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Journal of the Formosan Medical... Nov 2022The purpose of this study is to compare the clinical characteristics and surgical outcomes of thoracotomy and video-assisted thoracoscopic surgery (VATS) in children...
BACKGROUND
The purpose of this study is to compare the clinical characteristics and surgical outcomes of thoracotomy and video-assisted thoracoscopic surgery (VATS) in children with congenital lung malformations (CLMs) in a tertiary referring center and to report our modified biportal VATS setting.
METHODS
This is a single-center retrospective chart review study including children who underwent surgical resection for CLMs between January 2007 and December 2020. Patient characteristics and surgical outcomes were compared between open and thoracoscopy, as well as conventional VATS and biportal VATS. Biportal setting included an anterior utility wound and a camera trocar wound with one-lung ventilation.
RESULTS
A total of 100 patients were identified. Twenty patients received thoracotomy, and 80 patients received VATS (67 conventional and 13 biportal VATS). The median age at operation was 0.4 months in the thoracotomy group and 4.7 months in the VATS group. More patients in the thoracotomy group had preoperative symptoms, comorbidities, and emergent operations. The patients who underwent thoracotomy had significantly longer postoperative ICU stays, chest tube durations, hospital stays, and more complications. The pathological analysis revealed 67 congenital pulmonary airway malformations, 27 pulmonary sequestration, 6 hybrid lesions, and one accompanying pleuropulmonary blastoma. Compared to conventional VATS, the ICU stay was shorter in the biportal VATS group, with comparable operative durations, hospital stay and complications.
CONCLUSION
VATS for CLMs is associated with better postoperative recovery and fewer complications. Biportal VATS is also a safe and feasible approach.
Topics: Child; Humans; Length of Stay; Lung; Lung Diseases; Lung Neoplasms; One-Lung Ventilation; Pneumonectomy; Postoperative Complications; Retrospective Studies; Thoracic Surgery, Video-Assisted; Thoracotomy; Treatment Outcome
PubMed: 35331621
DOI: 10.1016/j.jfma.2022.03.003 -
Medicine Nov 2022Pulmonary blastoma is an extremely rare and highly aggressive tumor. Only a few hundred cases of pulmonary blastoma have been reported. In other cases, a definitive...
RATIONALE
Pulmonary blastoma is an extremely rare and highly aggressive tumor. Only a few hundred cases of pulmonary blastoma have been reported. In other cases, a definitive diagnosis is often made through surgical resection. The use of preoperative histopathological sampling in diagnosing was of limited value because of the variety of pulmonary blastoma histology. And there was no literature that the first biopsy was attempted with medical thoracoscopy for diagnosis.
PATIENT CONCERNS
A 65-year-old man presented to our hospital with pleural effusion and lung mass.
DIAGNOSES
The patient was initially diagnosed with dedifferentiated chondrosarcoma by medical thoracoscopic biopsy but the final diagnosis was pulmonary blastoma through bilobectomy.
INTERVENTIONS
Medical thoracoscopy, and video-assisted thoracoscopic surgery (bilobectomy) followed by adjuvant chemotherapy.
OUTCOMES
After surgical resection of the tumor, adjuvant chemotherapy has been performed 5 cycles at 3 weeks intervals, and there was no evidence of recurrence on follow-up computed tomography performed 4 months after surgery.
LESSONS
Medical thoracoscopy is useful for the diagnosis of indeterminate pleural effusion; however, caution is needed when confirming rare malignancies, such as pulmonary blastoma. Although surgical resection is the treatment of choice, appropriate adjuvant chemotherapy to improve the prognosis may be necessary if there is pleural metastasis.
Topics: Male; Humans; Aged; Pulmonary Blastoma; Thoracic Surgery, Video-Assisted; Pleural Effusion; Lung Neoplasms; Chondrosarcoma
PubMed: 36451398
DOI: 10.1097/MD.0000000000031377 -
Annals of Surgery Dec 2021To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer.
OBJECTIVE
To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer.
SUMMARY BACKGROUND DATA
Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors.
METHODS
The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients <21 years of age who underwent pneumonectomy from 1990 to 2017 for primary or metastatic tumors at 12 institutions. Clinical information was collected; outcomes included operative complications, long-term function, recurrence, and survival. Univariate log rank, and multivariable Cox analyses determined factors associated with survival.
RESULTS
Thirty-eight patients (mean 12 ± 6 yrs) were identified; median (IQR) follow-up was 19 (5-38) months. Twenty-six patients (68%) underwent pneumonectomy for primary tumors and 12 (32%) for metastases. The most frequent histologies were osteosarcoma (n = 6), inflammatory myofibroblastic tumors (IMT; n = 6), and pleuropulmonary blastoma (n = 5). Median postoperative ventilator days were 0 (0-1), intensive care 2 (1-3), and hospital 8 (5-16). Early postoperative complications occurred in 10 patients including 1 death. Of 25 (66%) patients alive at 1 year, 15 reported return to preoperative pulmonary status. All IMT patients survived while all osteosarcoma patients died during follow-up. On multivariable analysis, metastatic indications were associated with nonsurvival (HR = 3.37, P = 0.045).
