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Combined sellar fibrosarcoma and prolactinoma with neuronal metaplasia: Report of a case unassociated with radiotherapy

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Abstract

We report the occurrence of a primary pituitary fibrosarcoma causally unrelated to radiotherapy, admixed in association with a prolactin cell pituitary adenoma showing neuronal metaplasia. These unique findings were associated with multiple endocrine neoplasia type 1 (MEN 1).

Primary fibrosarcoma involving the sella is a very rare tumor. The majority of cases have been associated with prior irradiation of either a pituitary adenoma or a craniopharyngioma. Pituitary adenoma with neuronal metaplasia is also rare and usually occurs in the setting of acromegaly. Despite the intimate association of both elements in our lesion, no transition of adenoma to sarcoma was demonstrable by immunohistochemistry or in situ hybridization studies.

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References

  1. Alpers B, et al. The ganglioneurons of the central nervous system. Arch Neurol Psychiatry 26:501–523, 1931.

    Google Scholar 

  2. Amine AR, Sugar O. Suprasellar osteogenic sarcoma following radiation for pituitary adenoma. Case report. J Neurosurg 44(1):88–91, 1976.

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  4. Angelstein I. [Pathogenesis of acromegaly.]. Dtsch Z Nervenheilkd 170(4):337–348, 1953.

    Article  PubMed  CAS  Google Scholar 

  5. Arseni C, Horvath L, Carp N, Ciurea V. Intracranial ganglioneuromas in children. Acta Neurochir (Wien) 32(3–4):279–286, 1975.

    Article  CAS  Google Scholar 

  6. Asa SL, Bilbao JM, Kovacs K, Linfoot JA. Hypothalamic neuronal hamartoma associated with pituitary growth hormone cell adenoma and acromegaly. Acta Neuropathol (Berl) 52(3):231–234, 1980.

    Article  CAS  Google Scholar 

  7. Asa SL, Scheithauer BW, Bilbao JM, et al. A case for hypothalamic acromegaly: a clinicopathological study of six patients with hypothalamic gangliocytomas producing growth hormone-releasing factor. J Clin Endocrinol Metab 58(5):796–803, 1984.

    Article  PubMed  CAS  Google Scholar 

  8. Asa SL, Kovacs K, Tindall GT, Barrow DL, Horvath E, Vecsei P. Cushing’s disease associated with an intrasellar gangliocytoma producing corticotrophin-releasing factor. Ann Intern Med 101(6):789–793, 1984.

    PubMed  CAS  Google Scholar 

  9. Baysefer A, Gezen F, Kayali H, Erdogan E, Timurkaynak E, Celasun B. Intrasellar gangliocytoma resembling pituitary adenoma. Minim Invasive Neurosurg 40(3):107–109, 1997.

    PubMed  CAS  Google Scholar 

  10. Bevan JS, Asa SL, Rossi ML, Esiri MM, Adams CB, Burke CW. Intrasellar gangliocytoma containing gastrin and growth hormone-releasing hormone associated with a growth hormone-secreting pituitary adenoma. Clin Endocrinol (Oxf) 30(3):213–224, 1989.

    CAS  Google Scholar 

  11. Burchiel KJ, Shaw CM, Kelly WA. A mixed functional microadenoma and ganglioneuroma of the pituitary fossa. Case report. J Neurosurg 58(3):416–420, 1983.

    PubMed  CAS  Google Scholar 

  12. Casper J. Ueber neurogene Geschwulste im Hinterlappen der Hypopyyse. Zentralb Allg Pathol 56:404–411, 1933.

    Google Scholar 

  13. Corbetta S, Pizzocaro A, Peracchi M, Beck-Peccoz P, Faglia G, Spada A. Multiple endocrine neoplasia type 1 in patients with recognized pituitary tumours of different types. Clin Endocrinol (Oxf) 47(5):507–512, 1997.

    Article  CAS  Google Scholar 

  14. Fischer EG, Morris JH, Kettyle WM. Intrasellar gangliocytoma and syndromes of pituitary hypersecretion. Case report. J Neurosurg 59(6):1071–1075, 1983.

    PubMed  CAS  Google Scholar 

  15. Geddes JF, Jansen GH, Robinson SF, et al. “Gangliocytomas” of the pituitary: a heterogeneous group of lesions with differing histogenesis. Am J Surg Pathol 24(4):607–613, 2000.

    Article  PubMed  CAS  Google Scholar 

  16. Bots GT, Tijssen CC, Wijnalda D, Teepen JL. Alveolar soft part sarcoma of the pituitary gland with secondary involvement of the right cerebral ventricle. Br J Neurosurg 2(1):101–107, 1988.

