兒童遺傳性多發(fā)性骨軟骨瘤軟骨帽的超微結(jié)構(gòu)觀察
發(fā)布時(shí)間：2018-07-20 13:08
【摘要】：兒童遺傳性多發(fā)性骨軟骨瘤(Hereditary Multiple Exostoses, HME)是一種涉及人體骨骼系統(tǒng),影響正常軟骨化骨過(guò)程的遺傳性骨骼疾病。本病是小兒骨科中最常見(jiàn)的良性骨腫瘤,發(fā)病率較低約為1/5萬(wàn),男性發(fā)病率高于女性,男女比例約為3：1。兒童HME的瘤體由軟骨組織的帽狀結(jié)構(gòu)和幾乎與骨骼骨面垂直突出的骨組織構(gòu)成,大多數(shù)分布在關(guān)節(jié)周圍,即長(zhǎng)骨的干骺端,肋骨、肩胛骨、骨盆等處有少量分布。腫瘤的特點(diǎn)是生長(zhǎng)于瘤體的頂部有一形似帽子的軟骨層即軟骨帽,通過(guò)其深層的軟骨化骨作用,使瘤體持續(xù)生長(zhǎng),腫瘤增長(zhǎng)較大時(shí)可引起局部疼痛或者導(dǎo)致骨骼畸形,近關(guān)節(jié)的可引起活動(dòng)障礙,或可壓迫鄰近的血管神經(jīng)而引起相應(yīng)的癥狀,若腫瘤突然長(zhǎng)大或生長(zhǎng)迅速,應(yīng)考慮有惡變的可能。很多學(xué)者從遺傳致病基因定位、發(fā)病機(jī)制、臨床表現(xiàn)及分型、影像學(xué)等方面對(duì)HME進(jìn)行過(guò)深入的研究,軟骨帽過(guò)度生長(zhǎng)已被認(rèn)為是HME惡變的主要原因之一。 目的 本課題通過(guò)掃描電鏡和透射電鏡觀察兒童遺傳性多發(fā)性骨軟骨瘤軟骨帽的超微結(jié)構(gòu),以提高對(duì)兒童HME的超微病理認(rèn)識(shí),探尋該病的發(fā)病機(jī)制,為兒童HME超微病理診斷提供可靠依據(jù)。闡述兒童HME復(fù)發(fā)原因,為手術(shù)治療提供一定的指導(dǎo)。 方法 1.研究對(duì)象 實(shí)驗(yàn)組：病例選擇我院小兒骨科病區(qū)2010年10月-2012年6月收治的32例HME患兒。對(duì)照組：病例選擇我院小兒骨科病區(qū)相同時(shí)期收治的多指患兒32例,因手術(shù)切除的多指骨骺軟骨。對(duì)照組與實(shí)驗(yàn)組在年齡、性別、標(biāo)本處理方法等相配對(duì)；明確無(wú)家族遺傳病史；術(shù)前檢查無(wú)代謝系統(tǒng)及它處骨骼疾病。以上病例均經(jīng)醫(yī)院倫理委員會(huì)審批和家屬簽署知情同意書(shū)。 2.研究方法 將所取新鮮的瘤體軟骨帽或多指正常軟骨用生理鹽水沖洗后,放入10%福爾馬林溶液中,制作光學(xué)顯微鏡切片,應(yīng)用光鏡觀察。 去除所取HME軟骨帽及多指正常軟骨標(biāo)本表面附著物并用磷酸鹽緩沖液或生理鹽水將標(biāo)本清洗后,立即置于25g/L的戊二醛固定液中4℃恒溫固定2h以上。根據(jù)實(shí)驗(yàn)的需要,按要求制作成掃描電鏡及透射電鏡標(biāo)本,應(yīng)用掃描電鏡及透射電鏡觀察研究。 結(jié)果 1.多指正常軟骨和兒童HME瘤體軟骨帽的光鏡觀察 多指正常軟骨HE染色后光鏡觀察：鏡下見(jiàn)軟骨膜以及軟骨組織,近軟骨膜處有呈散在分布,體積較小,呈扁平狀的軟骨細(xì)胞；組織深部可見(jiàn)分裂增生,體積增大,呈同源群分布的軟骨細(xì)胞,軟骨陷窩明顯,軟骨基質(zhì)均質(zhì)狀分布,呈紫藍(lán)色。 兒童HME瘤體軟骨帽光鏡觀察：軟骨帽表面可見(jiàn)粉紅色纖維性骨膜；軟骨細(xì)胞都在隱窩內(nèi),常垂直于基底骨排列成柱狀,在基底部它們形成骨小梁。 2.多指正常軟骨和兒童HME瘤體軟骨帽的掃描電鏡觀察結(jié)果 對(duì)照組(多指正常軟骨)：冷凍斷裂的軟骨組織內(nèi)見(jiàn)少量的軟骨細(xì)胞位于軟骨陷窩內(nèi),細(xì)胞呈圓形或不規(guī)則,細(xì)胞表面有少量的突起；軟骨組織表面可見(jiàn)大量散亂、稀疏的膠原纖維。 實(shí)驗(yàn)組(兒童HME瘤體軟骨帽)：冷凍斷裂的軟骨組織內(nèi)見(jiàn)大量不規(guī)則的軟骨陷窩,每個(gè)軟骨陷窩內(nèi)均含有軟骨細(xì)胞,細(xì)胞增生明顯,軟骨細(xì)胞體積較大,形狀不規(guī)則,細(xì)胞表面有豐富的細(xì)胞突起；軟骨組織表面的膠原纖維較對(duì)照組結(jié)構(gòu)致密。 3.多指正常軟骨和兒童HME瘤體軟骨帽的透射電鏡觀察結(jié)果 對(duì)照組(多指正常軟骨)：軟骨細(xì)胞數(shù)量不多,細(xì)胞多呈橢圓形,細(xì)胞表面有少量短小的微絨毛,細(xì)胞核形狀不規(guī)則,核內(nèi)染色質(zhì)凝集、邊集；細(xì)胞質(zhì)內(nèi)可見(jiàn)到少量的細(xì)胞器,線粒體較小,糖原顆粒呈簇狀分布,粗面內(nèi)質(zhì)網(wǎng)成條索樣分散在細(xì)胞質(zhì)內(nèi)。 實(shí)驗(yàn)組(兒童HME瘤體軟骨帽)：大量的瘤樣細(xì)胞增生,瘤細(xì)胞體積較大,聚集分布；細(xì)胞核較大,核內(nèi)常染色質(zhì)豐富,核仁明顯,核質(zhì)比較高；細(xì)胞質(zhì)內(nèi)可見(jiàn)到擴(kuò)張的粗面內(nèi)質(zhì)網(wǎng)以及圓形或橢圓形的線粒體,溶酶體數(shù)量較少；瘤細(xì)胞間可見(jiàn)到毛細(xì)血管,血管腔內(nèi)充滿紅細(xì)胞及血漿蛋白,細(xì)胞間亦有蛋白樣物質(zhì)的沉積；在瘤樣細(xì)胞附近可見(jiàn)到明顯增多的軟骨細(xì)胞,軟骨細(xì)胞體積較對(duì)照組增大,形態(tài)呈梭形,細(xì)胞核圓形,核內(nèi)常染色質(zhì)豐富,有明顯的核仁；細(xì)胞質(zhì)內(nèi)可見(jiàn)到大量粗面內(nèi)質(zhì)網(wǎng),粗面內(nèi)質(zhì)網(wǎng)呈池樣擴(kuò)張,擴(kuò)張的池內(nèi)充滿細(xì)顆粒樣物質(zhì)：線粒體數(shù)量較少,游離核糖體�？梢�(jiàn)到。 結(jié)論 1.兒童HME瘤體軟骨帽軟骨組織代謝旺盛,細(xì)胞增生及蛋白質(zhì)合成活動(dòng)活躍,部分瘤體軟骨帽膠原纖維致密有鈣化傾向,具有使瘤體惡變的一定物質(zhì)基礎(chǔ),這與兒童HME的發(fā)病、發(fā)展、轉(zhuǎn)歸等因素密切相關(guān)。 2.兒童HME軟骨帽是瘤體惡變、復(fù)發(fā)的根源所在。
