ABSTRACT
In-transit metastases (ITM) are defined as metastatic lymph nodes or deposits occurring between the primary tumor and proximal draining lymph node basin. In extremity rhabdomyosarcoma (RMS), they have rarely been reported. This study evaluates the frequency, staging and survival of patients with ITM in distal extremity RMS. METHODS: Patients with extremity RMS distal to the elbow or knee, enrolled in the EpSSG RMS 2005 trial between 2005 and 2016 were eligible for this study. RESULTS: One hundred and nine distal extremity RMS patients, with a median age of 6.2 years (range 0-21 years) were included. Thirty seven of 109 (34%) had lymph node metastases at diagnosis, 19 of them (51%) had ITM, especially in lower extremity RMS. 18F-FDG-PET/CT detected involved lymph nodes in 47% of patients. In patients not undergoing 18F-FDG-PET/CT lymph node involvement was detected in 22%. The 5-yr EFS of patients with ITM vs proximal lymph nodes vs combined proximal and ITM was 88.9% vs 21.4% vs 20%, respectively (p = 0.01) and 5-yr OS was 100% vs 25.2% vs 15%, respectively (p = 0.003). CONCLUSION: Our study showed that in-transit metastases constituted more than 50% of all lymph node metastases in distal extremity RMS. 18F-FDG-PET/CT improved nodal staging by detecting more regional and in-transit metastases. Popliteal and epitrochlear nodes should be considered as true (distal) regional nodes, instead of in-transit metastases. Biopsy of these nodes is recommended especially in distal extremity RMS of the lower limb. Patients with proximal (axillary or inguinal) lymph node involvement have a worse prognosis.
Subject(s)
Fluorodeoxyglucose F18 , Rhabdomyosarcoma , Adolescent , Child , Child, Preschool , Clinical Trials as Topic , Humans , Infant , Infant, Newborn , Lower Extremity , Lymph Nodes/pathology , Lymphatic Metastasis/pathology , Neoplasm Staging , Positron Emission Tomography Computed Tomography , Rhabdomyosarcoma/diagnostic imaging , Rhabdomyosarcoma/pathology , Young AdultABSTRACT
OBJECTIVE: Pneumatosis intestinalis (PI) is a radiological sign that can be accompanied by pneumoperitoneum. It is not exclusive of neonatal necrotizing enterocolitis. It can also appear after bone marrow transplantation. We describe our experience with 6 patients diagnosed of PI after bone marrow transplantation (BMT) who were treated conservatively without surgery in any case and good outcome. PATIENTS AND METHOD: We have reviewed the patients diagnosed of PI from 2000 to 2007 after BMT in our center. RESULTS: Six patients have had 7 episodes of PI with pneumoperitoneum in 3. All cases previously developed intestinal graft-versus-host disease. PI was diagnosed from 1 to 4 months after transplantation. At diagnosis, any patient presented peritoneal signs. Computed tomography was used for PI diagnosis with colonic predominance (5), pneumomediastinum (1) and retropneumoperitoneum (2). The treatment was conservative with intestinal rest, antibiotics and total parenteral nutrition. Enteral feeding was initiated progressively between 1 and 2 months after diagnosis but in one case PI reappeared and it required to start again the conservative treatment. In the other cases, outcome was very satisfactory, improving the pneumatosis and with a correct oral feeding without needing of surgery in any case. COMMENTS: PI with or without pneumoperitoneum is an condition to have in mind in bone marrow transplantation patients. Pneumoperitoneum with good general condition and no sign of peritonitis is not indicative of surgery in these patients. Conservative treatment with antibiotics and parenteral nutrition allows resolution spontaneously
