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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., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

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., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Pleuropulmonary blastoma (PPB) is a rare malignancy of childhood which when left untreated often shows pathologic progression resulting in a more aggressive neoplasm with an increasingly poor prognosis. Because of this it is important to diagnose and initiate treatment early. However, early stage PPB can appear as a cystic lung lesion on imaging and can be easily misdiagnosed given the rarity of the malignancy. Moreover, current therapeutic guidelines for these lesions are not well established, making treatment decisions and management difficult for clinicians. DICER1 mutations are known to be present in a majority of PPBs with or without a germline mutation and may be part of a familial tumor predisposition syndrome. The clinical, pathologic, and genetic data of 6 patients are summarized here. Two patients with type I PPB and 4 patients with type II PPB underwent surgical and chemotherapeutic treatment and all are alive and without recurrence 1 to 13 years after treatment. With increasing awareness of PPB, it is important for clinicians to consider this malignant entity in the evaluation and treatment of patients presenting with a cystic lung abnormality, especially in cases with a history strongly suggestive of a DICER1 mutation.

The sarcomatous element in pleuropulmonary blastoma (PPB) is often histologically indistinguishable from embryonal rhabdomyosarcoma (ERMS). A diagnosis of PPB is often made after definitive surgical resection based on pathologic features, most notably the presence of hamartomatous pulmonary elements. Samples from seven PPB patients were obtained from the rhabdomyosarcomatous portion of the tumor by macrodissection. Representative ERMS tumor tissue was selected from 21 ERMS patient samples. Formalin-fixed paraffin-embedded tissue scrolls from each sample were analyzed using the Affymetrix Human Exon arrays. All PPB patients and 7 of 21 ERMS patients were 3 years old and younger. Twenty transcripts (10 annotated, 10 noncoding RNAs) were significantly differentially expressed in ERMS when compared with PPB samples. Insulin-like growth factor 2 (IGF2) was uniformly overexpressed in ERMS (19/21>400) but was expressed at low levels in PPB (P<0.001). Two ERMS cases that had low level IGF2 expression were 3 years and younger of age. No other differences between the 2 approached this degree of significance, despite a common rhabdomyogenic phenotype in the sarcomatous areas of PPB. PPB, unlike most ERMS, appears not to be driven by autocrine IGF2 signaling.

Pediatric tumors are heterogenous and can be quite varied in appearance. However, those in the infamous "small round blue-cell tumor" group, with their hyperchromatic nuclei and small amount of cytoplasm can be challenging, and their diagnosis and prognostication require cost-efficient and focused immunohistochemistry and ancillary testing. Ideally, ample material should be obtained for routine histology and ancillary testing, including immunohistochemistry, fluorescent in situ hybridization, fresh tissue for cytogenetic studies, and snap-frozen tumor for DNA/RNA extraction both for routine molecular testing (ie, reverse-transcription PCR studies), as well as future research study protocols (genome wide studies, targeted gene sequencing). This review focuses on the main pediatric tumors with emphasis on immunophenotype, keeping in mind that a directed panel approach yields the highest yield with combination of clinical history, histologic features, and ancillary molecular testing.

Malignant biphasic tumors of the lungs are rare primary tumors of the bronchopulmonary system. These tumors are composed of malignant epithelial and mesenchymal elements and together comprise <2% of all primary pulmonary neoplasms. The tumors belonging to this group include pulmonary blastoma, pleuropulmonary blastoma, and carcinosarcoma. In this study, the clinicopathologic features, immunohistochemical phenotype, molecular biological characteristics, and the differential diagnosis of these uncommon neoplasms are discussed.

Introduction: Pneumonectomy syndrome is a rare complication after pneumonectomy with variable onset and presentation. It is characterized by extrinsic airway compression secondary to mediastinal shift. As far as we are aware, we are reporting the first pediatric case of pneumonectomy syndrome presenting as a cardio-pulmonary arrest., Patient and Intervention: A two-year-old boy presented with a cardiac arrest 8 months after his pneumonectomy. He failed extubation twice. A flexible bronchoscopy was then performed which revealed external compression of the left mainstem bronchus. He underwent surgical correction with a prosthetic, thoracic implant., Result: The patient's trachea was subsequently extubated to room air on postoperative day 1. He remains clinically stable at 6 month follow-up., Conclusion: Postpneumonectomy syndrome is a rare but serious complication after pneumonectomy. If the airway compression occurs in conjunction with compromised vena caval return, cardio-pulmonary arrest can occur. Therefore, timely diagnosis and early intervention is required.