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F1000Research 2020Primary malignant pericardial mesothelioma (PMPM) is a rare cardiac tumor. The patient usually presents with pericardial constriction, usually misdiagnosed and wrongly...
Primary malignant pericardial mesothelioma (PMPM) is a rare cardiac tumor. The patient usually presents with pericardial constriction, usually misdiagnosed and wrongly managed. We present the case of a 21-year-old woman with a history of pericarditis and cardiac tamponade. The patient was referred from a clinic due to nausea, vomiting and ascites with lower extremity edema, soft and watery diarrhea, and right upper quadrant pain. Surgery (sternotomy and partial pericardiectomy) was proposed after looking at the different relevant investigations; it was not until that the patient was operated on that it was established that this wasn't a mere constriction but a malignancy. The patient shortly died after the operation. Pathology made a diagnosis of PMPM. Along with the classical symptoms those who present with level 1 thoracic adenopathy a decision to operate should be very carefully made, this may lead to a misdiagnosis of PMPM which postoperatively results in patient's death.
PubMed: 35186278
DOI: 10.12688/f1000research.23998.1 -
Cancers May 2020Mesothelioma is a malignancy of serosal membranes including the peritoneum, pleura, pericardium and the tunica vaginalis of the testes. Malignant mesothelioma (MM) is a... (Review)
Review
Mesothelioma is a malignancy of serosal membranes including the peritoneum, pleura, pericardium and the tunica vaginalis of the testes. Malignant mesothelioma (MM) is a rare disease with a global incidence in countries like Italy of about 1.15 per 100,000 inhabitants. Malignant Pleural Mesothelioma (MPM) is the most common form of mesothelioma, accounting for approximately 80% of disease. Although rare in the global population, mesothelioma is linked to industrial pollutants and mineral fiber exposure, with approximately 80% of cases linked to asbestos. Due to the persistent asbestos exposure in many countries, a worldwide progressive increase in MPM incidence is expected for the current and coming years. The tumor grows in a loco-regional pattern, spreading from the parietal to the visceral pleura and invading the surrounding structures that induce the clinical picture of pleural effusion, pain and dyspnea. Distant spreading and metastasis are rarely observed, and most patients die from the burden of the primary tumor. Currently, there are no effective treatments for MPM, and the prognosis is invariably poor. Some studies average the prognosis to be roughly one-year after diagnosis. The uniquely poor mutational landscape which characterizes MPM appears to derive from a selective pressure operated by the environment; thus, inflammation and immune response emerge as key players in driving MPM progression and represent promising therapeutic targets. Here we recapitulate current knowledge on MPM with focus on the emerging network between genetic asset and inflammatory microenvironment which characterize the disease as amenable target for novel therapeutic approaches.
PubMed: 32392897
DOI: 10.3390/cancers12051186 -
European Heart Journal. Case Reports Apr 2020Primary pericardial mesothelioma is a rare primary cardiac malignancy, with three main histopathological types, sarcomatoid histotype being the rarest. The imaging...
BACKGROUND
Primary pericardial mesothelioma is a rare primary cardiac malignancy, with three main histopathological types, sarcomatoid histotype being the rarest. The imaging features were atypical due to concomitant extensive calcification, which resulted in aggravated differential diagnosis.
CASE SUMMARY
A 45-year-old man presented to our hospital with non-specific clinical symptoms. According to clinical history, a mediastinal tumour had been suspected with a previous unsuccessful attempt of transthoracic computed tomography-guided biopsy at an overseas hospital with limited data of performed imaging procedures. Multimodality imaging at our centre revealed extensively calcified solid masses in the pericardium, invading the left atrium. As the imaging features suggested an atypical primary pericardial malignancy, a diagnostic thoracoscopy was performed. Histopathological analysis of specimen revealed sarcomatoid type of pericardial mesothelioma with areas of necrosis and foci of osteogenic differentiation. Despite planned treatment, 2 weeks after histological diagnosis, the patient passed away due to perforated peptic ulcer-related sepsis.
