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Presentation Clinique

Clinical setting

 
  M 7 ans , tumeur du fémur, biopsie chirurgicale M 7 yo, tumor of the femur, surgical biopsy.  
       
 

Radiologie

Radiology

 
         
     
 

Lesion metaphysaire, atteignant le cartilage de conjugaison.

Lesion of metaphysis, reaching the cartilaginous plate

 

Os cortical soufflé

Cortical bone thinned

Presence d'images kystiques, avec des niveaux.

Presence of cystic lesions, with level pattern.

Microscopie

Microscopy

Deux specimens biopsiques soumis

 

Two biopsies submitted

 

Espaces enfractueux, hematiques, et cellularité pléomorphe avec mitoses atypiques.

 

Irregular cystic spaces and pleomorphic cellular pattern with atypical mitotic figures.

 

Foyer de formation d'ostéoide et dé maturation osseuse avec figure de mitose.

 

Foci of osteoid and bone formation, with mitotic figures.

 

Foyers de production d'osteoide atypique

 

Atypical osteoid production

 

 

 

 

 

Diagnostic proposé :

Proposed Diagnosis:

 

Ostéosarcome télangiectasique

 

Telangiectatic osteosarcoma

 

 

 

 

 
  Telangiectatic osteosarcoma
(Ref. Diagnostic Histopathology of Tumors, CDM Fletcher, Churchill Livingstone, 2nd Ed.)


Telangiectatic osteosarcoma forms approximately 4% of all osteosarcomas in the Mayo Clinic files. There is controversy in the literature concerning the importance of differentiating telangiectatic osteosarcoma from conventional osteosarcoma. Part of this controversy relates to the different criteria used to make the diagnosis in different institutions. The criteria that we use are as follows:

1. The roentgenogram shows a purely lytic destructive lesion, usually in the metaphysis of a long bone of a young patient.
2. The gross appearance is that of a cavity separated by septa or a cavity containing a blood clot.
3. Microscopically, there are septa separating spaces and simulating the appearance of an aneurysmal bone cyst. However, the lining cells are pleomorphic. Bone production by the tumor cells is minimal.

We do not include osteosarcomas that are partly telangiectatic in this group. In the original study from the Mayo Clinic, it was thought that telangiectatic osteosarcoma was associated with an extremely poor prognosis. However, a series from Memorial Hospital suggested that the prognosis for telangiectatic osteosarcoma is the same as for conventional osteosarcoma. A later study from the Mayo Clinic found that the prognosis for more recent patients with telangiectatic osteosarcoma is the same as for those who have conventional osteosarcoma. This did not seem to be associated with the use of chemotherapy; however, in our experience, telangiectatic osteosarcoma is extremely chemosensitive.

We believe that telangiectatic osteosarcoma should still be retained as a separate entity because of the characteristic gross and microscopic features. Most importantly, it helps to remind us of the possibility of telangiectatic osteosarcoma when an aneurysmal bone cyst is diagnosed. Aneurysmal bone cysts and telangiectatic osteosarcomas resemble each other in many respects, including roentgenographic, gross, and low power microscopic features. The only difference is the presence of markedly atypical cells in the septa of telangiectatic osteosarcoma.

 
 

 

Some abstracts about Telangiectatic Osteosarcoma

 
 


Radiology. 2003 Nov;229(2):545-53. Epub 2003 Sep 25.


Telangiectatic osteosarcoma: radiologic-pathologic comparison.


Murphey MD, wan Jaovisidha S, Temple HT, Gannon FH, Jelinek JS, Malawer MM.

Department of Radiologic Pathology, Armed Forces Institute of Pathology, 6825
16th Street NW, Bldg 54, Rm M127A, Washington, DC 20306, USA.
murphey@afip.osd.mil

