Usefulness of 99mTc-ASC lymphoscintigraphy and SPECT/CT in the evaluation of rare lymphatic disorders: Gorham-Stout disease, lymphangioma, and lymphangioleiomyomatosis.

The purpose of this study was to investigate the role of Tc-antimony sulfide colloid (ASC) lymphoscintigraphy and single photon emission computed tomography/computed tomography (SPECT/CT) in the evaluation of rare lymphatic disorders, including Gorham--Stout disease (GSD), lymphangioma, and lymphangioleiomyomatosis (LAM).Nine patients suspected to have rare lymphatic disorders were included in this retrospective study. All patients underwent Tc-ASC lymphoscintigraphy and SPECT/CT to evaluate the lesions. The lymphoscintigraphy results were compared with the clinical and immunopathological findings.Tc-ASC lymphoscintigraphy and SPECT/CT could provide lymphatic draining and anatomical information for rare lymphatic disorders. Among the 9 patients, 3 were diagnosed with GSD (1 female, 2 males; aged 15-34 years, range 27.0 ± 10.4 years), 3 with lymphangioma (1 female, 2 males; aged 17-42 years, range 32.0 ± 13.2 years), and 3 patients were diagnosed with LAM (3 females; aged 33-50 years, range 43.7 ± 9.3 years]. GSD is characterized by multiple bone destruction, including spine, ribs, ilium, pubis, ischium, and femur. The tracer uptake of involved bones and soft tissue around bone is increased, accompanied by chylothorax, chylopericardium, and chylous leakage in abdominal and pelvic cavity. Lymphangiomas present as multiple cystic lesions with increased tracer uptake in the peripancreatic, retroperitoneal, and iliac areas, and in the abdominopelvic cavity. LAM presents as multiple thin-walled cysts in the bilateral lungs and multiple retroperitoneal enlarged lymph nodes with increased tracer uptake.Tc-ASC lymphoscintigraphy and SPECT/CT could comprehensively and specifically detect some rare lymphatic disorders, namely, GSD, lymphangioma, and LAM. This technique is useful for the evaluation of GSD, lymphangioma, and LAM.