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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Alpers B, et al. The ganglioneurons of the central nervous system. Arch Neurol Psychiatry 26:501–523, 1931.
Amine AR, Sugar O. Suprasellar osteogenic sarcoma following radiation for pituitary adenoma. Case report. J Neurosurg 44(1):88–91, 1976.
Anderson WR, Cameron JD, Tsai SH. Primary intracranial leiomyosarcoma. Case report with ultrastructural study. J Neurosurg 53(3):401–405, 1980.
Angelstein I. [Pathogenesis of acromegaly.]. Dtsch Z Nervenheilkd 170(4):337–348, 1953.
Arseni C, Horvath L, Carp N, Ciurea V. Intracranial ganglioneuromas in children. Acta Neurochir (Wien) 32(3–4):279–286, 1975.
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.
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.
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.
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.
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.
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.
Casper J. Ueber neurogene Geschwulste im Hinterlappen der Hypopyyse. Zentralb Allg Pathol 56:404–411, 1933.
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.
Fischer EG, Morris JH, Kettyle WM. Intrasellar gangliocytoma and syndromes of pituitary hypersecretion. Case report. J Neurosurg 59(6):1071–1075, 1983.
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.
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.
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.
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.
Hourihane M, Moran M, Burke M. Intrasellar gangliocytoma associated with functional pituitary gland microadenoma. J Neuropathol Exp Neurol 51:364 (Abstract), 1992.
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.
Jakumeit HD, Zimmermann V, Guiot G. Intrasellar gangliocytomas. Report of four cases. J Neurosurg 40(5):626–630, 1974.
Kamel OW, Horoupian DS, Silverberg GD. Mixed gangliocytoma-adenoma: a distinct neuroendocrine tumor of the pituitary fossa. Hum Pathol 20(12):1198–1203, 1989.
Kiyono H. Die Histopathologie der hypophyse. Virchows Arch A Pathol Anat Histopathol 259:388–465, 1926.
Kleinsasser O, Albrecht H. Zur Kenntnis der Osteosarkome des Stirn-und Keilbeines. Arch Ohren Heilk 170:595–603, 1957.
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.
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.
Kroh H, Marchel A. Intrasellar neuronal hamartoma associated with pituitary adenoma. Case report. Folia Neuropathol 38(3):119–124, 2000.
Landolt AM, Froesch ER. [Prolactin producing hypophyseal adenoma: diagnosis and therapeutic possibilities]. Schweiz Med Wochenschr 115(23):803–809, 1985.
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.
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.
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.
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.
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.
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.
Muller W, Marcos F. [The occurrence of ganglion cells in a pituitary tumor.]. Virchows Arch 325(6):733–736, 1954.
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.
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.
Nikonov AA. [Hormonally active (somatotropin) hypophyseal ganglioneuroma.]. Arkh Patol 43(12):48–52, 1981.
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.
Paulus W, Slowik F, Jellinger K. Primary intracranial sarcomas: histopathological features of 19 cases. Histopathology 18(5):395–402, 1991.
Pearce SH. Multiple endocrine neoplasia type 1 (MEN1): recent advances. Clin Endocrinol (Oxf) 47(5):513–514, 1997.
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.
Powell HC, Marshall LF, Ignelzi RJ. Post-irradiation pituitary sarcoma. Acta Neuropathol (Berl) 39(2):165–167, 1977.
Puchner MJ, Herrmann HD. Intrasellar pituitary gangliocyto-adenoma presenting with acromegaly: case report. Neurosurgery 42(5):1197–1199, 1998.
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.
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.
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.
Sabel MC, Hans VH, Reifenberger G. Mixed gangliocytoma/pituitary adenoma. Arch Neurol 57(4):587–588, 2000.
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.
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.
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.
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.
Scindler C, Neurnberger F, Schachenmayer W. Invasive pituitary adenoma in a patient with hypothalamic neuronal hamartoma. Clin Neuropathol 12:264 (Abstract), 1993.
Serebrin R, Robertson DM. Ganglioneuroma arising in the pituitary fossa: a twenty year follow-up. J Neurol Neurosurg Psychiatry 47(1):97–98, 1984.
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.
Shi T, Farrell MA, Kaufmann JC. Fibrosarcoma complicating irradiated pituitary adenoma. Surg Neurol 22(3):277–284, 1984.
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.
Slowik F, Fazekas I, Balint K, et al. Intrasellar hamartoma associated with pituitary adenoma. Acta Neuropathol (Berl) 80(3):328–333, 1990.
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.
Teh BT, Zedenius J, Kytola S, et al. Thymic carcinoids in multiple endocrine neoplasia type 1. Ann Surg 228(1):99–105, 1998.
Terry R, Hyams V, Davidoff L. Combined non-metastasizing fibrosarcoma and chromophobe tumor of the pituitary. Cancer 12:791–798, 1958.
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.
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.
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.
Ule G, Waidelich FW. Neurosecretoric nerve-cell choristoma in the anterior pituitary (author’s transl)]. Acta Neuropathol (Berl) 36(1):81–84, 1976.
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.
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.
Willis R. Pathological study of tumors of the pituitary region. Med J Aust 1:281–291, 1938.
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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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DOI: https://doi.org/10.1385/EP:15:2:149