Pediatr Dev Pathol. 2004 Jan-Feb;7(1):35-8. PMID: 15255033 [PubMed - indexed for MEDLINE] Related articles
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ABSTRACTS PRESENTED AT CAP'10 MEETING
A Rare Case of Giant Cell Tumor in the Thumb of an 82-Year-Old Woman
Author Block: Aram Millstein, MD1 (aram.millstein@ucdmc.ucdavis.edu); Robert Tamurian, MD2; John Bishop, MD1; Dariusz Borys, MD.1 Departments of 1Pathology and 2Orthopedic Surgery, UC Davis, Sacramento, Calif.
Giant cell tumor of the bone is a benign tumor that commonly occurs in skeletally mature women with a peak incidence between 20 and 45 years of age. Although this tumor can occur anywhere in the skeleton, its most common locations are the distal femur, proximal tibia, distal radius, and sacrum. We present a case of a giant cell tumor occurring in the proximal phalanx of the thumb in an 82-year-old woman with a history of breast cancer. Imaging studies showed an expansile lytic lesion with cortical disruption of the proximal phalanx of the thumb. There was a soft tissue prominence suggesting an associated soft tissue mass. The lesion had the typical radiographic appearance of a giant cell tumor. Given the patient's age and history of breast cancer, the differential diagnosis consisted of a tumor metastasis, followed by a giant cell tumor. An excisional biopsy was performed and demonstrated a proliferation of round-to-oval and elongated mononuclear cells mixed with numerous osteoclast-like giant cells. This histology was characteristic for both a giant cell tumor and a brown tumor. As the patient’s calcium levels were in the normal range, a brown tumor was excluded. We found 27 articles reporting giant cell tumors in the carpals, metacarpals, or phalanges of the hand. There were only 6 cases of giant cell tumor in the thumb: 1 in the distal phalanx, 1 in the proximal phalanx, and 4 in the first metacarpal. Of these 6 cases, only one was older than age 45 (Table).
Metastatic Hepatocellular Carcinoma to the Bone Occurring in a 13-Year-Old Adolescent Boy
(Poster No. 36)
Jose Gorospe, MD1 (jose.gorospe@ucdmc.ucdavis.edu); John Bishop,MD1; George Rab, MD2; Dariusz Borys, MD.1 Departments of 1Pathology and Laboratory Medicine and 2Orthopedics, University of California Davis at Sacramento.
Hepatocellular carcinoma is a relatively uncommon condition in children and adolescents with an incidence rate of 0.5 to 1.0 case per million. Few cases of hepatocellular carcinoma with bone metastases in children have been reported, and most studies are adult series. We present a case of metastatic hepatocellular carcinoma in a 13-year-old adolescent boy who initially presented with a pathologic femur fracture. The boy first presented with leg pain after a fall at school with initial xrays
demonstrating a right femur fracture and possible underlying trochanteric bone cyst. To further evaluate the bone cyst, a pelvic computed tomography scan was completed, which incidentally revealed
a large hepatic mass. Curettage of the bone lesion was obtained during surgical reduction and fixation of the fracture. Microscopically, the curettage demonstrated neoplastic cells forming sheets and trabeculae
(Figure 9) with extensive necrosis, moderate nuclear atypia, and mitotic activity. The
immunohistochemical stains for carcinoembryonic antigen, hepatocyte paraffin 1, anti-cytokeratin (CAM 5.2), and a-fetoprotein (focally) were positive. Based on these findings, a diagnosis of metastatic
hepatocellular carcinoma to the bone was rendered with agreement from external consultation. To our knowledge, this is the first described case of hepatocellular carcinoma in a child diagnosed on bone biopsy.
Soft Tissue Angiosarcoma With Abundant Metaplastic Bone Formation Mimicking Osteosarcoma
(Poster No. 32)
Heidi A. Jess, MD1 (heidi.jess@ucdmc.ucdavis.edu); Robert Tamurian,MD2; Luigia Abramovici, MD3; Dariusz A. Borys, MD.1 Departments of 1Pathology and 2Orthopedic Surgery, University of California Davis Medical Center, Sacramento; 3Department of Pathology, New York University Hospital for Joint Diseases, New York.