    PubMed  CAS  Google Scholar 

  17. Horvath E, Kovacs K, Scheithauer BW, Lloyd RV, Smyth HS. Pituitary adenoma with neuronal choristoma (PANCH): composite lesion or lineage infidelity? Ultrastruct Pathol 18(6):565–574, 1994.

    PubMed  CAS  Google Scholar 

  18. Horvath E, Kovacs K, Tran A, Scheithauer BW. Ganglion cells in the posterior pituitary: result of ectopia or transdifferentiation? Acta Neuropathol (Berl) 100(1):106–110, 2000.

    Article  CAS  Google Scholar 

  19. Hourihane M, Moran M, Burke M. Intrasellar gangliocytoma associated with functional pituitary gland microadenoma. J Neuropathol Exp Neurol 51:364 (Abstract), 1992.

    Google Scholar 

  20. Iwase T, Nishizawa S, Baba S, et al. Intrasellar neuronal choristoma associated with growth hormone-producing pituitary adenoma containing amyloid deposits. Hum Pathol 26(8):925–928, 1995.

    Article  PubMed  CAS  Google Scholar 

  21. Jakumeit HD, Zimmermann V, Guiot G. Intrasellar gangliocytomas. Report of four cases. J Neurosurg 40(5):626–630, 1974.

    PubMed  CAS  Google Scholar 

  22. Kamel OW, Horoupian DS, Silverberg GD. Mixed gangliocytoma-adenoma: a distinct neuroendocrine tumor of the pituitary fossa. Hum Pathol 20(12):1198–1203, 1989.

    PubMed  CAS  Google Scholar 

  23. Kiyono H. Die Histopathologie der hypophyse. Virchows Arch A Pathol Anat Histopathol 259:388–465, 1926.

    Google Scholar 

  24. Kleinsasser O, Albrecht H. Zur Kenntnis der Osteosarkome des Stirn-und Keilbeines. Arch Ohren Heilk 170:595–603, 1957.

    Article  Google Scholar 

  25. Kovacs K, Lloyd R, Horvath E, et al. Silent somatotroph adenomas of the human pituitary. A morphologic study of three cases including immunocytochemistry, electron microscopy, in vitro examination, and in situ hybridization. Am J Pathol 134(2):345–353, 1989.

    PubMed  CAS  Google Scholar 

  26. Kovacs K, Stefaneanu L, Horvath E, et al. Effect of dopamine agonist medication on prolactin producing pituitary adenomas. A morphological study including immunocytochemistry, electron microscopy and in situ hybridization. Virchows Arch A Pathol Anat Histopathol 418(5):439–446, 1991.

    Article  PubMed  CAS  Google Scholar 

  27. Kroh H, Marchel A. Intrasellar neuronal hamartoma associated with pituitary adenoma. Case report. Folia Neuropathol 38(3):119–124, 2000.

    PubMed  CAS  Google Scholar 

  28. Landolt AM, Froesch ER. [Prolactin producing hypophyseal adenoma: diagnosis and therapeutic possibilities]. Schweiz Med Wochenschr 115(23):803–809, 1985.

    PubMed  CAS  Google Scholar 

  29. Lapresle J, Racadot J, Said G. [Myasthenia, thymoma and hypophyseal tumor associated with heterogenous adenomatous proliferation and ganglioneuroma of the sella turcica]. J Neurol Sci 28(2):249–254, 1976.

    Article  PubMed  CAS  Google Scholar 

  30. Li JY, Racadot O, Kujas M, Kouadri M, Peillon F, Racadot J. Immunocytochemistry of four mixed pituitary adenomas and intrasellar gangliocytomas associated with different clinical syndromes: acromegaly, amenorrhea-galactorrhea, Cushing’s disease and isolated tumoral syndrome. Acta Neuropathol (Berl) 77(3):320–328, 1989.

    Article  CAS  Google Scholar 

  31. 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 

  32. Marx S, Spiegel AM, Skarulis MC, Doppman JL, Collins FS, Liotta LA. Multiple endocrine neoplasia type 1: clinical and genetic topics. Ann Intern Med 129(6):484–494, 1998.

    PubMed  CAS  Google Scholar 

  33. McCowen KC, Glickman JN, Black PM, Zervas NT, Lidov HG, Garber JR. Gangliocytoma masquerading as a prolactinoma. Case report. J Neurosurg 91(3):490–495, 1999.