DISCUSSION
The presence of extensive calcification in the lesion resulted in a challenging imaging workup and diagnosis. Initial differential diagnosis included primary or metastatic calcification-prone tumour, secondary calcification due to haemorrhage after previous interventional procedure and other pathologies, such as tuberculous pericarditis, calcified amorphous tumour, among others. Calcification may be part of the histological tumour characteristics; however, proper history taking is crucial as concomitant diseases, previous treatment, and interventional procedures may alter the imaging pattern.
PubMed: 32352059
DOI: 10.1093/ehjcr/ytaa034 -
Scandinavian Journal of Work,... Nov 2020Objectives The purposes of this study are to describe the epidemiology of pericardial and tunica vaginalis testis mesothelioma and assess the role of asbestos exposure...
Objectives The purposes of this study are to describe the epidemiology of pericardial and tunica vaginalis testis mesothelioma and assess the role of asbestos exposure for these rare diseases. Methods Based on incident pericardial and tunica vaginalis testis mesothelioma cases collected from the Italian national mesothelioma registry (ReNaM) in the period 1993-2015, incidence rates, survival median period and prognostic factors have been evaluated. A case-control study has been performed to analyze the association with asbestos exposure (occupational and non-occupational) for these diseases. Results Between 1993 and 2015, 58 pericardial (20 women and 38 men) and 80 tunica vaginalis testis mesothelioma cases have been registered with a mean annual standardized (world standard population as reference) incidence rates of 0.049 (per million) in men and 0.023 in women for the pericardial site, and 0.095 for tunica vaginalis testis mesothelioma. Occupational exposure to asbestos was significantly associated with the risk of the diseases [odds ratio (OR) 3.68, 95% confidence interval (CI) 1.85-7.31 and OR 3.42, 95% CI 1.93-6.04 in pericardial and tunica vaginalis testis mesothelioma, respectively]. The median survival was 2.5 months for pericardial and 33.0 months for tunica vaginalis testis mesotheliomas. Age was the main predictive factor for survival for both anatomical sites. Conclusions For the first time in an analytical study, asbestos exposure was associated with pericardial and tunica vaginalis testis mesothelioma risk, supporting the causal role of asbestos for all anatomical sites. The extreme rarity of the diseases, the poor survival and the prognostic role of age have been confirmed based on population and nationwide mesothelioma registry data.
Topics: Adolescent; Adult; Aged; Asbestos; Case-Control Studies; Child; Child, Preschool; Female; Humans; Incidence; Infant; Infant, Newborn; Italy; Male; Mesothelioma, Malignant; Middle Aged; Occupational Diseases; Occupational Exposure; Pericardium; Registries; Testicular Neoplasms; Young Adult
PubMed: 32253443
DOI: 10.5271/sjweh.3895 -
American Journal of Industrial Medicine Jun 2020Asbestos is the primary known cause of malignant mesothelioma. Some cosmetic talc products have been shown to contain asbestos. Recently, repeated exposures to cosmetic...
BACKGROUND
Asbestos is the primary known cause of malignant mesothelioma. Some cosmetic talc products have been shown to contain asbestos. Recently, repeated exposures to cosmetic talc have been implicated as a cause of mesothelioma.
METHODS
Seventy-five individuals (64 females; 11 males) with malignant mesothelioma, whose only known exposure to asbestos was repeated exposures to cosmetic talcum powders, were reviewed in medical-legal consultation. Out of the 75 cases, 11 were examined for asbestiform fibers.
RESULTS
All subjects had pathologically confirmed malignant mesothelioma. The mean age at diagnosis was 61 ± 17 years. The mean latency from exposure to diagnosis was 50 ± 13 years. The mean exposure duration was 33 ± 16 years. Four mesotheliomas (5%) occurred in individuals working as barbers/cosmetologists, or in a family member who swept the barber shop. Twelve (16%) occurred in individuals less than 45 years old (10 females; 2 males). Forty-eight mesotheliomas were pleural (40 females; 8 males), 23 were peritoneal (21 females; 2 males). Two presented with concomitant pleural and peritoneal disease. There was one pericardial, and one testicular mesothelioma. The majority (51) were of the epithelioid histological subtype, followed by 13 biphasic, 8 sarcomatoid, 2 lymphohistiocytoid, and 1 poorly differentiated. Of the 11 individuals whose nontumorous tissues were analyzed for the presence of asbestiform fibers, all showed the presence of anthophyllite and/or tremolite asbestos.
