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At least 19 recordsLinked to original sources
PubMed1986

Pulmonary and extrapulmonary lymphangioleiomyomatosis. Report of a case with bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis, and a glial polyp of the endocervix.

The authors report an extraordinary case of a 33-year-old female who had pulmonary and extrapulmonary lymphangioleiomyomatosis, bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis involving the uterus, ovaries, periadrenal vessels, and liver. The patient also had a glial polyp of the endocervix which represented retained fetal parts from an abortion 15 years earlier. Both ovaries showed multiple follicle cysts which may have been a source of endogenous estrogen. The classical stigmata of the tuberous sclerosis complex were absent. The multifocal lymphangioleiomyomatosis of the various organs described presents an extremely rare manifestation of this disorder.

Adult↗
PubMed2004

Lymphangiogenesis in lymphangioleiomyomatosis: its implication in the progression of lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is characterized by the proliferation of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes, and/or other organs. We examined lymphangiogenesis using immunohistochemistry for Flt-4 (VEGFR-3), a new specific marker for lymphatic endothelial cells, as well as the expression of vascular endothelial growth factor (VEGF)-C in LAM. Specimens were obtained from 6 autopsy cases, a single lung transplant case, and 8 surgical cases for analyses. We demonstrated that lymphatics were extremely abundant in both pulmonary and extrapulmonary LAM and that lymphatic endothelial cells not only proliferated encompassing LAM foci but also infiltrated the intra-LAM foci, and that in advanced LAM, lymphangiogenesis involved vascular walls and interstitium surrounding the area where LAM cells proliferate. In contrast, angiogenesis, confirmed with CD31 immunostaining, was observed less in the LAM foci. LAM cells demonstrated positive reactivity against anti-VEGF-C antibody at varying intensities. Significant correlation (P < 0.001) was noted between the degree of lymphangiogenesis in LAM or VEGF-C expression on LAM cells and lymphagioleiomyomatosis histologic score (LHS), which represents the histologic severity of pulmonary LAM and has been reported to have prognostic significance. Our study is likely to provide a novel point of view on the pathophysiologic significance of lymphangiogenesis in LAM.

Adult↗
PubMed2006

Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features.

PURPOSE: To retrospectively compare the frequencies of computed tomographic (CT) findings in patients with lymphangioleiomyomatosis (LAM) and patients with tuberous sclerosis complex (TSC) and LAM. MATERIALS AND METHODS: Institutional review board approval and informed consent were obtained for the HIPAA-compliant study. In 256 patients with LAM (mean age, 44 years) and 67 patients with TSC/LAM (mean age, 40 years), CT scans of the chest, abdomen, and pelvis were reviewed by a single radiologist. The fraction of lung involvement with cysts was estimated from high-spatial-resolution CT scans. Other findings assessed included noncalcified pulmonary nodules, pleural effusion, thoracic duct dilatation, hepatic and renal angiomyolipomas (AMLs), lymphangioleiomyoma (LALM), ascites, nephrectomy, and renal embolization. Confidence intervals and hypothesis tests of differences in frequencies, comparison of age quartiles, RIDIT analysis, analysis of variance, and correlation coefficients were used in the statistical analysis. RESULTS: Patients with LAM had more extensive lung involvement (RIDIT score, 0.36) and higher frequency of LALM (29% vs 9%, P<.001), thoracic duct dilatation (4% vs 0, P=.3), pleural effusion (12% vs 6%, P=.2), or ascites (10% vs 6%, P=.3). Patients with TSC/LAM had higher frequency of noncalcified pulmonary nodules (12% vs 1%, P<.01), hepatic (33% vs 2%, P<.001) and renal (93% vs 32%, P<.001) AMLs, nephrectomy (25% vs 7%, P<.001), or renal artery embolization (9% vs 2%, P<.05). CONCLUSION: The extent of lung disease is greater in LAM than TSC/LAM. Hepatic and renal AMLs and noncalcified lung nodules are more common in TSC/LAM, while lymphatic involvement-thoracic duct dilatation, chylous pleural effusion, ascites, and LALM-is more common in LAM.

Adult↗
PubMed1984

Pulmonary lymphangioleiomyomatosis: a case of progesterone receptor positive lymphangioleiomyomatosis treated with medroxyprogesterone, oophorectomy and tamoxifen.

A 28-year-old woman with lymphangioleiomyomatosis had severe changes in the chest radiograph and recurrent pneumothoraces. Pathological tissue from the lung was progesterone receptor positive. After treatment with medroxyprogesterone her condition improved, but did not become clinically stable until after bilateral oophorectomy. Even after oophorectomy her condition deteriorated, when the dose of medroxyprogesterone was decreased. For one year after oophorectomy she has had no episodes of pneumothorax or breathlessness. In order to depress oestrogen activity further the anti-oestrogen drug tamoxifen was added to her usual treatment with medroxyprogesterone. During 2 weeks of treatment with tamoxifen and medroxyprogesterone marked increase of the changes in the chest radiograph were seen and a pneumothorax occurred. After discontinuation of tamoxifen the condition improved and has since been stable on medroxyprogesterone treatment.

Adult↗
PubMed2006

Frequent [corrected] hyperphosphorylation of ribosomal protein S6 [corrected] in lymphangioleiomyomatosis-associated angiomyolipomas.

Lymphangioleiomyomatosis is a progressive lung disease characterized by a diffuse proliferation of pulmonary smooth muscle cells and cystic degeneration. Lymphangioleiomyomatosis can occur either independently of other disease or in association with tuberous sclerosis complex, a tumor-suppressor gene syndrome caused by mutations that inactivate either TSC1 or TSC2. TSC2 mutations and loss of heterozygosity have been identified in sporadic lymphangioleiomyomatosis-associated angiomyolipomas, thus implicating the TSC/Ras homolog-enriched in brain (Rheb)/mammalian target of Rapamycin (mTOR)/p70 S6 kinase signaling pathway in their pathogenesis. This study was undertaken to determine whether the mTOR/p70 S6 kinase signaling pathway is activated in lymphangioleiomyomatosis-associated angiomyolipomas lacking TSC1/TSC2 loss of heterozygosity. Phospho-ribosomal protein S6 (Ser235/236) immunohistochemistry was performed on five lymphangioleiomyomatosis-associated angiomyolipomas, two matched lymphangioleiomyomatosis pulmonary samples, and three sporadic angiomyolipomas. TSC1/TSC2 loss of heterozygosity was previously excluded in these angiomyolipomas. Moderate or strong phospho-ribosomal protein S6 immunoreactivity was found in all lymphangioleiomyomatosis-associated and sporadic angiomyolipomas, suggesting a high incidence of mTOR/p70 S6 kinase signaling pathway activation despite a lack of TSC1/TSC2 loss of heterozygosity. Focally positive phospho-S6 staining was also evident in both lymphangioleiomyomatosis pulmonary samples. We hypothesized that this S6 hyperphosphorylation could reflect mutational activation of Rheb or Rheb-like protein (RhebL1), Ras family members which directly activate mTOR. Mutational analysis performed on DNA from these eight angiomyolipomas plus five additional sporadic angiomyolipomas did not reveal mutations in exons 3 and 4 (homologous sites of Ras activating mutations) of either Rheb or RhebL1. These data suggest that activation of the Rheb/mTOR/p70 S6 kinase pathway is related to the pathogenesis of lymphangioleiomyomatosis-associated and sporadic angiomyolipomas lacking TSC1/TSC2 loss of heterozygosity. This high incidence of mTOR signaling pathway activation suggests that treatment with mTOR inhibitors, such as Rapamycin, may benefit patients with angiomyolipomas independent of the detection of TSC1/TSC2 loss of heterozygosity.

Angiomyolipoma↗
PubMed2004

Lung transplantation for lymphangioleiomyomatosis.