    PubMed  CAS  Google Scholar 

  34. Morikawa M, Tamaki N, Kokunai T, Imai Y. Intrasellar pituitary gangliocyto-adenoma presenting with acromegaly: case report. Neurosurgery 40(3):611–614; discussion 614–615, 1997.

    Article  PubMed  CAS  Google Scholar 

  35. Muller W, Marcos F. [The occurrence of ganglion cells in a pituitary tumor.]. Virchows Arch 325(6):733–736, 1954.

    Article  PubMed  CAS  Google Scholar 

  36. Muller W. Uber das gemeinsame Vorkommen eines Hypophysenadenomas mit einem Gangliocytom in Zwei Fallen: ein Beitrag zur Frage der Neurosekretion. Acta Neurochir (Wien) 7:13–29, 1959.

    Article  Google Scholar 

  37. Nagasaka T, Nakashima N, Furui A, Wakabayashi T, Yoshida J. Sarcomatous transformation of pituitary adenoma after bromocriptine therapy. Hum Pathol 29(2):190–193, 1998.

    Article  PubMed  CAS  Google Scholar 

  38. Nikonov AA. [Hormonally active (somatotropin) hypophyseal ganglioneuroma.]. Arkh Patol 43(12):48–52, 1981.

    PubMed  CAS  Google Scholar 

  39. Oberg K, Skogseid B, Eriksson B. Multiple endocrine neoplasia type 1 (MEN-1). Clinical, biochemical and genetical investigations. Acta Oncol 28(3):383–387, 1989.

    PubMed  CAS  Google Scholar 

  40. Paulus W, Slowik F, Jellinger K. Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 18(5):395–402, 1991.

    Article  PubMed  CAS  Google Scholar 

  41. Pearce SH. Multiple endocrine neoplasia type 1 (MEN1): recent advances. Clin Endocrinol (Oxf) 47(5):513–514, 1997.

    Article  CAS  Google Scholar 

  42. Pieterse S, Dinning TA, Blumbergs PC. Postir-radiation sarcomatous transformation of a pituitary adenoma: a combined pituitary tumor. Case report. J Neurosurg 56(2):283–286, 1982.

    PubMed  CAS  Google Scholar 

  43. Powell HC, Marshall LF, Ignelzi RJ. Post-irradiation pituitary sarcoma. Acta Neuropathol (Berl) 39(2):165–167, 1977.

    Article  CAS  Google Scholar 

  44. Puchner MJ, Herrmann HD. Intrasellar pituitary gangliocyto-adenoma presenting with acromegaly: case report. Neurosurgery 42(5):1197–1199, 1998.

    Article  PubMed  CAS  Google Scholar 

  45. Reichenthal E, Cohen ML, Manor CR, Marshak G, Matz S, Shalit MN. Primary osteogenic sarcoma of the sellar region. Case report. J Neurosurg 55(2):299–302, 1981.

    PubMed  CAS  Google Scholar 

  46. Rhodes RH, Dusseau JJ, Boyd AS, Jr., Knigge KM. Intrasellar neural-adenohypophyseal choristoma. A morphological and immunocytochemical study. J Neuropathol Exp Neurol 41(3):267–280, 1982.

    Article  PubMed  CAS  Google Scholar 

  47. Russell D, Rubistein L. Pathology of tumours of the nervous system. In: D Russell, L Rubistein, eds. Pathology of tumours in the nervous system. 5th ed. London: Edward Arnold; 1989; 507–517.

    Google Scholar 

  48. Sabel MC, Hans VH, Reifenberger G. Mixed gangliocytoma/pituitary adenoma. Arch Neurol 57(4):587–588, 2000.

    Article  PubMed  CAS  Google Scholar 

  49. Saeger W, Puchner MJ, Ludecke DK. Combined sellar gangliocytoma and pituitary adenoma in acromegaly or Cushing’s disease. A report of 3 cases. Virchows Arch 425(1):93–99, 1994.

    Article  PubMed  CAS  Google Scholar 

  50. Samaan NA, Ouais S, Ordonez NG, Choksi UA, Sellin RV, Hickey RC. Multiple endocrine syndrome type I. Clinical, laboratory findings, and management in five families. Cancer 64(3):741–752, 1989.

    Article  PubMed  CAS  Google Scholar 

  51. Scheithauer BW, Kovacs K, Randall RV, Horvath E, Okazaki H, Laws ER, Jr. Hypothalamic neuronal hamartoma and adenohypophyseal neuronal choristoma: their association with growth hormone adenoma of the pituitary gland. J Neuropathol Exp Neurol 42(6):648–663, 1983.

