Skip to main content
Log in

Undifferentiated Sarcoma of the Sellar Region

  • Published:
Endocrine Pathology Aims and scope Submit manuscript

Abstract

Malignancies lacking specific features of cellular maturation are termed “undifferentiated” and represent 5–10% of all human tumors. They are encountered at a variety of sites but do not, as a rule, arise in the sellar region. A 39-year-old male with a history of testicular seminoma and an unsuccessful biopsy of a third ventricular neoplasm, presented with visual disturbances and memory loss. Light microscopically, the tumor consisted entirely of undifferentiated spindle cells. No germ cell component was noted. An exhaustive immunohistochemical study found immunoreactivity for vimentin and desmin, but for no other myoid markers. Polymerase chain reaction showed no X;18 translocation. Based upon these studies, a diagnosis of “undifferentiated sarcoma” was made. Our case, being highly unusual among reported sellar neoplasms, underscores the difficulties inherent in the differential diagnosis of undifferentiated neoplasms.

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.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. Gatter KC, Alcock C, Heryet A, Pulford KA, Heyderman E, Taylor-Papadimitriou J, Stein H, Mason DY: The differential diagnosis of routinely processed anaplastic tumors using monoclonal antibodies. Am J Clin Pathol 82 (1):33–43, 1984.

    PubMed  CAS  Google Scholar 

  2. Vege DS, Soman CS, Joshi UA, Ganesh B, Yadav JN: Undifferentiated tumors: An immunohistochemical analysis on biopsies. J Surg Oncol 57 (4):273–276, 1994.

    Article  PubMed  CAS  Google Scholar 

  3. Enzinger FM, Weiss SW. Immunohistochemistry of soft tissue lesions. In: Soft Tissue Tumors. FM Enzinger; SW Weiss, editors. Vol. 3. St. Louis: CV Mosby; 1995; p. 139–163.

  4. Huang BY, Castillo M: Nonadenomatous tumors of the pituitary and sella turcica. Top Magn Reson Imaging 16 (4):289–299, 2005.

    Article  PubMed  Google Scholar 

  5. Lopes MB, Lanzino G, Cloft HJ, Winston DC, Vance ML, Laws ER, Jr.: Primary fibrosarcoma of the sella unrelated to previous radiation therapy. Mod Pathol 11 (6):579–584, 1998.

    PubMed  CAS  Google Scholar 

  6. Kovacs K, Giannini C, Scheithauer BW, Stefaneanu L, Lloyd RV, Horvath E: Pituitary changes in ataxia-telangiectasia syndrome: An immunocytochemical, in situ hybridization, and DNA cytometric study of three cases. Endocr Pathol 8 (3):195–203, 1997.

    Article  PubMed  CAS  Google Scholar 

  7. Kovacs K, Stefaneanu L, Ezzat S, Smyth HS: Prolactin-producing pituitary adenoma in a male-to-female transsexual patient with protracted estrogen administration. A morphologic study. Arch Pathol Lab Med 118 (5):562–565, 1994.

    PubMed  CAS  Google Scholar 

  8. Singer S, Demetri GD, Baldini EH, Fletcher CD: Management of soft-tissue sarcomas: An overview and update. Lancet Oncol 1:75–85, 2000.

    Article  PubMed  CAS  Google Scholar 

  9. Carneiro SS, Scheithauer BW, Nascimento AG, Hirose T, Davis DH: Solitary fibrous tumor of the meninges: A lesion distinct from fibrous meningioma. A clinicopathologic and immunohistochemical study. Am J Clin Pathol 106 (2):217–224, 1996.

    PubMed  CAS  Google Scholar 

  10. Schultz AB, Brat DJ, Oyesiku NM, Hunter SB: Intrasellar pituicytoma in a patient with other endocrine neoplasms. Arch Pathol Lab Med 125 (4):527–530, 2001.

    PubMed  CAS  Google Scholar 

  11. Shinojima N, Ohta K, Yano S, Nakamura H, Kochi M, Ishimaru Y, Nakazato Y, Ushio Y: Myofibroblastoma in the suprasellar region. Case report. J Neurosurg 97 (5):1203–1207, 2002.

    Article  PubMed  Google Scholar 

  12. Mena H, Ribas JL, Pezeshkpour GH, Cowan DN, Parisi JE: Hemangiopericytoma of the central nervous system: A review of 94 cases. Hum Pathol 22 (1):84–91, 1991.

    Article  PubMed  CAS  Google Scholar 

  13. Allan CA, Kaltsas G, Evanson J, Geddes J, Lowe DG, Plowman PN, Grossman AB: Pituitary chondrosarcoma: An unusual cause of a sellar mass presenting as a pituitary adenoma. J Clin Endocrinol Metab 86 (1):386–391, 2001.

    Article  PubMed  CAS  Google Scholar 

  14. Ashkan K, Pollock J, D'Arrigo C, Kitchen ND: Intracranial osteosarcomas: Report of four cases and review of the literature. J Neurooncol 40 (1):87–96, 1998.

    Article  PubMed  CAS  Google Scholar 

  15. Scheithauer BW, Silva AI, Kattner K, Seibly J, Oliveira AM, Kovacs K: Synovial sarcoma of the sellar region. Neuro Oncol 9 (4):454–459, 2007.

    Article  PubMed  Google Scholar 

  16. Anderson WR, Cameron JD, Tsai SH: Primary intracranial leiomyosarcoma. Case report with ultrastructural study. J Neurosurg 53 (3):401–405, 1980.

