Resection of Extrapulmonary Lymphangiomyoma and Post-Operative Management Considerations.

Background: Lymphangiomyomatosis is a rare disease involving the lymph vessels, causing obstruction and cystic formation with an incidence of 3-8 per million women. The disease might be sporadic or inherited. Lymphangiomyomatosis mostly affects the pulmonary system, whereas extrapulmonary Lymphangiomyomatosis may present in various site, occasionally as a localized abdominal mass. The diagnostic process might entail surgical resection to obtain a specimen for pathology that may also help to achieve a long-term control of the disease. Methods: Herein, we present a case of a 45 years old female, who suffered from pulmonary symptoms, and during her workup an abdominal mass was found. The patient underwent exploratory laparotomy with resection of a left retroperitoneal bilobar mass. Results: Histopathological report revealed Lymphangiomyoma. She had a complication of a lymphatic leakage that required a second laparotomy with satisfactory clinical outcome. Conclusions: Surgeons should be aware of the pathological lymphatics and manage post-operative complications by a trial of conservative.

Chylopericardium Due to Residual Lymphangiomyoma Detected on 99mTc-Sulfur Colloid Lymphoscintigraphy.

ABSTRACT: Lymphangiomyomas are relatively rare, benign neoplasms. Many patients present with symptoms including effusions, and some cases are incidentally detected. Surgical excision is the treatment of choice, but because of its location, complete surgical resection of a lymphangioma can be technically difficult, and recurrent cases can present with symptoms including effusions. 99mTc-sulfur colloid scan can be used to confirm the leak and nature of the effusion fluid. Here, we present an 8-year-old girl with recurrent pleural and pericardial effusions after lymphocele excision and total pericardiectomy. 99mTc-sulfur colloid lymphoscintigraphy was done to rule out secondary chylopericardium.

Effect of fasting on the size of lymphangioleiomyomas in patients with lymphangioleiomyomatosis.

BACKGROUND: Lymphangioleiomyomas occur in 38% of patients with sporadic lymphangioleiomyomatosis (LAM) and may cause pain and increased abdominal girth, mimicking the presence of a malignancy. Lymphatic involvement in LAM is closely associated with elevated serum levels of vascular endothelium growth factor-D (VEGF-D). Because lymphangioleiomyomas undergo diurnal variation in volume, we hypothesized that daytime ingestion of food, by increasing chyle formation and lymphatic flow, is the cause of an increase in lymphangioleiomyoma volume. METHODS: Subjects had abdominopelvic sonograms and blood drawn for measurement of serum VEGF-D levels under nonfasting (day 1) and fasting (day 2) conditions. The size of the lymphangioleiomyomas was determined by a radiologist who was blinded to the subjects' status. The Wilcoxon signed rank test was used to determine whether the nonfasting tumor size was different from the fasting tumor size. RESULTS: Thirty-five women were studied (aged 45.2 ± 8.5 years; FEV1, 82% ± 25%; diffusing capacity of the lung for carbon monoxide, 64% ± 25% predicted). Images suitable for volume measurements were obtained in 30 subjects. Fasting decreased the tumor size by 20.7 ± 39.3 cm3 (24% ± 40%, P < .001). Fasting VEGF-D levels (10,650 ± 900 pg/mL) were not significantly different from nonfasting values (12,100 ± 800 pg/mL, P = .56). CONCLUSIONS: Lymphangioleiomyoma volume decreased during the fasting state. Conversely, a combination of food intake and decreased chyle flow through lymphatics partially obstructed by LAM cells may account for increases in lymphangioleiomyoma size. Imaging studies performed under fasting conditions may help in determining whether an abdominal tumor is a result of LAM or malignancy.

[Case of lymphangioleiomyomatosis (LAM) discovered during cesarean section under spinal anesthesia].