    PubMed  CAS  Google Scholar 

  52. Scheithauer BW, Horvath E, Kovacs K, et al. Prolactin-producing pituitary adenoma and carcinoma with neuronal components—a metaplastic lesion. Pituitary 1(3–4):197–205, 1999.

    Article  PubMed  CAS  Google Scholar 

  53. Scindler C, Neurnberger F, Schachenmayer W. Invasive pituitary adenoma in a patient with hypothalamic neuronal hamartoma. Clin Neuropathol 12:264 (Abstract), 1993.

    Google Scholar 

  54. Serebrin R, Robertson DM. Ganglioneuroma arising in the pituitary fossa: a twenty year follow-up. J Neurol Neurosurg Psychiatry 47(1):97–98, 1984.

    PubMed  CAS  Google Scholar 

  55. Sharma MC, Karak AK, Mahapatra AK, Sarkar C. Pituitary adenoma with neuronal choristoma: a report of two rare cases. Clin Neurol Neurosurg 101(2):128–132, 1999.

    Article  PubMed  CAS  Google Scholar 

  56. Shi T, Farrell MA, Kaufmann JC. Fibrosarcoma complicating irradiated pituitary adenoma. Surg Neurol 22(3):277–284, 1984.

    Article  PubMed  CAS  Google Scholar 

  57. Skogseid B, Rastad J, Oberg K. Multiple endocrine neoplasia type 1. Clinical features and screening. Endocrinol Metab Clin North Am 23(1):1–18, 1994.

    PubMed  CAS  Google Scholar 

  58. Slowik F, Fazekas I, Balint K, et al. Intrasellar hamartoma associated with pituitary adenoma. Acta Neuropathol (Berl) 80(3):328–333, 1990.

    Article  CAS  Google Scholar 

  59. Tajika Y, Kubo O, Takeshita M, Tajika T, Shimizu T, Kitamura K. [An intracranial collision tumor composed of intrasellar gangliocytoma and pituitary adenoma.]. No Shinkei Geka 17(12):1181–1186, 1989.

    PubMed  CAS  Google Scholar 

  60. Teh BT, Zedenius J, Kytola S, et al. Thymic carcinoids in multiple endocrine neoplasia type 1. Ann Surg 228(1):99–105, 1998.

    Article  PubMed  CAS  Google Scholar 

  61. Terry R, Hyams V, Davidoff L. Combined non-metastasizing fibrosarcoma and chromophobe tumor of the pituitary. Cancer 12:791–798, 1958.

    Article  Google Scholar 

  62. Thapar K, Kovacs K, Stefaneanu L, et al. Overexpression of the growth-hormone-releasing hormone gene in acromegaly associated pituitary tumors. An event associated with neoplastic progression and aggressive behavior. Am J Pathol 151(3):769–784, 1997.

    PubMed  CAS  Google Scholar 

  63. Towfighi J, Salam MM, McLendon RE, Powers S, Page RB. Ganglion cell-containing tumors of the pituitary gland. Arch Pathol Lab Med 120(4):369–377, 1996.

    PubMed  CAS  Google Scholar 

  64. Trojanowski JQ, Gordon D, Obrocka M, Lee VM. The developmental expression of neurofilament and glial filament proteins in the human pituitary gland: an immunohistochemical study with monoclonal antibodies. Brain Res 315(2):229–239, 1984.

    PubMed  CAS  Google Scholar 

  65. Ule G, Waidelich FW. Neurosecretoric nerve-cell choristoma in the anterior pituitary (author’s transl)]. Acta Neuropathol (Berl) 36(1):81–84, 1976.

    Article  CAS  Google Scholar 

  66. Waltz TA, Brownell B. Sarcoma: a possible late result of effective radiation therapy for pituitary adenoma. Report of two cases. J Neurosurg 24(5):901–907, 1966.

    PubMed  CAS  Google Scholar 

  67. Weil RJ, Vortmeyer AO, Huang S, et al. 11q13 allelic loss in pituitary tumors in patients with multiple endocrine neoplasia syndrome type 1. Clin Cancer Res 4(7):1673–1678, 1998.

    PubMed  CAS  Google Scholar 

  68. Willis R. Pathological study of tumors of the pituitary region. Med J Aust 1:281–291, 1938.

    Google Scholar 

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Moro, M., Giannini, C., Scheithauer, B.W. et al. Combined sellar fibrosarcoma and prolactinoma with neuronal metaplasia: Report of a case unassociated with radiotherapy. Endocr Pathol 15, 149–158 (2004). https://doi.org/10.1385/EP:15:2:149

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