Biomedical subjects

Robert J McKenna

Publications and source records attributed to Robert J McKenna.

At least 19 recordsLinked to original sources

PubMed2006

A multicenter trial of an intrabronchial valve for treatment of severe emphysema.

OBJECTIVES: Minimally invasive endoscopic treatment of emphysema could provide palliation with less risk than lung volume reduction surgery and offer therapy to patients currently not considered for lung volume reduction surgery. The Intrabronchial Valve is used to block bronchial airflow in the most emphysematous areas of lung. METHODS: Patients with severe chronic obstructive pulmonary disease and heterogeneous upper lobe-predominant emphysema were eligible. Patients underwent flexible bronchoscopic placement of valves into segmental or subsegmental airways in both upper lobes. Outcomes assessed over a minimum of 6 months of follow-up included the safety, feasibility, tolerance, and success of valve placement; health-related quality of life; exercise capacity; pulmonary function; and gas exchange. RESULTS: Five centers treated 30 patients. Patient follow-up ranged from 1 to 12 months. A mean of 6.1 valves were placed per patient. Valves were positioned by means of flexible bronchoscopy in 99% of desired airways, and the procedure duration ranged from 15 to 125 minutes (mean, 65 minutes). Hospital discharge occurred within 2 days in 27 of 30 patients. There were no deaths or episodes of valve migration, tissue erosion, or significant bleeding. Eighty-three percent of patients had no adverse events judged probably or definitely related to the device. Patients experienced significant improvement in health-related quality of life, although the physiologic and exercise outcomes did not show statistically significant improvements. CONCLUSIONS: These first multicenter results with the Intrabronchial Valve demonstrate significant improvements in health-related quality of life and acceptable safety, ease of use, and procedural complication rates. The valve might be a safer and less-invasive alternative to surgical therapy for patients with severe emphysema.

Adult↗

PubMed2006

Video-assisted thoracic surgery lobectomy: experience with 1,100 cases.

BACKGROUND: Although many video-assisted thoracic surgery (VATS) lobectomies have been performed over the 12 years since the first VATS lobectomy, controversies about the procedure remain regarding the safety and associated morbidity and mortality of that procedure. This series is reviewed to assess these issues. METHODS: Between 1992 and 2004, we performed 1,100 VATS lobectomies in 595 women (54.1%) and 505 men (45.9%), with a mean age of 71.2 years. Diagnoses were as follows: benign disease (53), pulmonary metastases (27), lymphoma (5), and lung cancer (1,015). Of the primary lung cancers, 641 (63.1%) were adenocarcinoma. With visualization on a monitor, anatomic hilar dissection and lymph node sampling or dissection were performed, primarily through a 5-cm incision without spreading the ribs. RESULTS: There were 9 deaths (0.8%), and none was intraoperative or due to bleeding; 932 patients had no postoperative complications (84.7%). Blood transfusion was required in 45 of 1,100 patients (4.1%). Length of stay was median 3 days (mean, 4.78). One hundred eighty patients (20%) were discharged on postoperative day 1 or 2. Conversion to a thoracotomy occurred in 28 patients (2.5%). Recurrence developed in the incisions in 5 patients (0.57%). In 2003, 89% of 224 lobectomies were performed with VATS. CONCLUSIONS: VATS lobectomy with anatomic dissection can be performed with low morbidity and mortality rates. The risk of intraoperative bleeding or recurrence in an incision seems minimal.

Adolescent↗

PubMed2006

Prevalence of in vitro extreme chemotherapy resistance in resected nonsmall-cell lung cancer.

