Pulmonary Blastoma Presenting as a Solitary Lip Metastasis: Case Report and Review of the Literature

Abstract

Pulmonary blastoma (PB) is an uncommon primary lung malignancy. This neoplasm was first described by Barrett and Barnard in 1945. The tumor is composed of immature epithelial and mesenchymal tissues which may recapitulate early embryological lung development. Under the microscope, the globular component resembles immature bronchus and connective tissue as seen in embryonic lung. More than one hundred cases have been reported in the literature. PB is more frequent in older people and in males and tends to affect blacks at younger ages. Symptomatology varies from asymptomatic to symptoms of a non-specific pulmonary disease. Cough, hemoptysis, dyspnea, chest pain, respiratory distress, fever, anorexia and weight loss are the most common presenting features. The most common roentgenologic pattern is a well-demarcated peripheral lesion, encapsulated by compression or atelectatic lung tissue, although in some cases there is a tendency to lobulation and cavitation. The size of the mass varies from a small peripheral nodule to a mass occupying the entire lobe or hemithorax. The treatment of choice has been surgical excision, radiation and, in selected cases, a combination of chemotherapy with radiation. The prognosis of this malignancy is poor; overall five-year survival is approximately 16 percent. No correlation has been established between histopathologic criteria and survival. The factors that indicate poor prognosis are tumor recurrence, metastasis at initial presentation, tumor size over 5 cm and lymph node metastasis. Liver, central nervous system and bones are the most frequent location of distant metastases. A rare case is presented of a pulmonary blastoma with an upper lip metastasis occurring in a paraplegic male. Diagnosis was confirmed by autopsy findings. To our knowledge, this is the first documented case of a pulmonary blastoma metastasis to the upper lip. (J Spinal Cord Med; 18:203–207)     

Primary malignant lung tumors in children: A report from the Australian Childhood Cancer Registry, 1983‐2015

Lung cancers in children under the age of 15 are very uncommon, with a scarcity of literature describing patient characteristics and survival. This study assessed first primary malignant cancers occurring in the trachea, bronchus, or lung (International Classification of Diseases for Oncology, 3rd edition [ICD‐O‐3] codes C33‐C34) for the period 1983‐2015, using data from the population‐based Australian Childhood Cancer Registry. Variables of interest included morphology, sex, age group, and metastatic status at diagnosis. Mode of treatment was also assessed where possible. The Kaplan‐Meier method was used to calculate 5‐year observed survival. Of the 53 in‐scope patients, almost half (n = 23, 43%) were diagnosed with pleuropulmonary blastoma and a further 8 (15%) had a carcinoid tumor. Few of the patients with details available on stage at diagnosis (n = 7 of 43, 16%) presented with metastatic disease. Surgical excision was the most common treatment (30 of 37 children, 81%), with two‐thirds (n = 28 of 43, 65%) receiving chemotherapy. Five‐year observed survival was estimated to be 74% (95% CI = 61%‐85%). Our results represent one of the largest and most complete population‐based cohorts of children with primary malignant lung cancers available to date. Detection of childhood lung cancer can be difficult due to the rarity of this disease and symptoms that are typically nonspecific.     

Diagnosis and multi-modality treatment of adult pulmonary plastoma:Analysis of 18 cases and review of literature

The clinical data of 18 patients with PB from April 1989 to April 2013 was analyzed retrospectively,including 11 men and 7 women,aged 45 and 76 years old(mean 53 years).There were 12 eases of PB occurrlng in right lung and other cases in left lung,AraBng them,3 patients had no symptoms,and 15 patients displayed symptoms of cough,chest pain,asthenia or minor hacmoptysis.Overall,11 patients had a preoperative diagnosis of lung cancer,7 patients were preoperatively diagnosed as the other disease,which included lung benign tumor(n=5)and mediastinal mass(n=2).All patients received a radical resection.Six patients received postoperative cisplalinbascd chemothcrapy,and two paticnts received postoperative irradiation with the dose of 55 Gy.Histologically.14 eases of 18 patients had biphasie pulmonary blastema and four cases had well differentiated fetal adenocarcinoma.A total of 12 patients died in a period of 6-36 months after operation,and 1 ease was lost after 2 years of follow up.The median survival time was 19 months,PB is a rare primary lung malignant embryonal neoplasm.Despite its assumed embyonal origin,the tumor has a predileetion for adults.A preoperative correct diagnosis is very difficult in spite of modern diagnostic imaging and biopsy techniques,Surgical resection is the main method for diagnosis and treatment.Postoperative chemotherapy or irradiation can help eliminate tumor remnants.Its prognosis is very poor,especially for the biphasie type.     

Pediatric Cystic Lung Lesions: Where Are We Now?

Pediatric cystic lung lesions have long been a source of confusion for clinicians, radiologists, and pathologists. They encompass a wide spectrum of entities with variable prognostic implications, including congenital lung malformations, pulmonary neoplasms, and hereditary conditions. As our understanding of the developmental and genetic origins of these conditions has evolved, revised nomenclature and classifications have emerged in an attempt to bring clarity to the origin of these lesions and guide clinical management. This review discusses cystic lung lesions and the current understanding of their etiopathogenesis.     

成人肺母细胞瘤一例病例报告并文献复习

肺母细胞瘤（pulmonary blastoma,PB）又称肺胚层瘤或胚胎型癌肉瘤,由原始间叶成分和/或上皮成分组成,类似于胚胎肺的结构,是一种非常罕见的肿瘤,至2011年全世界文献报道累积仅约200例［1］。     

USCAP Specialty Conference: Case 1-Type I Pleuropulmonary Blastoma

Pleuropulmonary blastoma (PPB) was defined in 1988 by Manivel et al. in a series describing 11 intrathoracic pulmonary neoplasms in young children [1]. The PPB is a unique peripheral pulmonary or pleural-based tumor of childhood that is characterized in its earliest form as a bland-appearing multiloculated cyst with small foci of tumor cells and in later forms as mixed and predominantly primitive, overtly malignant neoplasms [2,3]. Prior to the introduction of the PPB as a distinct entity, this tumor had been reported in the literature as pulmonary blastoma, sarcoma arising in mesenchymal cystic hamartoma, embryonal sarcoma, malignant mesenchymoma, primary pulmonary rhabdomyosarcoma and rhabdomyosarcoma arising in congenital adenomatoid malformation or bronchogenic cyst. Over the past 15 years, PPB has come to be recognized in centers around the world. With the establishment of the Pleuropulmonary Blastoma Registry by Jack Priest, MD, and colleagues, there has been improved understanding of this rare pediatric neoplasm. The registry Web site serves as an important resource for physicians and families ().     

Pulmonary blastoma in a child: report of a case

We describe herein the case of a 2-year-old girl found to have a pulmonary blastoma (PB). The child was admitted to our hospital with the chief complaints of coughing and left-sided chest pain. On admission, a chest X-ray revealed a large mass in the left lung, which measured 10 cm in diameter. Computed tomography and magnetic resonance imaging showed a marginally and heterogeneously enhanced tumor filling the left hemithorax. Pathologic findings of the fine-needle aspiration were suggestive of neuroblastoma. Subsequently, a left pneumonectomy with lymph node dissection was performed and histopathological examination confirmed that the tumor was a PB (type III). After the definitive diagnosis was made the patient received combination chemotherapy, and no evidence of recurrence has been seen in the 5 months since surgery. Received: September 4, 2000 / Accepted: March 6, 2001