A 40-year-old male presents with complaint of pain in the middle of his right calf that began after a period of toe-walking while hedge-trimming. Physical exam was notable for only localized tenderness in the area. Deep vein thrombosis was excluded twice. Rest did not alleviate his pain.
Plain radiographs of the lower extremity (tibia and fibula) demonstrate no osseous or soft tissue abnormality. Further evaluation with magnetic resonance imaging was performed. Axial STIR images demonstrate a heterogenous predominately hyperintense mass centered in the soleus. T1 weighted images of the mass demonstrate T1 heterogenous signal with areas of hyperintensity likely related to internal haemorrhage. Axial, coronal and sagittal T1 weighted images demonstrate heterogenous, predominately nodular peripheral enhancement of the mass.
Originally classified as Masson’s tumour, Intravascular Papillary Endothelial Hyperplasia (IPEH) is a reactive benign vascular proliferation that often resembles angiosarcoma. This tumour only occurs confined within the vasculature lumen and is associated with an organized thrombus [1]. This entity is estimated to represent anywhere from 2-4% of vascular tumours [2]. Primary IPEH most frequently presents in the superficial soft tissues of the head, neck or fingers. Secondary IPEH occurs as a reactive outgrowth from a previous vascular lesion.
In contrast to the primary type, secondary IPEH tends to be larger and arises in deep soft tissue locations [1]. In a study of 91 cases of IPEH, the tumour had a predilection for females, with a median age of presentation of 31 years old. The most common presenting sign of IPEH is oedema of the overriding skin, often with blue or red discoloration. Pain accompanies this pathology in roughly one-third of cases [3].
Despite the fact this pathology is relatively common in the general population, IPEH is rarely imaged [2]. On ultrasonography, the lesion appears hypoechoic with mixed or peripheral associated vascularization. The lesion appears as a small, spherical mass on MRI. On T2-weighted images, IPEH exhibits hyperintensity peripherally with discrete foci of low to intermediate intensity. Peripheral enhancement reflects vascular proliferation along the lesion’s borders, while the unpredictable pattern of the thrombus organization and vascular proliferation within the vessel accounts for the variability of the central signal [1]. Though T1-weighted imaging may vary, IPEH typically demonstrates hypointense or heterogeneous signal, possibly due to haemorrhage contained within the lesion [1,2].
Once correctly identified as IPEH, the prognosis is excellent. When necessary, simple excision of the mass generally proves curative. In the rare cases in which the lesion is refractory, one should highly suspect another underlying vascular pathology. Such cases are predicated upon treating the underlying pathology [4]. Secondary IPEH tends to recur at greater indices relative to the primary form [3].
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Intravascular papillary endothelial hyperplasia.
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Based on the provided X-ray and MRI images of the right lower leg, the bony structures (tibia and fibula) appear generally normal, with no obvious signs of bone destruction or fracture. In the soft tissue window, a solitary nodule-like lesion is visible in the deep soft tissues near the gastrocnemius muscle. On the T2-weighted MRI sequence, the lesion shows a relatively high signal around the periphery, with some disorganized internal signals, indicating the possibility of varying degrees of thrombosis or blood components. Contrast-enhanced imaging reveals peripheral ring or irregular enhancement with relatively low or heterogeneous signal in the center, consistent with neovascular proliferation in vascular or thrombotic tissue. No significant erosion or destruction is observed in adjacent muscle or bone cortex. Mild edema is occasionally noted in the surrounding soft tissue, and there is no obvious enlargement of nearby lymph nodes.
Combined with the patient's history of ruling out deep vein thrombosis and the local pain presentation, there is a clear relationship between the lesion and an intravascular condition (e.g., localized vascular proliferative changes).
Considering the patient's age, symptoms, imaging characteristics, and histological findings, the most likely diagnosis is Intravascular Papillary Endothelial Hyperplasia (IPEH). The pathological feature (papillary or villous endothelial proliferation within the vascular lumen accompanied by thrombotic components) further supports this conclusion.
Treatment Strategies:
1. Conservative Observation: If pain is mild and the lesion is small and localized, symptomatic management and regular follow-up can be considered to monitor for any increase in size or exacerbation of symptoms.
2. Surgical Excision: For lesions causing significant symptoms or affecting daily life, especially if other potential pathologies cannot be excluded, surgical removal of the lesion may be considered. Prognosis is generally good.
3. Other Treatments: If the lesion is related to previous vascular conditions (e.g., varicose veins or other vascular malformations), addressing the primary disease is necessary to reduce recurrence risk.
Rehabilitation/Exercise Prescription (FITT-VP Principle):
1. Type of Exercise: Start with moderate aerobic activities such as walking, using a stationary bike, or swimming. Avoid long periods of weight-bearing and strenuous lower-limb impact.
2. Frequency: 3-4 sessions per week initially, gradually increasing to 5 sessions depending on individual tolerance.
3. Intensity: Begin at low to moderate intensity (e.g., walking at 5-6 km/h, maintaining heart rate at 50-60% of maximal heart rate). Gradually increase as tolerated.
4. Time (Duration): Start with 20-30 minutes per session. Increase to 45 minutes or longer if no significant discomfort occurs.
5. Progression: Increase exercise volume in stages, provided there is no significant pain or other discomfort, and reassess every 2-4 weeks to adjust intensity and duration.
6. Precautions: If noticeable lower-leg pain, local swelling, or any other discomfort occurs during or after exercise, stop immediately and seek medical evaluation. Postoperative patients should increase activity gradually under a guided rehabilitation plan.
Disclaimer: This report is based on currently available information and serves as a reference for analysis. It cannot replace an in-person consultation or professional medical advice. Patients should follow individualized treatment and rehabilitation plans under the guidance of a qualified physician.
Intravascular papillary endothelial hyperplasia.