BACKGROUND: Lymphangioleiomyomatosis is a rare disease in women leading to respiratory failure. We describe a single-institution experience with lung transplantation for end-stage lymphangioleiomyomatosis. METHODS: We retrospectively reviewed records of patients transplanted for lymphangioleiomyomatosis between 1989 and 2001. Follow-up was complete on all patients (median 3.5 years). RESULTS: Seven single and 7 bilateral transplants were performed on 14 recipients (mean age 41.8 years). Eleven patients suffered the following intra-operative complications: dysrhythmia (1); blood loss > 1,000 ml (7); extensive pleural adhesions (10); hypothermia (1); phrenic nerve injury (1); and graft dysfunction (2). The following post-operative complications occurred in 11 recipients: dysrhythmia (7); respiratory failure (5); sepsis (3); airway dehiscence (2); vocal cord dysfunction (1); cholecystitis (1); deep vein thrombosis (1); acute renal failure (1); and pericarditis (1). Post-operative chylous fistulas necessitated thoracic duct ligation (1); sclerosis (6); and drainage of ascites (1). There were no peri-operative deaths. Late deaths occurred due to sepsis in 2 patients and obliterative bronchiolitis in 1 patient. Survival rates were 100%, 90% and 69% at (1, 2 and 5 years, respectively.) Mean FEV1 (1.77 +/- 1.06 vs 0.60 +/- 0.91) and 6-minute walk (1,519 +/- 379 vs 826 +/- 293 feet) improved at 1 year as compared with pre-transplant evaluation. Five patients reached criteria for bronchiolitis obliterans syndrome. One recipient has had a documented recurrence of lymphangioleiomyomatosis in the transplanted lung. CONCLUSIONS: Early and late survival after lung transplant are comparable in lymphangioleiomyomatosis patients versus patients with other diseases. Morbidity is common after transplant for lymphangioleiomyomatosis and is usually due to lymphangioleiomyomatosis-related complications. Lymphangioleiomyomatosis recurrence in the allograft does not pose a substantial clinical problem.

Adult↗
PubMed2002

An unusual renal angiomyolipoma with morphological lymphangioleiomyomatosis features and coexpression of oestrogen and progesterone receptors.

Angiomyolipoma is the most common mesenchymal renal tumour, the clonal origin of which has recently been demonstrated. It is composed of varying amounts of blood vessels, smooth muscle and fat. In this report, we describe a renal angiomyolipoma, which is unusual owing to the presence of a lymphangioleiomyomatosis-like component, occurring in a 41-year-old woman suffering from sporadic lymphangioleiomyomatosis. The diagnosis was based on histopathological and immunohistochemical findings. The tumour consisted of an intimate admixture of two components: one was typical of a classical angiomyolipoma and the other was reminiscent of lymphangioleiomyomatosis. HMB45 positivity was found on 5% of the cells of the angiomyolipoma component. Ten percent of the nuclei of the lymphangioleiomyomatosis and angiomyolipoma components expressed oestrogen receptors and 5% progesterone receptors. This case illustrates a very unusual pattern of a renal angiomyolipoma containing a lymphangioleiomyomatosis-like component. The oestrogen and progesterone immunoreactivity suggests that angiomyolipoma could be hormonally dependent. Therefore, we have emphasised the morphological and immunohistochemical similarities between angiomyolipoma and lymphangioleiomyomatosis.

Adult↗
PubMed2002

The TSC-2 product tuberin is expressed in lymphangioleiomyomatosis and angiomyolipoma.

AIMS: Lymphangioleiomyomatosis is categorized by proliferation of abnormal smooth muscle cells (LAM cells) in the lungs and lymphatics and the presence of angiomyolipomas. Recently mutations in the tuberous sclerosis complex-2 gene (TSC-2) have been described in LAM cells and angiomyolipomas. The TSC-2 protein tuberin is a tumour suppressor and its loss may result in cellular proliferation. We used immunohistochemistry to test the hypothesis that uncontrolled cellular proliferation in lymphangioleiomyomatosis is the result of reduced tuberin protein expression. METHODS AND RESULTS: Tissue from normal lung, normal kidney, lymphangioleiomyomatosis and angiomyolipomas was immunostained with three separate anti-tuberin antibodies. Tuberin staining in normal tissues was similar to that previously described. Surprisingly, tuberin was strongly expressed in the LAM cells of all cases of lymphangioleiomyomatosis and angiomyolipoma at a greater level than in normal smooth muscle cells. The perivascular cells of angiomyolipomas, however, did not stain for tuberin. CONCLUSIONS: Our results suggest that a loss of tuberin protein in LAM cells is not the cause of the cellular proliferation seen in lymphangioleiomyomatosis. Lymphangioleiomyomatosis may result either from the expression of a mutant tuberin with abnormal function, as a result of mutations in functionally related proteins, or from more than one mechanism.

Adult↗
PubMed2005

The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment.

