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Presented by Fred Askin, M.D. and prepared by Natasha Rekhtman, M.D., Ph.D.
Case 1: This patient was a 28 year-old television news reporter.
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1. Question
Week 231: Case 1
This patient was a 28 year-old television news reporter. She was discovered to have 4 cm subpleural nodule in the lower level of the right lung when radiographs were taken following an automobile accident.images/7 15 05 case 1 2.jpg
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images/7 15 05 case 1 5.jpgCorrect
Answer: Well-differentiated fetal adenocarcinoma
Histology: This lesion has a characteristic appearance with branching glandular elements composed of tubules and glycogen-rich, non-ciliated cells that resemble fetal lung tubules. Glycogen vacuoles, either in a subnuclear or supranuclear location may give the tumor an “endometrioid” appearance. A characteristic feature of this lesion is rounded morules of polygonal cells with abundant eosinophilic and finely granular cytoplasm.
Discussion: The latest World Health Organization (WHO) Classification places this lesion under the category of Adenocarcinoma with the relatively unhelpful note that “when fetal adenocarcinoma is associated with a sarcomatous primitive blastemal stroma, the tumor is classified as pulmonary blastoma”. There is, however, evidence that WDFA and classic biphasic pulmonary blastoma are related. When WDFA is carefully defined as a glandular lesion with morules, immunocytochemical studies often show beta-catenin nuclear and cytoplasmic staining in both classic biphasic pulmonary blastoma and WDFA.
Reference(s):
– Travis WD, Brambella E, Muller-Hermelink HK, and Harris CC, Editors. Pathology and genetics of the lung, pleura, thymus, and heart. IARC Press, Lyon, France, 2004.
– Sekine S, Shabatta T, Matsuno Y, et al. Beta-catenin mutations in pulmonary blastomas: Association with morule formation. J Pathol 2003; 200:214-221.Incorrect
Answer: Well-differentiated fetal adenocarcinoma
Histology: This lesion has a characteristic appearance with branching glandular elements composed of tubules and glycogen-rich, non-ciliated cells that resemble fetal lung tubules. Glycogen vacuoles, either in a subnuclear or supranuclear location may give the tumor an “endometrioid” appearance. A characteristic feature of this lesion is rounded morules of polygonal cells with abundant eosinophilic and finely granular cytoplasm.
Discussion: The latest World Health Organization (WHO) Classification places this lesion under the category of Adenocarcinoma with the relatively unhelpful note that “when fetal adenocarcinoma is associated with a sarcomatous primitive blastemal stroma, the tumor is classified as pulmonary blastoma”. There is, however, evidence that WDFA and classic biphasic pulmonary blastoma are related. When WDFA is carefully defined as a glandular lesion with morules, immunocytochemical studies often show beta-catenin nuclear and cytoplasmic staining in both classic biphasic pulmonary blastoma and WDFA.
Reference(s):
– Travis WD, Brambella E, Muller-Hermelink HK, and Harris CC, Editors. Pathology and genetics of the lung, pleura, thymus, and heart. IARC Press, Lyon, France, 2004.
– Sekine S, Shabatta T, Matsuno Y, et al. Beta-catenin mutations in pulmonary blastomas: Association with morule formation. J Pathol 2003; 200:214-221.
