Background: Lymphangioleiomyomatosis (LAM) is a rare disease involving the bronchi, lymphatic vessels, and veins. However, there are few reports about lymphatic vessel abnormalities associated with LAM. Purpose: To evaluate computed tomography (CT) lymphangiography findings in cases of LAM, especially lymphatic vessel abnormalities. Material and Methods: Twenty-seven patients with LAM underwent direct lymphangiography (DLG), followed by a post-procedural thoracoabdominal CT examination. Results: All 27 patients were diagnosed with LAM, including 25 (93%) with chylothorax, eight (30%) with chyloperitoneum, seven (26%) with chylous fluid in the chest and abdomen, and one (4%) with lower-limb lymphedema. The CT lymphangiography showed that 27/27 (100%) patients presented iliac or retroperitoneal lymphatic vessel dilation; 20/27 (74%) presented thoracic duct outlet obstruction; 9/27 (33%) presented pleural lymph leakage; and 13/27 (48%) presented an abnormal distribution of contrast agent in the contralateral lumbar trunk, 3/27 (11%) in the pulmonary field, 1/27 (4%) in the abdominal cavity, and 4/27 (15%) in the pelvic cavity. Five of the 27 patients (19%) had retroperitoneal lymphangioleiomyomas, with contrast agent accumulation in three cases during CT lymphangiography. Two of the 27 (7%) patients had enlarged lymph nodes in the retroperitoneum. All 27 patients (100%) diagnosed with LAM displayed various lung cysts on thoracic high-resolution CT scans. Conclusion: CT lymphangiography findings can be used to diagnose most LAM cases accurately. Moreover, these findings also display the various lymphatic vessel abnormalities associated with LAM.

Objective: In this work, we studied the clinical effect of liposuction followed by lymphovenous anastomosis (LVAs) for the treatment of breast cancer-related lymphedema (BCRL).Methods: We analyzed 158 patients with unilateral upper limb BCRL who underwent liposuction followed by LVAs 2–4 months later. Arm circumferences before and 7 days after the combined treatments were prospectively recorded. Circumferences of different upper extremities were measured before the procedure, 7 days after LVAs, and during the follow-ups. Volumes were calculated with the frustum method. During the follow-ups, the conditions of patients’ treated arms, i.e., the frequency of erysipelas episodes and dependence on compression garments, were recorded.Results: The mean circumference difference between two upper limbs decreased significantly from M (P25, P75) of 5.3 (4.1, 6.9) preoperatively to 0.5 (−0.8, 1.0) (P < 0.05) 7 days after treatments, while at follow-up 0.3 (−0.4, 1.0). The mean volume difference decreased significantly from M (P25, P75) of 838.3 (662.4, 1,129.0) preoperatively to 7.8 (−120.3, 151.4) (P < 0.05) 7 days after treatments, while at follow-up 43.7 (−59.4, 161.1). The incidence of erysipelas also significantly decreased (P < 0.05). 6.3% of patients were already independent of compression garments during the past six months or even more.Conclusion: Liposuction followed by LVAs is an effective method for the treatment of BCRL.

Introduction: 22q13.3 deletion syndrome is a well-known syndrome characterized by typical clinical findings including neonatal hypotonia, absent or severely delayed speech, intellectual disability, and other various features, and detection of a heterozygous deletion of chromosome 22q13.3 with the involvement of at least part of SHANK3. It is reported that 10% to 29% of patients with 22q13.3 deletion syndrome present lymphedema. Protein-losing enteropathy (PLE) has never been reported in 22q13.3 deletion syndrome. Patient concerns: The patient presented to our institution for refractory hypoalbuminemia and chronic lymphedema in both legs. Diagnosis: The patient manifested intellectual disability, absent speech, tooth grinding, dysmorphic face, and abnormal hands and toenails. Copy-number variation sequencing confirmed the maternal deletion in 22q13.31-q13.33 (chr22:46285592–51244566, hg19). The patient was genetically diagnosed with 22q13.3 deletion syndrome. Interventions: Low-fat diets and medium-chain triglycerides supplements were prescribed. The patient was recommended to wear compression garments and elevate legs. Outcomes: The symptom of diarrhea was resolved, but hypoalbuminemia persisted. Lower extremities lymphedema was gradually becoming severe. Conclusions: Primary lymphedema and PLE can occur simultaneously in a patient with 22q13.3 deletion syndrome. The 2 phenotypes could share the same genetic etiology of congenital lymphatic abnormalities. CELSR1 deletion may play a role in lymphatic dysplasia. The case also provides additional proof of the pathogenic effect of CELSR1 on hereditary lymphedema.

Stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.

Background: Chylothorax is caused by accumulation of chylous fluid in the thoracic cavity following injury to the thoracic duct or its tributaries. It is a rare but relatively severe complication of lung cancer surgery, especially after lung resection and mediastinal lymph node dissection. If chylothorax is not treated promptly and effectively, it may result in relatively high mortality rate. Although the majority of patients with chylothorax secondary to lung cancer surgery can be treated with conservative measures, there are still some patients who fail to respond to conservative treatment or suffer from recurrent episodes. This study aims to explore the causes of postoperative chylothorax and how targeted treatment can help achieve better outcomes. Methods: Four cases of chylothorax secondary to lung cancer surgery are reported in which patients underwent direct lymphangiography (DLG) after failed conservative treatment. Results: Thoracic duct obstruction was found in all patients on DLG, as well as dilated intrathoracic part of the thoracic duct. Thoracic duct reconstruction was performed to successfully treat 3 of the 4 patients. Conclusions: DLG is of great value in the diagnosis of chylothorax secondary to lung cancer surgery, for which thoracic duct reconstruction proves to be an effective procedure. DLG provides a new objective basis for the diagnosis and treatment of chylothorax. It is our best wish to help more patients with chylothorax secondary to lung cancer surgery.

Lymphedema is a chronic condition that can follow cancer treatment, and liposuction has been shown to be an effective approach for reducing limb volume in advanced cases. However, recurrence rates vary widely, with prior erysipelas identified as a potentially significant factor influencing prognosis. This study aimed to identify key risk factors for recurrence following liposuction in patients with cancer-associated secondary lymphedema, develop a predictive nomogram model, and investigate the molecular mechanisms by which previous erysipelas may affect recurrence. In a retrospective analysis of 1,016 patients, multivariate logistic regression and propensity score matching identified four independent risk factors, namely, prior erysipelas, hyperlipidemia, severe distal limb edema, and older age, with erysipelas showing the strongest association with poor outcomes (OR 3.98; 95% CI: 2.81–5.69). A validated nomogram demonstrated high predictive accuracy (C-index 0.757, Brier score 0.176) and net clinical benefit in estimating recurrence risk. The nomogram supports personalized treatment strategies, potentially improving patient outcomes. Transcriptome sequencing further revealed that previous erysipelas exacerbates lymphedema through inflammation, tissue remodeling, and metabolic dysregulation, suggesting potential therapeutic targets.