儿童遗传性多发性骨软骨瘤软骨帽的超微结构观察
发布时间：2018-07-20 13:08
【摘要】：儿童遗传性多发性骨软骨瘤(Hereditary Multiple Exostoses, HME)是一种涉及人体骨骼系统,影响正常软骨化骨过程的遗传性骨骼疾病。本病是小儿骨科中最常见的良性骨肿瘤,发病率较低约为1/5万,男性发病率高于女性,男女比例约为3：1。儿童HME的瘤体由软骨组织的帽状结构和几乎与骨骼骨面垂直突出的骨组织构成,大多数分布在关节周围,即长骨的干骺端,肋骨、肩胛骨、骨盆等处有少量分布。肿瘤的特点是生长于瘤体的顶部有一形似帽子的软骨层即软骨帽,通过其深层的软骨化骨作用,使瘤体持续生长,肿瘤增长较大时可引起局部疼痛或者导致骨骼畸形,近关节的可引起活动障碍,或可压迫邻近的血管神经而引起相应的症状,若肿瘤突然长大或生长迅速,应考虑有恶变的可能。很多学者从遗传致病基因定位、发病机制、临床表现及分型、影像学等方面对HME进行过深入的研究,软骨帽过度生长已被认为是HME恶变的主要原因之一。 目的 本课题通过扫描电镜和透射电镜观察儿童遗传性多发性骨软骨瘤软骨帽的超微结构,以提高对儿童HME的超微病理认识,探寻该病的发病机制,为儿童HME超微病理诊断提供可靠依据。阐述儿童HME复发原因,为手术治疗提供一定的指导。 方法 1.研究对象 实验组：病例选择我院小儿骨科病区2010年10月-2012年6月收治的32例HME患儿。对照组：病例选择我院小儿骨科病区相同时期收治的多指患儿32例,因手术切除的多指骨骺软骨。对照组与实验组在年龄、性别、标本处理方法等相配对；明确无家族遗传病史；术前检查无代谢系统及它处骨骼疾病。以上病例均经医院伦理委员会审批和家属签署知情同意书。 2.研究方法 将所取新鲜的瘤体软骨帽或多指正常软骨用生理盐水冲洗后,放入10%福尔马林溶液中,制作光学显微镜切片,应用光镜观察。 去除所取HME软骨帽及多指正常软骨标本表面附着物并用磷酸盐缓冲液或生理盐水将标本清洗后,立即置于25g/L的戊二醛固定液中4℃恒温固定2h以上。根据实验的需要,按要求制作成扫描电镜及透射电镜标本,应用扫描电镜及透射电镜观察研究。 结果 1.多指正常软骨和儿童HME瘤体软骨帽的光镜观察 多指正常软骨HE染色后光镜观察：镜下见软骨膜以及软骨组织,近软骨膜处有呈散在分布,体积较小,呈扁平状的软骨细胞；组织深部可见分裂增生,体积增大,呈同源群分布的软骨细胞,软骨陷窝明显,软骨基质均质状分布,呈紫蓝色。 儿童HME瘤体软骨帽光镜观察：软骨帽表面可见粉红色纤维性骨膜；软骨细胞都在隐窝内,常垂直于基底骨排列成柱状,在基底部它们形成骨小梁。 2.多指正常软骨和儿童HME瘤体软骨帽的扫描电镜观察结果 对照组(多指正常软骨)：冷冻断裂的软骨组织内见少量的软骨细胞位于软骨陷窝内,细胞呈圆形或不规则,细胞表面有少量的突起；软骨组织表面可见大量散乱、稀疏的胶原纤维。 实验组(儿童HME瘤体软骨帽)：冷冻断裂的软骨组织内见大量不规则的软骨陷窝,每个软骨陷窝内均含有软骨细胞,细胞增生明显,软骨细胞体积较大,形状不规则,细胞表面有丰富的细胞突起；软骨组织表面的胶原纤维较对照组结构致密。 3.多指正常软骨和儿童HME瘤体软骨帽的透射电镜观察结果 对照组(多指正常软骨)：软骨细胞数量不多,细胞多呈椭圆形,细胞表面有少量短小的微绒毛,细胞核形状不规则,核内染色质凝集、边集；细胞质内可见到少量的细胞器,线粒体较小,糖原颗粒呈簇状分布,粗面内质网成条索样分散在细胞质内。 实验组(儿童HME瘤体软骨帽)：大量的瘤样细胞增生,瘤细胞体积较大,聚集分布；细胞核较大,核内常染色质丰富,核仁明显,核质比较高；细胞质内可见到扩张的粗面内质网以及圆形或椭圆形的线粒体,溶酶体数量较少；瘤细胞间可见到毛细血管,血管腔内充满红细胞及血浆蛋白,细胞间亦有蛋白样物质的沉积；在瘤样细胞附近可见到明显增多的软骨细胞,软骨细胞体积较对照组增大,形态呈梭形,细胞核圆形,核内常染色质丰富,有明显的核仁；细胞质内可见到大量粗面内质网,粗面内质网呈池样扩张,扩张的池内充满细颗粒样物质：线粒体数量较少,游离核糖体常可见到。 结论 1.儿童HME瘤体软骨帽软骨组织代谢旺盛,细胞增生及蛋白质合成活动活跃,部分瘤体软骨帽胶原纤维致密有钙化倾向,具有使瘤体恶变的一定物质基础,这与儿童HME的发病、发展、转归等因素密切相关。 2.儿童HME软骨帽是瘤体恶变、复发的根源所在。
[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.
【学位授予单位】：郑州大学
【学位级别】：硕士
【学位授予年份】：2013
【分类号】：R738.1
本文编号：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.
【学位授予单位】：郑州大学
【学位级别】：硕士
【学位授予年份】：2013
【分类号】：R738.1
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