BACKGROUND: Recent clinical trials suggest that adjuvant chemotherapy provides a survival advantage for patients with completely resected nonsmall-cell lung cancer (NSCLC) yet many patients receive chemotherapy without benefit. Tumor in vitro resistance to antineoplastic agents is highly predictive of clinical unresponsiveness to chemotherapy for some cancers; however, little is known of the prevalence of extreme chemotherapy drug resistance for human NSCLC tumors. Chemoresistance testing may be a way to predict treatment failure, choose alternative agents, and to avoid unnecessary chemotherapy toxicity. This study describes the prevalence of in vitro chemotherapy resistance in NSCLC patient tumor cultures. METHODS: A total of 3,042 NSCLC specimens were cultured in a proliferation assay and tested for resistance to carboplatin, cisplatin, doxorubicin, etoposide, gemcitabine, navelbine, paclitaxel, taxotere, or topotecan. The percentage of cell-growth inhibition measured by 3H-Thymidine uptake was used to determine extreme drug resistance, intermediate drug resistance, or low drug resistance. RESULTS: Extreme drug resistance or intermediate drug resistance to carboplatin was found in 1,056 of 1,565 NSCLC cultures (68%), to cisplatin in 1,409 of 2,227 (63%), to doxorubicin in 1,101 of 1,471 (75%), to etoposide in 1,581 of 2,505 (63%), to gemcitabine in 594 of 823 (72%), to navelbine in 603 of 1,444 (42%), to paclitaxel in 689 of 1,706 (40%), to taxotere in 273 of 521 (52%), and to topotecan in 280 of 896 (31%). CONCLUSIONS: Chemotherapy resistance is prevalent among NSCLC clinical cell cultures. This may account for the small survival seen with empiric adjuvant chemotherapy. The use of viable tumor culture for in vitro chemoresistance testing should be considered when formulating a plan of adjuvant therapy for resected NSCLC. Future trials comparing patient survival after tailored versus empiric adjuvant therapy appear justified.

Antineoplastic Agents↗

PubMed2006

Patient and surgical factors influencing air leak after lung volume reduction surgery: lessons learned from the National Emphysema Treatment Trial.

BACKGROUND: Although staple line buttressing is advocated to reduce air leak after lung volume reduction surgery (LVRS), its effectiveness is unknown. We sought to identify risk factors for air leak and its duration and to estimate its medical consequences for selecting optimal perioperative technique(s), such as buttressing technique, to preempt or treat post-LVRS air leak. METHODS: Detailed air leak data were available for 552 of 580 patients receiving bilateral stapled LVRS in the National Emphysema Treatment Trial. Risk factors for prevalence and duration of air leak were identified by logistic and hazard function analyses. Medical consequences were estimated in propensity-matched pairs with and without air leak. RESULTS: Within 30 days of LVRS, 90% of patients developed air leak (median duration = 7 days). Its occurrence was more common and duration prolonged in patients with lower diffusing capacity (p = 0.06), upper lobe disease (p = 0.04), and important pleural adhesions (p = 0.007). Duration was also protracted in Caucasians (p < 0.0001), patients using inhaled steroids (p = 0.004), and those with lower 1-second forced expiratory volume (p = 0.0003). Surgical approach, buttressing, stapler brand, and intraoperative adjunctive procedures were not associated with fewer or less prolonged air leaks (p >/= 0.2). Postoperative complications occurred more often in matched patients experiencing air leak (57% vs 30%, p = 0.0004), and postoperative stay was longer (11.8 +/- 6.5 days vs 7.6 +/- 4.4 days, p = 0.0005). CONCLUSIONS: Air leak accompanies LVRS in 90% of patients, is often prolonged, and is associated with a more complicated and protracted hospital course. Its occurrence and duration are associated with characteristics of patients and their disease, not with a specific surgical technique.

Administration, Inhalation↗

PubMed2005

Predictors of operative mortality and cardiopulmonary morbidity in the National Emphysema Treatment Trial.

OBJECTIVE: We sought to identify predictors of operative mortality, pulmonary morbidity, and cardiovascular morbidity after lung volume reduction surgery. METHODS: Univariate and multivariate logistic regression analyses were performed. Candidate predictors included demographic characteristics, physical condition characteristics, pulmonary function measures, measures of the distribution of emphysema as determined by radiologists and by means of computerized analysis of chest computed tomographic scans, and measures of exercise capacity, dyspnea, and quality of life. End points analyzed were operative mortality (death within 90 days of the operation), major pulmonary morbidities (tracheostomy, failure to wean, reintubation, pneumonia, and ventilator for > or =3 days), and cardiovascular morbidities (infarction, pulmonary embolus, or arrhythmia requiring treatment). RESULTS: Five hundred eleven patients in the non-high-risk group of the National Emphysema Treatment Trial underwent lung volume reduction. The incidence of operative mortality was 5.5%, major pulmonary morbidity occurred in 29.8% of patients, and cardiovascular morbidity occurred in 20.0% of patients. Predictors for these end points are as follows: [table: see text]. CONCLUSIONS: Although lung volume reduction can be performed in selected patients with acceptable mortality, the incidence of major cardiopulmonary morbidity remains high. The lone predictor for operative mortality of lung volume reduction was the presence of non-upper-lobe-predominant emphysema, as assessed by the radiologist. Pulmonary morbidity can be expected in elderly patients who have a low diffusing capacity for carbon monoxide and forced expiratory volume in 1 second. When assessing morbidity, the computer-assisted chest computed tomographic analysis proved useful only in predicting cardiovascular complications.

