Isolated sporadic uterine lymphangioleiomyoma with unusual clinical and pathological features.

We report a unique uterine neoplasm, favoured to represent an isolated extrapulmonary lymphangioleiomyoma with unusual pathological features, in a postmenopausal woman without tuberous sclerosis complex. The large neoplasm consisted of smooth muscle fascicles and cystic spaces lined by lymphatic cells, which were negative for the melanocytic staining that is characteristically positive in lymphangioleiomyomatosis (LAM). There are fewer than 30 cases of uterine LAM reported, none of which have demonstrated this morphology or these immunohistochemical findings. The origin of LAM cells in the more typical pulmonary LAM remains unclear; the unusual features in this case may represent a distinct pathological entity or a rare variant of typical extrapulmonary LAM, and may contribute to determining the cellular origin of these rare tumours. Conversely, this may represent a case of 'prepulmonary' LAM, providing supporting evidence for a possible gynaecological origin of these tumours in the broader affected (almost exclusively female) population.

Retroperitoneal lymphangioleiomyoma mimicking ovarian tumor emerging after tamoxifen therapy.

BACKGROUND: Lymphangioleiomyomas are lymphatic masses that can be associated with lymphangioleiomyomatosis. They are usually associated with pulmonary involvement. CASE: A 44-year-old premenopausal woman with breast cancer treated with adjuvant tamoxifen presented with abdominal distension. A thoraco-abdominopelvic enhanced computed tomography scan showed a 22 x 21 x 12 cm well-encapsulated, complex pelvic mass. An ovarian cystadenocarcinoma was suspected. Surgery revealed a retroperitoneal mass that was removed with uterus and both adnexae. Histological and immunohistochemical studies diagnosed a lymphangioleiomyoma. Estrogen and progesterone receptors were positive on smooth muscle cells and human melanoma black 45 was negative. CONCLUSION: Isolated retroperitoneal lymphangioleiomyoma is rare and difficult to detect in the absence of pulmonary lymphangioleiomyomatosis. We speculate that tamoxifen treatment may play a role in the development of this benign tumor.

Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score.

Correlations were made between clinical and follow-up data and histopathologic findings in 105 women (mean age +/- standard deviation, 38.3 +/- 9.0 years) with pulmonary lymphangioleiomyomatosis (LAM). The actuarial survival (to pulmonary transplantation or death) of the patients from the time of lung biopsy was 85.1% and 71.0% after 5 and 10 years respectively. The histologic severity of LAM, graded as a LAM histologic score (LHS), was determined on the basis of semiquantitative estimation of the percentage of tissue involvement by the two major features of LAM: the cystic lesions and the infiltration by abnormal smooth muscle cells (LAM cells) in each case: LHS-1, <25%; LHS-2, 25% to 50%; and LHS-3, >50%. Analysis using the Kaplan-Meier method revealed significant differences in survival for patients with LHS-1, -2, and -3 (p = 0.01). The 5-and 10-year survivals were 100% and 100% for LHS-1, 81.2% and 74.4% for LHS-2, and 62.8% and 52.4% for LHS-3. Increased degrees of accumulation of hemosiderin in macrophages also were associated with higher LHS scores (p = 0.029) and a worse prognosis (p = 0.0012). Thus, the current study suggests that the LHS may provide a basis for determining the prognosis of LAM.

Treatment of lymphangioleiomyomatosis. A meta-analysis.

Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. It is sufficiently uncommon that randomization or any other systematic evaluation of regimens of treatment has been difficult. Review of scattered case reports implies that a number of hormonal manipulations may be equally effective. A comprehensive review of the literature revealed 30 cases of LAM treated with eight regimens of treatment. Evaluation with predetermined criteria (meta-analysis) shows that administration of progesterone or oophorectomy or both are the most effective treatments, resulting in improvement or stabilization of the disease in the majority of cases.

Unilateral auto-PEEP in the recipient of a single lung transplant.

We report a patient who received a right single lung transplant (SLT) for progressive lymphangioleiomyomatosis and required reintubation for postoperative respiratory distress. She developed hemodynamic instability due to mediastinal shift from unilateral auto-PEEP with hyperinflation of the native lung. Placement of a double lumen endotracheal tube (DLET) and institution of differential lung ventilation restored equal lung inflation and hemodynamic stability.

How to predict forced vital capacity after living-donor lobar-lung transplantation.