RATIONALE: Pulmonary lymphangioleiomyomatosis is a progressive cystic lung disease that is associated with infiltration of atypical smooth muscle-like cells. Previous descriptions of clinical characteristics of subjects with lymphangioleiomyomatosis have been based on a limited number of patients. OBJECTIVES: To describe the clinical characteristics of subjects with pulmonary lymphangioleiomyomatosis, both sporadic and tuberous sclerosis-related forms. METHODS: Over a 3-yr period, from 1998 to 2001, 243 subjects with pulmonary lymphangioleiomyomatosis were enrolled into a national registry; 13 subjects who had already undergone lung transplantation were excluded for the purposes of this report. MEASUREMENTS AND MAIN RESULTS: All 230 subjects were women, aged 18 to 76 yr (mean +/- SE, 44.5 +/- 0.65 yr). The average age at onset of symptoms was 38.9 +/- 0.73 yr and at diagnosis was 41.0 +/- 0.65 yr. Tuberous sclerosis complex was present in 14.8% of subjects. Pulmonary manifestations, most commonly spontaneous pneumothorax, were the primary events leading to the diagnosis in 86.5% of cases. Nearly 55% of the subjects were being treated with a progesterone derivative. An obstructive pattern on pulmonary function testing was observed in 57.3% of the subjects, whereas 33.9% had normal spirometric results. Women with tuberous sclerosis-related lymphangioleiomyomatosis were younger and had less impaired lung function compared with those with the sporadic form. CONCLUSIONS: The age range of women afflicted with pulmonary lymphangioleiomyomatosis is broader than previously appreciated and the degree of pulmonary function can be quite variable, with one-third of subjects having normal spirometry at enrollment into this registry.

Adolescent↗
PubMed1999

[Pulmonary lymphangioleiomyomatosis: an often fortuitous diagnosis. A case report].

INTRODUCTION: Lymphangioleiomyomatosis is an uncommon disorder of unknown origin, which exclusively occurs in women of reproductive age. The condition is characterized by proliferation of immature smooth muscle cells throughout the lungs, i.e., in the peribronchial, perilymphatic, and perivascular areas. This results in obliteration of the respiratory tract and in the development of cysts. Lymphangioleiomyomatosis has a poor prognosis due to both numerous lung complications and progression of the disease to respiratory failure. EXEGESIS: We report the case of a patient in whom lymphangioleiomyomatosis was fortuitously diagnosed from chest CT scan, itself performed for the diagnosis of pulmonary embolism. This case is therefore of particular interest. CONCLUSION: Our results suggest that the prevalence of lymphangioleiomyomatosis is probably underestimated due to its clinical latency and the absence of specific laboratory tests. Therefore, the development of non-invasive radiological methods should permit early diagnosis of the disease.

Adult↗
PubMed1996

Lung transplantation for lymphangioleiomyomatosis.

BACKGROUND: Lymphangioleiomyomatosis is a rare disease of unknown origin that usually leads to progressive deterioration of lung function and eventual death from respiratory failure. It occurs in women of reproductive age and people with tuberous sclerosis. Lung transplantation is a recent therapeutic approach. METHODS: We conducted a retrospective study by questionnaire of 34 patients, treated at 16 transplantation centers, who underwent lung transplantation for end-stage lymphangioleiomyomatosis between 1983 and 1995. RESULTS: Of the 34 patients, 27 received single-lung transplants; 6, bilateral transplants; and 1, a heart-lung transplant. As of August 31, 1995, the actuarial survival calculated by the Kaplan-Meier method was 69 percent after one year and 58 percent after two years. Eighteen patients were alive 33 +/- 20 months (range, 3 to 74) after transplantation. Forced expiratory volume in one second increased from 24 +/- 12 percent of the predicted value before transplantation to 48 +/- 16 percent six months after transplantation. Five early deaths (within one month) were due to hemorrhage (in one patient), acute lung injury (in three), and dehiscence of the bronchial anastomosis (in one). Eleven late deaths (after one month) were due to infections (in eight patients), bronchiolitis obliterans (in two), and metastatic nephroblastoma (in one). Disease-associated problems were extensive pleural adhesions in 18 patients, leading to moderate-to-severe intraoperative hemorrhage in 4; pneumothorax in the native lung after single-lung transplantation in 6 patients; postoperative chylothorax in 3; and recurrent lymphangioleiomyomatosis in the allograft in 1 patient, who died of disseminated aspergillosis. CONCLUSIONS: Although disease-related complications are frequent, lung transplantation can be a valuable therapy for patients with end-stage lymphangioleiomyomatosis.

