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内生软骨瘤

Enchondroma

作者信息

Biondi Nicholas L., Tiwari Vivek, Varacallo Matthew A.

机构信息

Campbell Un / Cape Fear Valley MC

Apollo Sage Hospital, Bhopal, India

PMID:30725623
Abstract

Bone is a unique connective tissue type, being one that undergoes mineralization. Bone's inorganic component consists of calcium hydroxyapatite, which imparts strength and toughness. This mineral is also the body's main calcium (99%) and phosphate (85%) reservoir and stores 65% of its sodium and magnesium. Meanwhile, bone's organic component is comprised of bone cells and matrix proteins. The bone cells include the following: Osteoprogenitor cells: pluripotent mesenchymal stem cells in bone surfaces that growth factors can stimulate to differentiate into osteoblasts. Osteoblasts: initiate mineralization and produce, transport, and arrange matrix proteins. Osteoblast actions are regulated by parathyroid hormone, vitamin D, estrogen, growth factors, cytokines, leptin, and low-density lipoprotein-related protein 5. Osteoblasts may become osteocytes or surface-lining cells of bones. Osteocytes: the most numerous cells in bone. Osteocytes participate in key bodily functions like calcium and phosphate homeostasis and cyclic adenosine monophosphate activation. Osteoclast: hematopoietic progenitor cell-derived bone cells responsible for bone resorption. Bone matrix proteins include type 1 collagen and noncollagenous osteoblast-derived proteins.   encode the skeletal morphogenesis regulators. Mesenchyme from the following regions gives rise to different parts of the skeleton: Cranial neural crest: craniofacial skeleton. Paraxial mesoderm: axial skeleton. Lateral plate mesoderm: appendicular skeleton. In the embryo, craniofacial and clavicular bones undergo intramembranous ossification, whereby osteoblasts form bone directly from mesenchyme. These bones enlarge only when new bone deposits on the surface—a process called "appositional growth." Meanwhile, most other bones begin as a cartilage anlage, undergoing enchondral ossification around the 8th week of gestation. Bones that form this way increase in length due to the development of the physis or growth plate at their ends. Growth plates have a cartilaginous core covered by a layer of bone (primary spongiosa). Enchondral ossification within growth plates increases bone length and diameter.  Hyaline-cartilage bone tumors arise either within the medullary cavity or on the bone surface. Enchondromas are benign medullary cavity hyaline-cartilage tumors occurring in bones of endochondral origin. These growths are usually solitary, central, metaphyseal tubular bone lesions. In contrast, subperiosteal or juxtacortical chondromas form on the bone surface. Enchondromas arise most commonly from the hand and foot bones but may occasionally appear in the femur and humerus. These neoplasms are also the most common primary bone tumors of the hand, where they commonly appear in the proximal phalanges. Other common sites include the middle phalanges, metacarpals, and distal phalanges. Enchondromatous tumors typically begin and grow in childhood, arising from rests of growth plate cartilage or chondrocytes that initially proliferate but stop developing normally and persist throughout adulthood. These tumors are most frequently noted in the 3rd and 4th decades of life. The small finger is the most commonly affected digit. Hand enchondromas are unique, as they may also demonstrate cellular atypia, resembling chondrosarcoma on histopathological examination. Enchondromas have malignant potential and may transform into chondrosarcoma. However, malignancies arising from enchondromas are low-grade and rarely metastasize to other body regions.

摘要

骨是一种独特的结缔组织类型,是一种会发生矿化的组织。骨的无机成分由羟基磷灰石钙组成,赋予骨强度和韧性。这种矿物质也是人体主要的钙(99%)和磷(85%)储存库,储存了其65%的钠和镁。同时,骨的有机成分由骨细胞和基质蛋白组成。骨细胞包括以下几种:骨祖细胞:存在于骨表面的多能间充质干细胞,生长因子可刺激其分化为成骨细胞。成骨细胞:启动矿化并产生、运输和排列基质蛋白。成骨细胞的活动受甲状旁腺激素、维生素D、雌激素、生长因子、细胞因子、瘦素和低密度脂蛋白相关蛋白5的调节。成骨细胞可能会变成骨细胞或骨的表面衬里细胞。骨细胞:骨中数量最多的细胞。骨细胞参与关键的身体功能,如钙和磷的稳态以及环磷酸腺苷的激活。破骨细胞:造血祖细胞衍生的骨细胞,负责骨吸收。骨基质蛋白包括I型胶原蛋白和成骨细胞衍生的非胶原蛋白。编码骨骼形态发生调节因子。来自以下区域的间充质产生骨骼的不同部分:颅神经嵴:颅面骨骼。轴旁中胚层:轴骨骼。侧板中胚层:附属骨骼。在胚胎中,颅面骨和锁骨经历膜内成骨,即成骨细胞直接从中胚层形成骨。这些骨头只有在新骨沉积在表面时才会增大——这个过程称为“附加生长”。同时,大多数其他骨头最初是软骨原基,在妊娠第8周左右经历软骨内成骨。以这种方式形成的骨头由于其末端的骨骺或生长板的发育而长度增加。生长板有一个软骨核心,被一层骨(初级骨小梁)覆盖。生长板内的软骨内成骨增加骨的长度和直径。透明软骨骨肿瘤发生在髓腔内或骨表面。内生软骨瘤是发生在软骨内起源骨的良性髓腔透明软骨肿瘤。这些生长通常是孤立的、中央性的、干骺端管状骨病变。相比之下,骨膜下或皮质旁软骨瘤形成于骨表面。内生软骨瘤最常见于手和足部骨骼,但偶尔也可能出现在股骨和肱骨。这些肿瘤也是手部最常见的原发性骨肿瘤,通常出现在近端指骨。其他常见部位包括中指骨、掌骨和远端指骨。内生软骨瘤性肿瘤通常在儿童期开始并生长,起源于生长板软骨或软骨细胞的残余物,这些细胞最初增殖但停止正常发育并在整个成年期持续存在。这些肿瘤最常出现在生命的第三个和第四个十年。小指是最常受影响的手指。手部内生软骨瘤很独特,因为它们在组织病理学检查中也可能表现出细胞异型性,类似于软骨肉瘤。内生软骨瘤有恶变潜能,可能转变为软骨肉瘤。然而,由内生软骨瘤引起的恶性肿瘤级别较低,很少转移到身体其他部位。