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Bone & Soft Tissue Pathology
Sunday, March 21, 2010, 7:30 PM
Salon 1 and Balconies
Clinical histories are printed below.
Click on the case numbers for text and references of each case.
Click on each slide thumbnail image for an enlarged view
Advances in the Molecular Biology of Bone and Soft Tissue Tumors
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Moderator:
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ANDREW E. ROSENBERG
Massachusetts General Hospital
Boston, MA
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Disclosure:
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In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Symposium) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. The speakers listed below have indicated they have nothing to disclose.
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Panelists:
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ROBERTO ANTONIO GARCIA, Mount Sinai Medical Center, New York, NY
ANDREW E. HORVAI, University of California, San Francisco, San Francisco, CA
VAIYAPURI P. SUMATHI, The Royal Orthopaedic Hospital NHS Trust, University of Birmingham, Birmingham, UK
YONG-KOO PARK, Kyung Hee University Hospital, Seoul, South Korea
JULIE FANBURG-SMITH, AIP Laboratories, Silver Spring, MD
JOHN R. GOLDBLUM, Cleveland Clinic, Cleveland, OH
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Clinical histories are displayed below.
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for Text and References
Submitted by: Julie Fanburg-Smith - AIP Laboratories, Silver Spring, MD
A twenty-four year old female presented with back pain. She was found to have a left posterior chest wall mass, primarily involving the L1 vertebra and the adjacent rib. The mass extended into soft tissue. A biopsy of the mass was performed that measured 3.5 cm (see slide). A definitive resection was performed elsewhere. Despite report that the patient received chemotherapy and radiation therapy, the patient died of disease one year later.
Case 1 - Slide 1
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for Text and References
Submitted by: Vaiyapuri P. Sumathi - The Royal Orthopaedic Hospital NHS Trust, University of Birmingham, Birmingham, UK
The patient is a 40 year old man who was referred to the Royal Orthopaedic Hospital for evaluation of left knee pain. The pain was present for six months but had become worse at the time of presentation. Imaging studies revealed a pathological fracture through a large lytic lesion in the distal femur. Following a biopsy the distal femur was resected. He was under close observation and 10 months following surgery he complained of left knee pain.Subsequently, 14 months following surgery he complained of chest pain and investigations revealed multiple nodules in both lung fields. One of the nodules was biopsied.
Case 2 - Slide 1
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Case 2 - Figure 1
Coronal STIR image demonstrates an expansile lesion in the subarticular location of the distal femur. It shows some internal cystic change with a multi-locular appearance and cortical thinning. |
Case 2 - Figure 2
Macroscopic appearance of the bisected specimen of the left distal femur shows a fairly well circumscribed large haemorrhagic tumour involving the epi-metaphysis. The lesion contains several blood filled cystic spaces. Tan coloured solid areas are noted towards the edge of the tumour. The tumour has breached the cortex laterally with soft tissue extension. |
Case 2 - Figure 3
Lower power view of the lesion shows a narrow rim of reactive new bone formation at the edge of the lesion. Cystic spaces resembling aneurysmal bone cyst are prominent. |
Case 2 - Figure 4
High power view which shows a mixture of cytologically bland mononuclear cells which have indistinct cell borders. There is very little intercellular matrix. The multinucleated giant cells have numerous nuclei and resemble the nuclei of the stromal cells. |
Case 2 - Figure 5
Section shows vascular invasion. The tumour emboli consist of mononuclear cells and osteoclast type giant cells. |
Case 2 - Figure 6
Low power view of the resected lung nodule showed a striking resemblance to the primary tumour and consists of haemorrhagic cystic and solid areas. |
Case 2 - Figure 7
High power view of the lung nodule composed of mononuclear stromal cells and multinucleated osteoclast - like giant cells. |
for Text and References
Submitted by: Andrew Horvai - University of California, San Francisco, CA
A 65 year old woman with vague abdominal discomfort was found to have a 10 cm tumor of the retroperitoneum. The tumor was resected.
