Mondor disease
Mondor disease is a rare benign breast condition characterized by thrombophlebitis of the subcutaneous veins of the breast and anterior chest wall. It can also occur in the dorsal veins of the penis.
Epidemiology
Although Mondor disease is rarely reported in the literature, this is likely in part due to a lack of awareness of the entity. It tends to dominate in women between 30-60 years.
Incidence rates of 0.5-0.8% have been reported, however, it reflects only the symptomatic population.
Clinical presentation
Patients usually present with a painful breast (cord-like) mass. There may be overlying skin erythema. It may be accentuated when the ipsilateral arm is raised.
Pathology
The pathogenesis includes the formation of venous thrombosis with total or partial occlusion and vascular recanalization causing fibromuscular hyperplasia of the vessel wall and infiltration plus fibrosis of surrounding subcutaneous cellular tissue. The thrombotic vessel can adhere to the superjacent skin causing retraction and formation of characteristic cordiform grooves secondary to local fibroblastic proliferation.
The vessels most commonly involved are the thoraco-epigastric, lateral thoracic vein, and superior epigastric veins.
Associations
In most patients, the condition is idiopathic . Recognized associations include:
- trauma, preceding thoracic or breast surgical procedures, ultrasound-guided or stereotactic biopsies
- previous central venous catheters
- hypercoagulable states
- dehydration
- although an association with breast cancer (~12% ) has been reported, the veracity of this claim is difficult to confirm due to the over-representation of patients being investigated for other breast lesions in reported cases
Location - distribution
Most commonly affects the thoraco-epigastric and/or lateral thoracic veins towards the upper outer quadrant. The upper inner quadrants of the breasts are almost never involved .
Radiographic features
Mammography
Typically, Mondor disease appears as superficially located tubular beaded density corresponding to a palpable rope-like mass. Mammography can be normal in a significant proportion of cases.
Ultrasound
On ultrasound, Mondor disease appears as a tubular anechoic or isoechoic structure with multiple areas of narrowing, giving a beaded appearance. Sometimes low-level internal echoes may be present representing clot. The surrounding soft tissues may be hyperechoic due to the associated inflammatory response.
No flow is present on color or spectral Doppler studies and in some situations, an abrupt cut off with the normal vessel may be seen.
Treatment and prognosis
It is a benign self-limiting condition, and the natural history is for the thrombosed vein to recanalize and for clinical symptoms to resolve gradually (~ 6 weeks). Anti-inflammatory medications are sometimes given for symptomatic relief. Otherwise, management is supportive, and anticoagulants, antibiotics or surgical intervention is not indicated due to thrombophlebitis subsiding spontaneously and without complications, or persistent deformity. A close interval follow-up scan is usually recommended to ensure resolution and to exclude any other entity.
History and etymology
In the year 1869, Faage was the first one who described this condition. Further characterization of this condition by the French surgeon Henry Mondor in 1939, hence the name.
Siehe auch:
- paraneoplastisches Syndrom
- Polycythaemia vera
- Venae thoracoepigastricae
- Schwangerschaft
- Polyglobulie
- Neoplasien der Mamma
und weiter:
Assoziationen und Differentialdiagnosen zu Mondor disease:
