scholarly journals Pulmonary Benign Metastasizing Leiomyomas: a Case Series of 23 Patients at a Single Facility

2020 ◽  
Author(s):  
Rong Fan ◽  
Fengzhi Feng ◽  
Hua Yang ◽  
Kaifeng Xu ◽  
Shanqing Li ◽  
...  
Keyword(s):  
Single Case ◽  
Case Series ◽  
Progesterone Receptors ◽  
Premenopausal Women ◽  
Complete Response ◽  
Gynecologic Surgery ◽  
Curative Surgery ◽  
College Hospital ◽  
First Choice ◽  
S 100

Abstract Background: Pulmonary benign metastasizing leiomyoma (PBML) is a rare disease characterized by leiomyoma of benign histopathology existing in the lungs. Because of its rarity, limited literature with a single case or small number of cases has been regarding to the clinical course, pathology or management of PBML. Methods: A retrospective study was performed of all PBML cases diagnosed and managed at Peking Union Medical College Hospital (PUMCH) from 2001 to 2019. The clinical characteristics, pathology, treatment and outcomes of each case were studied. Results: There were 25 PBML patients identified in the 19-year period in PUMCH, and 23 patients’ data was analyzed. The median age at diagnosis was 46 years. There were 7 patients (30.4%) diagnosed with postmenopausal status. 2 patients (8.7%) had no uterine leiomyoma, and 3 patients (13.0%) had no gynecologic surgery history. Immunohistochemistry of most lesions demonstrated positive for desmin, SMA and Estrogen/Progesterone Receptors; and negative for S-100 were shown in 7 cases. After curative or diagnostic surgeries for the PBML, several treatments from observation to medical or surgical castration were performed. 9 premenopausal patients preserved their ovaries at first. At a median follow-up of 8 years, 3 patients finally had oophorectomy.Conclusions: PBML is a disease that not only be found in premenopausal women. Treatment should be individualized. Curative surgery for patients with limited lesions can achieve the complete response. For patients that are young and asymptomatic, close observation is recommended as the first choice. All patients should undergo long-term surveillance.

scholarly journals Pulmonary Benign Metastasizing Leiomyomas: a Case Series of 23 Patients at a Single Facility

2020 ◽  
Author(s):  
Rong Fan ◽  
Fengzhi Feng ◽  
Hua Yang ◽  
Kaifeng Xu ◽  
Shanqing Li ◽  
...  
Keyword(s):  
Single Case ◽  
Case Series ◽  
Progesterone Receptors ◽  
Premenopausal Women ◽  
Complete Response ◽  
Gynecologic Surgery ◽  
Curative Surgery ◽  
College Hospital ◽  
First Choice ◽  
S 100

Abstract Background: Pulmonary benign metastasizing leiomyoma (PBML) is a rare disease characterized by leiomyoma of benign histopathology existing in the lungs. Because of its rarity, limited literature with a single case or small number of cases has been regarding to the clinical course, pathology or management of PBML. Methods: A retrospective study was performed of all PBML cases diagnosed and managed at Peking Union Medical College Hospital (PUMCH) from 2001 to 2019. The clinical characteristics, pathology, treatment and outcomes of each case were studied. Results: There were 25 PBML patients identified in the 19-year period in PUMCH, and 23 patients’ data was analyzed. The median age at diagnosis was 46 years. There were 7 patients (30.4%) diagnosed with postmenopausal status. 2 patients (8.7%) had no uterine leiomyoma, and 3 patients (13.0%) had no gynecologic surgery history. Immunohistochemistry of most lesions demonstrated positive for desmin, SMA and Estrogen/Progesterone Receptors; and negative for S-100 were shown in 7 cases. After curative or diagnostic surgeries for the PBML, several treatments from observation to medical or surgical castration were performed. 9 premenopausal patients preserved their ovaries at first. At a median follow-up of 8 years, 3 patients finally had oophorectomy.Conclusions: PBML is a disease that not only be found in premenopausal women. Treatment should be individualized. Curative surgery for patients with limited lesions can achieve the complete response. For patients that are young and asymptomatic, close observation is recommended as the first choice. All patients should undergo long-term surveillance.

