Pulmonary Benign Metastasizing Leiomyoma Research Articles

A Case Report on Pulmonary Benign Metastasizing Leiomyoma with Recurrent Uterine Fibroids

Pulmonary benign metastasizing leiomyoma (PBML) is a type of benign metastasizing leiomyoma (BML), which metastasizes to lungs. In view of the rarity and nonspecific imaging characteristics of PBML, this condition is easily misdiagnosed as malignant lung cancer. This is a case report of a 39-year-old lady in whom multiple bilateral lung nodules were found during a health checkup and was diagnosed with PBML. The patient had recurrent uterine fibroids and underwent myomectomy twice, 7 years and 3 years ago, respectively, before the diagnosis of PBML was made. This patient was asymptomatic, and regular examinations were normal. Her laboratory investigations were all within normal range. Computed tomography (CT) showed multiple nodules in bilateral lungs. In order to confirm the disease, thoracoscopic nodule resection and tissue biopsy were performed. Hematoxylin eosin (HE) staining showed spindle-shaped cells, while immunohistochemical staining revealed positive for desmin, smooth muscle actin (SMA), estrogen receptor (ER), and progesterone receptor (PR), which are all PBML markers. The patient was followed-up without any further treatment. After 8 months of follow-up, CT scan revealed an increased number of nodules. This is unique case because of the recurrence of uterine fibroids, which means that the patient is susceptible to it. Our report may give a new insight to the relationship between the susceptibility of uterine fibroids and the onset and progression of PBML.

Pulmonary Benign Metastasizing Leiomyoma: Case Report of Late Occurrence in Postmenopausal Patient

Introduction: Pulmonary benign metastasizing leiomyoma (PBML) is a rare condition that occurs most commonly in young women of reproductive age some years after hysterectomy. The aim of this study was to report a PBML case which occurred 33 years after hysterectomy in a postmenopausal patient. Case report: A 59-year-old female diabetic patient, G2P2A0, with dyslipidemia, a non-smoker and ex-alcoholic (quit in the 90s), with pulmonary symptoms was referred to the health service. A chest computed tomography (CT) revealed multiple pulmonary nodules. A previous history of hysterectomy at 26 years of age led to a diagnostic hypothesis of PBML. The patient underwent segmentectomy of the largest nodule (measuring 1.2 x 0.9 cm) located in the left lung. Histopathology and immunohistochemistry studies confirmed the diagnosis of PBML. Conclusion: The patient is currently in good physical condition (regression of previous pulmonary symptoms) and scheduled for clinical follow-up. A new chest CT will be performed in 6 months.

Intravenous leiomyomatosis: Case series and review of the literature.

IntroductionIntravenous leiomyomatosis (ILV) is a rare pathology, part of leiomyoma beyond the uterus (LBU), characterized by benign smooth muscle cell tumor outside of the uterus and mainly affecting premenopausal woman with a medical history of leiomyoma or gynecologic surgical treatment. The treatment depends on the localization of the tumor, age of the patient, initial size, symptoms and the suitability for surgery but should always aims in toto surgical resection.Case presentationRetrospective case series and review of literature.Clinical discussionSymptoms presented by the patient were aspecific and only localized in the pelvic area. All cases were fortuitous histopathological diagnosis. No relapse was. Two out of 5 patients have pulmonary nodules, only one was biopsied and diagnosed with PBML (pulmonary benign metastasizing leiomyoma).ConclusionIVL and BML are rare disease that can co-exist. Because of tumoral hormonal receptors, hormonotherapy could be an optional treatment but to date no clear efficacy is demonstrated. In case of high recurrence risk such as voluminous initial mass, impairment of broad ligament, failure of total surgical resection, adjuvant hormonotherapy could be useful. Recurrence rate is about 16.6-30% and can occur even dozen years later and even after radical surgery, justifying a regular follow up.

The role of miRNA-221 and miRNA-126 in patients with benign metastasizing leiomyoma of the lung: an overview with new interesting scenarios

