Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Telangiectatic Osteosarcoma Workup

  • Author: Nirag C Jhala, MD, MBBS; Chief Editor: Harris Gellman, MD  more...
 
Updated: Jun 16, 2016
 

Imaging Studies

In patients with suspected bone tumors, imaging studies are the initial examinations for determining the nature and extent of the lesion. Plain radiography, magnetic resonance imaging (MRI), computed tomography (CT), and radionuclide bone imaging (ie, bone scanning) help in the differential diagnosis. Patient treatment is often based on the findings from these studies.

On conventional radiographs, pure lytic lesions define these tumors. The tumor margins are frequently permeative; however, well-defined margins have been noted. A sclerotic rim suggests another diagnosis. The lytic lesions may have fluid-filled spaces. Cortical destruction and infiltration into the surrounding soft tissues can occur. This tumor may also evoke a periosteal bone reaction, and it can be associated with the Codman triangle. A pattern of parallel striations is highly suggestive of telangiectatic osteosarcoma.

The literature regarding CT and MRI features of telangiectatic osteosarcoma is relatively sparse.

The differential diagnosis of telangiectatic osteosarcoma, based on imaging studies, often includes the following[33] :

  • Aneurysmal bone cyst
  • Ewing sarcoma
  • Langerhans cell histiocytosis
  • Fibrosarcoma
  • Malignant fibrous histiocytoma
Next

Histologic Findings

The use of fine-needle aspiration biopsy (FNAB) and the pathologic evaluation of tissue sections obtained during surgery in the diagnosis of telangiectatic osteosarcoma have been reviewed.[3, 4, 34]

Fine-needle aspiration biopsy

The examination of samples obtained by means of FNAB reveals sheets of highly polymorphous cells. The cells include spindled cells reminiscent of fibroblasts, as well as round or oval malignant cells. Multinucleated cells also are frequently identified. Nuclear hyperchromasia, nuclear membrane irregularity, and prominent nucleoli are noted in the malignant cells. Increased mitotic activity may be noted as well. The cytoplasm is variable and granular.

Although FNAB results, in conjunction with highly suggestive clinical and radiographic findings, may be of value in determining the malignant nature of the underlying process, they are not the mainstays in the diagnosis of telangiectatic osteosarcomas. Rather, core-needle or conventional biopsy permits a definitive diagnosis of telangiectatic osteosarcoma.

Gross appearance of the resected tumors

The tumors, which may be 10-20 cm in diameter, have the appearance of a hemorrhagic mass. An aneurysmal bone cyst is often suspected. Sometimes, these lesions have multicystic channels filled with blood that correspond to the radiographic appearance of fluid-filled spaces. A solid, fleshy, sarcomalike appearance is not appreciated in these lesions.

Microscopic features

Malignant cells are noted in a background of blood and necrotic debris. Because the pleomorphic hyperchromatic malignant cells may be diluted in the necrotic and hemorrhagic background, a careful examination to recognize these elements is imperative. Blood lakes, rather than endothelium-lined spaces, are present (see the image below).

Large blood lakes seen at a low magnification are Large blood lakes seen at a low magnification are reminiscent of findings in an aneurysmal bone cyst (hematoxylin and eosin, original magnification X4).

In some cases, an osteoid matrix may not be visualized except within the septal walls, which may be thin and difficult to find. In such cases, a characteristic radiographic appearance, when correlated with a careful microscopic search for features suggestive of malignancy, helps in the correct interpretation of the findings.[35] Occasionally, low-power examination reveals a morphologic pattern that is reminiscent of an aneurysmal bone cyst.

A potential trap is created by a radiologic impression of an aneurysmal bone cyst and the characteristic gross features of that cyst. However, examination of the cyst lining reveals overt malignant cells, often with increased mitotic activity (see the first image below). These cells may lie adjacent to the benign osteoclastic giant cells. In some cases, these giant cells are numerous, and the tumor mimics a giant cell–rich osteosarcoma (see the second image below).

Careful examination of the lining of blood-filled Careful examination of the lining of blood-filled lakes shows overt malignant cells. Atypical tripolar mitosis is noted in the field (hematoxylin and eosin, original magnification X20).
Numerous giant cells may be noted in the tumor, wh Numerous giant cells may be noted in the tumor, which mimics a giant, cell–rich osteosarcoma (hematoxylin and eosin, original magnification X20).

Unlike an aneurysmal bone cyst, telangiectatic osteosarcoma has an osteoid matrix that is delicate and lacelike in appearance. Also, the stroma between the dilated vascular spaces often contains malignant cells.