scholarly journals Pulmonary benign metastasizing leiomyomas: a case series of 23 patients at a single facility

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Rong Fan ◽  
Fengzhi Feng ◽  
Hua Yang ◽  
Kaifeng Xu ◽  
Shanqing Li ◽  
...  
Keyword(s):  
Rare Disease ◽  
Single Case ◽  
Case Series ◽  
Progesterone Receptors ◽  
Complete Response ◽  
Gynecologic Surgery ◽  
Curative Surgery ◽  
College Hospital ◽  
First Choice ◽  
S 100

Abstract Background Pulmonary benign metastasizing leiomyoma (PBML) is a rare disease characterized by leiomyoma of benign histopathology existing in the lungs. Because of its rarity, limited literature with a single case or small number of cases has been regarding to the clinical course, pathology or management of PBML. Methods A retrospective study was performed of all PBML cases diagnosed and managed at Peking Union Medical College Hospital (PUMCH) from 2001 to 2019. The clinical characteristics, pathology, treatment and outcomes of each case were studied. Results There were 25 PBML patients identified in the 19-year period in PUMCH, and 23 patients’ data was analyzed. The median age at diagnosis was 46 years. There were 7 patients (30.4%) diagnosed with postmenopausal status. Two patients (8.7%) had no uterine leiomyoma, and 3 patients (13.0%) had no gynecologic surgery history. Immunohistochemistry of most lesions demonstrated positive for desmin, SMA and Estrogen/Progesterone Receptors; and negative for S-100 were shown in 7 cases. After curative or diagnostic surgeries for the PBML, several treatments from observation to medical or surgical castration were performed. Nine premenopausal patients preserved their ovaries at first. At a median follow-up of 8 years, 3 patients finally had oophorectomy. Conclusions PBML is a rare disease and should be treated by individualization according to the patients’ age, symptoms and extent of lesion. Curative surgery for patients with limited lesions can achieve the complete response. For patients that are young and asymptomatic, close observation is recommended as the first choice. All patients should undergo long-term surveillance.

scholarly journals Pulmonary Benign Metastasizing Leiomyomas: a Case Series of 23 Patients at a Single Facility

2020 ◽  
Author(s):  
Rong Fan ◽  
Fengzhi Feng ◽  
Hua Yang ◽  
Kaifeng Xu ◽  
Shanqing Li ◽  
...  
Keyword(s):  
Rare Disease ◽  
Single Case ◽  
Case Series ◽  
Progesterone Receptors ◽  
Complete Response ◽  
Gynecologic Surgery ◽  
Curative Surgery ◽  
College Hospital ◽  
First Choice ◽  
S 100

Abstract Background: Pulmonary benign metastasizing leiomyoma (PBML) is a rare disease characterized by leiomyoma of benign histopathology existing in the lungs. Because of its rarity, limited literature with a single case or small number of cases has been regarding to the clinical course, pathology or management of PBML. Methods: A retrospective study was performed of all PBML cases diagnosed and managed at Peking Union Medical College Hospital (PUMCH) from 2001 to 2019. The clinical characteristics, pathology, treatment and outcomes of each case were studied. Results: There were 25 PBML patients identified in the 19-year period in PUMCH, and 23 patients’ data was analyzed. The median age at diagnosis was 46 years. There were 7 patients (30.4%) diagnosed with postmenopausal status. 2 patients (8.7%) had no uterine leiomyoma, and 3 patients (13.0%) had no gynecologic surgery history. Immunohistochemistry of most lesions demonstrated positive for desmin, SMA and Estrogen/Progesterone Receptors; and negative for S-100 were shown in 7 cases. After curative or diagnostic surgeries for the PBML, several treatments from observation to medical or surgical castration were performed. 9 premenopausal patients preserved their ovaries at first. At a median follow-up of 8 years, 3 patients finally had oophorectomy.Conclusions: PBML is a rare disease and should be treated by individualization according to the patients’ age, symptoms and extent of lesion. Curative surgery for patients with limited lesions can achieve the complete response. For patients that are young and asymptomatic, close observation is recommended as the first choice. All patients should undergo long-term surveillance.