Benign metastasizing leiomyoma (BML) is a rare disease characterized by extrauterine benign leiomyomatosis in patients with a previous or concomitant history of uterine leiomyoma. Currently, there are no specific criteria to predict the metastasizing ability of the uterine leiomyoma and the risk of malignant degeneration of pulmonary BML, and these are the aims of this study. We analyzed 10 uterine (three leiomyomas, four leiomyomas that gave rise to lung BML, three healthy tissues) and 11 pulmonary tissue samples (eight lung BML, three healthy tissues). Interestingly, one of the BML lesions exceptionally evolved into a leiomyosarcoma (case 2). Uterine leiomyoma microvascular density (MVD) was higher in the patients with uterine leiomyomas that gave rise to lung BML, reaching a peak in case 2. Strong positivity for the estrogen (ER) and progesterone (PR) receptors and a low proliferation index (Ki67 < 1%) were discovered both in patients with uterine leiomyoma and in patients with BML. Interestingly, in case 2, the last dedifferentiated leiomyosarcoma showed a weaker ER and PR positivity with a higher proliferation index (Ki67:30%). Regarding the uterine miRNA-126, a trend toward a hypo-expression between uterine leiomyoma and uterine leiomyoma that gave rise to lung BML was discovered, reaching the lowest level in case 2. Considering the pulmonary samples, we observed a higher miRNA-221 and a lower miRNA-126 expression in the leiomyosarcoma. We tried to better elucidate the biological behaviour of this rare disease. The analysis of the miRNA-221 and miRNA-126 could offer new diagnostic, prognostic and therapeutic perspectives.

Pulmonary benign metastasizing leiomyoma.

Pulmonary benign metastasizing leiomyoma (PBML) is a rare hormone dependent disease that refers to benign hysteromyoma metastasizing to the lung. Lung biopsy is indispensable for the diagnosis for PBML. Therapeutic options include surgical removing of the tumor, hormonal therapy (hysterectomy, bilateral adnexectomy, and long term oral drugs such tamoxifene, raloxifene, and aromatase inhibitors), and inferior vena cava filter implantation. A 57 years old postmenopausal woman underwent total hysterectomy for uterine leiomyoma. Eight years later, she presented with a chronic cough and dyspnea. A thoracic computed tomography scan showed a solitary proximal nodule of 4 cm on the right lung (A). Histological examination of biopsies revealed a benign leiomyoma of the lung. As the surgery would be mutilating, a regular follow up was recommended but she lost sight of. The patient was lost of view for one year before she appears with increased cough, dyspnea and chest pain. A new CT-scan showed a significant increase in size of the initial tumor of about 4 cm with compression of the right bronchus (B). Subsequently, biopsies have been performed as a leiomyosarcomatous transformation has been suspected. Histopathological analysis disclosed the diagnosis of a benign metastasizing leiomyoma of the lung. The patient was given the aromatase inhibitor (Letrozole 2.5 mg). This treatment yielded a disease progression after six months. Second line Tamoxifene was then started. The disease is still stable for 19 months now and the patient will continue to be followed.

A case of pulmonary benign metastasizing leiomyoma: clinicopathologic, immunohistochemical, and PET/CT analyses

The pulmonary benign metastasizing leiomyoma (BML) is an extremely rare disease case, of which the pathogenesis was not elucidated. The accumulation of additional case reports and analyses is needed. A case of pulmonary BML is presented. A 48-year-old asymptomatic female patient that underwent a hysteromyomectomy 11 years ago was found to have abnormal shadows on a chest X-ray during a regular health check-up in 2012. Multiple nodular was observed in both lungs and uterine under computed tomography (CT) examination. Transbronchial lung biopsy surgery with a resection of the lesion was performed. The results revealed the pulmonary nodule was formed by benign smooth muscle cells which displayed very low mitotic activity. The samples showed highly positive signals for α-smooth muscle actin (αSMA), Desmin and Vimentin. Estrogen receptor (ER) and progesterone receptor (PR) were also immunoreactive positive. HMB-45, CD117 (C-kit) and S-100 were found to be negative with low Ki-67 index in the tumor cells. The pathological diagnosis was pulmonary BML. A total hysterectomy with adnexectomy were performed for the multiple uterine leiomyomas and pulmonary BML one week after diagnosis was made. From then on, the pulmonary nodules in both lungs were stable in 7 years postoperation, based on chest CT scan. When a solitary lung nodule is detected in a patient with a history of uterine leiomyoma, as an option, pulmonary BML, accurately diagnosing and proper treatment to has important implications for clinical outcome.

Benign metastasizing leiomyoma and 18-FDG-PET/CT: A case report and literature review.

Pulmonary benign metastasizing leiomyoma (PBML) is a rare disease entity that usually occurs in females of reproductive age with a previous history of uterine myoma. It is typically characterized by multiple pulmonary tumors consisting of benign leiomyoma cells. In the present study, two cases of PBML are discussed. The patient in each case underwent 2-deoxy-2-(fluorine-18)-fluoro-D-glucose positron emission tomography/computed tomography (18-FDG-PET/CT) scans. One patient demonstrated a lack of 18-FDG uptake and a quiescent clinical course. However, the second patient exhibited a markedly high uptake of 18-FDG and aggressive cell proliferation. The two tumors revealed significant differences in metabolic behavior and in clinical course; however, they were similar with regard to cellular appearance. A review of previous studies concerning the findings of 18-FDG-PET/CT in published cases of PBML was also conducted and is presented here.