Aged↗

PubMed2005

Troubleshooting video-assisted thoracic surgery lobectomy.

PURPOSE: Surgeons converting their open lobectomy skills to video-assisted thoracic surgery (VATS) techniques have sought traditional technical courses, publications, and physician mentoring. While these are useful in teaching basic principles, it is more difficult to promulgate the numerous advanced techniques or technical "tricks" that deal with anatomical variations or pathologic changes in the lung tissue. DESCRIPTION: Engineers have simplified the process of rolling out complex technology by using troubleshooting guides. Accordingly, helpful video-assisted lobectomy maneuvers have been categorized according to the specific problems occasionally encountered at different points in the operation. EVALUATION: These maneuvers were compiled and reviewed by a panel of thoracic surgeons experienced in video-assisted lobectomies and have been active in teaching and mentoring of thoracic surgeons, residents and fellows. The techniques described have been used successfully by the authors to overcome exposure and instrumentation limitations, to achieve the outcomes reported in their series, and to guide trainees. CONCLUSIONS: Troubleshooting guides offer an organized means for surgeons to improve the parts of the video-assisted lobectomy procedure that they find tedious or challenging.

Humans↗

PubMed2005

New approaches to the minimally invasive treatment of lung cancer.

PURPOSE OF REVIEW: The momentum for minimally invasive thoracic surgery has been growing. Thousands of video-assisted thoracoscopic surgery lobectomies have been performed since the first video-assisted thoracoscopic surgery lobectomy was performed in 1992, but currently most lobectomies are still performed via a thoracotomy. Although most lobectomies could be performed with video-assisted thoracoscopic surgery, less than 5% are currently performed that way. Compared with a thoracotomy, video-assisted thoracoscopic surgery offers patients a shorter length of stay, less pain, and a quicker recovery, without compromising the adequacy of the operation. The purpose of this review is to identify the current uses for minimally invasive procedures in thoracic surgery and to present the current data regarding these procedures. RECENT FINDINGS: Complete anatomic resections and node dissections are routinely being performed at several centers internationally. The median length of stay after video-assisted thoracoscopic surgery lobectomy is 3 days, and 84.7% of patients had no complications. Studies comparing video-assisted thoracoscopic surgery and thoracotomy suggest that minimally invasive surgery causes less pain, has a smaller impact on postoperative pulmonary function, and provides a quicker return to regular activity, with at least comparable survival for cancer patients. SUMMARY: Current data suggest that, compared with a thoracotomy, video-assisted thoracoscopic surgery has advantages for anatomic pulmonary resections.

Humans↗

PubMed2005

Swyer-James (MacLeod) syndrome with placental transmogrification of the lung: a case report and review of the literature.

Swyer-James (MacLeod) syndrome is an acquired form of unilateral hyperlucency of the lung and is characterized by the development of severe emphysema, bronchiectasis, and/or bronchiolitis obliterans. It may develop as a complication of repeated episodes of pulmonary infection resulting in bronchiolitis obliterans and obstruction of small airways. Most patients with Swyer-James (MacLeod) syndrome can be managed clinically, and the pathologic features of the syndrome have been described in only a few reports. Placental transmogrification of the lung is a rare histopathologic finding that has been described in patients with severe emphysema associated with cigarette smoking, congenital bullous emphysema, and fibrochondromatous hamartomas of the lung and is characterized by the development of peculiar structures in the pulmonary parenchyma that resemble placental villi. To our knowledge, placental transmogrification of the lung has not been previously described in patients with Swyer-James (MacLeod) syndrome. We encountered a 32-year-old man with a history of childhood asthma who presented with progressively severe exertional dyspnea and had unilateral right lung hyperlucency. The patient underwent a right pneumonectomy. Examination of the lung revealed severe mixed centriacinar-panacinar emphysema in all lobes, bullous emphysema in the upper lobe, bronchiectases, mild interstitial pneumonia with fibrosis, and placental transmogrification of the pulmonary parenchyma of all 3 lobes. Here, we review the pathology of Swyer-James (MacLeod) syndrome and the possible pathogenesis of villous-like changes in the lung tissues.

