袁振超 黃保華

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長(zhǎng)節(jié)段胸椎管內(nèi)硬膜外血管脂肪瘤一例報(bào)告

袁振超 黃保華

作者單位：530021 南寧，廣西醫(yī)科大學(xué)附屬腫瘤醫(yī)院骨軟組織神經(jīng)外科 ( 袁振超)；530011 南寧，廣西中醫(yī)藥大學(xué)附屬瑞康醫(yī)院脊柱微創(chuàng)中心 ( 黃保華)

【關(guān)鍵詞】脂肪瘤；椎管；胸椎；硬膜外腔；血管脂肪瘤

椎管內(nèi)硬膜外血管脂肪瘤 ( Angiolipoma) 為包含成熟脂肪組織和異常血管成分的良性腫瘤，為一種少見(jiàn)的椎管內(nèi)腫瘤，其患病率約占所有椎管腫瘤的 0.14%～1.2%，占椎管內(nèi)硬膜外腫瘤的 2%～3%，常見(jiàn)的發(fā)病年齡為 40 歲左右。該病確切病因尚未明了，Berenbruch[1]于 1860 年首次報(bào)道 1960 年 Howard 和 Helwig[2]首次將血管脂肪瘤歸為一個(gè)獨(dú)立的病種。2012 年 2 月我院經(jīng)治 1 例，現(xiàn)報(bào)告如下。

患者，男，46 歲。進(jìn)行性左下肢乏力、行走困難 10 余年，于 2012 年 2 月 7 日入院?；颊咦栽V 10 余年前無(wú)誘因出現(xiàn)左下肢乏力，當(dāng)時(shí)無(wú)明顯麻木，行走較為困難，時(shí)常伴左肩部、腰背部疼痛，曾在外院診治，療效不佳，后患者左下肢無(wú)力癥狀呈漸進(jìn)性加重，近 3 個(gè)月來(lái)，患者左下肢乏力、活動(dòng)困難癥狀明顯加重，并伴有左側(cè)身體自乳頭以下麻木。專科檢查：患者蹣跚步態(tài)，需拄拐行走，平地最遠(yuǎn)行走約 50 m，無(wú)法上下樓梯，脊柱呈駝背畸形，無(wú)側(cè)凸畸形，左側(cè)身體自乳頭以下感覺(jué)功能減退，左下肢肌肉萎縮。左髂腰肌肌力 3 級(jí)，左股四頭肌肌力 3 級(jí)，左背伸肌力減弱約 4 級(jí)，左側(cè)膝腱反射亢進(jìn)，Babinski’s sign 左 ( +) 右 ( -)；Hoffmann’s sign 左 ( -) 右 ( -)。胸椎 MRI及增強(qiáng)檢查示 T1～9椎管內(nèi)長(zhǎng)節(jié)段占位病變，T1、T2加權(quán)像為內(nèi)部混雜不均勻的中高信號(hào)影 ( 圖 1，2)，T2加權(quán)像為高信號(hào)影，左側(cè)胸段脊髓受壓，以 T4～5水平最明顯。于2012 年 2 月 23 日，在全麻下行 T1～9全椎板切除減壓硬膜外血管脂肪瘤切除術(shù)，術(shù)中見(jiàn) T1～9硬膜外有一條索樣腫物，長(zhǎng)約 20 cm，色暗紅、質(zhì)韌、邊界清楚，血供豐富，與硬脊膜粘連不緊，易于完整剝離，相應(yīng)節(jié)段硬膜及脊髓明顯受壓，搏動(dòng)性差，術(shù)中予 500 mg 甲潑尼龍靜滴保護(hù)脊髓。完整切除腫瘤組織，標(biāo)本送病理檢查。術(shù)后病理檢查報(bào)告：血管脂肪瘤 ( 圖 3)?；颊咝g(shù)后恢復(fù)良好，左側(cè)肢體麻木感緩解，左肩部、腰背部疼痛癥狀未再發(fā)作，左下肢肌力較術(shù)前改善。術(shù)后 2 周復(fù)查 MRI 見(jiàn)胸椎管內(nèi)腫瘤完全摘除 ( 圖 3)，椎管通暢，硬膜及髓脊無(wú)受壓。術(shù)后 2 年隨訪 ( 圖 4)，患者左肩部、腰背部疼痛消失，左下肢肌力恢復(fù)至 5 級(jí)，肢體感覺(jué)功能恢復(fù)，能自行行走、上樓，可負(fù)重。但負(fù)重、長(zhǎng)時(shí)間行走時(shí)仍左下肢乏力感。復(fù)查 MRI見(jiàn)胸椎管通暢，腫瘤未見(jiàn)復(fù)發(fā)，腰椎正側(cè)位片未出現(xiàn)明顯脊柱側(cè)凸、后凸畸形。