CONCLUSION
This is the largest review of children who underwent pneumonectomy for cancer. There is decreased procedure-related morbidity and mortality than reported for adults. Survival is worse with preoperative metastatic disease, especially osteosarcoma.
Topics: Adolescent; Child; Child, Preschool; Humans; Length of Stay; Lung Neoplasms; Myofibroma; Neoplasm Metastasis; Neoplasm Recurrence, Local; Operative Time; Osteosarcoma; Pneumonectomy; Postoperative Complications; Proportional Hazards Models; Pulmonary Blastoma; Survival Analysis
PubMed: 32209902
DOI: 10.1097/SLA.0000000000003795 -
European Journal of Cardio-thoracic... Dec 2016
Topics: Child, Preschool; Female; Humans; Lung; Pleura; Pulmonary Blastoma; Radiography; Sternotomy; Tomography, X-Ray Computed
PubMed: 27341849
DOI: 10.1093/ejcts/ezw217 -
The New England Journal of Medicine Jun 2018
Topics: DNA Helicases; Female; Germ-Line Mutation; Humans; Infant; Lung Neoplasms; Male; Nuclear Proteins; Pedigree; Pulmonary Blastoma; Teratoma; Transcription Factors
PubMed: 29874541
DOI: 10.1056/NEJMc1803354 -
Virchows Archiv : An International... Sep 2020
Topics: Cystic Adenomatoid Malformation of Lung, Congenital; Humans; Pulmonary Blastoma
PubMed: 32300881
DOI: 10.1007/s00428-020-02811-x -
Zhonghua Zhong Liu Za Zhi [Chinese... Jul 2020To explore the clinical characteristics, therapeutic methods and prognosis of pleuropulmonary blastoma in children. The clinical data of 28 patients with...
To explore the clinical characteristics, therapeutic methods and prognosis of pleuropulmonary blastoma in children. The clinical data of 28 patients with pleuropulmonary blastoma diagnosed in Guangzhou Women and Children's Medical Centre from November 2008 to May 2018 were collected and retrospectively analyzed. Of the 28 patients, 18 were male and 10 were female, aged from 22 days to 5 years 10 month, the average age was 2 years 6 months. One patient underwent biopsy and other 27 underwent operation, 14 patients with type Ⅱ/Ⅲ pleuropulmonary blastoma received postoperative chemotherapy. Five patients were pathologically diagnosed as typeⅠpleuropulmonary blastoma, 5 were type Ⅱ pleuropulmonary blastoma and 18 were type Ⅲ pleuropulmonary blastoma. During the follow-up period of 24 patients, 15 patients were disease free survival, 3 patients relapsed within 6 months, 10 months and 18 months after chemotherapy, respectively. One patient who received postoperative chemotherapy suffered a bone metastasis within 11 months, 2 patient without chemotherapy relapsed within 2 months and suffered bone or renal metastasis within 3 months, respectively. Three patients who left hospital voluntarily died within 1 month. Pleuropulmonary blastoma is a highly malignant and rapidly progressed neoplasm. Patients with type Ⅰ pleuropulmonary blastoma have good prognoses while the prognoses of Ⅱ/Ⅲ pleuropulmonary blastoma are poor. Postoperative chemotherapy seems to improve the survival of patients withⅡ/Ⅲ pleuropulmonary blastoma.
Topics: Child; Child, Preschool; Disease-Free Survival; Female; Humans; Lung Neoplasms; Male; Prognosis; Pulmonary Blastoma; Retrospective Studies
PubMed: 32842446
DOI: 10.3760/cma.j.cn112152-20190316-00165 -
Urology Aug 2021DICER1 syndrome is a rare hereditary cancer predisposition syndrome that has relevance to pediatric urology providers due to its association with many various pediatric... (Review)
Review
DICER1 syndrome is a rare hereditary cancer predisposition syndrome that has relevance to pediatric urology providers due to its association with many various pediatric genitourinary malignancies. We describe the case of a pediatric patient who was eventually diagnosed with a pathogenic DICER1 germline variant after undergoing resection of a cystic nephroma and pleuropulmonary blastoma.
Topics: Child, Preschool; DEAD-box RNA Helicases; Genetic Predisposition to Disease; Germ-Line Mutation; Humans; Kidney Diseases, Cystic; Kidney Neoplasms; Lung Neoplasms; Male; Pneumothorax; Pulmonary Blastoma; Ribonuclease III; Syndrome; Tomography, X-Ray Computed
PubMed: 33571543
DOI: 10.1016/j.urology.2021.01.049 -
Medicine Dec 2017Pulmonary blastoma is a rare primary lung cancer that can be categorized into adult type and child type. The clinical symptoms and imaging features of pulmonary blastoma...
