Lymphoscintigraphy in Angiomyomatous Hamartomas and Primary Lower Limb Lymphedema

Purpose: Angiomyomatous hamartoma (AH) of the lymph node is a rare vascular benign disease of unknown etiology with a predisposition for the lymph nodes of the inguinal area. Only 18 cases have been described up to now in the literature and the disorder was reported to be associated with lymphedema or swelling of the ipsilateral limb in 4 patients. However, scintigraphic investigation of the lymphatic system in these patients was reported in only 2 cases. Material and Methods: Five patients where the biopsy of inguinal nodes for suspected lymphadenitis led to the diagnosis of angiomyomatous hamartoma were investigated using lymphoscintigraphic techniques (1 girl aged 15; 1 boy aged 9 at the time of first biopsy and 11 at the time of the second one; and 3 men aged 30, 50, and 57). The operated limb was lymphedematous in 3 and 1 developed lymphedema after biopsy. The fifth patient developed a contralateral lymphedema after his second nodal biopsy. Results: In all cases, lymphoscintigraphic investigation of the limbs showed extensive lymph node abnormalities on the operated side and in 4 cases on the opposite side. Conclusions: These observations support not only the hypothesis that lymphatic disturbance was involved in the pathogenesis of these tumors but also the proposition that lymphoscintigraphy should be performed in cases of inguinal lymphadenitis of unknown origin to diagnose the underlying situation of latent lymphedema.