圖1　胸椎 MRI 檢查示 T1～9 椎管內(nèi)長(zhǎng)節(jié)段占位病變，T1、T2 加權(quán)像為內(nèi)部混雜不均勻的信號(hào)影；胸段脊髓受壓，以 T4～5 水平最明顯圖2　胸椎脂肪抑制像、增強(qiáng) MRI 進(jìn)一步證實(shí)椎管內(nèi)占位病變圖3　術(shù)后 1 周復(fù)查 MRI 見(jiàn)胸椎管內(nèi)腫瘤完全摘除，椎管通暢，硬膜及髓脊無(wú)受壓；病理檢查報(bào)告：血管脂肪瘤 ( HE 染色，10 × 20)圖4　術(shù)后 2 年復(fù)查 MRI 見(jiàn)胸椎管通暢，腫瘤未見(jiàn)復(fù)發(fā)，胸椎正側(cè)位 X 線片未出現(xiàn)明顯脊柱側(cè)凸、后凸畸形Fig.1 Thoracic vertebral MRI examination shows T1-9 intraspinal long segment space occupying lesion, T1 weighted imaging is internally unevenly mixed medium and high signal image, left thoracic segment suffers spinal cord compression, obviously at T4-5 levelFig.2 The thoracic fat-suppression and enhanced MRI further confirms spinal canal space-occupying lesionsFig.3 The thoracic MRI check shows that thoracic intraspinal tumor was completely removed after 1 week, spinal canal is patent, dura mater and spinal cord are not compressed. Pathological examination report after the operation: angiolipoma ( hematoxylin and eosin stain, 10 × 20)Fig.4 The thoracic MRI check shows that thoracic spinal canal was after 2 years, The X-ray ( anteroposterior and lateral view) confirm no spinal deformity

討 論

本病為良性腫瘤，生長(zhǎng)緩慢，病程較長(zhǎng)，臨床表現(xiàn)以脊髓受壓癥狀即運(yùn)動(dòng)、感覺(jué)受累為主，臨床癥狀缺乏特異性，容易造成誤診。病情輕重與進(jìn)展快慢取決于腫瘤的生長(zhǎng)速度及其對(duì)脊髓的壓迫程度，癥狀急性加重往往提示瘤內(nèi)急性出血引起壓迫癥狀。

Gelabert-González 等[3]統(tǒng)計(jì)，至 2009 年，在可查閱到的文獻(xiàn)中，椎管內(nèi)硬膜外腫瘤報(bào)道病例共 121 例，其中女 72 例，男 49 例，平均年齡 ( 44.03±5.9) 歲。

本例患者，病程長(zhǎng)達(dá) 10 年之久，病情進(jìn)展緩慢，未出現(xiàn)急性出血癥狀加重情況，臨床特點(diǎn)與文獻(xiàn)報(bào)道相符。

椎管內(nèi)血管脂肪瘤以胸段最常見(jiàn)[4]，其次為腰段和頸段，嚴(yán)寧等[5]統(tǒng)計(jì) 132 例，其中 97 例 ( 73.5%) 腫瘤位于胸段，5 例 ( 3.8%) 位于胸腰段。椎管內(nèi)血管脂肪瘤好發(fā)于胸段，其原因可能與胸段脊髓血供差，從而導(dǎo)致胸段脊髓代償性新生血管形成，繼而引發(fā)腫瘤生于該處。椎管內(nèi)血管脂肪瘤累及的節(jié)段通常 2～3 個(gè)椎體，臨床上病變累及 6 個(gè)節(jié)段以上者罕見(jiàn)，國(guó)內(nèi)外文獻(xiàn)，檢索到 14 例[6-17]。本例病灶位于胸椎，腫瘤組織自 T1～9椎管內(nèi)占位，長(zhǎng)約20 cm，為長(zhǎng)節(jié)段椎管內(nèi)血管脂肪瘤。

Lin 等[18]將 459 例椎管內(nèi)血管脂肪瘤病例進(jìn)行分析歸納，根據(jù)腫瘤是否向周圍生長(zhǎng)將椎管內(nèi)血管脂肪瘤分為浸潤(rùn)性和非浸潤(rùn)性，非浸潤(rùn)性多發(fā)生于椎管背側(cè)，而浸潤(rùn)性多發(fā)生于前部或前外側(cè)。本例腫瘤位于椎管背側(cè)，沒(méi)有向周圍組織生長(zhǎng)，為非浸潤(rùn)型。

