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Presented by Fred Askin, M.D. and prepared by Natasha Rekhtman, M.D., Ph.D.
Case 6: The patient is a 2 year-old child with a large mass in the left lung.
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Week 231: Case 6
The patient is a 2 year-old child with a large mass in the left lung.images/7 15 05 case 6 2.jpg
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images/7 15 05 case 6 5.jpgCorrect
Answer: Congenital peribronchial myofibroblastic tumor (bronchopulmonary fibrosarcoma)
Histology: The lung parenchyma is replaced by fascicles of uniform spindle cells. These fascicles often intersect, with or without a so-called “herringbone” pattern. The tumor cell nuclei are elongated and have finely dispersed chromatin. Pleomorphism and anaplasia are absent and there is variable mitotic activity. The tumor may invade bronchi, may diffusely obliterate lung parenchyma or form islands or nodules of spindle cells separated by areas of uninvolved parenchyma. As in this case, tumor growth in the septa may be a prominent feature.
Discussion: This lesion usually occurs as a congenital lesion and may be associated with hydramnios or a non-immune hydrops. It should be possible to detect this lesion by prenatal ultrasound. Although the tumor has many histologic features similar to those of congenital mesoblastic nephroma¸ there are no reports to this date of the detection of the t(12;15) t(13;q25-26) translocation (seen in congenital mesoblastic nephroma) having been found in the congenital bronchopulmonary fibrosarcoma. (This case is used through the courtesy of Dr. JC Manivel).
Reference(s):
– Petlinato G, Manivel JC, Saldane MJ et al. Hum Pathol 1989, 20: 463-471.Incorrect
Answer: Congenital peribronchial myofibroblastic tumor (bronchopulmonary fibrosarcoma)
Histology: The lung parenchyma is replaced by fascicles of uniform spindle cells. These fascicles often intersect, with or without a so-called “herringbone” pattern. The tumor cell nuclei are elongated and have finely dispersed chromatin. Pleomorphism and anaplasia are absent and there is variable mitotic activity. The tumor may invade bronchi, may diffusely obliterate lung parenchyma or form islands or nodules of spindle cells separated by areas of uninvolved parenchyma. As in this case, tumor growth in the septa may be a prominent feature.
Discussion: This lesion usually occurs as a congenital lesion and may be associated with hydramnios or a non-immune hydrops. It should be possible to detect this lesion by prenatal ultrasound. Although the tumor has many histologic features similar to those of congenital mesoblastic nephroma¸ there are no reports to this date of the detection of the t(12;15) t(13;q25-26) translocation (seen in congenital mesoblastic nephroma) having been found in the congenital bronchopulmonary fibrosarcoma. (This case is used through the courtesy of Dr. JC Manivel).
Reference(s):
– Petlinato G, Manivel JC, Saldane MJ et al. Hum Pathol 1989, 20: 463-471.