Case 3 - Figure 1
Gross examination demonstrates a fatty and myxoid neoplasm with areas of hemorrhage. |
Case 3 - Figure 2
Low magnification shows myxoid matrix and areas of cyst formation reminiscent of a so-called "pulmonary edema" pattern. |
Case 3 - Figure 3
Some areas of tumor demonstrate immature fat cells (lipoblasts), a subset of which are reminiscent of signet ring cells. |
Case 3 - Figure 4
A transitional area of the tumor shows juxtaposition of myxoid areas with small lipoblasts to cells with higher grade cytomorphology. |
Case 3 - Figure 5
Higher grade areas have more fibrous stroma and striking nuclear pleomorphism, yet retain adipocytic differentiation |
Case 3 - Figure 6
Tumor cells show nuclear positivity with antibodies to CDK4 and MDM2 |
Case 3 - Figure 7
The tumor shows high level amplification of chromosome region 12q13-15 by array comparative genomic hybridization |
for Text and References
Submitted by: Roberto Antonio Garcia - Mount Sinai Medical Center, New York, NY
A 50 year old male presented with a large mass in the right iliac wing. He recently underwent right nephrectomy for renal cell carcinoma. A resection of the iliac mass was performed.
Case 4 - Slide 1
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Case 4 - Figure 1
Preoperative radiograph showing a large lytic lesion in the right iliac wing. |
Case 4 - Figure 2
The resected tumor is well circumscribed with a fleshy, tan-pink to brown cut surface. |
Case 4 - Figure 3
Low power view shows a nodular configuration with discernible giant cells. |
Case 4 - Figure 4
Intermediate power showing fibrovascular stroma with numerous giant cells, abundant hemosiderin deposition and focal hemorrhage. |
Case 4 - Figure 5
High power showing a proliferation of bland spindle cells with osteoclast-like giant cells aggregated around areas of hemorrhage. |
Case 4 - Figure 6
Very high power view of the vascular spindle cell stroma with osteoclast-like giant cells and hemosiderin-laden macrophages. |
Case 4 - Figure 7
Low power view of the trabecular bone adjacent to the tumor showing osteopenia and normocellular marrow. |
Case 4 - Figure 8
Intermediate power showing tunneling resorption of trabecular bone with associated fibrosis (dissecting osteitis). |
Case 4 - Figure 9
High power shows increased bone remodeling with marked osteoblastic and osteoclastic activity, irregular cement lines and peritrabecular fibrosis. |
Case 4 - Figure 10
Very high power demonstrating tunneling resorption by osteoclasts (cutting cones) with fibrosis and increased osteoblastic activity. |
for Text and References
Submitted by: Yong-Koo Park - Kyung Hee University Hospital, Seoul, South Korea
A 56-year-old woman presented with a 3-year history of diffuse, increasing bone pain at multiple locations. She also suffered from generalized fatigue and difficulty walking.
There was a more recent history of protruding mass on her right foot sole.
Laboratory data at the admission showed the following: serum phosphorus 1.1 mg/dl (normal range 2.5-4.5 mg/dl), calcium 9 mg/dl (normal range 8.2-10.8 mg/dl) and alkaline phosphatase 625 IU/l (normal range 96-254 IU/l). Urinary excretion of phosphorus was 392 mg/day and tubular reabsorption of phosphorus was markedly decreased at 59.6% (normal range 92-95%).
Pelvic X-ray revealed a marked decrease in bone densities and multiple pseudogractures.
Plain radiograph of the foot showed marked osteopenia and an ill-defined lobulated mass with pressure erosion of the second and third metatarsal bones.
A bone biopsy was performed.
Case 5 - Slide 1
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Case 5 - Figure 1
Heavy calcification and granulation tissue-like blood vessel proliferation are noted. |
Case 5 - Figure 2
Higher magnification shows prominent capillary endothelial cells and loose myxoid stroma and some scattered histiocyes. |
Case 5 - Figure 3
In the area, there is cystic change and some necrosis with calcification. |
Case 5 - Figure 4
Higher magnification shows the cyst wall with calcification. |
Case 5 - Figure 5
In some areas, the cyst wall is composed of some histiocytes and calcified materials. |
Case 5 - Figure 6
In solid parts, cellular areas show thick blood vessel calcification and some histiocytes. |
Case 5 - Figure 7
Higher magnification shows osteoclast-like giant cells and some plasma cells. |
Case 5 - Figure 8
There is also noted prominent hemangiopericytomatous blood vessel proliferation and some giant cell calcification. |
for Text and References
Submitted by: John R. Goldblum - Cleveland Clinic, Cleveland, OH
12-year-old male with a 3.5-cm soft tissue mass of the left forearm.
Immunophenotype / molecular genetic findings
AE1/AE3 negative
Desmin focal positive
CD99 focal positive
EMA negative
S100 protein negative
SMA negative
EWS FISH positive
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