Role of pathologists

A high degree of suspicion is necessary with a purely destructive long-bone lesion in adolescents. Examinations of bone tumors and tumorlike conditions have been reviewed.[11] Briefly, in the resected specimen, the size and location of the tumor and the extent of disease should be noted. The involvement of the resected margins and of the vessels, nerves, skin, and soft tissues along the biopsy tract should be included in the report.

After preoperative chemotherapy, the resected specimen should be mapped, and multiple sections representing the complete face of the bone should be obtained to document the amount of necrosis and viable tumor in the specimen. This observation is particularly important because the amount of necrosis has strong prognostic significance and affects the subsequent management of telangiectatic osteosarcoma. Tumor necrosis in more than 95-98% of the resected specimen is considered a good response to chemotherapy.[36, 37]

Previous
Next

Staging

The American Joint Committee on Cancer (AJCC) staging system for primary bone tumors is based on a combination of the primary tumor (T), regional lymph node involvement (N), distant metastasis (M), and histopathologic grade (G). Because regional lymph node involvement is rare in bone tumors, the pathologic stage grouping involves any combination of these four grades.

With respect to these bone tumors, AJCC definitions for primary tumor characteristics are as follows:

  • TX - The primary tumor cannot be assessed
  • T0 - No evidence of a primary tumor is present
  • T1 - The tumor is confined within the cortex
  • T2 - The tumor invades beyond the cortex

Definitions for regional lymph node involvement are as follows:

  • NX - Regional lymph nodes cannot be assessed
  • N0 - No regional lymph node metastasis is present
  • N1 - Regional lymph node metastasis is present

Definitions for distant metastasis are as follows:

  • MX - Distant metastasis cannot be assessed
  • M0 - No distant metastasis is present
  • M1 - Distant metastasis is present

Definitions for histopathologic grades are as follows:

  • GX - Grade not assessable
  • G1 - Well differentiated, low grade
  • G2 - Moderately differentiated, low grade
  • G3 - Poorly differentiated, high grade
  • G4 - Undifferentiated, high grade

Definitions for pathologic stages are as follows:

  • Stage IA - G1 or G2, T1, N0, M0
  • Stage IB - G1 or G2, T2, N0, M0
  • Stage IIA - G3 or G4, T1, N0, M0
  • Stage IIB - G3 or G4, T2, N0, M0
  • Stage III - Not defined
  • Stage IVA - Any G, any T, N1, M0
  • Stage IVB - Any G, any T, any N, M1

Enneking initially proposed a system for staging bone tumors that was based on the histopathologic grade, the site of the lesion, and evidence of metastasis (see Table 1 below).

Table 1. Initial Enneking Clinical Staging System for Primary Malignant Bone Tumors (Open Table in a new window)

Stage Grade Location Metastasis
IA Low grade, G1 T1 M0, intracompartmental
IB Low grade, G1 T2 M0, intracompartmental
IIA High grade, G2 T1 M0, intracompartmental
IIB High grade, G2 T2 M0, extracompartmental
IIIA Low or high grade, G1 or G2 T1 M1, intracompartmental with metastasis
IIIB Low or high grade, G1 or G2 T2 M1, extracompartmental with metastasis

In the Enneking method of staging malignant bone tumors, histologic grade is defined as follows:

  • Low grade, G1 - Well-differentiated tumor with few mitoses and moderate nuclear atypia
  • High grade, G2 - Poorly differentiated tumor with high cell-to-matrix ratio, many mitoses, and marked nuclear atypia

The site of the lesion is specified as follows:

  • Intracapsular, T0 - Lesion surrounded by an intact capsule of fibrous tissue or reactive bone
  • Extracapsular, T1 - Lesion within the compartment of its origin (the lesion remains within an intraosseous, intrafascial and/or muscular plane or periosteal or parosteal compartment)
  • Extracapsular, T2 - Lesion extension beyond its compartment of origin or lesion origin within an incompletely bound space, such as the popliteal fossa, axilla, or groin

Metastasis is specified as follows:

  • M0 - No known metastasis
  • M1 - Metastasis present
Previous
 
 
Contributor Information and Disclosures
Author

Nirag C Jhala, MD, MBBS Director of Anatomic Pathology, Director of Cytopathology, Temple University Hospital; Professor of Pathology and Laboratory Medicine, Temple University School of Medicine

Nirag C Jhala, MD, MBBS is a member of the following medical societies: American Society of Cytopathology, Biomedical Engineering Society, College of American Pathologists, International Academy of Cytology, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Coauthor(s)

