Skip to main content
Log in

Diaphyseal medullary stenosis with pleomorphic malignant fibrous histiocytoma of the bone:99mTc hydroxymethylenediphosphonate and201Tl chloride scintigraphy findings

  • Case Report
  • Published:
Annals of Nuclear Medicine Aims and scope Submit manuscript

Abstract

Diaphyseal medullary stenosis (DMS) is an extremely rare hereditary bone dysplasia, which was first described by Arnold in 1973. DMS has a high incidence of pleomorphic malignant fibrous histiocytoma (MFH). In this paper, we report the imaging findings of DMS with pleomorphic MFH of the bone, mainly describing99mTc hydroxymethylenediphosphonate (HMDP) and thallium-201 (2OIT1) chloride scintigraphy findings. On99mTc HMDP scintigraphy, focal increased uptake area of the right femur corresponded to the area of bone marrow invasion of the tumor and bone infarction. The mechanism of the uptake of99mTc HMDP to the extraosseous lesion was not clear. On201Tl chloride scintigraphy, the increased uptake of the periphery of the mass seemed to reflect the aggressiveness of invasion and the cellularity.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Arnold WH. Hereditary bone dysplasia with sarcomatous degeneration: Study of a family.Ann Intern Med 1973; 78:902–906.

    PubMed  CAS  Google Scholar 

  2. Hardcastle P, Nade S, Arnold WH. Hereditary bone dysplasia with malignant change: Report of three families.J Bone Joint Surg 1986; 68A: 1079–1089.

    Google Scholar 

  3. Norton KI, Wagreich JM, Granowetter L, Martignetti JA. Diaphyseal medullary stenosis (sclerosis) with bone malignancy (malignant fibrous histiocytoma): Hardcastle syndrome.Pediatr Radiol 1996; 26 (9): 675–677.

    Article  PubMed  CAS  Google Scholar 

  4. Kenan S, Abdelwahab IF, Hermann G, Klein MJ. Malignant fibrous histiocytoma associated with a bone infarct in a patient with hereditary bone dysplasia.Skeletal Radiol 1998; 27: 463–467.

    Article  PubMed  CAS  Google Scholar 

  5. Martignetti JA, Desnick RJ, Aliprandis E, et al. Diaphyseal medullary stenosis with malignant fibrous histiocytoma: A hereditary bone dysplasia/cancer syndrome maps to 9p217#x2013;22.Am J Hum Genet 1999; 64: 801–807.

    Article  PubMed  CAS  Google Scholar 

  6. Martignetti JA, Gelb BD, Pierce H, Picci P, Desnick RJ. Malignant fibrous histiocytoma: inherited and sporadic forms have loss of heterozygosity at chromosome bands 9p21-22-evidence for a common genetic defect.Genes Chromosomes Cancer 2000; 27: 191–195.

    Article  PubMed  CAS  Google Scholar 

  7. Muroya K, Nishimura G, Douya H, Hasegawa T, Ogata T. Diaphyseal medullary stenosis with malignant fibrous histiocytoma: further evidence for loss of heterozygosity involving 9p21-22 in tumor tissue.Genes Chromosomes Cancer 2002; 33: 326–328.

    Article  PubMed  CAS  Google Scholar 

  8. Douya H, Yokoyama R, Beppu Y, Hasegawa T. Malignant fibrous histiocytoma associated with diaphyseal medullary stenosis.Clin Orthop 2002; 400: 211–221.

    Article  PubMed  Google Scholar 

  9. Gay BB Jr, Elsas LJ, Wyly JB, Pasquali M. Osteopathia striata with cranial sclerosis.Pediatr Radiol 1994; 24: 56–60.

    Article  PubMed  Google Scholar 

  10. Higuchi T, Taki J, Nakajima N, et al. Differentiation of soft tissue haemangioma by201Tl scintigraphy.Nucl Med Commun 2003; 24: 327–330.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Miyake, M., Tateishi, U., Maeda, T. et al. Diaphyseal medullary stenosis with pleomorphic malignant fibrous histiocytoma of the bone:99mTc hydroxymethylenediphosphonate and201Tl chloride scintigraphy findings. Ann Nucl Med 20, 233–236 (2006). https://doi.org/10.1007/BF03027436

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF03027436

Key words

Navigation