Humans ; Child ; Pulmonary Blastoma/pathology* ; Lung Neoplasms/pathology*

Pleuropulmonary bastoma (PPB) is a rare intrathoracic malignant neoplasm in children, differ from pulmonary blastoma in adults. PPB is usually aggressive and has wide-metastases at the time of diagnosis. The therapeutic modality of PPB is extensive surgical resection with neoadjuvant or adjuvant chemotherapy. We report a case of a cystic pleuropulmonary blastoma treated with surgical resection and adjuvant chemotherapy.
Adult ; Chemotherapy, Adjuvant ; Child ; Diagnosis ; Humans ; Lung Neoplasms ; Lung* ; Pathology ; Pulmonary Blastoma

Objective: To investigate the clinical manifestation, pathological type, treatment and prognosis of primary lung tumors in children. Methods: We collected and retrospectively analyzed the clinical manifestation, pathological type, therapeutic method and prognosis of 56 primary lung tumors patients who diagnosed from 2009 to 2019 in Guangzhou Women and Children Medical Center. Results: There were 56 patients identified as the primary lung tumors, including pleuropulmonary blastoma (PPB, n=28), pulmonary inflammatory myofibroblastic tumor(IMT, n=20), mucoepidermoid carcinoma(n=6), infantile hemangioma (n=1), pulmonary sclerosing hermangioma(n=1). Respiratory symptoms were the most manifestation at the time of diagnosis including 26 patients with cough, 3 with hemoptysis, and 17 with dyspnea. Others included 15 with fever, 3 with chest pain, and 2 with epigastiric pain. The primary tumor of 18 cases were located in the lower lobe of left lung, 11 cases in the lower lobe of right lung, 10 cases in the upper lobe of left lung, 7 cases in the upper lobe of right lung, 6 cases in the middle lobe of right lung, and 4 cases in pulmonary hilum. Among the 56 patients, 41 patients underwent thoracotomy, 13 thoracoscopy, and 2 fiberoptic bronchoscopy. Five patients with type Ⅰ PPB were still alive at the end of follow-up without chemotherapy. Among 5 patients with type Ⅱ PPB, 2 patients without chemotherapy died after recurrence, 3 patients suffered postoperative chemotherapy were still alive at the end of follow-up. All of the 18 patients with type Ⅲ PPB underwent postoperative chemotherapy with IVADo regimen. Recurrence occurred in 6 cases, distant metastasis occurred in 3 cases, and cancer-related deaths occurred in 8 cases. For 20 patients with IMT, recurrence occurred in 5 of 13 patients experienced wedge resection, 1 of 6 patients experienced lobectomy and 1 of 6 underwent fiberoptic bronchoscopy, respectively. For 6 mucoepidermoid carcinoma patients, lobectomy was carried on 5 patients, wedge resection on 1 patient, all of them were still alive at the end of follow-up. One hermangioma patient underwent fiberoptic bronchoscopy and other 1 sclerosing hermangioma patient underwent wedge resection, both of them were still alive at the end of follow-up. Conclusions: The clinical manifestations of the primary lung tumors in children are nonspecific. Complete resection and achieving negative marginattribute to the excellent outcome. Adjunctive treatment such as chemotherapy is necessary for patients with type Ⅱ and type Ⅲ PPB.
Bronchoscopy ; Child ; Female ; Humans ; Lung/pathology* ; Lung Neoplasms/surgery* ; Pulmonary Blastoma/surgery* ; Retrospective Studies

BACKGROUND AND OBJECTIVEPleuropulmonary blastoma (PPB) is a rare malignant tumor with unique clinicopathological features. The aim of this study is to investigate the clinicopathological features, the diagnosis and differential diagnosis of pleuropulmonary blastoma.

METHODSFive cases of PPB were analyzed by light microscopy, immunohistochemistry and their clinical data, and the relative literatures were reviewed.

RESULTSFive cases of patients suffered from PPB were aged from 21 to 47 months (mean 32.8 months). Most of the masses were located in the thoracic cavities and 4 cases accompanied with pleural effusions. Histologically, these tumors included 1 case of type I PPB which showed pure cystic architecture; 2 cases were type II PPB which showed cystic and solid masses accompanied with rhabdomyoblastic differentiation and nodules of cartilage; the other 2 cases were type III PPB and characterized by absolute solid masses with anaplastic undifferentiated sarcomatous components. Immunohistochemical studies showed that tumor cells were positive for Vimentin and some for Desmin and Myogenin, the nodules of cartilage were positive for S-100. The tumor cells were negative for PCK, EMA and CD99.

