Abstract
Background
Sellar melanocytomas represent a small subgroup of primary melanocytic tumors. They arise from melanocytes located in the meningeal lining of the sellar floor or in the diaphragma sellae and this location is very uncommon. Usually, sellar melanocytomas are benign and slow-growing tumors with a high likelihood of recurrence.
Purpose
To our knowledge, due to the rarity of this condition, there are no guidelines regarding their diagnosis and treatment in the medical literature to date. We have developed a narrative review, analyzing the available studies regarding primary sellar melanocytomas reported in the medical literature. We have found ten papers on this topic and all of them are case reports. In all patients, tumor diagnosis was performed after the occurrence of neurological symptoms, in particular progressive visual loss or endocrinological disorders. The diagnosis is difficult, and it requires several preoperative and postoperative investigations, but histological examination is crucial.
Conclusions
Transsphenoidal surgery is the first-choice treatment. In case of tumor’s recurrence or regrowth, the role of radiation therapy and chemotherapy is not entirely clear.
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References
Goldgeier MH, Klein LE, Klein-Angerer S, Moellmann G, Nordlund JJ (1984) The distribution of melanocytes in the leptomeninges of the human brain. J Invest Dermatol 82(3):235–238
Smith AB, Rushing EJ, Smirniotopoulos JG (2009) Pigmented lesions of the central nervous system: radiologic-pathologic correlation. Radiographics 29(5):1503–1524. https://doi.org/10.1148/rg.295095109
Tewari MK, Radotra BD, Sharma BS, Mathuriya SN, Pathak A, Banerjee AK, Kak VK (1990) Meningeal melanocytoma: report of two cases. Indian J Cancer 27(3):133–137
Classen J, Hehr T, Paulus W, Plate K, Bamberg M (2002) Suprasellar melanocytoma: a case of primary radiotherapy and review of the literature. J Neurooncol 58(1):39–46
Rousseau A, Bernier M, Kujas M, Varlet P (2005) Primary intracranial melanocytic tumor simulating pituitary macroadenoma: case report and review of the literature. Neurosurgery 57(2):E369 (discussion E369)
Vezzosi D, Capuani C, Loubes-Lacroix F, Lagarrigue J, Bennet A, Delisle MB, Caron P (2009) Primary sellar melanocytic tumor: report of new case and literature review. Pituitary 12(1):51–56. https://doi.org/10.1007/s11102-007-0076-0
Coulibaly B, Bouvier C, Paula AM, Fernandez C, Dufour H, Figarella-Branger D (2011) Pituitary melanocytoma mimicking an adenoma. Ann Pathol 31(1):50–52. https://doi.org/10.1016/j.annpat.2010.09.007
Sakata K, Miyoshi J, Takeshige N, Komaki S, Miyagi N, Nakashima S, Morioka M, Sugita Y (2015) Primary meningeal melanocytoma of the sellar region: review of the literature and differential diagnosis with special reference to angiographical features. Pituitary 18(5):685–694. https://doi.org/10.1007/s11102-015-0635-8
Zhou HJ, Zhan RY, Ma YH, Cao F, Zheng XJ (2015) Primary sellar melanocytic tumor mimicking hemorrhagic pituitary macroadenoma: case report and literature review. Br J Neurosurg 29(2):298–302. https://doi.org/10.3109/02688697.2014.967752
Brito AB, Rogerio F, Reis F, Garmes HM, Vassallo J, Lima CS (2016) Primary meningeal melanocytoma mimicking a nonfunctioning pituitary adenoma. Clin Neuropathol 35(3):158–161. https://doi.org/10.5414/NP300885
Wang F, Ling S (2018) Primary meningeal melanocytoma in sellar region, simulating a nonfunctioning pituitary adenoma: case report and literature review. World Neurosurg 112:209–213. https://doi.org/10.1016/j.wneu.2018.01.145
Albano L, Losa M, Spatola G, Panni P, Terreni MR, Barzaghi LR, Mortini P (2019) Primary sellar melanocytoma: report of two cases treated at the same institution and their long term outcome. Br J Neurosurg 20:1–5. https://doi.org/10.1080/02688697.2019.1667485
Nmkc LD (1999) The neural crest. Cambridge University Press, Cambridge