scholarly journals Pulmonary Benign Metastasizing Leiomyomas: a Case Series of 23 Patients at a Single Facility

2020 ◽  
Author(s):  
Rong Fan ◽  
Fengzhi Feng ◽  
Hua Yang ◽  
Kaifeng Xu ◽  
Shanqing Li ◽  
...  
Keyword(s):  
Rare Disease ◽  
Single Case ◽  
Case Series ◽  
Progesterone Receptors ◽  
Complete Response ◽  
Gynecologic Surgery ◽  
Curative Surgery ◽  
College Hospital ◽  
First Choice ◽  
S 100

Abstract Background: Pulmonary benign metastasizing leiomyoma (PBML) is a rare disease characterized by leiomyoma of benign histopathology existing in the lungs. Because of its rarity, limited literature with a single case or small number of cases has been regarding to the clinical course, pathology or management of PBML.Methods: A retrospective study was performed of all PBML cases diagnosed and managed at Peking Union Medical College Hospital (PUMCH) from 2001 to 2019. The clinical characteristics, pathology, treatment and outcomes of each case were studied.Results: There were 25 PBML patients identified in the 19-year period in PUMCH, and 23 patients’ data was analyzed. The median age at diagnosis was 46 years. There were 7 patients (30.4%) diagnosed with postmenopausal status. 2 patients (8.7%) had no uterine leiomyoma, and 3 patients (13.0%) had no gynecologic surgery history. Immunohistochemistry of most lesions demonstrated positive for desmin, SMA and Estrogen/Progesterone Receptors; and negative for S-100 were shown in 7 cases. After curative or diagnostic surgeries for the PBML, several treatments from observation to medical or surgical castration were performed. 9 premenopausal patients preserved their ovaries at first. At a median follow-up of 8 years, 3 patients finally had oophorectomy.Conclusions: PBML is a rare disease and should be treated by individualization according to the patients’ age, symptoms and extent of lesion. Curative surgery for patients with limited lesions can achieve the complete response. For patients that are young and asymptomatic, close observation is recommended as the first choice. All patients should undergo long-term surveillance.

scholarly journals Primary Melanoma of the Lung: A Systematic Review

Medicina ◽  
2020 ◽  
Vol 56 (11) ◽  
pp. 576
Author(s):  
Panagiotis Paliogiannis ◽  
Antonella M. Fara ◽  
Gianfranco Pintus ◽  
Wael M. Abdel-Rahman ◽  
Maria Colombino ◽  
...  
Keyword(s):  
Malignant Melanoma ◽  
Single Case ◽  
Left Lung ◽  
Case Series ◽  
Primary Melanoma ◽  
Primary Malignant Melanoma ◽  
S 100 ◽  
Main Determinants ◽  
Mitotic Figures ◽  
Rare Malignancy

Background and Objectives: The respiratory apparatus, generally affected by highly aggressive tumors like lung cancer and mesothelioma, is rarely affected by primary malignant melanoma. The aim of this review was to identify cases of primary malignant melanoma of the lung (PMML) published in the modern scientific literature, and to describe their main clinical, pathological and therapeutic features. Materials and Methods: A systematic search of publications in the electronic database PubMed has been performed using keywords, and the references of the selected articles were checked to identify additional missing studies. Results: Globally 52 papers reporting on 76 cases were identified. Among them there were 47 reports of a single case, three papers reporting on two cases each, and two larger case series published in 1997 and 2005 including eight and 15 cases, respectively. Conclusions: PMML was generally diagnosed in middle-aged males, without any apparent correlation with cigarette smoking. It was more frequently found in the lower lobes and the left lung. The tumors were generally pigmented, composed by epithelial and/or spindle cells with large nuclei and prominent nucleoli, nuclear atypia, and numerous mitotic figures; they commonly showed immunostaining for S-100, HMB 45 and Melan-A. Early detection and surgical resection were the main determinants of survival from this rare malignancy.