[Abstract]:Hereditary multiple osteochondroma (Hereditary Multiple Exostoses, HME) is a genetic skeleton disease involving the human skeleton system and affecting the normal osteochondral bone process. This disease is the most common benign bone tumor in the pediatric department of orthopedics with a low incidence of about 1/5 million. The incidence of male is higher than that of women, and the proportion of men and women is about 3:1. children. The tumor body of HME is composed of a cap like structure of cartilage tissue and a bone tissue that is almost perpendicular to the bone surface. Most of the tumors are distributed around the joints, that is, the metaphysis of the long bone, the ribs, the scapula, and the pelvis. The tumor is characterized by a hat like cartilaginous cap at the top of the tumor. The action of cartilaginous osseous causes the tumor to grow continuously. When the tumor grows larger, it can cause local pain or cause bone malformation. The near joint can cause activity disorder, or the adjacent vascular nerve can be oppressed to cause the corresponding symptoms. If the tumor grows suddenly or grows rapidly, it should be considered to have a malignant possibility. Many scholars from the genetic pathogenicity gene. HME has been studied deeply in the aspects of location, pathogenesis, clinical manifestation, typing and imaging. The overgrowth of cartilage cap has been considered as one of the main causes of HME malignancy.
objective
The ultrastructure of children's hereditary multiple osteochondroma cartilage hats was observed by scanning electron microscopy and transmission electron microscopy, so as to improve the ultrastructure of HME in children and explore the pathogenesis of the disease, and provide a reliable basis for the pathological diagnosis of HME in children. The reasons for the recurrence of HME in children were explained and some guidance for the surgical treatment was provided.
Method
1. research objects
In the experimental group, 32 cases of HME were selected in the pediatric department of orthopedics of our hospital in October 2010 -2012 year in June. Control group: cases selected from the same period of the pediatric department of orthopedics in our hospital were treated with multiple finger epiphyseal cartilage. The control group was paired with the experimental group in age, sex, and sample treatment. There was no history of family history. No metabolic system and bone disease were found before operation. All cases were examined and approved by the hospital ethics committee and informed consent was signed by the family members.
