99mTc-SC lymphoscintigraphy and SPECT/CT findings in a case report of Gorham–Stout disease presenting with chylothorax and bone pain

Abstract

Rationale: Gorham–Stout disease (GSD) is a rare disorder characterized by multiple osteolytic lesions, sometimes complicated by chylothorax. The aim of this case report is to introduce a very rare case of Gorham–Stout syndrome, which involved several bones along with chylous pericardial and pleural effusions detected by 99m Tc-sulfur colloid (SC) lymphoscintigraphy and single photon emission computed tomography/computed tomography (SPECT/CT). Patient concerns: A 15-year-old girl presented to our hospital complaining of shortness of breath and bone pain. Diagnosis: The CT showed multiple osteolytic lesions, left-sided pleural effusion, and pericardial effusion. 99m Tc-SC lymphoscintigraphy showed discontinuation of thoracic duct and tracer accumulation on the left side chest. SPECT/CT revealed increased radioactivity uptake in pleural, pericardial effusions, and some thoracolumbar spines. Diagnostic thoracentesis to identify the nature of pleural effusion and histopathology of biopsy in the right femoral to that of the bone lesion were performed. Based on the clinical information, histopathologic, and radiographic findings, the diagnosis of GSD was made. Interventions: The patient received thoracic duct ligation and bisphosphonates treatment. Outcomes: After receiving thoracic duct ligation and bisphosphonates treatment, the patient's symptoms of bone pain and dyspnea were relieved, and the pericardial and pleural fluid was diminished dramatically. At the 3-month and 9-month follow-up visit, the patient had nearly complete remission without any complication. Lessons: The 99m Tc-SC lymphoscintigraphy and SPECT/CT could provide significant value assessing the lymphatic abnormity and evaluating the extent of disease, therefore aiding to guide decision making in the clinic.

Usefulness of 99mTc-ASC lymphoscintigraphy and SPECT/CT in the evaluation of rare lymphatic disorders

“…The histopathological diagnosis of GSD was based on extensive loss of bony matrix replaced by proliferation of thin-walled capillary-sized vascular channels, and at a later stage, by fibrous connective tissue. [ 10 ] Dilated lymph vessels are the typical histopathological finding of lymphangioma, which is usually present as a cystic structure on imaging. [ 3 ] According to the diagnosis guidelines, a definite diagnosis of LAM can be made based on the presence of cystic changes on high-resolution CT of the chest and any of the following confirmatory features: renal angiomyolipoma, chylous effusion, lymphangioleiomyoma, adenopathy, lymphatic vessel dilation, and either definite or probable tuberous sclerosis complex.…”

“…[ 1 – 7 ] GSD is characterized by massive osteolysis resulting from bone-replacing abnormal capillary proliferation early in the disease or fibrous hyperplasia in the later stage. [ 8 – 10 ] Only about 300 cases of GSD have been reported in the literature. [ 11 ] All bones could be affected, but the most common areas are the skull, shoulder girdle, pelvis, and extremities.…”

“…Accurate diagnosis and evaluation of lymphatic disorders are crucial for the therapeutic approach. [ 10 , 15 , 24 , 25 ]…”

Current concepts from diagnosis to management in Gorham–Stout disease: a systematic narrative review of about 350 cases

“…These findings are often associated with other lymphatic diffuse anomalies such as chylothorax (since the establishment mechanism is the same), lymphatic flow into the pericardial area or involvement of thoracic duct. However, these are rare findings in GSD (less than 10 cases reported in the literature), but they can be life-threatening (56, 71,72,73,74,75,76,77,78).…”

The relationship between the drainage function of inguinal lymph nodes and unilateral pelvic cancer-related lymphedema

“…However, the number and size of lymph nodes only indirectly reflect the drainage function of lymph nodes. Lymphatic system imaging in nuclear medicine directly reflects the function of lymphatic drainage [9–16] . For many years, lymphoscintigraphy has been widely considered as the first-line imaging to confirm the diagnosis of lymphedema and to visualize the functional state of the lymphatic system [15,17–23] …”