MRI 檢查是診斷椎管內(nèi)硬膜外血管脂肪瘤最可靠的影像學(xué)檢查方法，腫瘤多呈縱行梭狀生長(zhǎng)，長(zhǎng)軸與椎管縱軸平行，相應(yīng)椎管內(nèi)脊髓受壓變窄。根據(jù)病灶內(nèi)脂肪與血管兩種成分所占的比例不同，MRI 表現(xiàn)為混雜不均信號(hào)影。當(dāng)腫瘤以脂肪為主時(shí)，T1像為高信號(hào)，比硬膜外脂肪略低；如果腫瘤以血管成分為主，則 T1像低信號(hào)區(qū)域增大，可表現(xiàn)為斑駁影像或條塊狀低信號(hào)灶，增強(qiáng)后同樣呈現(xiàn)混雜不均一的信號(hào)，脂肪抑制成像有助于明確診斷( 圖 2)。胡春洪等[19]將本病 MRI 影像表現(xiàn)分為 3 種類型：I 型為腫瘤局限于椎管內(nèi)。瘤體以脂肪信號(hào)為主，血管成分較少；II 型為腫瘤局限于椎管內(nèi)，血管成分占病灶體積的 1 / 2 以上，呈團(tuán)塊狀位于瘤體的中央部分，脂肪成分圍繞其周圍；III 型為腫瘤沿一側(cè)或雙側(cè)椎間孔向椎管外生長(zhǎng)，形成啞鈴狀腫塊。

無(wú)論對(duì)于浸潤(rùn)型還是非浸潤(rùn)型的椎管內(nèi)硬膜外血管脂肪瘤，手術(shù)切除腫瘤是最為行之有效的治療方法。手術(shù)切除療效確切[20]，復(fù)發(fā)極少[21]( 圖 4)。非浸潤(rùn)型脂肪血管瘤往往覆蓋于脊髓背側(cè)硬膜外，邊界清楚，與硬膜囊粘連不緊，易于完全切除。而對(duì)于浸潤(rùn)型血管脂肪瘤無(wú)法完全切除時(shí)，部分切除也可獲得滿意效果。

參 考 文 獻(xiàn)

[1] Berenbruch K. Ein fall von multiple angiolipomen kombiniert miteinem angiom des ruckenmarks. Tibingen, 1890.

[2] Howard WR, Helwig E. Angiolipoma. Arch Dermntol, 1960, 82:924-931.

[3] Gelabert-González M, García-Allut A. Spinal extradural angiolipoma: report of two cases and review of the literature. Eur Spine J, 2009, 18(3):324-335.

[4] 韓蕓峰, 王振宇, 林國(guó)中, 等. 椎管內(nèi)硬膜外侵襲型血管脂肪瘤1例報(bào)告及文獻(xiàn)復(fù)習(xí). 中國(guó)脊柱脊髓雜志, 2012, 22(5): 487-489.

[5] 嚴(yán)寧, 侯鐵勝, 曾紹林. 椎管內(nèi)血管脂肪瘤9例報(bào)告. 中國(guó)矯形外科雜志, 2008, 23(16):1834-1836.

[6] Kasper JA, Cowan A. Extradural lipoma of the spinal canal. Arch Pathol, 1929, 8:800-802.

[7] Anson JA, Cybulski GR, Reyes M. Spinal extradural angiolipoma: a report of two cases and review of the literature. Surg Neurol, 1990, 34:173-178.

[8] Yamashita K, Fuji T, Nakai T, et al. Extradural spinal angiolipoma: report of a case studied with MRI. Surg Neurol, 1993, 39(1):49-52.

[9] Michilli R, Tzonos P, Iglesias-Rozas JR. Spinal extradural angiolipoma: case report and literatura review. Neurochirurgia (Stuttg), 1993, 36:63-65.

[10] Fernández JJ, Abad RM, Ribas T, et al. Spinal angiolipoma causing acuteparaplegia. Report of two cases. Neurocirugia, 1994, 5:242-245.

[11] Bouramas D, Korres DS, Roussos L, et al. Spinal extradural angiolipoma. J Spinal Disord, 1995, 8:324-327.

[12] O’Donovan NA, Naik K, Maloney WJ, et al. Spinal angiolipoma mimicking extradural lipomatosis. Can Assoc Radiol J, 1996, 47:51-53.

[13] Trabulo A, Cerqueira L, Monteiro J, et al. Spinal angiolipomas revisited: two case reports. Acta Neurochir (Wien), 1996, 138:1311-1319.

[14] Boockvar JA, Black K, Malik S, et al. Subacute paraparesis induced by venous thrombosis of a spinal angiolipoma: a case report. Spine, 1997, 22:2304-2308.