RATIONALE
Pulmonary blastoma is a rare primary lung cancer that can be categorized into adult type and child type. The clinical symptoms and imaging features of pulmonary blastoma are nonspecific, making it difficult to diagnose preoperatively. Postoperative pathology with immunohistochemical staining can help diagnosis.
PATIENT CONCERNS
A 53-year-old male had chest tightness and shortness of breath.
DIAGNOSES
The patient was diagnosed as pleural pulmonary blastoma based on computed tomography (CT) scan, pathology, immunohistochemistry, and molecular pathology. CT examination showed solid mass on the upper lobe of the left lung Intraoperative observation found that tumor tissue was gray with tough texture. The surrounding lung tissue showed AE1/AE3 (+), Vimentin (+), and CD34 (+) staining. No epidermal growth factor receptor gene mutation was detected.
INTERVENTIONS
The left lobe resection plus mediastinal lymph node dissection were performed. After the operation, patient received paclitaxel combined with nedaplatin chemotherapy for 4 times.
OUTCOMES
Four months later, left pleural metastasis, and mediastinal lymph node metastasis was found. The patient died 15 months later.
LESSONS
Pleural pulmonary blastoma is a malignant tumor with rare pathological features that is easy to relapse and metastasis with poor prognosis. Surgical treatment preferably, lobectomy plus mediastinal lymph node dissection, is the first treatment option. The overall prognosis is poor.
Topics: Diagnosis, Differential; Fatal Outcome; Humans; Immunohistochemistry; Lung Neoplasms; Lymph Node Excision; Male; Middle Aged; Pulmonary Blastoma; Tomography, X-Ray Computed
PubMed: 29390280
DOI: 10.1097/MD.0000000000008918 -
Journal of Clinical Oncology : Official... Mar 2019DICER1 syndrome is an autosomal-dominant, pleiotropic tumor-predisposition disorder caused by pathogenic germline variants in DICER1. We sought to quantify risk, hazard... (Clinical Trial)
Clinical Trial Observational Study
PURPOSE
DICER1 syndrome is an autosomal-dominant, pleiotropic tumor-predisposition disorder caused by pathogenic germline variants in DICER1. We sought to quantify risk, hazard rates, and the probability of neoplasm incidence accounting for competing risks ("cumulative incidence") of neoplasms (benign and malignant) and standardized incidence ratios for malignant tumors in individuals with DICER1 pathogenic variation.
PATIENTS AND METHODS
We combined data from three large cohorts of patients who carry germline pathogenic variation in DICER1. To reduce ascertainment bias, we distinguished probands from nonprobands. Neoplasm diagnoses were confirmed by review of pathology reports and/or central review of surgical pathology materials. Standardized cancer incidence ratios were determined relative to the SEER program, which does not capture all DICER1-associated neoplasms. For all malignancies and benign tumors ("neoplasms," excluding type Ir pleuropulmonary blastoma and thyroid nodules), we used the Kaplan-Meier method and nonparametric cumulative incidence curves to estimate neoplasm-free survival.
RESULTS
We calculated the age at first neoplasm diagnosis (systematically ascertained cancers plus DICER1-associated neoplasms pleuropulmonary blastoma, cystic nephroma, and nasal chondromesenchymal hamartoma) in 102 female and male nonproband DICER1 carriers. By age 10 years, 5.3% (95% CI, 0.6% to 9.7%) of nonproband DICER1 carriers had developed a neoplasm (females, 4.0%; males, 6.6%). By age 50 years, 19.3% (95% CI, 8.4% to 29.0%) of nonprobands had developed a neoplasm (females, 26.5%; males, 10.2%). After age 10 years, female risk was elevated compared with male risk. Standardized cancer incidence ratio analysis of 102 nonproband DICER1 carriers, which represented 3,344 person-years of observation, showed significant cancer excesses overall, particularly of gynecologic and thyroid cancers.
CONCLUSION
This work provides the first quantitative analysis of site-specific neoplasm risk and excess malignancy risk in 102 systematically characterized nonproband DICER1 carriers. Our findings inform DICER1 syndrome phenotype, natural history, and genetic counseling.
Topics: Adolescent; Adult; Child; Cohort Studies; DEAD-box RNA Helicases; Female; Genetic Predisposition to Disease; Genotype; Germ-Line Mutation; Humans; Kaplan-Meier Estimate; Lung Neoplasms; Male; Middle Aged; Pulmonary Blastoma; Registries; Ribonuclease III; Risk Factors; Young Adult
PubMed: 30715996
DOI: 10.1200/JCO.2018.78.4678