scholarly journals Peripartum hysterectomy: a five year review at a tertiary care centre

2017 ◽  
Vol 6 (8) ◽  
pp. 3590
Author(s):  
Deepak A. V. ◽  
K. J. Jacob ◽  
Sumi P. Maria
Keyword(s):  
Tertiary Care ◽  
Case Series ◽  
Case Fatality ◽  
Tertiary Care Centre ◽  
Retrospective Case ◽  
College Hospital ◽  
Peripartum Hysterectomy ◽  
Medical College ◽  
Obstetric Haemorrhage ◽  
Suboptimal Care

Background: Peripartum hysterectomy is a life-saving procedure resorted to when conservative measures fail to control obstetric haemorrhage. Several predisposing factors, suboptimal care and lack of infrastructure may lead to this emergency procedure. We wanted to find out factors associated with peripartum hysterectomy and the adverse maternal outcomes at our centre.Methods: A retrospective case series analysis of 40 cases of peripartum hysterectomy performed over a period of 5 years from January 2010 to December 2014 at Government Medical College Hospital, Thrissur, Kerala was done.Results: The incidence of peripartum hysterectomy was 0.29%. The most common indication for peripartum hysterectomy was hysterectomy was uterine atony (50%). Thirty-five women (88%) were between 20 and 35 years. Most of the subjects were unbooked. There were two maternal deaths (case fatality rate of 5%) following peripartum hysterectomy during this period. All the subjects required blood transfusion.Conclusions: Prompt performance hysterectomy before the patient’s clinical condition deteriorates is the key to success. The incidence of adherent placenta is increasing, so every effort should be taken to reduce the caesarean section rates globally. 

scholarly journals Post-Infectious Guillain–Barré Syndrome Related to SARS-CoV-2 Infection: A Systematic Review

Life ◽  
2021 ◽  
Vol 11 (2) ◽  
pp. 167
Author(s):  
Pasquale Sansone ◽  
Luca Gregorio Giaccari ◽  
Caterina Aurilio ◽  
Francesco Coppolino ◽  
Valentina Esposito ◽  
...  
Keyword(s):  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Guillain Barre Syndrome ◽  
Response To Treatment ◽  
Comprehensive Overview ◽  
Fisher Syndrome ◽  
Immunomodulating Therapy ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

Background. Guillain-Barré syndrome (GBS) is the most common cause of flaccid paralysis, with about 100,000 people developing the disorder every year worldwide. Recently, the incidence of GBS has increased during the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) epidemics. We reviewed the literature to give a comprehensive overview of the demographic characteristics, clinical features, diagnostic investigations, and outcome of SARS-CoV-2-related GBS patients. Methods. Embase, MEDLINE, Google Scholar, and Cochrane Central Trials Register were systematically searched on 24 September 2020 for studies reporting on GBS secondary to COVID-19. Results. We identified 63 articles; we included 32 studies in our review. A total of 41 GBS cases with a confirmed or probable COVID-19 infection were reported: 26 of them were single case reports and 6 case series. Published studies on SARS-CoV-2-related GBS typically report a classic sensorimotor type of GBS often with a demyelinating electrophysiological subtype. Miller Fisher syndrome was reported in a quarter of the cases. In 78.1% of the cases, the response to immunomodulating therapy is favourable. The disease course is frequently severe and about one-third of the patients with SARS-CoV-2-associated GBS requires mechanical ventilation and Intensive Care Unit (ICU) admission. Rarely the outcome is poor or even fatal (10.8% of the cases). Conclusion. Clinical presentation, course, response to treatment, and outcome are similar in SARS-CoV-2-associated GBS and GBS due to other triggers.