Vinod B Shidham, MD, FRCPath Professor, Vice-Chair-AP, and Director of Cytopathology, Department of Pathology, Wayne State University School of Medicine, Karmanos Cancer Center and Detroit Medical Center; Co-Editor-in-Chief and Executive Editor, CytoJournal

Vinod B Shidham, MD, FRCPath is a member of the following medical societies: American Association for Cancer Research, American Society of Cytopathology, College of American Pathologists, International Academy of Cytology, Royal College of Pathologists, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Donald A Hackbarth, Jr, MD, FACS Professor of Clinical Orthopedic Surgery, Division Chief, Musculoskeletal Oncology, Department of Orthopedic Surgery, Medical College of Wisconsin

Donald A Hackbarth, Jr, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Tissue Banks, American College of Surgeons, Christian Medical and Dental Associations, Clinical Orthopaedic Society, Children's Oncology Group, Wisconsin Medical Society

Disclosure: Received honoraria from Musculoskeletal Transplant Foundation for board membership.

Stuart Wong, MD Assistant Professor, Department of Medicine, Section of Hematology/Oncology, Froedert Memorial Lutheran Hospital

Disclosure: Nothing to disclose.

Gene P Siegal, MD, PhD Director, Division of Anatomic Pathology, Professor, Departments of Pathology and Surgery, University of Alabama at Birmingham

Gene P Siegal, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Medical Association, American Society for Investigative Pathology, American Society for Clinical Pathology, College of American Pathologists, International Academy of Pathology, International Skeletal Society, New York County Medical Society, Royal Society of Medicine, Sigma Xi, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Sean P Scully, MD 

Sean P Scully, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, Society of Surgical Oncology

Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine, Clinical Professor, Surgery, Nova Southeastern School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, Arkansas Medical Society

Disclosure: Nothing to disclose.

References
  1. Ewing J. A review and classification of bone sarcomas. Arch Surg. 1922. 485-533.

  2. Huvos AG, Rosen G, Bretsky SS. Telangiectatic osteogenic sarcoma: a clinicopathologic study of 124 patients. Cancer. 1982 Apr 15. 49(8):1679-89. [Medline].

  3. Larsson SE, Lorentzon R, Wedren H, et al. Osteosarcoma. A multifactorial clinical and histopathological study with special regard to therapy and survival. Acta Orthop Scand. 1978 Dec. 49(6):571-81. [Medline].

  4. Larsson SE, Lorentzon R, Boquist L. Telangiectatic osteosarcoma. Acta Orthop Scand. 1978 Dec. 49(6):589-94. [Medline].

  5. Mervak TR, Unni KK, Pritchard DJ, et al. Telangiectatic osteosarcoma. Clin Orthop Relat Res. 1991 Sep. (270):135-9. [Medline].

  6. Berner K, Johannesen TB, Berner A, Haugland HK, Bjerkehagen B, Bøhler PJ, et al. Time-trends on incidence and survival in a nationwide and unselected cohort of patients with skeletal osteosarcoma. Acta Oncol. 2014 Jun 24. 1-9. [Medline].

  7. Sangle NA, Layfield LJ. Telangiectatic osteosarcoma. Arch Pathol Lab Med. 2012 May. 136(5):572-6. [Medline].

  8. Metcalf DJ, Nightingale TD, Zenner HL, et al. Formation and function of Weibel-Palade bodies. J Cell Sci. 2008 Jan 1. 121(Pt 1):19-27. [Medline].

  9. Roessner A, Hobik HP, Immenkamp M, et al. Ultrastructure of telangiectatic osteosarcoma. J Cancer Res Clin Oncol. 1979 Oct. 95(2):197-207. [Medline].

  10. Nishida J, Abe M, Shiraishi H, et al. Familial occurrence of telangiectatic osteosarcoma: cousin cases. J Pediatr Orthop. 1994 Jan-Feb. 14(1):119-22. [Medline].

  11. Bell W, Siegal GP. Osteosarcoma. Cullinane C, Burchill S, Squire J, et al, eds. Molecular Biology and Pathology of Paediatric Cancer. London, England: Oxford University Press; 2003.

  12. Ottaviani G, Jaffe N. The epidemiology of osteosarcoma. Cancer Treat Res. 2010. 152:3-13. [Medline].

  13. Bacci G, Picci P, Ferrari S. Primary chemotherapy and delayed surgery for non-metastatic telangiectatic osteosarcoma of the extremities. Results in 28 patients. Eur J Cancer. 1994. 30A(5):620-6. [Medline].