Angiosarcomas represent fewer than 1% of soft tissue sarcomas andmost commonly occur in cutaneous tissue. Fewer than 25% of angiosarcomas occur in the deep soft tissue and then most commonly in the deep muscles of the lower extremities. Typically, angiosarcomas consist predominantly of epitheliod cells with abundant eosinophilic cytoplasm and large vesicular nuclei arranged in sheets, nests, cords, or rudimentary, freely anastomosing vascular channels with dilated vascular spaces. Metaplastic bone formation within angiosarcoma has not been reported in the literature and is a very unusual characteristic of this case. We describe a case of soft tissue angiosarcoma with metaplastic bone formation that
was originally diagnosed as extraskeletal osteosarcoma by biopsy at an outside facility. The patient was a 57-year-old man who was performing heavy labor when he noted a ‘‘pop’’ in his right thigh, which was followed by mild to severe, sharp, burning pain. An x-ray of the right lower extremity showed a large soft tissue mass. A biopsy was performed and showed abundant osteoid bordered by small, flat osteoblasts admixed with neoplastic cells that formed nests and irregular vascular spaces with a hobnail appearance (Figure 70). The neoplastic cells had abundant eosinophilic cytoplasm, irregular nuclear membranes, and hyperchromatic nuclei. The initial diagnosis was extraskeletal osteosarcoma. Review of the pathology at the University of California Davis Medical Center revealed that the neoplastic cells expressed vascular immunohistochemical markers CD31 and CD34. We determined the osteoblasts to be reactive in nature. A diagnosis of soft tissue angiosarcoma with abundantmetaplastic bone formation was made.
ABSTRACT ACCEPTED FOR PRESENTATION AT VISION'10
Application of Virtual Pathology with Radiology Correlation in Daily Clinical Orthopedic Pathology Practice and Research
Dariusz Borys M.D.
Pathology, University of California davis
Telepathology is the use of telecommunications technology to facilitate the transfer of image-rich pathology data between remote locations for the purposes of diagnosis, education, and research. Departmental use of the Aperio virtual system has multiple practical application in daily clinical practice and research. Presenting histolopathologic slides during orthopedic tumor board gives a better view of the case from different aspects and makes possible correlation with virtual radiology and gross pictures. This provides opportunity to review slides anywhere, anytime and save time for taking pictures for power point presentation. Other positive aspect of virtual slide application is easy communication with other pathologists from outside institutions for second opinion and knowledge exchange. Virtual slides review is powerful teaching tool for colleagues and residents. Scanned slides for research purposes give better view for immunohistochemistry stain adjustment and percentage expression. All these applications make pathology slides easy to access and give freedom for pathologists to review them anytime, anywhere and correlate with radiological and clinical data the same time.
CTOS ANNUAL MEETING IN PARIS PRESENTATIONS:
896336 POSTOPERATIVE EXTERNAL BEAM RADIATION HAS NO IMPACT ON
SURVIVAL AMONG PATIENTS WITH RETROPERITONEAL SARCOMA
W.H. Tseng; S.R. Martinez; L. Do; R.M. Tamurian; D. Borys; J.E. Goodnight; R.J. Canter
825910 EXPRESSION OF MTA1, FIP 1, RBM9 AND CTR9 IN SMALL BLUE CELL
TUMORS IN CHILDREN
D. Borys; R. Canter; J. Bishop; L. Wilson; R. Tamurian; A. Greco
ABSTRACT ACCEPTED FOR PRESENTATION
GENE DELETION UNDERLIES LOSS OF P16 EXPRESSION IN OSTEOSARCOMA TUMORS WITH POOR RESPONSE TO NEOADJUVANT CHEMOTHERAPY
1. Dariusz Borys1, 2. Robert Canter2, 3. Jeffrey Gregg1, 4. Ben Hoch3, 5. Ryan Davis1 and 6. Andrew Horvai4
Background: Although pathologic response to neoadjuvant chemotherapy predicts survival among patients with osteosarcoma (OS), there are currently no established molecular markers to predict response to chemotherapy. We have previously shown that immunohistochemical (IHC) expression of p16 (the product of the CDKNA2 gene) in OS is significantly correlated with pathologic response to neoadjuvant chemotherapy. The objective of the current study was to assess for copy number alternations at the CDKNA2 gene on chromosome 9 in p16 IHC-expressing and IHC-non-expressing OS specimens. Design: Genomic DNA was obtained from paraffin-embedded pretreatment biopsy specimens of OS patients prior to receiving neoadjuvant chemotherapy. We selected three cases of p16 IHC-expressing tumors with pathologic response to chemotherapy ( 90% tumor necrosis) and three cases of P16 IHCnon- expressing tumors without pathologic response (< 90% tumor necrosis). Human genomic array was performed on 44K arrays using 80 Gb per human genome at 30X coverage. The CDKN2A locus (p16) on chromosome 9 was probed with A_14_P129522 chr9, A_14_P130650 chr9 and A_14_P112983 chr9. Results: Four of the 6 cases yielded complete copy number information (2 p16 IHC-expressing, 2 p16 IHC-non-expressing). Among p16 expressing tumors, both demonstrated a wildtype CDKNA2 locus, while one of two p16 non-expressing tumors had a deletion on the short arm of chromosome 9 including the CDKNA2 locus. Conclusion: Deletion of the entire CDKNA2 locus explains loss of p16 expression in some OS patients with poor response to neoadjuvant chemotherapy. However diverse mechanisms may be responsible for loss of p16 expression across the population of OS patients. Additional molecular studies are needed to validate these findings. . .