BACKGROUND: Living-donor lobar-lung transplantation (LDLLT) has evolved from a rarely performed experimental procedure to an accepted therapy for selected patients who are unlikely to survive the long wait for cadaveric lungs. However, a convincing study has not been performed that shows the effects of small grafts and of pre-operative variables in predicting functional outcome of recipients after LDLLT. METHODS: From October 1998 to March 2002, 2 male and 11 female patients underwent LDLLT. Mean age was 27.3 years (range, 8-53 years). Diagnoses included primary pulmonary hypertension (n = 5), idiopathic interstitial pneumonia (n = 2), bronchiolitis obliterans (n = 2), bronchiectasis (n = 2), lymphangioleiomyomatosis (n = 1), and cystic fibrosis (n = 1). Donors included 12 men and 14 women with a mean age of 40 years. Given that the right lower lobe consists of 5 segments, the left lower lobe of 4, and the whole lung of 19, we estimated the graft forced vital capacity (FVC) based on the donor's measured FVC and compared this with the recipient's FVC measured after LDLLT. RESULTS: Currently, all patients are alive, with a mean follow-up of 22.2 months (range, 10-51 months). The recipients' FVC measured at 6 months (1,813 +/- 86 ml) correlated well with the graft FVC (1,803 +/- 70 ml), estimated based on the donors' measured FVC (r = 0.802, p = 0.00098). CONCLUSIONS: Recipient FVC after LDLLT can be predicted by measuring donor FVC before surgery regardless of the diagnosis of the recipient.

Retroperitoneal lymphangiomyoma in an infant.

Lymphangiomyomatosis (LAM) is a benign tumor-like lesion of lymphatic vessels with unknown etiology. 80 cases of LAM are presently described. So far, it appears that only women in their reproductive years seem to be affected. Here, we describe the first LAM in an 11-months-old infant with a presumably local form of LAM. This leads us to consider the possibility that this tumor-like lesion may originate from a hamartomateous malformation. The diagnosis is based on pre-operative sonography and CT, as well as on histological and immunohistochemical examination of the tumor.

Early onset of childhood pulmonary lymphangiomyomatosis.

The authors present the first case of lymphangiomyomatosis in a pediatric patient. Radiographic findings, biopsy, computerized tomography, and electron microscopy were useful in detailing the presentation of early onset of pulmonary lymphangiomyomatosis. The unusual presentation of this child includes a description of her disease prior to any pulmonary symptomatology and 4 years of follow-up.

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.

[Lymphangioleiomyomatosis--a rare disease of the lymphatic system]. / Die Lymphangioleiomyomatose--eine seltene Erkrankung des Lymphgefässsystems.

Lymphangioleiomyomatosis is a rare disease with proliferation of smooth muscle cells within the lymphatics, mediastinal and retroperitoneal lymph nodes and in the lungs. The clinical symptoms are increasing dyspnea, chylous effusion, intestinal obstruction and thoracic or abdominal pain. The authors report the case of a 42-year-old woman who primarily suffered from thoracic pain, dyspnea and chylous effusion. In further examinations we discovered a leftsided retroperitoneal tumor and a tumor in the mesentery. The diagnostic difficulties experienced are described and the necessity of explorative laparotomy for definite diagnosis is demonstrated. Furthermore, the article provides a review of the latest developments in pathology, diagnostics and therapy.

Referrals for lung transplantation in Japan. Unique indications and necessity of living-donor lobar lung transplantation.

OBJECTIVES: The purpose of this study was to review all referrals to our lung transplant program and to find realistic options for accepted candidates in Japan. METHODS: During the period from April 1992 to August 1999, 45 referrals were received. After screening, 22 of the referred patients were admitted for an extensive inpatient evaluation, and 15 of these were accepted as candidates for transplantation. RESULTS: The indication was primary pulmonary hypertension in 9, bronchiectasis in 2, lymphangioleiomyomatosis in 2, idiopathic pulmonary fibrosis in 1, and pneumoconiosis in the other 1. Two of those with primary pulmonary hypertension went to the USA and there received bilateral lung transplant. One with bronchiectasis received living-donor lobar lung transplantation in our center. These three recipients are alive and doing well during the follow-up period of 11 to 69 months. Among the 12 patients who have not received lung transplant, 5 patients have died while waiting. CONCLUSIONS: Indications for lung transplant are quite distinct in Japan, and primary pulmonary hypertension is the most frequent indication. Living-donor lobar lung transplantation is a realistic option for properly selected candidates.

[Two cases of diffuse pulmonary lymphangiomyomatosis].

We cared 2 patients with diffuse pulmonary lymphangiomyomatosis (LAM) through the perioperative period. LAM is a disease of uncertain origin and poor prognosis because of respiratory failure. Therefore, it is important to provide not only a good anesthetic care but also a good preoperative respiratory care. In the first case (a 35-yr-old woman), an open lung biopsy was performed after dyspnea and sputum had disappeared with preoperative medications of a bronchodilator and some antibiotics. In the second case (a 35-yr-old woman), oophorectomy was performed after FEV1.0% had remarkably increased with preoperative medication of a bronchodilator. Both patients did well through the perioperative period without any trouble or complications, such as pneumonia or severe hypoxemia, presumably owing to our perioperative management system.