2. research methods
After rinsing the fresh cartilage cap or multi finger cartilage with normal saline, it was put into 10% formalin solution and made the optical microscope section to observe the light microscope.
After cleaning the specimens of the HME cartilage cap and multi finger normal cartilage specimens and cleaning the specimens with phosphate buffer or physiological saline, the specimens were immediately placed in the 25g/L glutaraldehyde fixed solution at 4 degrees centigrade at more than 2H at constant temperature. The scanning electron microscope and transmission electron microscopy were made according to the requirements of the experiment, and the scanning electron microscope and transmission electron microscope were applied. Observation and research.
Result
1. multi finger normal cartilage and HME cartilage cap in children: light microscopic observation
After HE staining of normal cartilage, the optical microscopy was observed: cartilage membrane and cartilage tissue were seen under the microscope. There were scattered, small, flat cartilage cells near the cartilage membrane. The cartilage cells with split hyperplasia and volume increased in the deep tissue, the cartilage lacunae were obvious, the cartilage matrix was homogeneous and purple blue.
The light microscopic observation of the HME tumor cartilage cap of children: the pink fibrotic periosteum on the surface of the cartilage cap is visible; the chondrocytes are in the recess, often perpendicular to the basilar bone to form a columnar, and they form a trabecular bone in the basal part.
Scanning electron microscopic observation of 2. multi finger normal cartilage and HME cartilage cap in children
The control group (multi finger cartilage): a small number of chondrocytes in the cartilaginous lacunae were found in the cartilage tissue of the frozen broken cartilage. The cells were round or irregular, with a small number of protuberances on the surface of the cells; a large number of scattered and sparse collagen fibers were seen on the surface of cartilage tissue.
In the experimental group (HME tumor cartilage cap of children): a large number of irregular cartilaginous lacunae were found in the cartilage tissue of frozen fracture. The chondrocytes were contained in each cartilage lacunae, the cell proliferation was obvious, the size of the chondrocytes was large, the shape of the cartilage was irregular, the surface of the cell was rich in cell protuberance, and the collagen fiber on the cartilage tissue surface was more than the control group. Secret.