Adult↗

PubMed2005

Do cancer cells selectively mutate HFE to increase their intracellular iron?

Malignant cells compared to their normal counter-parts, have an increased requirement for iron in order to achieve an enhanced cell growth. Transferrin receptor (TfR), an essential transport protein that enables cells to satisfy their need of iron, is upregulated in cancer cells. The hemochromatosis gene (HFE) produces a protein that interacts with TfR; and we hypothesized that tumor cells would selectively mutate HFE to improve their iron-uptake and thus provide themselves a growth advantage over non-tumor cells. A total of 36 non-small cell lung cancer (NSCLC) samples and matched tumor-free tissue from the same individuals were examined for HFE mutations using polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP). Sequencing analysis of the shifted bands in three of 36 cases (in both, the tumor and matching normal tissue of the same individuals) revealed the cysteine to tyrosine substitution at amino acid residue 282 (C282Y) in the protein. Surprisingly, matching samples from one patient with a heterozygous C282Y mutation in the germline, showed loss of heterozygosity in the tumor sample at the mutant HFE allele resulting in loss of the C282Y and retention of the normal allele. In addition, we examined 45 tumor cell lines from 11 different tissues. Five cell lines had a heterozygous mutation of HFE, none had a homozygous HFE mutation in the coding region. In summary, our experiments suggest that HFE mutations are not associated with a growth advantage for cancer cells.

Base Sequence↗

PubMed2004

Short-term influences of lung volume reduction surgery on the diaphragm in emphysematous hamsters.

With emphysema, diaphragm length adaptation results in shortened fibers. We hypothesize that passive diaphragm stretch occurring acutely after lung volume reduction surgery (LVRS) results in fiber injury. Bilateral LVRS was performed in emphysematous hamsters. Studies were performed 1 (D1) and 4 (D4) days after LVRS, and compared with sham-treated groups. Sarcolemmal rupture was evident in 10.9% of fibers in LVRS-D1 and reduced to 1.6% in LVRS-D4. Ultrastructural analysis revealed focal abnormalities in both LVRS-D1 and LVRS-D4 animals in over one-third of fibers. Myofibrillar disruption was not observed in sham-treated animals. Diaphragm insulin-like growth factor-I (IGF-I) was increased in LVRS-D4 compared with other emphysematous groups. Increased IGF-I immunoreactivity was localized to types IIA and I fibers. The abundance of the splice variant of IGF-I mRNA sensitive to muscle stretch (IGF-IEb) increased 3.2-fold in LVRS D-4 diaphragms, compared with emphysema-sham animals. The main form of IGF-I mRNA was unchanged. Marked force deficit was observed in the LVRS-D1 diaphragm, compared with emphysema-sham and emphysema (no surgery) animals. These data highlight a markedly compromised ventilatory pump acutely after LVRS. Acute fiber stretch predisposes to muscle fiber injury and may also be a necessary mechanotransductive stimulus for fiber remodeling as the diaphragm adapts to reduced lung volume.

Adaptation, Physiological↗

PubMed2004

Video-assisted thoracic surgery upper lobe trisegmentectomy for early-stage left apical lung cancer.

Resection of the left upper lobe with preservation of the lingula is the anatomic equivalent of a right upper lobectomy with preservation of the right middle lobe. Therefore, our standard operation for a small apical tumor in the left upper lobe has been an apical trisegmentectomy. The purpose of this article is to review our experience with the procedure by video-assisted thoracic surgery (VATS) and to describe the operative technique for a VATS apical trisegmentectomy. Eleven patients underwent the procedure with no deaths, low morbidity, and good initial disease-free survival. A VATS apical trisegmentectomy seems to be a feasible and reasonable treatment for small stage I lung cancers at the apex of the left upper lobe.

Adenocarcinoma↗

PubMed2004

Prospective randomized study evaluating a biodegradable polymeric sealant for sealing intraoperative air leaks that occur during pulmonary resection.