Domingues MJ, Larue L, Bonaventure J (2013) Migration of melanocytic lineage-derived cells. Med Sci (Paris) 29(3):287–292. https://doi.org/10.1051/medsci/2013293015
Hwang HW, Pavan WJ (2010) Melanocytes don’t always take the high road. Pigment Cell Melanoma Res 23(1):11–13. https://doi.org/10.1111/j.1755-148X.2009.00665.x
Wjkt M (2007) Melanin, melanocytes, skin. In: Shaw P (ed) Pathology of melanocytic disorders. Hodder Arnold, London, p 6
Holbrook KA, Underwood RA, Vogel AM, Gown AM, Kimball H (1989) The appearance, density and distribution of melanocytes in human embryonic and fetal skin revealed by the anti-melanoma monoclonal antibody, HMB-45. Anat Embryol (Berl) 180(5):443–455
Wjkt M (2007) Melanin, melanocytes, skin. In: Shaw P (ed) Pathology of melanocytic disorders. Hodder Arnold, London, p 4
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW (2016) The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol 131(6):803–820. https://doi.org/10.1007/s00401-016-1545-1
WHO (2016) WHO classification of tumours of the central nervous system, 4th edn. IARC, Lyon
Kusters-Vandevelde HV, Kusters B, van Engen-van Grunsven AC, Groenen PJ, Wesseling P, Blokx WA (2015) Primary melanocytic tumors of the central nervous system: a review with focus on molecular aspects. Brain Pathol 25(2):209–226. https://doi.org/10.1111/bpa.12241
Brat DJPA (2007) Melanocytic lesions. In: Wiestler OD, Cavenee WK, Louis DN (eds) WHO classification of tumours of the central nervous system. International agency for research on cancer, Lyon, pp 181–183
Litofsky NS, Zee CS, Breeze RE, Chandrasoma PT (1992) Meningeal melanocytoma: diagnostic criteria for a rare lesion. Neurosurgery 31(5):945–948
Chappell PM, Kelly WM, Ercius M (1990) Primary sellar melanoma simulating hemorrhagic pituitary adenoma: MR and pathologic findings. Am J Neuroradiol 11(5):1054–1056
Tuttenberg J, Fink W, Back W, Wenz F, Schadendorf D, Thome C (2004) A rare primary sellar melanoma. Case report. J Neurosurg 100(5):931–934. https://doi.org/10.3171/jns.2004.100.5.0931
Beseoglu K, Knobbe CB, Reifenberger G, Steiger HJ, Stummer W (2006) Supratentorial meningeal melanocytoma mimicking a convexity meningioma. Acta Neurochir (Wien) 148(4):485–490. https://doi.org/10.1007/s00701-005-0705-1
Leonardi MA, Lumenta CB, Stolzle A, Muller-Hocker J (1998) Unusual clinical presentation of a meningeal melanocytoma with seizures: case report and review of the literature. Acta Neurochir (Wien) 140(6):621–628
Jellinger K, Bock F, Brenner H (1988) Meningeal melanocytoma. Report of a case and review of the literature. Acta Neurochir (Wien) 94(1–2):78–87
Navas M, Pascual JM, Fraga J, Pedrosa M, Shakur S, Carrasco R, Martinez P, Manzanares R, de Sola RG (2009) Intracranial intermediate-grade meningeal melanocytoma with increased cellular proliferative index: an illustrative case associated with a nevus of Ota. J Neurooncol 95(1):105–115. https://doi.org/10.1007/s11060-009-9907-3
Wang H, Zhang S, Wu C, Zhang Z, Qin T (2013) Melanocytomas of the central nervous system: a clinicopathological and molecular study. Eur J Clin Invest 43(8):809–815. https://doi.org/10.1111/eci.12109
Davies H, Bignell GR, Cox C, Stephens P, Edkins S, Clegg S, Teague J, Woffendin H, Garnett MJ, Bottomley W, Davis N, Dicks E, Ewing R, Floyd Y, Gray K, Hall S, Hawes R, Hughes J, Kosmidou V, Menzies A, Mould C, Parker A, Stevens C, Watt S, Hooper S, Wilson R, Jayatilake H, Gusterson BA, Cooper C, Shipley J, Hargrave D, Pritchard-Jones K, Maitland N, Chenevix-Trench G, Riggins GJ, Bigner DD, Palmieri G, Cossu A, Flanagan A, Nicholson A, Ho JW, Leung SY, Yuen ST, Weber BL, Seigler HF, Darrow TL, Paterson H, Marais R, Marshall CJ, Wooster R, Stratton MR, Futreal PA (2002) Mutations of the BRAF gene in human cancer. Nature 417(6892):949–954. https://doi.org/10.1038/nature00766
Van Raamsdonk CD, Bezrookove V, Green G, Bauer J, Gaugler L, O’Brien JM, Simpson EM, Barsh GS, Bastian BC (2009) Frequent somatic mutations of GNAQ in uveal melanoma and blue naevi. Nature 457(7229):599–602. https://doi.org/10.1038/nature07586