Analysis of Clinical, Imaging, and Pathologic Features of 36 Patients with Primary Intraspinal Primitive Neuroectodermal Tumors: A Case Series and Literature Review

2021 ◽  
Author(s):  
Xuefeng Wei ◽  
Xu Zhang ◽  
Zimu Song ◽  
Feng Wang
Keyword(s):  
Literature Review ◽  
Single Case ◽  
Case Reports ◽  
Case Series ◽  
Subtotal Resection ◽  
Study Cohort ◽  
Primitive Neuroectodermal Tumors ◽  
Pathologic Features ◽  
Neuroectodermal Tumors ◽  
The Mean

Abstract Background and Study Aims Primary intraspinal primitive neuroectodermal tumors (PNETs) account for ∼0.4% of all intraspinal tumors, but information about these tumors in the medical literature is limited to single case reports. We report four cases of primary intraspinal PNETs and present a systematic literature review of the reported cases. Materials and Methods We retrospectively reviewed and analyzed the clinical data of 4 patients with primary intraspinal PNETs who underwent neurosurgical treatment at our clinic between January 2013 and January 2020, and of 32 cases reported in the literature. Results The female-to-male ratio was 2.6:1. The mean patient age was 21.42 ± 15.76 years (range: 1–60 years), and patients <36 years of age accounted for 83.30% of the study cohort. Progressive limb weakness and numbness were the chief symptoms (accounting for ∼55.6%). The mean complaint duration was 0.89 ± 0.66 months for males and 2.72 ± 3.82 months for females (p = 0.028). Epidural (41.7%) was the most common site, and thoracic (47.3%) was the most frequent location. Most PNETs were peripheral, and magnetic resonance imaging (MRI) appearance was isointense or mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous contrast enhancement was observed. The 1-year survival rate of patients who underwent chemoradiation after total or subtotal lesion resection was better compared with patients who did not undergo chemotherapy, radiotherapy, or total or subtotal resection. The modality of treatment was associated with survival time (p = 0.007). Conclusion Primary intraspinal PNETs mainly occur in young people with a female preponderance. In patients with a rapid loss of lower limb muscle strength and large intraspinal lesions on MRI, PNETs should be considered. Surgical resection and adjuvant radio chemotherapy are key prognostic factors.

scholarly journals Characterization of the clinical and genetic spectrum of autoimmune polyendocrine syndrome type 1 in Chinese case series

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Ya-Bing Wang ◽  
Ou Wang ◽  
Min Nie ◽  
Yan Jiang ◽  
Mei Li ◽  
...  
Keyword(s):  
Case Series ◽  
Pure Red Cell Aplasia ◽  
Hereditary Disease ◽  
Chinese Patients ◽  
Chronic Mucocutaneous Candidiasis ◽  
Syndrome Type ◽  
Myeloproliferative Disease ◽  
College Hospital ◽  
Autoimmune Polyendocrine Syndrome

Abstract Background Autoimmune polyendocrine syndrome type 1 (APS1) is a hereditary disease caused by mutations in the AIRE gene with both endocrine and non-endocrine organ involvement. The existing data from China are limited, and this study aims to describe the phenotypes and genetic characterization in Chinese APS1 patients. In this single-center, retrospective, observational study, comprehensive endocrine and extra-endocrine manifestations were collected, and genetic analysis in AIRE was conducted in patients with APS1 between the years of 1984 and 2018 at Peking Union Medical College Hospital. Results In total, 13 patients from 12 unrelated families were enrolled, seven of whom were female, with hypoparathyroidism, chronic mucocutaneous candidiasis, and Addison’s disease being the most frequently observed manifestations. Up to 84.7% presented with two or three of the above-mentioned manifestations, and nearly 4.9 ± 1.8 components presented in patients aged 21.2 ± 7.9 years old. Several less common phenotypes, such as myeloproliferative disease, pure red cell aplasia, renal tubular acidosis, asplenia, autoimmune hepatitis, and ankylosing spondylitis, were also observed in patients. Altogether, seven different AIRE mutations were found in six patients, four of which (K161fs, G208V, A246fs, and L308F) had not been previously reported in patients with APS1. Conclusion We have provided a comprehensive profile of Chinese patients with APS1, with less commonly observed features being observed in addition to more regularly seen manifestations. Additionally, different AIRE mutations that were observed have expanded the genetic spectrum, which will help with future understanding of the molecular pathogenesis of APS1.

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