Well-differentiated fetal adenocarcinoma (WDFA) is a rare tumor of the lung, which has gone by many names over the years. The lesion was first described by Kradin et al., in 1982, who called it "pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung)." Since then, there have been at least 65 cases reported in the literature. Although there has been no consensus in the literature as to the best pathological term for this entity, the most recent World Health Organization classification of lung and pleural tumors uses the term well-differentiated fetal adenocarcinoma. Characteristically, this lesion consists of an epithelium, which recapitulates fetal lung at 3-5 months of gestation and demonstrates neuroendocrine differentiation. Although the classic age range is 30-40 years, there have been seven reports of WDFA in the pediatric age. We report an additional pediatric case of this tumor and review the pediatric cases in the existing literature.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1007/s10024-003-4046-x | DOI Listing |
Am J Surg Pathol
February 2024
Department of Human Genetics.
Germline and somatic pathogenic variants (PVs) in DICER1 , encoding a miRNA biogenesis protein, are associated with a wide variety of highly specific pathologic entities. The lung tumors pleuropulmonary blastoma, pulmonary blastoma (PB), and well-differentiated fetal lung adenocarcinoma (WDFLAC) are all known to harbor DICER1 biallelic variants (loss of function and/or somatic hotspot missense mutations), and all share pathologic features reminiscent of the immature lung. However, the role of DICER1 PVs in non-small cell lung cancer (NSCLC) is relatively unknown.
View Article and Find Full Text PDFJ Surg Case Rep
May 2023
Department of Surgery and Surgical Oncology, HCA East Florida, Fort Lauderdale, Florida, USA.
Hibernomas are rare and benign lipomatous tumors which consist of lobules of cells derived from brown fat. Less than 200 overall cases are reported in the literature which necessitates the need to report more cases for better understanding of this neoplasm. Hibernomas are slowly progressing, painless tumors most frequently located in the subcutaneous thigh, upper trunk, and neck.
View Article and Find Full Text PDFCancer Treat Res Commun
December 2022
Department of Pathology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia.
Background: Endometrial cancer in young women (less than 40-year-old) is associated with anovulatory menses, polycystic ovarian syndrome (PCOS) and subfertility. Endometrial cancer occurring in a miscarriage is rare. We highlight a case of endometrial cancer occurring during miscarriage of a non-viable pregnancy, its management and the outcome.
View Article and Find Full Text PDFJ Pediatr Surg
October 2022
Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, 38105; Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Memphis, Tennessee, 38105. Electronic address:
J Pediatr Surg
October 2022
Nemours Children's Hospital, 1600 Rockland Road, Wilmington DE 19803, United States. Electronic address:
Background: Hepatoblastoma (HB) requires surgical resection for cure, but only 20-30% of patients have resectable disease at diagnosis. Patients who undergo partial hepatectomy at diagnosis have historically received 4-6 cycles of adjuvant chemotherapy; however, those with 100% well-differentiated fetal histology (WDF) have been observed to have excellent outcomes when treated with surgery alone.
Patients And Methods: Patients on the Children's Oncology Group non randomized, multicenter phase III study, AHEP0731, were stratified based on Evan's stage, tumor histology, and serum alpha-fetoprotein level at diagnosis.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!