局部侵入性乳头内淋巴管内血管内膜瘤在淋巴/静脉形内发生
Reham Al-Refai1, Doaa Morrar1, Ahmed Bendari1
1Department of Pathology and Laboratory Medicine, Northwell Health - Lenox Hill Hospital, New York City, NY, USA.
The American journal of case reports
|October 7, 2023
概括
乳头内淋巴管内血管内膜瘤 (PILA) 是一种罕见的血管瘤. 这一案例突出了PILA的重点.
科学领域:
- 血管新生体 血管新生体
- 儿科瘤学 儿科瘤学
- 皮肤病理学 皮肤病理学
背景情况:
- 乳头内淋巴血管内膜瘤 (PILA) 是一种罕见的血管瘤,报告的病例不到50例.
- 通常影响儿童和年轻成年人,经常在四肢表现出边缘行为和淋巴特征.
- 以前被认为是恶性,现在被归类为罕见转移的淋巴血管瘤.
研究的目的:
- 在儿科患者中呈现PILA病例.
- 讨论PILA的诊断挑战和组织学特征.
- 为了强调这种罕见的血管瘤的长期随访的重要性.
主要方法:
- 一个10岁的女孩的病例报告,有面部静脉形的病史.
- 对右面软组织瘤的手术切除.
- 组织病理学检查包括免疫组织化学染色 (D2-40,ERG,CD34,CD31,CD3,CD20).
主要成果:
- 在淋巴/静脉形 (LVM) 的背景下诊断PILA.
- 组织病理学揭示了 papillary 和 Kaposiform 形态与细胞学上平淡的状细胞.
- 免疫组织化学证实了内皮标记物和淋巴细胞透,没有显著的形或线粒活动.
结论:
- 皮质血管新生瘤 (PILA) 构成诊断挑战,应在皮肤血管新生瘤的差异诊断中加以考虑.
- 边界行为需要长期的随访,因为潜在的局部侵入性和转移.
- 通过特征性组织学和免疫组织化学特征进行准确的诊断对于管理至关重要.
相关概念视频
01:15Development of the Lymphatic System
760
The development of lymphatic tissues and vessels in embryonic life begins around the fifth week. These structures originate from the mesoderm layer, with lymph sacs emerging from developing veins.
The first lymph sacs to form are the paired jugular lymph sacs located at the junction of the internal jugular and subclavian veins. From these sacs, lymphatic capillary plexuses extend to the thorax, upper limbs, neck, and head, eventually forming lymphatic vessels. Each jugular lymph sac maintains a...
The first lymph sacs to form are the paired jugular lymph sacs located at the junction of the internal jugular and subclavian veins. From these sacs, lymphatic capillary plexuses extend to the thorax, upper limbs, neck, and head, eventually forming lymphatic vessels. Each jugular lymph sac maintains a...
760
01:16Lymphatic Vessels and Lymph Transport
3.6K
Lymphatic vessels, known as lymphatics, are crucial in transporting lymph from peripheral tissues to our venous system. This process begins with lymph entering through tiny capillaries that branch through tissues. These capillaries have unique features such as larger diameters, thinner walls, and a distinctive one-way valve system formed by overlapping endothelial cells.
This one-way system allows fluids, solutes, and even pathogens to enter but prevents their return to the intercellular...
This one-way system allows fluids, solutes, and even pathogens to enter but prevents their return to the intercellular...
3.6K
01:20Venous Thrombosis II: Clinical Manifestations and Diagnostic Studies
13
The key difference between Superficial Vein Thrombosis (SVT) and Deep Vein Thrombosis (DVT) lies in their location and severity.Clinical ManifestationsSVT typically presents with localized pain, tenderness, and redness along the course of a superficial vein, often accompanied by a palpable, cord-like structure under the skin. This condition is usually less dangerous than DVT but can be uncomfortable and may lead to complications such as cellulitis or, rarely, a clot extension into the deep...
13
01:23Detailed Structure and Function of Lymph Nodes
1.8K
Lymph nodes are bean-shaped structures that cluster along the lymphatic vessels in the inguinal, axillary, and cervical regions. Each node is divided into compartments by a capsule that extends trabeculae inward.
From a histological perspective, lymph nodes can be split into two main areas: the superficial cortex and the deep medulla. The outer cortex is populated by dendritic cells, macrophages, and B lymphocytes, which are densely packed into follicles. When these B-lymphocytes are presented...
From a histological perspective, lymph nodes can be split into two main areas: the superficial cortex and the deep medulla. The outer cortex is populated by dendritic cells, macrophages, and B lymphocytes, which are densely packed into follicles. When these B-lymphocytes are presented...
1.8K