Kusters-Vandevelde HV, Klaasen A, Kusters B, Groenen PJ, van Engen-van Grunsven IA, van Dijk MR, Reifenberger G, Wesseling P, Blokx WA (2010) Activating mutations of the GNAQ gene: a frequent event in primary melanocytic neoplasms of the central nervous system. Acta Neuropathol 119(3):317–323. https://doi.org/10.1007/s00401-009-0611-3
Ross EM, Wilkie TM (2000) GTPase-activating proteins for heterotrimeric G proteins: regulators of G protein signaling (RGS) and RGS-like proteins. Annu Rev Biochem 69:795–827. https://doi.org/10.1146/annurev.biochem.69.1.795
Murali R, Wiesner T, Rosenblum MK, Bastian BC (2012) GNAQ and GNA11 mutations in melanocytomas of the central nervous system. Acta Neuropathol 123(3):457–459. https://doi.org/10.1007/s00401-012-0948-x
Bauer J, Kilic E, Vaarwater J, Bastian BC, Garbe C, de Klein A (2009) Oncogenic GNAQ mutations are not correlated with disease-free survival in uveal melanoma. Br J Cancer 101(5):813–815. https://doi.org/10.1038/sj.bjc.6605226
Koopmans AE, Vaarwater J, Paridaens D, Naus NC, Kilic E, de Klein A, Rotterdam Ocular Melanoma Study G (2013) Patient survival in uveal melanoma is not affected by oncogenic mutations in GNAQ and GNA11. Br J Cancer 109(2):493–496. https://doi.org/10.1038/bjc.2013.299
Koelsche C, Hovestadt V, Jones DT, Capper D, Sturm D, Sahm F, Schrimpf D, Adeberg S, Bohmer K, Hagenlocher C, Mechtersheimer G, Kohlhof P, Muhleisen H, Beschorner R, Hartmann C, Braczynski AK, Mittelbronn M, Buslei R, Becker A, Grote A, Urbach H, Staszewski O, Prinz M, Hewer E, Pfister SM, von Deimling A, Reuss DE (2015) Melanotic tumors of the nervous system are characterized by distinct mutational, chromosomal and epigenomic profiles. Brain Pathol 25(2):202–208. https://doi.org/10.1111/bpa.12228
Gessi M, Hammes J, Lauriola L, Dorner E, Kirfel J, Kristiansen G, Muehlen A, Denkhaus D, Waha A, Pietsch T (2013) GNA11 and N-RAS mutations: alternatives for MAPK pathway activating GNAQ mutations in primary melanocytic tumours of the central nervous system. Neuropathol Appl Neurobiol 39(4):417–425. https://doi.org/10.1111/j.1365-2990.2012.01288.x
Czarnecki EJ, Silbergleit R, Gutierrez JA (1997) MR of spinal meningeal melanocytoma. Am J Neuroradiol 18(1):180–182
Naul LG, Hise JH, Bauserman SC, Todd FD (1991) CT and MR of meningeal melanocytoma. Am J Neuroradiol 12(2):315–316
Uematsu Y, Yukawa S, Yokote H, Itakura T, Hayashi S, Komai N (1992) Meningeal melanocytoma: magnetic resonance imaging characteristics and pathological features. Case report. J Neurosurg 76(4):705–709. https://doi.org/10.3171/jns.1992.76.4.0705
O’Brien TF, Moran M, Miller JH, Hensley SD (1995) Meningeal melanocytoma. An uncommon diagnostic pitfall in surgical neuropathology. Arch Pathol Lab Med 119(6):542–546
Hamasaki O, Nakahara T, Sakamoto S, Kutsuna M, Sakoda K (2002) Intracranial meningeal melanocytoma. Neurol Med Chir (Tokyo) 42(11):504–509
Barkovich AJ, Frieden IJ, Williams ML (1994) MR of neurocutaneous melanosis. Am J Neuroradiol 15(5):859–867
Chen CJ, Hsu YI, Ho YS, Hsu YH, Wang LJ, Wong YC (1997) Intracranial meningeal melanocytoma: CT and MRI. Neuroradiology 39(11):811–814
Stevens G, Firth I, Coates A (1992) Cerebral metastases from malignant melanoma. Radiother Oncol 23(3):185–191
Komninos J, Vlassopoulou V, Protopapa D, Korfias S, Kontogeorgos G, Sakas DE, Thalassinos NC (2004) Tumors metastatic to the pituitary gland: case report and literature review. J Clin Endocrinol Metab 89(2):574–580. https://doi.org/10.1210/jc.2003-030395
Freda PU, Wardlaw SL, Post KD (1996) Unusual causes of sellar/parasellar masses in a large transsphenoidal surgical series. J Clin Endocrinol Metab 81(10):3455–3459. https://doi.org/10.1210/jcem.81.10.8855784
Post KD, McCormick PC, Bello JA (1987) Differential diagnosis of pituitary tumors. Endocrinol Metab Clin N Am 16(3):609–645
Leung GK, Chow WS, Tan KC, Fan YW, Lam KS (2003) Metastatic melanoma of the pituitary gland. Case report. J Neurosurg 99(5):913–915. https://doi.org/10.3171/jns.2003.99.5.0913