CONCLUSIONS
Mesotheliomas can develop following exposures to cosmetic talcum powders. These appear to be attributable to the presence of anthophyllite and tremolite contaminants in cosmetic talcum powder.
Topics: Adult; Air Pollutants, Occupational; Asbestos, Amphibole; Barbering; Beauty Culture; Female; Humans; Lung Neoplasms; Male; Mesothelioma, Malignant; Middle Aged; Occupational Diseases; Occupational Exposure; Pleural Neoplasms; Talc; Time Factors
PubMed: 32175619
DOI: 10.1002/ajim.23106 -
International Journal of Molecular... Mar 2020Malignant mesothelioma is an infrequent tumor that initiates from the mesothelial cells lining of body cavities. The great majority of mesotheliomas originate in the... (Review)
Review
Malignant mesothelioma is an infrequent tumor that initiates from the mesothelial cells lining of body cavities. The great majority of mesotheliomas originate in the pleural cavity, while the remaining cases initiate in the peritoneal cavity, in the pericardial cavity or on the tunica vaginalis. Usually, mesotheliomas grow in a diffuse pattern and tend to enclose and compress the organs in the various body cavities. Mesothelioma incidence is increasing worldwide and still today, the prognosis is very poor, with a reported median survival of approximately one year from presentation. Thus, the development of alternative and more effective therapies is currently an urgent requirement. The aim of this review article was to describe recent findings about the anti-cancer activity of curcumin and some of its derivatives on mesotheliomas. The potential clinical implications of these findings are discussed.
Topics: Antineoplastic Agents; Curcumin; Humans; Mesothelioma, Malignant; Phytochemicals; Pleura; Pleural Neoplasms; Prognosis
PubMed: 32155978
DOI: 10.3390/ijms21051839 -
Journal of Surgical Case Reports Feb 2020Cardiac herniation is a fatal complication in patients undergoing pneumonectomy with pericardial resection. A 53-year-old man underwent right-sided extrapleural...
Cardiac herniation is a fatal complication in patients undergoing pneumonectomy with pericardial resection. A 53-year-old man underwent right-sided extrapleural pneumonectomy for malignant pleural mesothelioma. He underwent right-sided pericardial resection and reconstruction with an expanded polytetrafluoroethylene sheet. Routine chest radiography performed 18 h postoperatively revealed cardiac herniation into the right-sided thoracic cavity. The patient was immediately transferred to the operating room, and the defect was repaired. He died of tumor progression. However, cardiac herniation did not recur over 2 years postoperatively.
PubMed: 32104562
DOI: 10.1093/jscr/rjaa011 -
Journal of Thoracic Oncology : Official... Mar 2020Children and young adults diagnosed with malignant mesothelioma may have unique genetic characteristics. In this study, we evaluated for the presence of the anaplastic...
INTRODUCTION
Children and young adults diagnosed with malignant mesothelioma may have unique genetic characteristics. In this study, we evaluated for the presence of the anaplastic lymphoma kinase (ALK) translocations in these patients.
METHODS
In a prospective study of mesothelioma natural history (ClinicalTrials.gov number NCT01950572), we assessed for the presence of the ALK translocation in patients younger than 40 years, irrespective of the site of disease. The presence of this translocation was assessed by means of fluorescence in situ hybridization (FISH). If the patients tested positive for the ALK translocation, both immunohistochemistry and RNA sequencing were performed on the tumor specimen.