The clinical characteristics of pulmonary benign metastasizing leiomyoma

Objective: Pulmonary benign metastasizing leiomyoma (PBML) is a rare entity that leiomyoma of uterus metastasized to the lung. The clinical characteristics of this rare disease were analyzed in this article. Methods: The detailed clinical records of 7 patients diagnosed as PBML at Peking Union Medical College Hospital between January 2001 and June 2015 were reviewed. Results: All patients were women with median age of 44 years (range 28-62). Symptoms included dyspnea (2/7), chest pain (1/7), cyanosis (1/7), cough (1/7) and bloody sputum (1/7), while 4/7 cases were asymptomatic. Six patients had the past-history of leiomyoma of uterus 20 months to 14 years ago among whom 5 patients received hysterectomy. Chest CT showed bilateral, random-distributed multiple round solid nodules, or diffuse-distributed miliary nodules, or single solid nodule, even some small cavities. Extra-pulmonary metastasis was found in left superclavicular lymph node (1 case) and right heart (1 case). Histological tissues were obtained by video-assisted thoracic surgery lung biopsy (4/7), mass resection on tricuspid valve (1/7), transbronchil lung biopsy (1/7), and CT-guided percutaneous lung biopsy (1/7). Pathology showed an interlacing pattern by spindle cells having elongated nuclei without cellular atypia. Ki-67 index was less than 1%. Molecules such as smooth muscle antibody, estrogen receptor (ER) and progestrone receptor (PR) were positive in immunohistochemistry staining. Neither letrozole nor zoladex was effective. Two patients responded to bilateral adnexectomy, presenting as shrunk nodules. No relapsed disease was seen in one patient with single nodule after resection. There was only one patient with disease-related mortality, whose chest CT showed milliary nodules. Conclusion: Although CT findings of PBML are similar to malignancies, the clinical outcome is good. Despite the positive expression of ER and PR, the effectiveness of hormone related treatment is limited. And periodical follow up is suggested even to those uneventful patients.

Pulmonary Cyst of Uterine Genesis

SESSION TITLE: Fellow Case Report Poster - Imaging SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Benign metastasizing leiomyoma (BML) is a rare disease characterized by well differentiated smooth musle cells of uterine myomatous origin that are present in sites distant from the uterus, most commonly in the lung. Pulmonary BML typically presents as solid discrete nodules of tumor within lung parenchyma in an asymptomatic patient. CASE PRESENTATION: A 65 year old woman presented with episodes of marked diaphoresis for one year. She had history of a hysterectomy for uterine fibroids 17 years prior. Her medical evaluation was unremarkable save for a chest radiograph which identified a left lower lobe mass. Computed tomography of the chest demonstrated a 4.9 cm low density mass along the left major fissure, consistent with a bronchogenic cyst (Figure 1A-B). Also noted were multiple small nodules, 3-6mm in size. The cyst was resected; its cytology was benign and fluid bacterial culture had no growth. The wall of the cyst demonstrated smooth muscle cells staining positive for actin, desmin, as well as estrogen and progesterone receptors, identical to histopathology from her prior hysterectomy. The cyst was also lined by a single layer of cuboidal epithelial cells staining positive for TTF-1 (Figure 2A-F). After cyst removal, the patient reported complete resolution of her symptoms. DISCUSSION: BML lesions are thought to arise as hematogenous metastases from benign tumors, with leiomyomatous smooth muscle cells entering the blood stream during surgical resection. The most well described radiographic presentation is multiple pulmonary nodules with a distribution typical of metastatic lesions. In very rare cases, BML has been reported to present with multiple thin walled pulmonary cysts, invoking the radiographic differential of diffuse cystic lung disease. In these cases,cyst formation is theorized to be due to pulmonary elements entrapped in tumor cells. We believe this case is the first described radiographic presentation of BML consistent with a predominant large fluid filled bronchogenic cyst. While the vast majority of BML nodules are found incidentally in aysmptomatic patients, in this case, the serous filled cyst was associated with pronounced diaphoresis that resolved with cyst resection. CONCLUSIONS: The radiographic differential for localized cystic pulmonary disease should include the rare finding of BML in patients with a history of uterine leiomyomata. Reference #1: Aboualfa K, et al. Benign metastasizing leiomyoma presenting as cystic lung disease: a diagnostic pitfall. Histopathology.2001;59(4):796-799. DISCLOSURE: The following authors have nothing to disclose: Anna Moniodis, Douglas Lin, Paul VanderLaan, Majd Mouded No Product/Research Disclosure Information

Benign metastasizing leiomyoma of the lung: A case report and literature review.