We experienced a case of scheduled cesarean section under spinal anesthesia in a patient with LAM which had been missed in spite of preoperative medical examination and consultation with specialists but discovered because of perioperative hypoxia A 35-year-old woman, Gravida 1 Para 0, with breech presentation was scheduled to undergo cesarean section under spinal anesthesia at 38 weeks of gestation. She had no history of asthma or abnormal findings at annual medical examination. She had suffered from dry cough and nocturnal dyspnea for 7 weeks and an inhaled bronchodilator was administered with diagnosis of inflammatory airway disease by her respiratory physicians. Spinal anesthesia was performed with bupivacaine 12.5 mg. At the beginning of anesthesia SPO2 was 97% in supine position, but it rapidly decreased to less than 90% and 3 l x min(-1) oxygen was supplied with a facial mask. The anesthetic level was thoracal 4 bilaterally and her breathing was stable. The circulatory state, Apgar score and other vital signs were within normal ranges. Postoperative chest X-ray showed bilateral numerous grained spots and computed tomography scans showed multiple thin-walled cysts. The characteristic history and the fluoroscopic data gave her clinical diagnosis of LAM.

Efficacy and safety of low-dose sirolimus for treatment of lymphangioleiomyomatosis.

BACKGROUND: Lymphangioleiomyomatosis (LAM) is a rare disease caused by dysregulated activation of the mammalian target of rapamycin (mTOR). Sirolimus, an inhibitor of mTOR, has been reported to decrease the size of angiomyolipomas and stabilize pulmonary function in patients with LAM. However, the optimal dose for the treatment of LAM remains unclear. METHODS: We conducted a retrospective, observational study of 15 patients with LAM who underwent sirolimus therapy for more than 6 months. The efficacy was evaluated by reviewing the patients' clinical courses, pulmonary function and chest radiologic findings before and after the initiation of sirolimus treatment. RESULTS: All patients had blood trough levels of sirolimus lower than 5ng/mL. Sirolimus treatment improved the annual rates of change in FVC and FEV1 in the 9 patients who were free from chylous effusion (FVC, -101.0 vs. +190.0mL/y, p=0.046 and FEV1, -115.4 vs. +127.8mL/y, p=0.015). The remaining 7 patients had chylous effusion at the start of sirolimus treatment; the chylothorax resolved completely within 1-5 months of treatment in 6 of these cases. These results resembled those of previous studies in which blood trough levels of sirolimus ranged from 5 to 15ng/mL. CONCLUSIONS: Low-dose sirolimus (trough level, 5ng/mL or less) performed as well as the higher doses used previously for improving pulmonary function and decreasing chylous effusion in patients with LAM.

Pulmonary lymphangioleiomyomatosis associated with retroperitoneal lymphangioleiomyomas.

A 36-year-old woman presented with worsening symptoms of abdominal pain, diarrhoea, low fever, dyspnoea on exertion and bilateral leg during the last 2 months. Chest radiograph showed mild diffuse reticular pattern with preserved lung volumes. Lung high resolution CT disclosed multiple thin-walled lung cysts throughout the lungs bilaterally with no zonal predominance and with normal intervening parenchyma and abdominal CT revealed bilateral retrocrural and retroperitoneal hypodense masses. Imaging findings were consistent with lymphangioleiomyomatosis.

Thoracic computed tomographic manifestations of tuberous sclerosis in adults.

The purpose of this article is to illustrate the various manifestations that can be encountered on thoracic computed tomography of tuberous sclerosis in adults. The pulmonary findings include lymphangioleiomyomatosis and multifocal micronodular pneumocyte hyperplasia. The extrapulmonary manifestations are divided into cardiac, vascular, mediastinal, osseous, and upper abdominal findings.

Broncheoalveolar carcinoma associated with pulmonary lymphangioleiomyomatosis and Tuberous Sclerosis Complex: case report.

Lymphangioleiomyomatosis (LAM) is a rare disorder that predominantly affects the lung parenchyma of young women and it's characterized by pulmonary cyst. Tuberous sclerosis complex (TSC) is a rare genetic disorder presenting with hamartomas and neurologic symptoms. The two renal pathologies most commonly seen in TSC are angiomyolipomas and cysts; less commonly, TSC co-exist with polycystic kidney disease. In this report is described an uncommon case of a patient with broncheoalveolar carcinoma, pulmonary LAM and TSC with polycystic kidney disease.