Hayward RD (1976) Malignant melanoma and the central nervous system: A guide for classification based on the clinical findings. J Neurol Neurosurg Psychiatry 39(6):526–530
Albrecht SBJ, Kovacs K (2002) Non pituitary tumors of the sellar region. In: Melmed S (ed) The pituitary, 2nd edn. Black-well Publishing, Massachusetts, pp 592–609
Mortini P, Barzaghi LR, Albano L, Panni P, Losa M (2018) Microsurgical therapy of pituitary adenomas. Endocrine 59(1):72–81. https://doi.org/10.1007/s12020-017-1458-3
Mortini P, Barzaghi R, Losa M, Boari N, Giovanelli M (2007) Surgical treatment of giant pituitary adenomas: strategies and results in a series of 95 consecutive patients. Neurosurgery 60(6):993–1002. https://doi.org/10.1227/01.neu.0000255459.14764.ba(discussion 1003-1004)
Zhang X, Fei Z, Zhang J, Fu L, Zhang Z, Liu W, Chen Y (1999) Management of nonfunctioning pituitary adenomas with suprasellar extensions by transsphenoidal microsurgery. Surg Neurol 52(4):380–385
Bydon A, Gutierrez JA, Mahmood A (2003) Meningeal melanocytoma: an aggressive course for a benign tumor. J Neurooncol 64(3):259–263
Clarke DB, Leblanc R, Bertrand G, Quartey GR, Snipes GJ (1998) Meningeal melanocytoma. Report of a case and a historical comparison. J Neurosurg 88(1):116–121. https://doi.org/10.3171/jns.1998.88.1.0116
Crouzet J, Richard S, Pillet G, Muckensturm B, Srour A, Pradat P (1992) Meningeal melanocytoma or multiple pigmented meningioma of the spinal canal. Report of a case. Review of the literature. Rev Rhum Mal Osteoartic 59(11):738–743
Verma DS, Spitzer G, Legha S, McCredie KB (1979) Chemoimmunotherapy for meningeal melanocytoma of the thoracic spinal cord. Report of a case. JAMA 242(22):2435–2436
Chapman PB, Hauschild A, Robert C, Haanen JB, Ascierto P, Larkin J, Dummer R, Garbe C, Testori A, Maio M, Hogg D, Lorigan P, Lebbe C, Jouary T, Schadendorf D, Ribas A, O’Day SJ, Sosman JA, Kirkwood JM, Eggermont AM, Dreno B, Nolop K, Li J, Nelson B, Hou J, Lee RJ, Flaherty KT, McArthur GA, Group B-S (2011) Improved survival with vemurafenib in melanoma with BRAF V600E mutation. N Engl J Med 364(26):2507–2516. https://doi.org/10.1056/NEJMoa1103782
Dubbink HJ, Deans ZC, Tops BB, van Kemenade FJ, Koljenovic S, van Krieken HJ, Blokx WA, Dinjens WN, Groenen PJ (2014) Next generation diagnostic molecular pathology: critical appraisal of quality assurance in Europe. Mol Oncol 8(4):830–839. https://doi.org/10.1016/j.molonc.2014.03.004
Louis DN, Perry A, Burger P, Ellison DW, Reifenberger G, von Deimling A, Aldape K, Brat D, Collins VP, Eberhart C, Figarella-Branger D, Fuller GN, Giangaspero F, Giannini C, Hawkins C, Kleihues P, Korshunov A, Kros JM, Beatriz Lopes M, Ng HK, Ohgaki H, Paulus W, Pietsch T, Rosenblum M, Rushing E, Soylemezoglu F, Wiestler O, Wesseling P, International Society Of N-H (2014) International Society Of Neuropathology-Haarlem consensus guidelines for nervous system tumor classification and grading. Brain Pathol 24(5):429–435. https://doi.org/10.1111/bpa.12171
Acknowledgements
The authors are grateful to Taylor & Francis Group for approving the license to reproduce Figs. 1 and 2 previously published in “Primary sellar melanocytomas: report of two cases treated at the same institution and their long-term outcome”; Albano et al. British Journal of Neurosurgery, copyright © 2019 The Neurosurgical Foundation, reprinted by permission of Informa UK Limited.
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Work and concept were initiated by LA; literature research and data analysis were performed by LA, GS, PP. The manuscript was written by LA, ML, LRB, GS, PP and MRT. ML and PM critically reviewed the manuscript.
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Albano, L., Losa, M., Barzaghi, L.R. et al. Primary sellar melanocytoma: pathological, clinical and treatment review. J Endocrinol Invest 43, 575–585 (2020). https://doi.org/10.1007/s40618-019-01158-8
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DOI: https://doi.org/10.1007/s40618-019-01158-8