Pulmonary benign metastasizing leiomyoma (BML) is a rare event characterized by benign soft-tissue tumors that occur when uterine leiomyomas metastasize to the lung. The present study reports the case of a 47-year-old female patient who presented with multiple bilateral pulmonary nodules on a chest X-ray during a health checkup nine years after a hysterectomy due to uterine fibroids. Chest computed tomography (CT) revealed multiple well-defined nodular shadows in the lung. One tumor of the left upper lung was resected by thoracoscopic surgery. Pathologically, the resected lesion consisted of benign spindle cells and was diagnosed as BML. The post-operative course was uneventful. Other lung nodules have been meticulously monitored at follow-up, and repeat CT two years later showed that these nodules had not increased at all in size and that no new lobe nodules had appeared. The present study indicates that pulmonary BML occurs in a low proportion of female with a history of uterine leiomyoma and treatment methods for it are diverse and controversial.

Benign pulmonary metastasizing leiomyoma of the uterus: A case report

Pulmonary benign metastasizing leiomyoma (BML), is characterized by multiple pulmonary nodular lesions and is a rare disease. The present study reports the case of a 45-year-old asymptomatic woman who underwent an excision of uterine leiomyoma 11 years previously. Chest computed tomography (CT) revealed multiple bilateral pulmonary nodules five months prior to admission, during a regular check-up. Intravenous levofloxacin (0.5 g/day) was administered for one week, which demonstrated no effect. Positron emission tomography combined with CT (PET/CT) revealed no evident radioactivity concentration. Due to the suspicion of metastasizing leiomyoma, video-assisted thoracoscopic surgery, with a wedge resection of the right pulmonary lesion, was performed. Post-operative pathological examination revealed the lesion to be a pulmonary leiomyoma accompanied by local necrosis. Immunohistochemical staining revealed that the lesion was positive for the expression of smooth muscle actin, desmin, estrogen receptor, progesterone receptor and B-cell lymphoma-2. Cytokeratin and epithelial membrane antigen were not expressed in the tumor cells. Staining for Ki-67 revealed expression of Ki-67 in ~1% of the spindle cells. The overall morphological and immunohistochemical features, accompanied by the remote patient history of primary uterine leiomyoma, supported the diagnosis of pulmonary BML.

Difficulty in complete transarterial embolization for pulmonary benign metastasizing leiomyoma with massive hemoptysis

A 43-year-old woman suffering from massive hemoptysis from pulmonary benign metastasizing leiomyoma (BML) at the left lung hilum underwent bronchial and nonbronchial transarterial embolization (TAE) using gelatin sponge particles and n-butyl 2-cyanoacrylate during three interventional procedures. However, since complete embolization of the tumor was difficult despite decreased tumor size, the tumor was surgically resected 3 months after the last interventional procedure. This case demonstrates the difficulty of complete TAE for pulmonary BML because of its hypervascularity and the rich communications between bronchial and nonbronchial anastomotic arteries.

Benign metastasizing leiomyoma: ‘a sheep in wolf’s clothing’

Benign metastasizing leiomyoma (BML) is a rare entity, of which a small number of cases, about 75 to 120, have been described in the literature since the condition was first described in 1939. It usually affects women of reproductive age with a history of uterine leiomyomatosis who have undergone a hysterectomy. The lung is the most common site of involvement. The pathologic features of this entity are described as having originated from the metastasis of the histologically benign uterine leiomyoma and are an uncommon lesion characterized by the presence of multiple smooth-muscle nodules that are often located in the lung, lymph nodes, or abdomen. Although these lesions are slow growing and asymptomatic and are usually incidentally diagnosed, they may rarely cause debilitating symptoms. Optimal treatment is controversial, but careful follow-up of these patients is recommended because the lesions show a lowgrade clinical malignant behavior although their appearance is benign. We describe here a rare case of pulmonary BML.

Pulmonary and retroperitoneal benign metastasizing leiomyoma

Benign metastasizing leiomyoma (BML) is a rare disease, which usually occurs in women with a history of a prior hysterectomy or myomectomy for benign uterine leiomyoma, and has the potential to metastasize to distant sites, such as the lung, lymph nodes, muscular tissue, heart, or retroperitoneum. These lesions are slow-growing, asymptomatic, and usually found incidentally. The prognosis of BML is also excellent. However, there has been debate on the origin and the correct classification of BML, and there are no guidelines for the treatment of BML. We report here on a rare case of BML in both the retroperitoneal cavity and lung in a 48-year-old woman with a history of hysterectomy due to histologically benign uterine leiomyoma. The patient underwent retroperitoneal mass excision and bilateral salpingo-oophorectomy, and then wedge biopsy of two pulmonary nodules was performed additionally 9 days later. Until now, there has been no sign of recurrence and the patient remains asymptomatic. To our knowledge, pulmonary BML is rare and the co-existence of the retroperitoneal metastases after previous hysterectomy is even rarer.