Introduction:
Diagnosing any condition in the beginning stage indicates the state of the disease and its prognosis. In the case of IPEH, as it is very uncommon, both microscopic and radiographic incidence plays a major role in primarily diagnosing it. Intravascular papillary endothelial hyperplasia is also commonly called Masson tumors. Although various forms of diagnostic methods are used, the most preferred in the case of IPEH is the microscopic and radiological examination. They reveal the shape and form of the structure along with the quality of the cell content and the structural changes happening within it. These diagnostic methods also help in differentiating the condition to be cancerous and non-cancerous.
What Is Intravascular Papillary Endothelial Hyperplasia?
Intravascular papillary endothelial hyperplasia is a lesion of the blood vessels that occurs as a proliferative reaction of the endothelial cells lining the blood vessels. The condition is very uncommon and is seen commonly among two percent of the cases in both cancerous and non-cancerous tumors. This type of tumor occurs in various parts of the body, most commonly involving the neck, head, trunk of the body, and fingers.
How Is Intravascular Papillary Endothelial Hyperplasia Seen Clinically?
Intravascular papillary endothelial hyperplasia is seen clinically as a very small, firm, reddish blue mass present superficially on the skin. Also, the occurrence of intravascular papillary endothelial hyperplasia is extremely rare in the case of the oral lesion and the forearm region; however, it can still spread in the mucosa of the lip and oral cavity. The intravascular papillary endothelial hyperplasia is seen to be a deep routed lesion along with conditions like intramuscular, pre-existing, and cavernous (large space) hemangiomas ( non-cancerous growth of the blood vessels that appear reddish blue in color) that are very rare conditions.
The prevalence of intravascular papillary endothelial hyperplasia (IPEH) does not have any specific predilection and is seen equally in male and female patients. Intravascular papillary endothelial hyperplasia can be of two types, namely pure and mixed. The pure type originates from the blood vessels commonly involving the vein, while the mixed type arises alongside pathological conditions like venous hemorrhoids (venous swelling seen in the lower portion of the rectus), pyogenic granuloma (an elevated non-cancerous tumor seen lying on the surface of the skin or mucosa, having a very high tendency to bleed) or cavernous hemangioma.
What Causes Incidence of Intravascular Papillary Endothelial Hyperplasia?
The incidence of intravascular papillary endothelial hyperplasia is believed to occur due to two reasons. One theory states that the condition occurs as a result of a thrombotic eruption (thrombus is a blood clot seen in the blood vessels) that triggers the proliferation of the endothelium (single colonization of cells forming a layer of skin). However, in contrast to this theory, in 1976, Clearkin and Enzinger suggested that the structure of the papilla is a depiction of an already existing structure of the thrombus. The condition becomes reactive, showing proliferation commonly after any traumatic incident.
What Are the Diagnostic Indications Seen in Intravascular Papillary Endothelial Hyperplasia?
Microscopic examinations play a very important role in the diagnosis of papillary endothelial hyperplasia. This is because the vesicles are composed of connective tissue and endothelial surfaces that distinguish one variant from another. Although many other structures resemble this condition, the most predominant features that provided standout diagnostic criteria are:
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The IPEH is commonly encapsulated or well-circumscribed in nature.
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The condition seems to be entirely contained within the intra-vascular spaces.
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The cells appear in a hyperchromatic pigmentation with an increased number of nuclear elements and mitotic characters (characters of cell division and proliferation). The papilla appears as a fibrohyalinized tissue with multiple layers of cells covering it.
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The endothelial does not appear true to the origin to help in aiding the diagnosis of IPEH.
When the structures are dissected, the tissues appear as a tangential section with pseudo channels present in them; however, necrosis of the structure is rare.
What Are the Radiographic Indications Seen in Intravascular Papillary Endothelial Hyperplasia?
A number of imaging techniques are used in the field of medicine; the imaging method most preferred in the diagnosis of intravascular papillary endothelial hyperplasia (IPEH) is magnetic resonance imaging (MRI), which is a three-dimensional imaging method used to examine the changes in the structure of the desired soft tissue. In this condition, MRI is used to examine the size of the lesion, the site of presentation, its intensity, replication, and appearance.
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Ultrasound Imaging: This imaging technique uses ultrasound wave frequencies to record the imaging. In this case of IPEH, ultrasound imaging is used to assess the size, tumor shape, and the ability of the cells to bounce the ultrasound waves in the form of echoes (echogenicity), diversity of the cellular complex, its blood supply, etc.
While these are some of the most commonly used diagnostic methods, the radiographic features that are to be taken under consideration after performing diagnostic imaging methods for a proper diagnosis of intravascular papillary endothelial hyperplasia (IPEH) are mentioned below:
- The tumor lesions are often situated in the subcutaneous tissue in close association with the origination of blood vessels.
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The size of the tumors is often around or less than two centimeters.
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High-intensity signals were seen while hitting the foci of the tumor.
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Masses that are well defined in the structure can be hyperechoic in nature with hyperintensity and also show the presentation of mixed septal lesions, whereas it can also show images that are intense only in the peripheries with foci showing low intensity.
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The images of the tumor appear more distinguishingly colored in the MRI.
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The epithelium appears with densely packed cells showing finger-like projections on the outer layer and a core that is fibrously vascularised.
Conclusion:
The significance of early diagnosis of IPEH is important to reduce the rate of progression of the disease. Both these diagnostic and radiological markers show specific features that distinguish these conditions and help in easy treatment and recovery of the patient. The diagnostic variations mentioned above are often missed out in general analysis and hence require specialized imaging techniques. The knowledge of these features gives us an idea of tumor features that clearly demarcates from other common and similar diagnosis.