PURPOSE: To describe the imaging characteristics of a large series of
telangiectatic osteosarcomas with pathologic findings for comparison. MATERIALS
AND METHODS: The authors retrospectively reviewed 40 pathologically confirmed
telangiectatic osteosarcomas. Patient demographics and images from radiography
(n = 36), bone scintigraphy (n = 17), angiography (n = 4), computed tomography
(CT) (n = 25), and magnetic resonance (MR) imaging (n = 27) were evaluated by
three authors in consensus for lesion location, size, and intrinsic
characteristics. There were 27 men (68%) and 13 women (32%) in the study, with
an age range of 4-83 years (mean age, 24 years). RESULTS: Lesions frequently
affected the femur, tibia, and humerus. Radiographs showed geographic bone
lysis, a wide zone of transition, and matrix mineralization. CT demonstrated low
attenuation, MR demonstrated high signal intensity on T2-weighted images, and
both demonstrated hemorrhage, which simulated the appearance of aneurysmal bone
cyst. Viable sarcomatous tissue surrounding hemorrhagic and/or necrotic regions
was best seen at contrast material-enhanced CT and MR imaging, with thick
peripheral, septal, and nodular enhancement in all cases. Subtle matrix
mineralization in this viable tissue was best seen at CT. An associated
soft-tissue mass was also seen in 19 of 25 cases (76%) at CT and in 24 of 27
cases (89%) at MR imaging. CONCLUSION: CT and MR imaging findings of
telangiectatic osteosarcoma often include thick nodular tissue (and matrix
mineralization at CT) in a largely hemorrhagic and/or necrotic osseous lesion
with an associated soft-tissue mass, which allows distinction from aneurysmal
bone cyst.


Cancer. 2003 Jun 15;97(12):3068-75.

Neoadjuvant chemotherapy for high-grade central osteosarcoma of the extremity.
Histologic response to preoperative chemotherapy correlates with histologic
subtype of the tumor.

Bacci G, Bertoni F, Longhi A, Ferrari S, Forni C, Biagini R, Bacchini P, Donati
D, Manfrini M, Bernini G, Lari S.

Department of Musculoskeletal Oncology, Istituto Ortopedico Rizzoli, Bologna,
Italy. gaetano.bacci@ior.it

BACKGROUND: In primary central high-grade osteosarcoma, a number of distinct
subtypes have been identified, but little is known about the response to
chemotherapy. METHODS: The authors investigated whether the subtypes correlated
with histologic response to chemotherapy in 1058 patients with osteosarcoma of
the extremities who were treated with neoadjuvant chemotherapy over the last 20
years. The tumors were classified as osteoblastic (70%), chondroblastic (13%),
fibroblastic (9%), and telangiectatic (6%). At diagnosis, 911 patients had
localized disease and 147 had resectable lung metastases. RESULTS: The response
to preoperative chemotherapy was good (90% or more tumor necrosis) in 59% of
patients and poor (< 90% tumor necrosis) in 41% of patients. The rate of good
responses was significantly higher (P = 0.0001) in the fibroblastic (83%) and
telangiectatic (80%) tumors and significantly lower in chondroblastic tumors
(43%). Prognosis was significantly correlated with the histologic subtypes. The
5-year overall survival rate was significantly higher (P = 0.0001) in
fibroblastic (83%) and telangiectatic (75%) tumors than in osteoblastic (62%)
and chondroblastic (60%) tumors. In all subtypes, except for the chondroblastic
subtype, the 5-year overall survival rate was significantly higher (P = 0.0001)
in good responders P = 0.0001 (68%) than in poor responders (52%). CONCLUSIONS:
The authors concluded that the histologic subtype of primary central high-grade
osteosarcoma of the extremity was strictly correlated with histologic response
to chemotherapy and probably, as a consequence, also with prognosis. Further
studies are needed to establish whether these results justify a specific
therapeutic approach based on the histologic subtype of the tumor. Copyright
2003 American Cancer Society.

 

Zhonghua Bing Li Xue Za Zhi. 2002 Jun;31(3):213-6.

Telangiectatic osteosarcoma: a clinicopathologic analysis of 14 cases.

Liu H, Huang X.

Department of Pathology, Beijing Jishuitan Hospital, Beijing 100035, China.

OBJECTIVE: To investigate the clinicopathologic characteritics and prognosis of
telangiectatic osteosarcoma. METHODS: The clinical and pathological data of 14
cases of telangiectatic osteosarcoma were reviewed. RESULTS: Most of these
patients were male (9/14). The mean age was 23 years. Most tumors were located
in the lower extremities (10/14). The roentgenograms showed a large purely
osteolytic lesion. Both medullar and cortical bone were extensively destroyed.
The gross specimen showed cystic cavities separated by septa, similar to an
aneurismal bone cyst. Microscopically, the septa contained anaplastic cells. A
few fine, lace-like osteoid were scattered among these sarcomatous cells. 12
patients were followed-up from 6 months to 84 months. Of seven patients who had
developed pulmonary metastasis, six patients died and one alive with lung
metastasis. The remaining five patients survived after operation. CONCLUSIONS:
Difficulties in making an early diagnosis and highly malignant of this disease
might be the important factors that affect the prognosis of telangiectatic
osteosarcoma.