Adult↗
PubMed2004

Conservative management of pulmonary lymphangioleiomyomatosis and tuberous sclerosis complicated by renal angiomyolipomas in pregnancy.

Both pulmonary lymphangioleiomyomatosis and tuberous sclerosis are rare diseases. The optimal management in pregnancy is unclear. A primigravida with pulmonary lymphangioleiomyomatosis and tuberous sclerosis complicated by worsening renal function secondary to angiomyolipomas was managed conservatively. Favorable maternal and neonatal outcomes were achieved. Pulmonary lymphangioleiomyomatosis is a consideration in tuberous sclerosis patients with respiratory symptoms. Tuberous sclerosis patients with pulmonary lymphangioleiomyomatosis require cautious and calculated expectant management in an effort to avoid adverse outcomes.

Adult↗
PubMed2000

Clinical experience of lymphangioleiomyomatosis in the UK.

BACKGROUND: Lymphangioleiomyomatosis is a rare lung disease that affects only women. No controlled trials of management have been performed and, until such data are available, management must be based on clinical experience. This study provides data on the natural history of lymphangioleiomyomatosis in the UK and compares this with experience from other centres. METHODS: We tried to identify all cases of lymphangioleiomyomatosis in the UK over a five year period by contacting all chest physicians. Cases were confirmed by lung biopsy or history and high resolution computed tomographic (CT) scanning. Details of disease and management were obtained from hospital notes. RESULTS: The 50 patients who fitted the diagnostic criteria for lymphangioleiomyomatosis had a median age at onset of 35 years (range 22-50). Five presented when postmenopausal (four taking hormone replacement therapy). Pneumothorax and dyspnoea were the most common presenting features. Extrapulmonary presentations included renal angiomyolipomas (3) and lymphangiomyomas (2). Only half the patients were assessed for renal angiomyolipoma and six were identified. Thirty patients had had one or more pneumothoraces, of which two thirds recurred if treated conservatively. Chylous effusions occurred in 11 patients, five requiring surgery. Pregnancy was uncommon once the diagnosis was made (n=7), but was associated with an increase in complications. Half the patients were taking a beta agonist and many showed a bronchodilator response in the laboratory. Thirty six patients had received hormone treatment. CONCLUSIONS: Our UK five year period prevalence was one per 1.1 million population. Since prophylactic interventions are sometimes indicated for renal angiomyolipoma, these data suggest that screening for angiomyolipoma, ideally by CT scanning, may be underused. Patients need to be aware of the increase in complications associated with pregnancy. Recurrence rate of pneumothorax was high in those not treated surgically. Hormone treatment was used variably and controlled trials are needed to determine their role and the optimum duration and dose.

Adult↗
PubMed1999

Decline in lung function in lymphangioleiomyomatosis: relation to menopause and progesterone treatment.

The progression of lymphangioleiomyomatosis, a rare lung disease in women, is thought to be influenced by hormonal factors. We studied the rate of decline in FEV(1) and carbon monoxide transfer factor (TL(CO)) in a national cohort of patients with lymphangioleiomyomatosis in the United Kingdom and its relation to two factors that might influence the disease, menopausal status and progesterone treatment. We used retrospective data from hospital notes, and of the 50 patients identified 43 had suitable lung function data spanning at least 3 mo. Mean (SD) annual decline in FEV(1) was 118 (142) ml for all patients, and these figures changed little when only data spanning at least 2 and 3 yr were analyzed. There was considerable variation in the rate of decline between subjects, however, and although it tended to be less among postmenopausal women and those receiving progesterone, patient numbers were smaller and the findings were not significant. There was a significant reduction in decline in TL(CO) in premenopausal patients receiving progesterone and in both FEV(1) and TL(CO) after starting progesterone in six patients who had data before and after starting treatment. This study documents the rapid decline in lung function in lymphangioleiomyomatosis, confirms the wide variation between patients, and provides some support for the suggestion that disease progression may be reduced by progesterone. The data provide a basis for designing prospective studies of treatment for lymphangioleiomyomatosis.

Adult↗
PubMed1995

[Pulmonary lymphangioleiomyomatosis].