3. transmission electron microscopic observation of normal cartilage and HME cartilage cap in children
The control group (mostly normal cartilage): the number of cartilage cells is not much, the cell is mostly oval, the cell surface has a small amount of short microvilli, the nucleus shape is irregular, the chromatin agglutination in the nucleus, the edge set, the cytoplasm can see a small number of organelles, the mitochondria are small, the glycogen granules are distributed in clusters, and the rough endoplasmic reticulum pattern is dispersed in thin. In the cytoplasm.
The experimental group (children's HME tumor cartilage cap): a large number of tumor like cells proliferate, the tumor cells are large and aggregative, the nuclei are large, the nuclei are often rich in chromatin, the nucleolus is obvious, and the nucleoplasm is high; the cytoplasm can see the dilated rough endoplasmic reticulum and the elliptical mitochondria in the cytoplasm, and the number of lysosomes is less; the tumor cells can be seen between the cells. The capillaries were filled with erythrocytes and plasma proteins in the blood vessel, and there was also a deposition of protein like material between the cells. There was a significant increase of chondrocytes in the vicinity of the tumor like cells. The volume of chondrocytes was larger than the control group. The morphology showed spindle shape, the nucleus was round, the nucleus was abundant in the nucleus, and the nucleolus was obvious. The cytoplasm could be seen large in the cytoplasm. Rough endoplasmic reticulum, rough endoplasmic reticulum and pool like dilatation. The dilated pool is filled with fine particulate matter: a small number of mitochondria and free ribosomes.
conclusion
1. children's HME tumor cartilage cap cartilage has strong metabolism, cell proliferation and protein synthesis activity are active. The cartilage cap of some tumors is dense and calcified, which has a certain material basis for the malignant transformation of the tumor, which is closely related to the pathogenesis, development and prognosis of children's HME.
2. children's HME cartilage cap is malignant transformation of tumor, the root cause of recurrence.
【學(xué)位授予單位】：鄭州大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2013
【分類號(hào)】：R738.1
本文編號(hào)：2133623
[Abstract]:Hereditary multiple osteochondroma (Hereditary Multiple Exostoses, HME) is a genetic skeleton disease involving the human skeleton system and affecting the normal osteochondral bone process. This disease is the most common benign bone tumor in the pediatric department of orthopedics with a low incidence of about 1/5 million. The incidence of male is higher than that of women, and the proportion of men and women is about 3:1. children. The tumor body of HME is composed of a cap like structure of cartilage tissue and a bone tissue that is almost perpendicular to the bone surface. Most of the tumors are distributed around the joints, that is, the metaphysis of the long bone, the ribs, the scapula, and the pelvis. The tumor is characterized by a hat like cartilaginous cap at the top of the tumor. The action of cartilaginous osseous causes the tumor to grow continuously. When the tumor grows larger, it can cause local pain or cause bone malformation. The near joint can cause activity disorder, or the adjacent vascular nerve can be oppressed to cause the corresponding symptoms. If the tumor grows suddenly or grows rapidly, it should be considered to have a malignant possibility. Many scholars from the genetic pathogenicity gene. HME has been studied deeply in the aspects of location, pathogenesis, clinical manifestation, typing and imaging. The overgrowth of cartilage cap has been considered as one of the main causes of HME malignancy.
objective
The ultrastructure of children's hereditary multiple osteochondroma cartilage hats was observed by scanning electron microscopy and transmission electron microscopy, so as to improve the ultrastructure of HME in children and explore the pathogenesis of the disease, and provide a reliable basis for the pathological diagnosis of HME in children. The reasons for the recurrence of HME in children were explained and some guidance for the surgical treatment was provided.
Method
1. research objects
In the experimental group, 32 cases of HME were selected in the pediatric department of orthopedics of our hospital in October 2010 -2012 year in June. Control group: cases selected from the same period of the pediatric department of orthopedics in our hospital were treated with multiple finger epiphyseal cartilage. The control group was paired with the experimental group in age, sex, and sample treatment. There was no history of family history. No metabolic system and bone disease were found before operation. All cases were examined and approved by the hospital ethics committee and informed consent was signed by the family members.