  14. Fechner R, Mills S. Tumors of the bone and joints. Rosai J, Sobin L, eds. Atlas of Tumor Pathology. Washington, DC: Armed Forces Institute of Pathology; 1993. 51-4.

  15. Xie L, Guo W, Li Y, Ji T, Sun X. Pathologic fracture does not influence local recurrence and survival in high-grade extremity osteosarcoma with adequate surgical margins. J Surg Oncol. 2012 Jun 27. [Medline].

  16. Papagelopoulos PJ, Mavrogenis AF, Savvidou OD, et al. Pathological fractures in primary bone sarcomas. Injury. 2007 Nov 29. [Medline].

  17. Raymond AK, Jaffe N. Osteosarcoma Multidisciplinary Approach to the Management from the Pathologist's Perspective. Cancer Treat Res. 2010. 152:63-84. [Medline].

  18. Kunze B, Bürkle S, Kluba T. Multifocal osteosarcoma in childhood. Chir Organi Mov. 2009 May. 93(1):27-31. [Medline].

  19. Brown MJ, Logan PM, O'Connell JX. Diaphyseal telangiectatic osteosarcoma as a second tumor after bilateral retinoblastomas. Skeletal Radiol. 1996 Oct. 25(7):685-8. [Medline].

  20. Chan CW, Kung TM, Ma L. Telangiectatic osteosarcoma of the mandible. Cancer. 1986 Nov 1. 58(9):2110-5. [Medline].

  21. Naik LK, Shetty P, Teerthanath S, Jagadeesh HM. Telangiectatic osteosarcoma affecting the mandible. J Oral Maxillofac Pathol. 2014 Sep. 18 (Suppl 1):S143-6. [Medline]. [Full Text].

  22. Donato G, Lavano A, Volpentesta G. Telangiectatic osteosarcoma of the skull. A post-Paget case. Clin Neuropathol. 1997 Jul-Aug. 16(4):201-3. [Medline].

  23. Merino S, Arrazola J, Saiz A, et al. Post-Paget telangiectatic osteosarcoma of the skull. Skeletal Radiol. 1999 Aug. 28(8):470-2. [Medline].

  24. Wick MR, Siegal GP, Unni KK, et al. Sarcomas of bone complicating osteitis deformans (Paget''s disease): fifty years'' experience. Am J Surg Pathol. 1981 Jan. 5(1):47-59. [Medline].

  25. Wines A, Bonar F, Lam P. Telangiectatic dedifferentiation of a parosteal osteosarcoma. Skeletal Radiol. 2000 Oct. 29(10):597-600. [Medline].

  26. Radhi JM, Loewy J. Dedifferentiated chondrosarcoma with features of telangiectatic osteosarcoma. Pathology. 1999 Nov. 31(4):428-30. [Medline].

  27. Adler CP. Case report 111. Skeletal Radiol. 1980 Feb. 5(1):56-60. [Medline].

  28. Kyriakos M, Hardy D. Malignant transformation of aneurysmal bone cyst, with an analysis of the literature. Cancer. 1991 Oct 15. 68(8):1770-80. [Medline].

  29. Janevska V, Spasevska L, Samardziski M, Nikodinovskai V, Zhivadinovik J, Trajkovskai E. FROM ANEURYSMAL BONE CYST TO TELANGIECTATIC OSTEOSARCOMA WITH METASTASIS IN INGUINAL LYMPH NODES - CASE REPORT. Med Pregl. 2015 Mar-Apr. 68 (3-4):127-32. [Medline].

  30. Mirra JM, Fain JS, Ward WG, et al. Extraskeletal telangiectatic osteosarcoma. Cancer. 1993 May 15. 71(10):3014-9. [Medline].

  31. Graadt van Roggen JF, Zonderland HM, Welvaart K, Peterse JL, Hogendoorn PC. Local recurrence of a phyllodes tumour of the breast presenting with widespread differentiation to a telangiectatic osteosarcoma. J Clin Pathol. 1998 Sep. 51(9):706-8. [Medline]. [Full Text].

  32. Hirakawa T, Tsuneyoshi M, Enjoji M, et al. Ovarian sarcoma with histologic features of telangiectatic osteosarcoma of the bone. Am J Surg Pathol. 1988 Jul. 12(7):567-72. [Medline].

  33. Azura M, Vanel D, Alberghini M, Picci P, Staals E, Mercuri M. Parosteal osteosarcoma dedifferentiating into telangiectatic osteosarcoma: importance of lytic changes and fluid cavities at imaging. Skeletal Radiol. 2009 Jul. 38(7):685-90. [Medline].