Linfangioleiomiomatosis / Lymphangioleiomyomatosis

La linfangioleiomiomatosis (LAM) es una enfermedad rara que afecta predominantemente a la mujer, sobre todo en edad fértil. Se presenta de forma esporádica o bien asociada al complejo de esclerosis tuberosa. Se caracteriza por una proliferación anormal de células musculares lisas inmaduras (células LAM), que crecen de manera aberrante en la vía aérea, parénquima, linfáticos y vasos sanguíneos pulmonares, lo que determina una evolución progresiva hacia la insuficiencia respiratoria. Tiene carácter multisistémico, afectando a ganglios linfáticos y produciendo tumores abdominales. Dadas su escasa prevalencia, la dificultad de establecer un diagnóstico precoz, la ausencia de un tratamiento curativo y la dificultad de obtener información, encuadran a la LAM dentro del capítulo de las denominadas Enfermedades Raras. Existe un creciente interés en el estudio de esta enfermedad, lo que ha determinado el establecimiento de registros de pacientes y un crecimiento exponencial en la investigación de la LAM, tanto a nivel clínico como celular(AU)

Lymphangioleiomyomatosis (LAM) is a rare disease that mainly affects women, particularly at fertile age. It is sporadic or associated with tuberous sclerosis complex. It is characterised by an abnormal proliferation of immature smooth muscle cells (SMC), which grow aberrantly in the airway, parenchyma, lymphatics and pulmonary blood vessels and which can gradually lead to respiratory failure. It affects several systems, affecting the lymphatic ganglia and causing abdominal tumours. Given its very low prevalence, a difficult to establish early diagnosis, absence of curative treatment and the difficulty in obtaining information, places LAM under the heading of the so-called Rare Diseases. There is a growing interest in the study of this disease which has led to the setting up of patient registers and an exponential growth in LAM research, both at a clinical level and cellular level(AU)

Atypical presentation of lymphangioleiomyomatosis as jugular vein thrombosis.

Lymphangioleiomyomatosis is a rare disorder of unknown origin that almost exclusively affects women of childbearing age. It is characterised by proliferation of abnormal smooth muscle cells (lymphangioleiomyomatosis cells) in the pulmonary interstitium and along the thoracic and abdominal lymphatics. Lymphangioleiomyomatosis may be associated with tuberous sclerosis complex. The most common manifestations of lymphangioleiomyomatosis are pulmonary symptoms, including progressive dyspnoea, recurrent pneumothoraces and chylous effusions. Extrapulmonary lymphangioleiomyomatosis as the initial presentation of the disease is highly unusual. We describe a patient with extrapulmonary lymphangioleiomyomatosis presenting as jugular vein thrombosis related to lower neck lymphangioleiomyoma. CT study showed bilateral lung cysts with left-sided hydropneumothorax and retroperitoneal lymphadenopathy. A left lower neck cystic lesion was seen with thrombosis of the adjacent left subclavian and internal jugular veins.

[Acute abdominal pain and fever in a young woman as the first symptoms of lymphangioleiomyomatosis]. / Acute buikpijn en koorts bij een jonge vrouw als eerste verschijnselen van lymfangioleiomyomatose.

A previously healthy 28-year-old woman presented to the emergency clinic with acute severe abdominal pain and high fever. A diagnostic laparoscopy was performed, during which a large retroperitoneal tumour was found. A CT-scan of the abdomen and thorax confirmed the presence of a retroperitoneal mass but also revealed multiple renal angiomyolipomas and extensive cystic lesions in all lung fields. Based on these findings, the diagnosis lymphangioleiomyomatosis (LAM) was suspected, and later confirmed by histological examination of a biopsy specimen. The acute abdomen and fever appeared to have been caused by a Streptococcus agalactiae infection of the retroperitoneal lymphangiomyoma, which was treated with intravenous antibiotics. LAM is a very rare disease affecting mostly women of childbearing age and presenting almost exclusively with pulmonary symptoms. This is the first description of LAM presenting with an acute surgical abdomen and fever due to infection of a lymphangiomyoma.