BACKGROUND: To evaluate the safety and effectiveness of a new biodegradable polymeric sealant to close intraoperative air leaks after pulmonary resection. METHODS: In a multicenter prospective randomized trial, 161 patients with a median age of 67 years old (range 18-85 years old), were randomized in a 2:1 ratio to receive sealant or control for at least one significant air leak (> or = 2.0 mm in size) after pulmonary resection. In the sealant group, all significant air leaks underwent attempted repair by standard methods (sutures, staples, or cautery) prior to the application of sealant. The control group underwent only standard methods. Blood was analyzed for immunologic response. Patients were followed up 1 month after surgery. RESULTS: Intraoperative air leaks were sealed in 77% of the sealant group compared with 16% in the control group (p < 0.001). The sealant group had significantly fewer patients with postoperative air leaks compared with the control group (65% vs 86%, p = 0.005). Median length of hospitalization was 6 days (range, 3-23 days) for the sealant group compared with 7 days (range 4-38 days) for controls (p = 0.028). There was no difference in mortality, morbidity, duration of chest tubes, or immune responses between the two groups. CONCLUSIONS: This study demonstrates the effectiveness of a biodegradable polymer when used as an adjunct to standard closure methods for sealing significant intraoperative air leaks that develop from pulmonary surgery. Use of the sealant led to a reduction in postoperative air leaks, which may have decreased the length of hospitalization.

Adolescent↗

PubMed2004

Frozen section diagnoses of small pulmonary nodules: accuracy and clinical implications.

BACKGROUND: Pulmonary nodules are frequently first diagnosed by frozen section, immediately followed by lobectomy or other procedures. The frozen section diagnosis of pulmonary nodules can be difficult, as inflammatory and fibrotic lesions can be confused for malignancy, creating intraoperative dilemmas for pathologists and thoracic surgeons. METHODS: We reviewed our experience at Cedars-Sinai Medical Center with the frozen section diagnoses of 183 consecutive pulmonary nodules smaller than 1.5 cm in diameter and calculated the sensitivity, specificity, and predictive values of this diagnostic procedure. RESULTS: One hundred and seventy four nodules were correctly classified by frozen section as neoplastic or nonneoplastic, six lesions were diagnosed equivocally, and two neoplasms were missed owing to sampling errors. The equivocal frozen section diagnoses included two bronchioloalveolar carcinomas (BAC) interpreted as "atypical hyperplasia, favor BAC," two BAC diagnosed as "alveolar hyperplasia," and two carcinoid tumors labeled as "atypical carcinoma" and "spindle cell lesion, carcinoid versus sclerosing hemangioma," respectively. The sensitivities for a diagnosis of neoplasia were 86.9% and 94.1% for nodules smaller than 1.1 cm in diameter and measuring 1.1 to 1.5 cm, respectively. The diagnostic accuracy of frozen sections was significantly better in nodules larger than 1.0 cm in diameter (p = 0.05). There were no false-positive diagnoses of malignancy, resulting in 100% specificity. CONCLUSIONS: Intraoperative consultation with frozen section is a sensitive and specific procedure for the diagnosis of malignancy from small pulmonary nodules. The distinction between BAC and atypical adenomatous hyperplasia, and of small peripheral carcinoid tumors from other lesions, can be difficult by frozen section. Thoracic surgeons need to become aware of these problems and develop appropriate therapeutic strategies.

Adenocarcinoma, Bronchiolo-Alveolar↗

PubMed2004

Safety and efficacy of median sternotomy versus video-assisted thoracic surgery for lung volume reduction surgery.

BACKGROUND: The National Emphysema Treatment Trial, a randomized trial comparing lung volume reduction surgery with medical therapy for severe emphysema, included randomized and nonrandomized comparisons of the median sternotomy and video-assisted thoracoscopic approaches for lung volume reduction surgery. METHODS: Lung volume reduction surgery was performed by median sternotomy only at 8 centers and video-assisted thoracoscopy only at 3 centers; 6 centers randomized the approach to lung volume reduction surgery. Mortality, morbidity, functional status, and costs were assessed. RESULTS: In the nonrandomized comparison, 359 patients received lung volume reduction surgery by median sternotomy, and 152 patients received lung volume reduction surgery by video-assisted thoracoscopy. The 90-day mortality was 5.9% for median sternotomy and 4.6% for video-assisted thoracoscopy (P =.67). Overall mortality was 0.08 deaths per person-year for median sternotomy and 0.10 deaths per person-year for video-assisted thoracoscopy (video-assisted thoracoscopy-median sternotomy risk ratio, 1.18; P =.42). Complication rates were low and not statistically different for the 2 approaches. The median hospital length of stay was longer for median sternotomy than for video-assisted thoracoscopy (10 vs 9 days; P =.01). By 30 days after surgery, 70.5% of median sternotomy patients and 80.9% of video-assisted thoracoscopy patients were living independently (P =.02). Functional outcomes were similar for median sternotomy and video-assisted thoracoscopy at 12 and 24 months. Costs for the operation and the associated hospital stay and costs in the 6 months after surgery were both less for video-assisted thoracoscopy than for median sternotomy (P <.01 in both cases). Similar results were noted for the randomized comparison. CONCLUSIONS: Morbidity and mortality were comparable after lung volume reduction surgery by video-assisted thoracoscopy or median sternotomy, as were functional results. The video-assisted thoracoscopic approach to lung volume reduction surgery allowed earlier recovery at a lower cost than median sternotomy.

