Primary pulmonary osteosarcoma: case report and molecular analysis.
BACKGROUND
Primary pulmonary osteosarcoma is an extremely rare malignancy. To date, only 12 cases have been reported, with a high mortality rate. The authors report on a newly diagnosed patient and describe investigations that were performed using immunohistochemistry and comparative genomic hybridization (CGH).


METHODS
The clinical course of a woman age 37 years is presented. Along with routine histologic examination, immunohistochemistry was used to demonstrate differentiation-associated proteins, oncoproteins, and other markers; CGH analysis for genomic alterations; and histochemistry to demonstrate alkaline phosphatase activity.


RESULTS
Immunohistochemical analysis showed varying expression patterns using antibodies against a panel of tumor markers. Most notable was high overexpression of BCL-2 and cyclin D. CGH analysis showed that this neoplasm contained a much higher level of genetic aberrations compared with skeletal osteosarcoma.


CONCLUSIONS
This tumor exhibited features common to skeletal osteosarcomas but also had some unique features. Genome analysis suggests that this tumor has several genetic aberrations in common with extraskeletal osteosarcoma. The novel regions of instability identified within the tumor genome may contribute toward the unique tumor phenotype and relative chemoresistance.
Department of Pathology, University of Aberdeen, Aberdeen, Scotland, United Kingdom.
A D
AD
Chapman
S C
SC
Pritchard
W W
WW
Yap
P H
PH
Rooney
J S
JS
Cockburn
A W
AW
Hutcheon
M C
MC
Nicolson
K M
KM
Kerr
H L
HL
McLeod
D000328:Adult; D015415:Biomarkers; D001859:Bone Neoplasms / Q000556:secondary*; D002869:Chromosome Aberrations; D003131:Combined Modality Therapy; D019938:Cyclin D1 / Q000378:metabolism; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000235:genetics* / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D009693:Nucleic Acid Hybridization; D012516:Osteosarcoma / Q000235:genetics* / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D010641:Phenotype; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000378:metabolism
D015415:Biomarkers; D019253:Proto-Oncogene Proteins c-bcl-2; D019938:Cyclin D1