RESULTS
Between September 2013 and December 2018, 373 patients were enrolled in the mesothelioma natural history study, of which 32 patients were 40 years old or younger at the time of their mesothelioma diagnosis. There were 25 patients with peritoneal mesothelioma, five with pleural mesothelioma, one with pericardial mesothelioma, and one with bicompartmental mesothelioma. Presence of an ALK translocation by FISH was seen in two of the 32 patients (6%) with mesothelioma. Both patients, a 14-year-old female and a 27-year-old male, had peritoneal mesothelioma and had no history of asbestos exposure, prior radiation therapy, or predisposing germline mutations. Neither had detectable ALK expression by immunohistochemistry. RNA sequencing revealed the presence of an STRN fusion partner in the female patient but failed to identify any fusion protein in the male patient.
CONCLUSIONS
Young patients with peritoneal mesothelioma should be evaluated for the presence of ALK translocations. Presence of this translocation should be assessed by FISH and these patients could potentially benefit from tyrosine kinase inhibitors targeting ALK.
Topics: Adolescent; Adult; Anaplastic Lymphoma Kinase; Child; Female; Gene Rearrangement; Humans; In Situ Hybridization, Fluorescence; Lung Neoplasms; Male; Mesothelioma; Prospective Studies; Receptor Protein-Tyrosine Kinases; Young Adult
PubMed: 31783178
DOI: 10.1016/j.jtho.2019.11.011 -
Cancer Cytopathology Dec 2019Sarcomas are uncommon findings in body cavity fluids. Diagnosis may be challenging because sarcoma cells in fluids can round up and lose their characteristic appearance...
BACKGROUND
Sarcomas are uncommon findings in body cavity fluids. Diagnosis may be challenging because sarcoma cells in fluids can round up and lose their characteristic appearance seen on smears and histologic sections. This study characterizes the cytologic features of sarcomas involving body cavity fluids.
METHODS
Effusion fluids and cerebrospinal fluids diagnosed as positive for sarcoma were reviewed.
RESULTS
Forty-three fluids from 28 patients (median age, 47 years) were positive for sarcoma. Four patients who presented with positive fluids were alive at 1 to 10.7 years' follow-up (median, 22.5 months). Twenty-four patients died 2 days to 2 years (median, 19 days) after their positive fluid diagnoses. Twenty-eight specimens from 20 patients had slides available for review. Although 18 of the 28 positive fluids had a morphology comparable to that of their primary, 4 small round blue cell tumors (SRBCTs) and 4 spindle cell tumors showed epithelioid morphology, 1 SRBCT had pleomorphic morphology, and 1 epithelioid primary had SRBCT morphology. Nine fluids had tumor cells in large, cohesive clusters mimicking carcinoma; workup was performed for 10, predominantly to rule out carcinoma and mesothelioma.
CONCLUSIONS
Sarcoma morphology may be altered in exfoliated cytology specimens. Workup on cell blocks aids in the differential diagnosis, especially for carcinoma and mesothelioma. Unsurprisingly, fluid cytology positive for sarcoma portends poor survival.
Topics: Adult; Aged; Ascitic Fluid; Biopsy, Needle; Body Fluids; Child, Preschool; Cohort Studies; Cytodiagnosis; Diagnosis, Differential; Exudates and Transudates; Female; Humans; Immunohistochemistry; Infant; Male; Middle Aged; Pericardial Effusion; Prognosis; Retrospective Studies; Sarcoma; Survival Analysis
PubMed: 31693303
DOI: 10.1002/cncy.22197 -
Case Reports in Surgery 2019This case report shows that pleural empyema limits the diagnostic significance of imaging techniques. Hereafter, we present the case of an 82-year-old patient with...
This case report shows that pleural empyema limits the diagnostic significance of imaging techniques. Hereafter, we present the case of an 82-year-old patient with primary pericardial mesothelioma, which was veiled by a pleural empyema. The patient met the typical triad of signs of heart failure (dyspnea, lower leg oedema), pericardial effusion, and pericarditis. Echocardiography in the identification of pericardial mesotheliomas is low. In this case, the cardiac function could be imaged well, but the tumor could not be imaged. The CT showed a pericardial effusion and a pleural effusion. Here, the tumor could not be diagnosed either. Only the operation led to diagnosis.
PubMed: 31612092
DOI: 10.1155/2019/2896810