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Introduction to Cancer
Published in David E. Thurston, Ilona Pysz, Chemistry and Pharmacology of Anticancer Drugs, 2021
A blastoma is a type of cancer thought to arise in embryonic tissue. The term is commonly used as part of the name for a tumor. For example, glioblastomas and medulloblastomas, hepatoblastomas, nephroblastomas (Wilms’ tumor of the kidney), neuroblastomas (a childhood tumor of neural origin), osteoblastomas, and retinoblastomas are tumors of the brain, liver, kidneys, neurological system, bone, and retina, respectively.
Pleuropulmonary Blastoma
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Raúl Barrera-Rodríguez, Carlos Pérez-Malagón
The term blastoma refers to a malignant neoplasm that occurs in immature or developing cells, suggesting a “primitive,” almost embryo-like, appearance. Therefore, it is considered as a dysontogenic neoplasm [4] and can be an analog neoplasm to Wilms tumor, neuroblastoma, pancreatoblastoma, retinoblastoma, or hepatoblastoma [3,5]. PPB has also been known with different names, including embryonal rhabdomyosarcoma (arising within a congenital bronchogenic cyst), rhabdomyosarcoma (arising in congenital cystic adenomatoid malformation [CCAM]), pulmonary sarcoma (arising in mesenchymal cystic hamartoma), and pulmonary blastoma (associated with cystic lung disease of childhood) [3].
Potential of Herbal Extracts and Bioactive Compounds for Human Healthcare
Published in Megh R. Goyal, Hafiz Ansar Rasul Suleria, Ramasamy Harikrishnan, The Role of Phytoconstitutents in Health Care, 2020
Ramasamy Harikrishnan, Chellam Balasundaram
The characteristic features of all tumor cells are continuous improper signals of cell growth or cell multiplication, programed cell death interruption, an endless number of cell growth, developing excessive blood vessel network, tissue invasion, and metastasis [332]. Carcinoma cancers are formed from epithelial cells that are most common among prostate, breast, pancreas, colon, and lung cancer. The sarcoma cancers (formed from cartilage, bone, fat, and nerve tissues) develop mesenchymal cells on the outside of bone marrow. However, the lymphoma or leukemia is formed from hematopoietic cells on bone marrow that mature in parts of lymph and blood vessels. The germ-cell tumor is formed from embryonic stem cells that are mostly present in the testis or ovary, particularly called seminoma and dysgerminoma. The blastoma cancers are also formed from immature embryonic stem cells.
Pleuropulmonary blastoma: Difficulty in diagnosis and treatment of a case in Vietnam
Published in Pediatric Hematology and Oncology, 2021
Bui Ngoc Lan, Le Thi Kim Ngoc, Hoang Ngoc Thach, Phan Canh Duy
PPB is a dysembryonic neoplasm of thoraco-pulmonary mesenchyme, which arises from either lungs or pleural surface, or even both. Histopathology of pleuropulmonary blastoma tumors are divided into 3 types: type I cystic form (14%) observed in infants and young children less than 10 months old, type II mixed (cystic/solid) form (48%) observed in children older than 2-3 years old, type III, completely solid (38%) observed older children older than 4 years old.2 Prognosis deteriorates from type I (cystic form) to type III (solid form).3 A germline mutation in DICER1 is the genetic cause in the majority of PPB cases.5 During the initial admission, the patient’s chest CT scan revealed a tumor dominantly cystic. The lesion was similar to CCAM. At 2 years old, this age is consistent with the study of Messinger YH et al., which shows that more than 97% of tumors in children under 3 years old are type I and in children under 4 years old are type II.2 This was the cause of the initial misdiagnosis.
Invasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report
Published in Acta Chirurgica Belgica, 2021
A. E. Frick, H. Decaluwé, B. Weynand, M. Proesmans, D. Van Raemdonck
The development of a congenital parenchymatous lung disorder, known as congenital pulmonary airway malformation (CPAM), has previously been described as congenital cystic adenomatoid malformation (CCAM) with an estimated incidence at 1:25,000–1:35,000 births. The classification by Stocker et al. in 1977 originally described 3 different subtypes and was later expanded and renamed into 5 subtypes on the basis of clinical and pathological features [1–3]. Most congenital CPAMs are uncommon and primarily identified in infants and in adults. Depending on the type of CPAM, clinical presentation and prognosis are different. Typical symptoms are recurrent pulmonary infection, productive cough, and hemoptysis. Some patients remain asymptomatic [4]. A number of reports have been published describing the association of adenocarcinoma with type 1 CPAM. The occurrence of rhabdomyosarcoma or pleuropulmonary blastoma arising in a CPAM has also been rarely documented [5,6].
Congenital Pulmonary Airway Malformation – 19-Year Experience from a Tertiary Care Center in India
Published in Fetal and Pediatric Pathology, 2019
Hema Kini, Saraswathy Sreeram, Saumya Shukla, Sadashiva Rao, Kausalya Sahu, Deepa Adiga, Pooja Suresh
CPAMs can show associated lesions on microscopy. Metaplastic change can be seen in cysts. Cartilage is usually detected in type 0 CPAM, however, chondroid metaplasia can be seen in any type, as was seen in one case with type 2 CPAM in our study (Table 1, case 7). About 5–10% of type 2 CPAMs show cartilage [18]. Unresected or incompletely resected lesions of type 1 CPAM are surmised to have some risk for adenocarcinoma (0.7%). Moreover, meticulous microscopic examination, especially of large cysts like in types 1 and 4, is crucial to detect malignant foci of spindled cells or immature chondroid, which is diagnostic of cystic pleuropulmonary blastoma (PPB) [15]. In fact, any degree of high cellularity is taken to be Type 1 PPB. The absence of blastemal component is touted to be a sign of regression of malignancy in PPB1, leading the morphology seen in type 4 CPAM [8]. Immunohistochemical reaction with desmin, myogenin or myoD1 is indicated to differentiate PPB from CPAM, because the mesenchymal fibroblasts in CPAM are not reactive. This distinction is important because cystic PPBs are known to recur with an increased grade of malignancy if incompletely resected [8, 15]. However, in our study, blastemal component was not detected in any case. Blastema is the diffuse or solid nests of small round blue cells, which are distinctly different from malignant spindle cells, which are pleomorphic, elongated cells.