Linfangioleiomiomatosis y esclerosis tuberosa con afectación pulmonar / Lymphangioleiomyomatosis and tuberous sclerosis with pulmonary involvement

Presentamos dos casos de linfangioleiomiomatosis pulmonar y un caso de esclerosis tuberosa con afectación pulmonar describiendo los hallazgos más característicos de estas entidades en la radiografía simple del tórax y en la tomografía computarizada de alta resolución (TCAR) (AU)

Intraabdominal lymphangiomyoma in an infant with protein-losing enteropathy and hemihypertrophy.

Lymphangiomyoma is an extremely rare tumor occurring exclusively in women of reproductive age. The tumor is characterized by proliferation of immature smooth muscle along the lymphatic vessels of the abdomen, thorax and lung. Although lymphangiomyoma has been reported in a young girl and a girl infant, none has been reported in boys. We report herein a case of lymphangiomyoma in a two-year-old boy. The unusual presentation in this patient was that the tumor arose from the small bowel mesentery without any evidence of lung involvement. The tumor was extirpated and lymphangiomyomatosis was confirmed pathologically.

[Tuberous sclerosis associated with renal angiomyolipoma, pulmonary lymphangioleiomyomatosis and subungual fibroma: report of a case].

We report a case of tuberous sclerosis associated with bilateral renal angiomyolipomas (AMLs), pulmonary lymphangioleiomyomatosis (LAM) and subungual fibroma of hands and feet. A 42-year-old woman who was diagnosed as tuberous sclerosis at the age of 18 complained of left flank pain and abdominal fullness. Bilateral renal AMLs were pointed out when complete examinations were performed for hypertension at the age of 32. She suffered from severe left flank pain and abdominal distension due to the left renal tumor. Left nephrectomy and excision of the renal hilar tumor were performed. The left renal tumor weighed 1120 g, the perirenal space was filled with the tumor. histopathological diagnosis of the left renal tumor and renal hilar tumor was AML. In our case, bilateral pneumothorax appeared, and chest CT scan revealed bilateral multiple pulmonary cysts. Histopathological diagnosis of pulmonary cysts was LAM. Other complications of our case are intracranial calcification and adenoma sebaceum.

Lung transplantation for respiratory failure resulting from systemic disease.

BACKGROUND: Lung transplantation for pulmonary failure resulting from systemic disease is controversial. We reviewed our transplant experience in patients with sarcoidosis, scleroderma, lymphangioleiomyomatosis, and graft-versus-host disease. METHODS: This retrospective review examined the outcome of 23 patients who underwent pulmonary transplantation for these systemic diseases. Group 1 included 15 patients with pulmonary hypertension who underwent transplantation (9 for sarcoidosis, 6 for scleroderma), and group 2 included 8 patients with normal pulmonary artery pressures who underwent transplantation (5 for lymphangioleiomyomatosis, 3 for graft-versus-host disease). The incidences of infection and rejection, pulmonary function, and survival were measured and compared with those of patients who underwent transplantation for isolated pulmonary disease. RESULTS: Although there were no differences in the rate of infection between patients who underwent transplantation for systemic versus isolated disease, patients with pulmonary hypertension who underwent transplantation for systemic disease had significantly lower rates of rejection. Four patients with sarcoidosis and 2 with lymphangioleiomyomatosis demonstrated recurrence in the allograft. Survival was similar between patients who underwent transplantation for systemic versus isolated disease. CONCLUSIONS: Patients with respiratory failure resulting from these systemic diseases can undergo transplantation with outcomes comparable to those obtained in patients who undergo transplantation for isolated pulmonary disease.