Pulmonary lymphangioleiomyomatosis is a rare disease with a haphazard proliferation of smooth muscle throughout the lung. Little knowledge about the disease, its clinical presentation and the diagnostic methods used may be a reason for underdiagnosing the disease. In Ostfold county, Norway, with only 250,000 inhabitants, we have seen four patients with this disease in the last few years. The diagnosis was based on histological examination of transbronchial biopsy material in three patients who underwent bronchoscopy the presence of lymphangioleiomyomatosis was not recognized at the initial pathological examination. The disease can be misinterpreted as fibrosis at histological examination. Specific procedures for detecting smooth muscle can be used, eradicating the need for open lung biopsy. Transbronchial biopsy is a valid and useful method for confirming the diagnosis of lymphangioleiomyomatosis. High resolution CT has also shown to be an important diagnostic tool. We emphasize the importance of raising the question of lymphangioleiomyomatosis with the pathologist when this rare, but probably underdiagnosed disease, is suspected by the clinician.

Diagnosis, Differential↗
PubMedDate not supplied

Lymphangioleiomyomatosis: recurrence after lung transplantation.

Pulmonary lymphangioleiomyomatosis is a disease principally affecting women during child-bearing years that eventually leads to respiratory failure. Recently, it has been listed as an indication for lung transplantation. To date, no cases of recurrent lymphangioleiomyomatosis after lung transplantation have been reported, unlike the experience with sarcoidosis and giant cell interstitial pneumonia. At the University of Pittsburgh Medical Center, four patients have undergone single-lung transplantation for lymphangioleiomyomatosis. We now report that one of these cases developed recurrent lymphangioleiomyomatosis in the allograft lung.

Female↗
PubMed2001

Lung transplantation for cystic lung diseases: lymphangioleiomyomatosis, histiocytosis x, and sarcoidosis.

Lung transplantation for respiratory failure due to lymphangioleiomyomatosis, histiocytosis X, and sarcoidosis is a therapeutic option in selected patients. These indications account for less than 6% of all lung transplant procedures, and experience is relatively scarce. Pulmonary function testing at evaluation usually shows an obstructive pattern in lymphangioleiomyomatosis, whereas histiocytosis presents with a mixed pattern, and sarcoidosis with a restrictive pattern. Lung diffusion capacity is similarly reduced, however, only histiocytosis and sarcoidosis are often associated with pulmonary hypertension. In all three diseases extrapulmonary manifestations, although of different kinds, are common and must be investigated before transplantation to avoid posttransplant diagnostic pitfalls. In all these diseases, single and bilateral transplantation show comparable results. Typical complications in lymphangioleiomyomatosis include intraoperative severe intrathoracic bleeding, posttransplant chylothorax, pneumothorax, and bleeding of angiomyolipomas. In histiocytosis, extrapulmonary manifestations in bones and pituitary gland may progress. These diseases typically recur in the allograft, leading to clinical symptoms in histiocytosis whereas recurrence in lymphangioleiomyomatosis and sarcoidosis is most often asymptomatic. Survival is similar to patients transplanted for other diseases.

Journal Article↗
PubMed1991

Estrogen and progesterone receptors in lymphangioleiomyomatosis, epithelioid hemangioendothelioma, and sclerosing hemangioma of the lung.

The therapeutic options in the treatment of lung neoplasia usually have not included hormonal therapy, unlike those for primary tumors of other sites (e.g., breast). However, two mesenchymal proliferations of lung, lymphangioleiomyomatosis and epithelioid hemangioendothelioma (EHE), and one epithelial tumor, sclerosing hemangioma (SH), have a significant female predilection and may benefit from such hormonal therapy. The authors investigated five cases each of EHE and lymphangioleiomyomatosis and four cases of SH for expression of estrogen and progesterone receptors and 17-beta estradiol in paraffin-embedded tissue. Only one case each of lymphangioleiomyomatosis and EHE expressed 17-beta estradiol. All of the other cases were negative. These findings are contrary to the viewpoint held in published literature, especially in case reports of lymphangioleiomyomatosis, describing patients with positive estrogen and progesterone receptor results. Consequently, a number of issues must be considered in the clinical and immunohistochemical evaluation of the estrogen and progesterone receptor status of these rare pulmonary neoplasms.

Adult↗