CONCLUSIONPleuropulmonary blastoma is a rare and highly aggressive malignancy arising in the lung and pleural of infancy and early childhood. The type I, II and III PPB have unique clinicopathological features respectively. This kind of tumor should be distinguished from some benign and malignant diseases such as congenital cystic adenomatoid malformation (CCAM) and embryonal rhabdomyosarcoma.

Adult ; Female ; Humans ; Lung Neoplasms ; mortality ; pathology ; surgery ; Middle Aged ; Pleural Neoplasms ; mortality ; pathology ; surgery ; Pulmonary Blastoma ; mortality ; pathology ; surgery

OBJECTIVETo investigate clinical and pathological features of lung lesions in children.

METHODSClinical manifestations, radiologic imaging, histopathological features and immunohistochemical results were analyzed in 215 cases of lung lesions in children.

RESULTSA total of 215 cases of lung lesions in children aged 0 day to 13 years (average age of 27.2 months and the median age of 18.0 months) were selected, including 137 male and 78 female patients with a male to female ratio of 1.76:1.00. The incidence of congenital lung disease was higher in patients of less than 1 year old than those of over 1 year old age, and the difference of the two groups was statistically significant (P = 0.004). 142 cases had acquired lung diseases, and 73 cases had congenital bronchopulmonary dysplasia. Lung abscess was the most common lesion seen in 86 cases (40.0%), including 1 case of fungal abscess. Congenital pulmonary airway malformation (CPAM) was the second most common, seen in 44 patients (20.5%), including 20 cases of type 1, 18 cases of type 2 and 6 cases of type 4 CPAM. Pulmonary sequestration was found in 25 cases (11.6%) including 14 cases of intralobar type and 11 cases of extralobar type. Two cases of extralobar pulmonary sequestration showed simultaneous CPAM2 type 2 lesion. Other lesions included tuberculosis (13 cases, 6.0%), emphysema (12 cases, 5.6%), interstitial pneumonia (7 cases, 3.2%), pulmonary hemorrhage (6 cases, 2.8%), bronchogenic cyst (4 cases, 1.9%), bronchiolitis obliterans (2 cases, 0.9%), idiopathic pulmonary hemosiderin deposition disease (2 cases, 0.9%) and 1 cases of lung non-specific changes. 13 cases of neoplastic lesions (6.0%) were found, of which 11 cases were primary tumors (5.1%), including inflammatory myofibroblastic tumor in 5 patients (2.3%), pleuropulmonary blastoma in 5 cases (1 case of type I, 2 type II and 2 type III) and 1 case of mucoepidermoid carcinoma (0.5%) and 2 cases of metastatic tumors (hepatoblastoma and Wilm's tumor, 0.9%).

CONCLUSIONSInfectious diseases are the most common lung diseases in children. Congenital bronchopulmonary dysplasia is the most common in children of less than 1 year old. Malignant lesions are rare.

Abscess ; pathology ; Adolescent ; Bronchopulmonary Sequestration ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Infant, Newborn ; Lung ; pathology ; Lung Diseases ; pathology ; Male ; Pulmonary Blastoma ; pathology

OBJECTIVETo study the clinicopathological and immunohistochemical features, histogenesis and prognosis of pleuropulmonary blastoma (PPB) in children.

METHODSPPB specimens from 16 pediatric cases with an age ranging from 1 year and 7 months to 5 years and 3 months (mean age of 3 years) were retrieved and analyzed by routine histological, immunohistochemical and electron methods.