[15] Labram EK, El-Shunnar K, Hilton DA, et al. Revisited: spinal angiolipoma, three additional cases. Br J Neurosurg, 1999, 13:25-29.

[16] Turgut M. Spinal angiolipomas: report of a case and review of the cases published since the discovery of the tumour in 1890. Br J Neurosurg, 1999, 13:30-40.

[17] Bailey D, Andrews J, Mazur E, et al. Thoracic cord compression from a spinal angiolipoma: case report and brief comment. Conn Med, 2000, 64:267-269.

[18] Lin JJ, Lin F. Two entities in angiolipoma. A study of 459 cases of lipoma with review of literature on infiltrating angiolipoma. Cancer, 1974, 34(3):720-727.

[19] 胡春洪, 丁乙, 王雪元. 椎管內(nèi)硬膜外血管脂肪瘤的MRI表現(xiàn). 中華放射學(xué)雜志, 2004, 11:1176-1179.

[20] Chotai S, Hur JS, Moon HJ, et al. Spinal Angiolipoma: case report. Neurol Med Chir (Tokyo), 2011, 51:539-542.

[21] Nanassis K, Tsitsopoulos P, Marinopoulos D, et al. Lumbar spinal epidural angiolipoma. J Clin Neurosci, 2008, 15(4): 460-463.

( 本文編輯：李貴存)

Long segmental thoracic intra-spinal canal epidural angiolipoma and review of literature: 1 case report YUAN Zhen-chao, HUANG Bao-hua. Department of Bone, Soft Tissue and Neurosurgery, the Oncology Hospital Affiliated to Guangxi Medical University, Nanning, Guangxi, 530021, PRC

【Abstract】Objective To report a case with long segmental thoracic intra-spinal canal epidural angiolipoma. Methods Retrospective analysis was done on the data of a case with long segmental thoracic intra-spinal canal epidural angiolipoma treated in our hospital in Feburary. 2012. Results The patient was male, 46 years old. More than 10 years ago, the patient developed left lower extremity weakness without remarkable precipitating factors. Numbness was not obvious, walking became more difficult, often accompanied of left shoulder and lower back pain, left lower extremity weakness was gradually worsened. In the past 3 months, left lower limb weakness and difficulty in walking exacerbated, and were accompanied by the numbness on the left side of the body from the nipple. Examination showed tottering gait. He could walk with crutches, and his longest walking distance on the flat ground was 50 m. He could not climb up and walk down the stairs, and had reduced sensation on the left side of the body from the nipple downward. Left lower limb muscle atrophy existed. His left iliopsoas muscle strength was grade 3, left femoral quadriceps muscle strength was grade 3, extensor digitorum longus had a grade 4 strength. He had left knee tendon hyperreflexia. Babinski sign was ( +) on the left and was ( -) on the right, Hoffmann’s sign was ( -) on the left andright. Thoracic and enhanced MRI examination revealed T1-9long segment spinal canal space-occupying lesions. T1, T2-weighted images showed internal high signal mixed uneven images, while T2-weighted images had high signal intensity. There was left thoracic spinal cord compression, which was more dramatic at T4-5levels. It was found intraoperatively that there was an epidural band-like mass at thoracic spinal canal at T1to T9levels, which was about 20 cm, dark red color, elastic, with clear boundary and rich blood supply, loose dural adhesions, easy to isolate. The dura and spinal cord at the corresponding section was obviously compressed and beating was poor. Pathological diagnosis: angiolipoma. Postoperative recovery was good: the left limb numbness remitted, left shoulder, lower back pain no longer existed, the left lower limb muscle strength was improved compared with preoperative improvement. Re-examination with MRI 2 weeks after the surgery showed that the tumor in the thoracic spinal canal was completely removed. In 2 years follow-up, the patient’s left shoulder and lower back pain disappeared, left lower limb muscle strength recovered to grade 5, limb sensory functional recovered. The patient could walk independently, climb up stairs, and bear some weight. The thoracic MRI re-checking showed that thoracic spinal canal was patent and there was no recurrence of the tumor. Conclusions The effect of early surgical treatment of long-segment spinal canal epidural angiolipoma was good and therefore early surgery is recommended.

【Key words】Lipoma; Spinal canal; Thoracic vertebrae; Epidural space; Angiolipoma

( 收稿日期：2015-08-17)

Corresponding author:HUANG Bao-hua, Email: 20hbh@163.com

通信作者：黃保華，Email: 20hbh@163.com

DOI:10.3969/j.issn.2095-252X.2016.02.018

中圖分類號(hào)：R738.6