  34. White VA, Fanning CV, Ayala AG, et al. Osteosarcoma and the role of fine-needle aspiration. A study of 51 cases. Cancer. 1988 Sep 15. 62(6):1238-46. [Medline].

  35. Vanel D, Tcheng S, Contesso G. The radiological appearances of telangiectatic osteosarcoma. A study of 14 cases. Skeletal Radiol. 1987. 16(3):196-200. [Medline].

  36. Khuu H, Moore D, Young S. Examination of tumor and tumor-like conditions of bone. Ann Diagn Pathol. 1999 Dec. 3(6):364-9. [Medline].

  37. Cui Q, Li D, Liu C, Guo J, Liu S, Liu Y, et al. The significance of MGMT protein detection in evaluation of osteosarcoma necrosis rate after cisplatin chemotherapy. Bosn J Basic Med Sci. 2011 May. 11(2):80-3. [Medline].

  38. Rosen G, Huvos AG, Marcove R, et al. Telangiectatic osteogenic sarcoma. Improved survival with combination chemotherapy. Clin Orthop Relat Res. 1986 Jun. (207):164-73. [Medline].

  39. Weiss A, Khoury JD, Hoffer FA, et al. Telangiectatic osteosarcoma: the St. Jude Children's Research Hospital's experience. Cancer. 2007 Apr 15. 109(8):1627-37. [Medline].

  40. Liu JJ, Liu S, Wang JG, Zhu W, Hua YQ, Sun W, et al. Telangiectatic osteosarcoma: a review of literature. Onco Targets Ther. 2013. 6:593-602. [Medline]. [Full Text].

  41. Rosen G, Caparros B, Huvos AG. Preoperative chemotherapy for osteogenic sarcoma: selection of postoperative adjuvant chemotherapy based on the response of the primary tumor to preoperative chemotherapy. Cancer. 1982 Mar 15. 49(6):1221-30. [Medline].

  42. Eftekhari F. Imaging assessment of osteosarcoma in childhood and adolescence: diagnosis, staging, and evaluating response to chemotherapy. Cancer Treat Res. 2010. 152:33-62. [Medline].

  43. Basaran M, Bavbek ES, Saglam S, et al. A phase II study of cisplatin, ifosfamide and epirubicin combination chemotherapy in adults with nonmetastatic and extremity osteosarcomas. Oncology. 2008 Jan 10. 72(3-4):255-260. [Medline].

  44. Ferguson WS, Harris MB, Goorin AM, et al. Presurgical window of carboplatin and surgery and multidrug chemotherapy for the treatment of newly diagnosed metastatic or unresectable osteosarcoma: Pediatric Oncology Group Trial. J Pediatr Hematol Oncol. 2001 Aug-Sep. 23(6):340-8. [Medline].

  45. Rashid M, Hafeez S, Zia Ul Islam M, et al. Limb salvage in malignant tumours of the upper limb using vascularised fibula. J Plast Reconstr Aesthet Surg. 2007 Dec 22. [Medline].

 
Previous
Next
 
Large blood lakes seen at a low magnification are reminiscent of findings in an aneurysmal bone cyst (hematoxylin and eosin, original magnification X4).
Careful examination of the lining of blood-filled lakes shows overt malignant cells. Atypical tripolar mitosis is noted in the field (hematoxylin and eosin, original magnification X20).
Numerous giant cells may be noted in the tumor, which mimics a giant, cell–rich osteosarcoma (hematoxylin and eosin, original magnification X20).
Areas of necrosis with persistent tumor cells are present after neoadjuvant chemotherapy (hematoxylin and eosin, original magnification X10).
Anteroposterior radiograph of the distal femur shows a large, aggressive lytic lesion replacing the distal femur with no appreciable intralesional matrix. Courtesy of Robert Lopez-Ben, MD.
Lateral radiograph of the distal femur shows a large, aggressive lytic lesion that replaces the distal femur with no appreciable intralesional matrix. Courtesy of Robert Lopez-Ben, MD.
Table 1. Initial Enneking Clinical Staging System for Primary Malignant Bone Tumors
Stage Grade Location Metastasis
IA Low grade, G1 T1 M0, intracompartmental
IB Low grade, G1 T2 M0, intracompartmental
IIA High grade, G2 T1 M0, intracompartmental
IIB High grade, G2 T2 M0, extracompartmental
IIIA Low or high grade, G1 or G2 T1 M1, intracompartmental with metastasis
IIIB Low or high grade, G1 or G2 T2 M1, extracompartmental with metastasis
Previous
Next
 
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.