非典型的纤维素瘤/多形皮肤肉瘤与骨质转化:一系列的三个案例
Taylor Novice1, Yitong Xu2, Thomas Brenn1,2
1Department of Pathology, University of Michigan, Ann Arbor, Michigan, USA.
Journal of cutaneous pathology
|January 6, 2026
概括
非典型的纤维素瘤和多形皮肤肉瘤可以呈现出骨质状体的产生. 在这些皮肤瘤中,这种罕见的发现对于老年患者的准确诊断和预后很重要.
科学领域:
- 皮肤病理学 皮肤病理学
- 在瘤学瘤学.
- 骨生物学 骨生物学
背景情况:
- 异型纤维素瘤 (AFX) 和多形皮肤肉瘤 (PDS) 是罕见的介质细胞性皮肤瘤.
- 这些瘤通常影响阳光损伤的头皮肤上的老年人.
- PDS比AFX更具侵略性,但它们具有相似的特征,使诊断复杂化.
研究的目的:
- 报告罕见的AFX/PDS病例,表现为骨质转化.
- 为了突出这些瘤中骨质产生的诊断挑战.
- 强调区分这些实体与其他骨形成瘤的重要性.
主要方法:
- 对3名患有AFX/PDS和骨质转化症的患者的病例系列分析.
- 瘤样本的组织病理学检查.
- 免疫组织化学分析以评估瘤标志物.
主要成果:
- 确定了三名老年患者 (91-93岁),他们的头部瘤被诊断为AFX/PDS,骨形质变形.
- 报告中的任何病例都没有出现复发或转移.
- 在这些病例中,骨质矩阵的产生是一个值得注意的特征,此前仅在一个PDS病例中报告.
结论:
- 带有骨质转化症的AFX/PDS是这些介质瘤的一个罕见变体.
- 准确的诊断至关重要,以区分这些瘤与其他骨性瘤.
- 提出的病例表明,AFX/PDS与骨质转化症的预后可能是有利的,因为没有观察到复发或转移.
相关概念视频
Fibril-associated Collagen
Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...
For example, the type II collagen fibrils in cartilage have covalently bound type IX fibril-associated collagens at regular intervals. Other types of fibril-associated collagens are...
Introduction to Connective Tissues
Connective tissues are one of the four main tissue types in humans that are extensively present in the body. They are characterized by cells embedded in an extracellular matrix (ECM) composed of a ground substance and three main types of protein fibers— collagen, elastic, and reticular fibers. The ground substance of connective tissues can range from a watery and jelly-like consistency to mineralized and hard. The wide variety of cells in the connective tissues include fibroblasts, osteocytes,...
Skin Diseases and Disorders
Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
Skin Cancer
Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
Fibrous Proteins
Fibrous proteins are either long and narrow proteins or assemble to form long and thin structures. They contain repetitive units and usually consist of either alpha helices or beta sheets and, in rare cases, a mix of both. The amino acids in the primary structure often consist of repeating amino acid sequences. The role of fibrous proteins is primarily structural. Many are located in the extracellular matrix and are present in connective tissues to impart strength and joint mobility. They are...
Healing II: Complications
Complications during healing arise when tissue repair is altered by local or systemic factors. These changes involve abnormal collagen deposition, altered biomechanics, and reduced vascular supply, impairing restoration of normal structure and function.Loss of FunctionScar tissue differs significantly from the original tissue it replaces. In the skin, fibrosis lacks adnexal structures such as hair follicles, sebaceous glands, and sweat glands. Their absence reduces tactile sensitivity, impairs...