RESULTSAmong 16 patients, there were 2 type I, 7 type II and 7 type III PPB cases. Type I PPB as multilocular cystic structure, consisted of thin fibrous wall lining the respiratory epithelium, subepithelial primitive blastema or immature mesenchymal cells, with or without rhabdomyoblastic differentiation or cartilage; Type II PPB as cystic-solid tumor, comparing with type I, consisted of intracystic components with appearance of anaplastic tumor cells. Type III PPB consisted of completely solid mass, the same as the solid region of type II, had mixed pattern including blastema, undifferentiated spindle-cell proliferations and sarcomas. In addition, anaplastic tumor cells and intra-and extra- cytoplasmic eosinophilic globules were also commonly present. Epithelial components in PPB were benign. Immunohistochemical study showed primitive mesenchymal differentiation of tumors. All cases were positive for vimentin, desmin, myogenin and SMA in tumors with skeletal muscle differentiation, S-100 was positive in tumors with cartilage differentiation. All tumors were negative for synaptophysin, CD99, and CD117. Benign epithelial components were positive for AE1/AE3 and EMA. In 12 cases, electron microscopy revealed few organelles in the primitive mesenchymal cells and rich heterochromatin in mesenchymal cells, the latter also demonstrating cytoplasmic myofilament dysplasia. Nine cases had clinical follow-up ranging from 5 to 48 months, of which 4 patients died.

CONCLUSIONSPPB is a rare lung neoplasm of children under the age of 6 years, with distinct pathological morphology. PPB may arise from lung or pleura mesenchymal cells and has a poor clinical outcome.

Child, Preschool ; Cysts ; pathology ; Desmin ; analysis ; Female ; Humans ; Infant ; Lung Neoplasms ; chemistry ; pathology ; Male ; Microscopy, Electron ; Myogenin ; analysis ; Prognosis ; Pulmonary Blastoma ; chemistry ; pathology ; Sarcoma ; pathology ; Vimentin ; analysis

Pleuropulmonary blastoma (PPB) is a rare dysontogenetic neoplasm in children and has been recognized as a distinct clinicopathological entity different from the ordinary pulmonary blastoma of adulthood. We report a very rare adult case of PPB. A 43-year-old female patient presented with massive pleural infusion, and a misdiagnosis of pleural tuberculosis (TB) was made on the basis of ultrasound scan and CT images, for which therapy with TB drugs was administered for 3 weeks. Subsequent operation and pathologic examination of the mass revealed a multicystic neoplasm consisting of malignant mesenchymal cells immunohistochemically positive for vimentin and actin. Local recurrence occurred in the left pleural 20 months after the surgical tumor resection and 4 cycles of adjuvant chemotherapy.
Adult ; Diagnosis, Differential ; Female ; Humans ; Lung Neoplasms ; diagnosis ; pathology ; surgery ; Pleural Effusion ; diagnosis ; etiology ; Pulmonary Blastoma ; diagnosis ; pathology ; surgery ; Tomography, X-Ray Computed

OBJECTIVETo study the clinicopathologic and immunohistochemical features of adult type pulmonary blastoma (PB).

METHODFour cases of adult type PB were studied with light microscopy and immunohistochemical staining.

RESULTSThree cases of PB were biphasic type and were composed of primitive epithelial component and primitive stromal component; one case of PB was epithelial type and was shown as branching glands with clustered and well-differentiated structure. Immunohistochemical studies showed that AE1/AE3 was positive in the epithelial component in four cases, Vimentin was positive in the stromal component in three cases, thyroid transricption factor-1 was positive in two cases, CD117 was positive in one case, Ki-67 was 2% positive in one case, while P53 was negative in all cases.

CONCLUSIONSThe adult type PB is a rarely seen biphasic tumor. It contains a primitive epithelial component and a primitive mesenchymal stroma, which looks like well-differentiated fetal adenocarcinoma. As highly invasive intrathoracic neoplasm, it is mainly found in adult with an unfavorable outcome.

Adult ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Ki-67 Antigen ; metabolism ; Lung Neoplasms ; metabolism ; pathology ; Male ; Pulmonary Blastoma ; metabolism ; pathology ; Retrospective Studies ; Vimentin ; metabolism

Humans ; Lung Neoplasms ; metabolism ; pathology ; surgery ; Lymph Node Excision ; Male ; Middle Aged ; Pneumonectomy ; methods ; Pulmonary Blastoma ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Biomarkers, Tumor ; metabolism ; Hemangioendothelioma, Epithelioid ; metabolism ; pathology ; Hemangiosarcoma ; metabolism ; pathology ; Humans ; Immunohistochemistry ; Leiomyosarcoma ; metabolism ; pathology ; Lung Neoplasms ; metabolism ; pathology ; Nerve Sheath Neoplasms ; metabolism ; pathology ; Pulmonary Blastoma ; metabolism ; pathology ; Sarcoma ; metabolism ; pathology ; Sarcoma, Synovial ; metabolism ; pathology ; Solitary Fibrous Tumors ; metabolism ; pathology