    Article  PubMed  CAS  Google Scholar 

  17. Arita K, Sugiyama K, Tominaga A, Yamasaki F: Intrasellar rhabdomyosarcoma: Case report. Neurosurgery 48 (3):677–680, 2001.

    Article  PubMed  CAS  Google Scholar 

  18. Stuart A, Radhakrishnan J: Rhabdomyosarcoma. Indian J Pediatr 71 (4):331–337, 2004.

    Article  PubMed  Google Scholar 

  19. Dagher R, Helman L: Rhabdomyosarcoma: An overview. Oncologist 4 (1):34–44, 1999.

    PubMed  CAS  Google Scholar 

  20. Herzog CE: Overview of sarcomas in the adolescent and young adult population. J Pediatr Hematol Oncol 27 (4):215–218, 2005.

    Article  PubMed  Google Scholar 

  21. Sohaib SA, Moseley I, Wright JE: Orbital rhabdomyosarcoma—the radiological characteristics. Clin Radiol 53 (5):357–362, 1998.

    Article  PubMed  CAS  Google Scholar 

  22. Lee JI, Nam DH, Kim JS, Hong SC, Shin HJ, Park K, Eoh W, Suh YL, Kim JH: Intracranial oculomotor nerve rhabdomyoma. J Neurosurg 93 (4):715, 2000.

    Article  PubMed  CAS  Google Scholar 

  23. Xu F, De Las Casas LE, Dobbs LJ, Jr.: Primary meningeal rhabdomyosarcoma in a child with hypomelanosis of Ito. Arch Pathol Lab Med 124 (5):762–765, 2000.

    PubMed  CAS  Google Scholar 

  24. Zhong J, Li ST, Yao XH, Jin B, Wan L: An intrasellar rhabdomyosarcoma misdiagnosed as pituitary adenoma. Surg Neurol 68 Suppl 2:S29-33; discussion S33, 2007.

    Article  PubMed  Google Scholar 

  25. Jalalah S, Kovacs K, Horvath E, Couldwell W, Weiss MH, Ezrin C: Rhabdomyosarcoma in the region of the sella turcica. Acta Neurochir (Wien) 88 (3–4):142–146, 1987.

    Article  CAS  Google Scholar 

  26. Coffin CM: The New International Rhabdomyosarcoma Classification, its progenitors, and considerations beyond morphology. Advances in Anatomic Pathology 4 (1):1–16, 1997.

    Article  Google Scholar 

  27. Dias P, Dilling M, Houghton P: The molecular basis of skeletal muscle differentiation. Semin Diagn Pathol 11 (1):3–14, 1994.

    PubMed  CAS  Google Scholar 

  28. Tonin PN, Scrable H, Shimada H, Cavenee WK: Muscle-specific gene expression in rhabdomyosarcomas and stages of human fetal skeletal muscle development. Cancer Res 51 (19):5100–5106, 1991.

    PubMed  CAS  Google Scholar 

  29. Furlong MA, Mentzel T, Fanburg-Smith JC: Pleomorphic rhabdomyosarcoma in adults: A clinicopathologic study of 38 cases with emphasis on morphologic variants and recent skeletal muscle-specific markers. Mod Pathol 14 (6):595–603, 2001.

    Article  PubMed  CAS  Google Scholar 

  30. Osborn M, Hill C, Altmannsberger M, Weber K: Monoclonal antibodies to titin in conjunction with antibodies to desmin separate rhabdomyosarcomas from other tumor types. Lab Invest 55 (1):101–108, 1986.

    PubMed  CAS  Google Scholar 

  31. Molenaar WM, Oosterhuis JW, Oosterhuis AM, Ramaekers FC: Mesenchymal and muscle-specific intermediate filaments (vimentin and desmin) in relation to differentiation in childhood rhabdomyosarcomas. Hum Pathol 16 (8):838–843, 1985.

    Article  PubMed  CAS  Google Scholar 

  32. Hicks J, Flaitz C: Rhabdomyosarcoma of the head and neck in children. Oral Oncol 38 (5):450–459, 2002.

    Article  PubMed  CAS  Google Scholar 

  33. Qualman SJ, Coffin CM, Newton WA, Hojo H, Triche TJ, Parham DM, Crist WM: Intergroup Rhabdomyosarcoma Study: Update for pathologists. Pediatr Dev Pathol 1 (6):550–561, 1998.

    Article  PubMed  CAS  Google Scholar 

  34. Crist WM, Anderson JR, Meza JL, Fryer C, Raney RB, Ruymann FB, Breneman J, Qualman SJ, Wiener E, Wharam M, Lobe T, Webber B, Maurer HM, Donaldson SS: Intergroup rhabdomyosarcoma study-IV: Results for patients with nonmetastatic disease. J Clin Oncol 19 (12):3091–3102, 2001.

    PubMed  CAS  Google Scholar 

  35. Breitfeld PP, Meyer WH: Rhabdomyosarcoma: New windows of opportunity. Oncologist 10 (7):518–527, 2005.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors thank Mrs. Denise Chase for her secretarial assistance as well as Mr. Jesus Rodriguez and the Jarislowsky and Lloyd Carr-Harris Foundations for their generous support.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Bernd W. Scheithauer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Manoranjan, B., Syro, L.V., Scheithauer, B.W. et al. Undifferentiated Sarcoma of the Sellar Region. Endocr Pathol 22, 159–164 (2011). https://doi.org/10.1007/s12022-011-9166-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12022-011-9166-7

Keywords

Navigation