童髂骨骨質(zhì)破壞性病變21例,總結(jié)分析其臨床特點(diǎn),旨在提高對(duì)此類病例的臨床診療水平。
1.1臨床資料
收集2013年1月至2015年12月西安市兒童醫(yī)院收治的髂骨骨質(zhì)破壞患兒資料共21例。對(duì)18例患兒行活檢術(shù)或病灶切除術(shù),經(jīng)手術(shù)后病理確診;2例骨髓炎經(jīng)血檢及復(fù)查X線片確診;1例白血病由骨髓檢查確診?;純耗挲g24天~11.5歲,中位年齡3.5歲;男:女=2.5:1。病史1天~1年?;純阂泽y部疼痛為首發(fā)癥狀9例,以下肢跛行為首發(fā)癥狀6例,無任何臨床癥狀者有5例,還有1例以髂骨包塊就診。血白細(xì)胞升高者8例,血沉及超敏C反應(yīng)蛋白升高者6例。朗格漢斯細(xì)胞增生癥患兒、尤文肉瘤患兒、臀部橫紋肌肉瘤以及急性淋巴細(xì)胞白血病患兒經(jīng)確診后均轉(zhuǎn)至本院血液腫瘤科進(jìn)行化療。多發(fā)性纖維結(jié)構(gòu)不良患兒術(shù)后予帕米膦酸二鈉(1mg·kg-1·d-1,每療程3天,每6個(gè)月1次,共4次)。骨髓炎患兒行抗感染治療,髂骨骨軟骨瘤患兒行手術(shù)切除。
1.2影像學(xué)資料
所有病例影像學(xué)均有髂骨骨質(zhì)破壞,發(fā)生在髂骨翼19例,髖臼2例。8例伴有大小不等的軟組織腫塊,5例見骨膜反應(yīng)。纖維結(jié)構(gòu)不良CT片表現(xiàn)為磨砂玻璃樣改變,見圖1A;骨軟骨瘤CT片表現(xiàn)為骨骺附近的骨性突起,見圖1B;橫紋肌肉瘤及尤文肉瘤均以溶骨性表現(xiàn)為主,見圖1C和圖1D;朗格漢斯細(xì)胞增生癥影像學(xué)表現(xiàn)各異,病變破壞范圍大小與病史長(zhǎng)短相關(guān),見圖1E和圖1F。
注:A為纖維結(jié)構(gòu)不良;B為髖臼骨軟骨瘤;C為橫紋肌肉瘤;D為尤文肉瘤;E為單發(fā)性朗格漢斯細(xì)胞增生癥;F為多發(fā)性朗格漢斯細(xì)胞增生癥;箭頭所示為病灶。
圖1 術(shù)前CT影像圖片
Fig.1 Preoperative CT pictures
在21例患兒中,朗格漢斯細(xì)胞增生癥11例(52.4%),其中9例為多處病灶,2例為單處病灶;骨髓炎3例(14.3%);尤文肉瘤2例(9.5%),其中1例已有遠(yuǎn)處轉(zhuǎn)移;多發(fā)性纖維結(jié)構(gòu)不良2例(9.5%),骨軟骨瘤、臀部橫紋肌肉瘤和急性淋巴細(xì)胞白血病各1例(4.8%)。有12例患兒在髂骨病灶之外還有多處病灶,主要分布于椎體、顱骨、肩胛骨、長(zhǎng)骨干骺端、肝臟和肺部,經(jīng)99锝全身骨掃描陽性率僅占57.1%(12/21),多病灶者準(zhǔn)確率66.7%(8/12)。
3.1髂骨腫瘤的發(fā)病情況
髂骨是人體最大的不規(guī)則扁骨,位置深在,由髂骨體和髂骨翼構(gòu)成,髓腔含有豐富的紅骨髓,具有造血功能,是彌漫性骨髓疾病和轉(zhuǎn)移瘤的好發(fā)部位。在成人中,髂骨原發(fā)惡性腫瘤更常見,惡性:良性腫瘤比例為1.82:1[1],主要以軟骨肉瘤、骨肉瘤、尤文氏肉瘤常見,髂骨和髖臼周圍為好發(fā)部位。本組病例中,以朗格漢斯細(xì)胞增生癥最多見,惡性腫瘤少見。患兒的中位年齡為3.5歲;就診年齡偏小,惡性腫瘤自然發(fā)病率低,故影響良惡性疾病的構(gòu)成比例。
兒童髂骨骨質(zhì)破壞性病變可由多種疾病所致,除本文中所提到的朗格漢斯細(xì)胞增生癥,骨髓炎,尤文肉瘤,纖維結(jié)構(gòu)不良,骨軟骨瘤、臀部橫紋肌肉瘤和急性淋巴細(xì)胞白血病以外,還可能為骨囊腫、成骨細(xì)胞瘤、骨肉瘤、淋巴瘤及轉(zhuǎn)移瘤所致[2]。其中兒童髂骨轉(zhuǎn)移瘤以腹膜后神經(jīng)母細(xì)胞瘤較多見,術(shù)前需完善腹部CT檢查,避免漏診和誤診。
3.2髂骨朗格漢斯細(xì)胞組織細(xì)胞增生癥情況
朗格漢斯細(xì)胞組織細(xì)胞增生癥(langerhans cell histiocytosis,LCH)是一組來源于骨髓的朗格漢斯細(xì)胞(langerhans cell,LC)的異常增生,伴有數(shù)量不等的中性粒細(xì)胞、嗜酸性粒細(xì)胞、淋巴細(xì)胞、漿細(xì)胞及多核巨細(xì)胞浸潤(rùn),引起組織破壞的疾患[3]。可發(fā)生于任何年齡組,但多見于兒童[4]。0~14歲發(fā)病率為3/100萬~5/100萬[5]。臨床上可分為三型:①單系統(tǒng)、單病灶(嗜酸性肉芽腫);②單系統(tǒng)、多病灶(hand-schuller-christian病);③多系統(tǒng)、多病灶(letterer-siwe病),多系統(tǒng)病變伴器官功能異常:若病變累及皮膚、骨、淋巴結(jié)及垂體為低風(fēng)險(xiǎn),累及肺、肝、脾及骨髓為高風(fēng)險(xiǎn)。該病臨床表現(xiàn)差異極大,輕者僅累及皮膚,重者可累及多器官并造成重要臟器功能損害且預(yù)后較差[6]。單病灶者可不用治療,多病灶未經(jīng)治療的病死率高達(dá)92.1%[7]。本組病例中,該病患兒最多,共11例,占52.4%;2例僅髂骨骨質(zhì)破壞,9例有多處病灶,其中8例為多處骨病灶,1例為多處骨病灶伴肝臟病變。由此可見,髂骨處LCH多以單系統(tǒng)、多病灶出現(xiàn),診斷該病時(shí)需全面檢查,準(zhǔn)確評(píng)估病情,避免出現(xiàn)重病輕治的情況,影響預(yù)后。
3.3兒童髂骨骨質(zhì)破壞性病變臨床表現(xiàn)分析