Aged↗

PubMed2004

Innovative approaches to lung volume reduction for emphysema.

The 10 years of resurgent interest in lung volume reduction surgery (LVRS) and recent National Emphysema Treatment Trial findings for emphysema have stimulated a range of innovative alternative ideas aimed at improving outcomes and reducing complications associated with current LVRS techniques. Concepts being actively investigated at this time include surgical resection with compression/banding devices, endobronchial blockers, sealants, obstructing devices and valves, and bronchial bypass methods. These novel approaches are reaching the stage of clinical trials at this time. Theory, design issues, methods, potential advantages and limitations, and available results are presented. Extensive research in the near future will help to determine the potential clinical applicability of these new approaches to the treatment of emphysema symptoms.

Animals↗

PubMed2003

The prognostic significance of intranodal isolated tumor cells and micrometastases in patients with non-small cell carcinoma of the lung.

OBJECTIVE: To study whether isolated tumor cells and micrometastases, as defined by the current American Joint Committee on Cancer criteria for extrapulmonary neoplasms, have prognostic value for patients with resected non-small cell carcinoma of the lung. METHODS: Intrathoracic lymph nodes (n = 1063) from 60 patients with non-small cell carcinoma of the lung were studied for the presence of metastases with serial histologic sections and keratin immunostains. Metastases were classified as isolated tumor cells, pN1mi, pN1, pN2mi, and pN2. Isolated tumor cells were smaller than 0.2 mm, while pN1mi and pN2mi measured 0.2 mm to 2 mm. Survival analysis was performed, stratifying by nodal status and stage. RESULTS: Isolated tumor cells were detected in 11 lymph nodes from 5 of 33 pN0 patients and in 9 pN1 and pN2 patients. The lymph nodes from 3 patients were reclassified as pN1mi. No pN2mi were detected. A survival model based on a stratification of the cohort into stages I to III was significant (chi-square = 7.426, df = 2, P =.024) but demonstrated considerable overlap between the survival curves of stage I and II patients. A model stratifying isolated tumor cells and pN1mi into stage I disease was significant (chi-square = 7.985, df = 2, P =.018) and showed no overlap between the survival curves of stage I and II patients. There were no significant survival function differences between patients with pN0, isolated tumor cells, and pN1mi. CONCLUSIONS: Patients with non-small cell carcinoma of the lung with isolated tumor cells and pN1mi have similar survivals to those with pN0, consistent with the findings reported for breast cancer patients. Future larger studies of patients with non-small cell carcinoma of the lung are needed to confirm whether current American Joint Committee on Cancer staging criteria should be modified to include the pN1mi category.

Adenocarcinoma↗

PubMed2003

Complications after lung volume reduction surgery.

LVRS has greater morbidity than most general thoracic surgical procedures. Proper care of patients after LVRS is a labor-intensive activity, but it is worthwhile because LVRS can be performed with acceptable risk. Patient selection, postoperative care, and an understanding of the potential complications are the keys to successful LVRS.

Humans↗

PubMed2003

Malignant monotypic epithelioid angiomyolipoma of the retroperitoneum.

Monotypic epithelioid angiomyolipoma (EAML) is an uncommon variant of angiomyolipoma, occurring primarily in the kidney and characterized by a predominance of HMB-45-positive epithelioid cells. EAML is generally considered a benign neoplasm, though rare cases exhibiting malignant behavior have been reported. We describe a patient with a primary retroperitoneal monotypic EAML, composed exclusively of atypical epithelioid cells, which metastasized to the liver and the mediastinum. This case represents, to our knowledge, the first description of metastatic disease occurring in an EAML of this particular site.

Adult↗