 

Mod Pathol. 2001 Dec;14(12):1277-83.

Clinicopathologic analysis of HER-2/neu immunoexpression among various
histologic subtypes and grades of osteosarcoma.

Kilpatrick SE, Geisinger KR, King TS, Sciarrotta J, Ward WG, Gold SH, Bos GD.

Department of Pathology, University of North Carolina, Chapel Hill, North
Carolina 27599-7525, USA. scott.kilpatrick@pathology.unc.edu

Overexpression of the HER-2/neu oncogene appears to have prognostic significance
in breast cancer. Recently, some have reported a relationship between increased
immunohistochemical expression in osteosarcoma and poor clinical outcome.
Despite limited data, a pilot trial of Herceptin, which targets the oncogene
product, has been initiated for the therapy of some metastatic osteosarcomas
(CCG-P9852). Archival formalin-fixed, paraffin-embedded tissue obtained from 41
patients diagnosed with osteosarcoma was examined immunohistochemically by 2
antibodies against the HER-2/neu oncogene product: CB-11 (monoclonal, 1/100) and
Oncor (polyclonal, 1/200). All but one tumor (case of recurrent dedifferentiated
parosteal osteosarcoma) represented primary tumor samples; when applicable, only
prechemotherapy biopsies were analyzed. The study sample included the full
spectrum of histologic subtypes and grades of osteosarcoma (25 conventional high
grade; 3 telangiectatic; 1 small cell; 6 parosteal; 1 periosteal; and 5
low-grade intramedullary). A case of metastatic breast cancer with known
overexpression of the HER-2/neu oncogene served as the positive control.
Complete membranous positivity, considered prognostically significant in breast
cancer, was not seen in any of our osteosarcoma cases. At least focal
cytoplasmic positivity was documented in 40 (98%) tumors using the CB11 antibody
and in 34 (83%) using the Oncor antibody. The intensity of the cytoplasmic
staining (0, 1-3+) did not correlate with histologic subtype/grade, response to
chemotherapy (<90% versus > or = 90% necrosis), metastasis, or survival.
Immunohistochemical overexpression of the HER-2/neu oncogene, defined as
complete membranous positivity, is not present in our series of osteosarcomas.
Cytoplasmic positivity is observed in most osteosarcomas, irrespective of
histologic subtype/grade, and is not associated with response to preoperative
chemotherapy or disease progression.

 

Pathology. 1999 Nov;31(4):428-30.

Dedifferentiated chondrosarcoma with features of telangiectatic osteosarcoma.

Radhi JM, Loewy J.

Department of Pathology, College of Medicine, Royal University Hospital,
University of Saskatchewan, Saskatoon, Canada.

We describe a 44-year-old female with a known history of a solitary
osteochondroma of the scapula followed on X-ray for five years. She then
presented with a rapidly growing lump. Imaging studies confirmed the presence of
an aggressive looking lesion. Excision was performed and pathology showed a
dedifferentiated chondrosarcoma with features of a telangiectatic osteosarcoma.

 

Hum Pathol. 1999 Oct;30(10):1254-9.

Large cell, epithelioid, telangiectatic osteoblastoma: a unique
pseudosarcomatous variant of osteoblastoma.

Angervall L, Persson S, Stenman G, Kindblom LG.

Department of Pathology, Sahlgrenska University Hospital, Goteborg, Sweden.

A previously undescribed large-cell, epithelioid, and aneurysmal variant of
osteoblastoma with minimal osteoid-production--simulating telangiectatic
osteosarcoma, epithelioid angiosarcoma, and metastatic carcinoma is reported.
The tumor occurred in the mandible of a 14-year-old girl. The light microscopic,
immunohistochemical, ultrastructural, cell proliferation, and DNA-ploidy
studies, as well as the 7-year disease-free follow-up period all indicate a
benign osteoblastic tumor. Cytogenetically, the tumor had a pseudodiploid
karyotype, distinguished by a complex t(1;5;17;22) and a terminal 1q deletion.
Recognition of this unique, pseudomalignant variant of osteoblastoma is
important to avoid an erroneous diagnosis of malignancy.