本組病例中,患兒病史從1天至1年不等,癥狀以髖部疼痛為首發(fā)癥狀9例,以下肢跛行為首發(fā)癥狀6例。所有病例影像學(xué)均有髂骨骨質(zhì)破壞,發(fā)生在髂骨翼19例,髖臼2例。有12例患兒在髂骨病灶之外還有多處病灶,主要分布于椎體、顱骨和肩胛骨、長(zhǎng)骨干骺端、肝臟和肺部。纖維結(jié)構(gòu)不良和骨軟骨瘤影像學(xué)具有特異性的形態(tài)和結(jié)構(gòu),一般可明確診斷。惡性腫瘤以溶骨性表現(xiàn)為主。朗格漢斯細(xì)胞增生癥影像學(xué)表現(xiàn)各異,病變范圍大小與病史長(zhǎng)短相關(guān)。經(jīng)99锝全身骨掃描陽性率僅占57.1%(12/21),多病灶者準(zhǔn)確率為66.7%(8/12),這與文獻(xiàn)報(bào)道相似[8]。
綜上所述,兒童髂骨骨質(zhì)破壞性病變臨床表現(xiàn)各異,常伴多處骨質(zhì)破壞,全身骨掃描假陰性率較高,易漏診。總之,兒童髂骨骨質(zhì)破壞以朗格漢斯細(xì)胞增生癥最多見,常合并多處病灶,可涉及多種疾病,全面細(xì)致的檢查有利于病情的整體評(píng)估,可有效指導(dǎo)后期的治療。因該類疾病臨床表現(xiàn)特異性不高,確診仍需病理檢查。
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[專業(yè)責(zé)任編輯:李 鵬]
Analysis of 21 children with iliac bone destruction
XING Shi-long,YANG Yi-jun,WANG Xiao-wei,ZHANG Yong,MA Yu-long,SONG De-fu
(Xi’anChildren’sHospital,ShaanxiXi’an710003,China)
Objective To summarize the clinical characteristics of iliac bone destruction in children. Methods The clinical features of 21 cases with iliac bone destruction admitted in Xi’an Children’s Hospital from January 2013 to December 2015 were analyzed retrospectively. The nature of lesions incised after surgery in 21 cases was confirmed to analyze their clinical features. Results The children aged from 24 days to 11.5 years old with median age of 3.5 years. The proportion of male and female was 2.5:1. Eleven cases were found with Langerhans cell histiocytosis (9 cases of multiple histiocytosis and 2 cases of single histiocytosis), 3 cases with osteomyelitis, 2 cases with Ewing’s sarcoma, 2 cases with multiple fibrous dysplasia, 1 case with osteochondroma, 1 case with hip rhabdomyosarcoma and 1 case with acute lymphocytic leukemia. In 12 patients there were multiple lesions besides iliac lesions. Imaging features of all cases showed iliac bone destruction in different shapes, with 19 cases in iliac wing and 2 cases in acetabulum. Eight cases had soft tissue masses of various sizes, and 5 cases had periosteal reaction. Hip pain as the first symptom was in 9 cases, and limping as the first symptom was in 6 cases. Technetium-99 bone scan revealed positive rate of 57.1%, and the accuracy for patients with more lesions was 66.7%. Conclusion Langerhans cell histiocytosis is the most common disease in children with iliac bone destruction, which often is associated with multiple bone lesions. The specificity of clinical manifestations is not high. Diagnosis still requires pathological examination.
iliac bone destruction, children, diagnosis
2016-08-16
幸世龍(1983-),男,主治醫(yī)師,碩士,主要從事小兒骨腫瘤的研究。
楊毅軍,副主任醫(yī)師。
10.3969/j.issn.1673-5293.2017.04.035
R726.8
A
1673-5293(2017)04-0467-03