[2 cases of lymphangioleiomyomatosis treated by hormonal manipulation]. / To tilfaelde af lymfangioleiomyomatose behandlet med hormonal manipulation.

The course of disease in two women with bioptically verified lymphangioleiomyomatosis (LAM) is demonstrated. One was oestrogen- and progesterone-receptor negative, but responded promptly to hysterosalpingo-oophorectomy and has had no symptoms later. The other was receptor positive, but in spite of hysterosalpingo-oophorectomy and hormonal manipulation with tamoxifen and medroxyprogesterone, has had a complicated course of the disease with symptoms for ten years. LAM is a hormone dependent disease, but our knowledge is still incomplete. Hormonal manipulation should be tried in receptor positive as well as negative patients. Tamoxifen seems to have deleterious effects on the course of disease.

[Anesthetic management of a patient with diffuse pulmonary hamartoangiomyomatosis associated with spontaneous pneumothorax].

We reported successful anesthetic management of a 34 year-old woman with diffuse pulmonary hamartoangiomyomatosis associated with spontaneous pneumothorax. She had recurrent pneumothorax and chest X-ray film showed reticulo-granular shadow, and chest CT revealed multiple bullae. Induced by thiopental and succinylcholine, anesthesia was maintained safely with enflurane in oxygen. Nitrous oxide was used after thoracotomy. This disease accompanies multiple bullae in the lung and some patients have complications such as tuberous sclerosis or hemangioma of the kidney. Therefore, we have to pay attention to both bullae and other complications.

[Anesthetic approach in 2 patients with pulmonary lymphangiomyomatosis]. / Conducta anestésica en 2 pacientes afectadas de linfangiomiomatosis pulmonar.

Pulmonary lymphangiomyomatosis (PLM) is an idiopathic disease of females in fertile age. It results in respiratory failure characterized by obliteration of the small airways, emphysema, formation of bullae, hemoptysis, pneumothorax, pulmonary fibrosis, severe hypoxemia and reduced carbon monoxide diffusion capacity. The major lymphatic ducts are also involved, resulting in chylous pleural effusion and ascites. Pulmonary abnormalities improve objectively and subjectively after surgical therapy, which consists in bilateral oophorectomy. Its evolution results in death in no more than 10 years. We report the anesthetic approach to 2 patients with PLM. Patient 1 was a female who had already been diagnosed of PLM and had received medical and surgical therapy, requiring reoperation for the resection of cystic intestinal masses and abdominal eventration. Patient 2 had been scheduled for bilateral oophorectomy after a diagnosis of PLM. We discuss the clinical condition of both patients, the course of the disease, the previous treatments and the anesthetic technique in each case.

[Lymphangiomyoma of the thoracic duct]. / A ductus thoracicus lymphangiomyomája.

From the right superior mediastinum of a 31-year old woman the authors extirpated a lymphangiomyoma originating from the thoracic duct. The clinical feature and pathology of this rare lesions are discussed. This case is the first successfully removed thoracic duct lymphangiomyoma in Hungary.

Solitary lymphangioleiomyoma of pancreas mimicking pancreatic pseudocyst--a case report.

INTRODUCTION: Lymphangioleiomyomatosis (LAM) is a rare disease characterized by proliferation of morphologically distinguishable smooth muscle cells in the lymphatics and lymph nodes of the pulmonary parenchyma in most cases. Extrapulmonary LAM is a rare condition and is found to occur concurrently, before or after pulmonary LAM, and show strong association with tuberous sclerosis. DISCUSSION: The literature regarding extrapulmonary LAM without associated pulmonary LAM is limited due to the extreme rarity of the cases. We hereby describe clinical, pathological and radiological features of primary pancreatic LAM presenting clinicoradiologically as pseudocyst of pancreas in a 43-year-old lady. CONCLUSION: The present case is unique as LAM in pancreas without associated pulmonary LAM has never been reported in the literature before.

Reduction of persistent air leak with endoscopic valve implants.

The standard management of air leaks due to persistent bronchopleural fistula involves chest drainage and occasionally pleurodesis, with intractable cases requiring surgical decortication or surgical repair. However, some of these patients may be at high risk for surgery, particularly if they have already had thoracic surgery or have other medical problems; for this group there is a need for less invasive methods of stopping or reducing air leaks. Emphasys endobronchial valves (EBV) are occlusive devices designed primarily for endoscopic lung volume reduction in emphysema. Because the device is a one-way inspiratory airway blocker, it is possible that it could be used in controlling persistent air leaks while maintaining the drainage of secretions. Two cases are reported of persistent air leaks that were managed by endoscopic occlusion with EBV. In one case complete stoppage of the air leak was achieved with immediate clinical benefits. The second patient died 5 days after treatment from additional complications apparently not related to the procedure. Endobronchial blockage may be a useful salvage procedure for patients with persistent air leak for whom there is no other treatment available.