2. research methods
After rinsing the fresh cartilage cap or multi finger cartilage with normal saline, it was put into 10% formalin solution and made the optical microscope section to observe the light microscope.
After cleaning the specimens of the HME cartilage cap and multi finger normal cartilage specimens and cleaning the specimens with phosphate buffer or physiological saline, the specimens were immediately placed in the 25g/L glutaraldehyde fixed solution at 4 degrees centigrade at more than 2H at constant temperature. The scanning electron microscope and transmission electron microscopy were made according to the requirements of the experiment, and the scanning electron microscope and transmission electron microscope were applied. Observation and research.
Result
1. multi finger normal cartilage and HME cartilage cap in children: light microscopic observation
After HE staining of normal cartilage, the optical microscopy was observed: cartilage membrane and cartilage tissue were seen under the microscope. There were scattered, small, flat cartilage cells near the cartilage membrane. The cartilage cells with split hyperplasia and volume increased in the deep tissue, the cartilage lacunae were obvious, the cartilage matrix was homogeneous and purple blue.
The light microscopic observation of the HME tumor cartilage cap of children: the pink fibrotic periosteum on the surface of the cartilage cap is visible; the chondrocytes are in the recess, often perpendicular to the basilar bone to form a columnar, and they form a trabecular bone in the basal part.
Scanning electron microscopic observation of 2. multi finger normal cartilage and HME cartilage cap in children
The control group (multi finger cartilage): a small number of chondrocytes in the cartilaginous lacunae were found in the cartilage tissue of the frozen broken cartilage. The cells were round or irregular, with a small number of protuberances on the surface of the cells; a large number of scattered and sparse collagen fibers were seen on the surface of cartilage tissue.
In the experimental group (HME tumor cartilage cap of children): a large number of irregular cartilaginous lacunae were found in the cartilage tissue of frozen fracture. The chondrocytes were contained in each cartilage lacunae, the cell proliferation was obvious, the size of the chondrocytes was large, the shape of the cartilage was irregular, the surface of the cell was rich in cell protuberance, and the collagen fiber on the cartilage tissue surface was more than the control group. Secret.
3. transmission electron microscopic observation of normal cartilage and HME cartilage cap in children
The control group (mostly normal cartilage): the number of cartilage cells is not much, the cell is mostly oval, the cell surface has a small amount of short microvilli, the nucleus shape is irregular, the chromatin agglutination in the nucleus, the edge set, the cytoplasm can see a small number of organelles, the mitochondria are small, the glycogen granules are distributed in clusters, and the rough endoplasmic reticulum pattern is dispersed in thin. In the cytoplasm.
The experimental group (children's HME tumor cartilage cap): a large number of tumor like cells proliferate, the tumor cells are large and aggregative, the nuclei are large, the nuclei are often rich in chromatin, the nucleolus is obvious, and the nucleoplasm is high; the cytoplasm can see the dilated rough endoplasmic reticulum and the elliptical mitochondria in the cytoplasm, and the number of lysosomes is less; the tumor cells can be seen between the cells. The capillaries were filled with erythrocytes and plasma proteins in the blood vessel, and there was also a deposition of protein like material between the cells. There was a significant increase of chondrocytes in the vicinity of the tumor like cells. The volume of chondrocytes was larger than the control group. The morphology showed spindle shape, the nucleus was round, the nucleus was abundant in the nucleus, and the nucleolus was obvious. The cytoplasm could be seen large in the cytoplasm. Rough endoplasmic reticulum, rough endoplasmic reticulum and pool like dilatation. The dilated pool is filled with fine particulate matter: a small number of mitochondria and free ribosomes.
conclusion
1. children's HME tumor cartilage cap cartilage has strong metabolism, cell proliferation and protein synthesis activity are active. The cartilage cap of some tumors is dense and calcified, which has a certain material basis for the malignant transformation of the tumor, which is closely related to the pathogenesis, development and prognosis of children's HME.
2. children's HME cartilage cap is malignant transformation of tumor, the root cause of recurrence.
【學(xué)位授予單位】：鄭州大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2013
【分類號(hào)】：R738.1
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