Skip to main content

Advertisement

SpringerLink
Log in

Primary sellar melanocytoma: pathological, clinical and treatment review

  • Short Review
  • Published:
Journal of Endocrinological Investigation Aims and scope Submit manuscript

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.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Fig. 1

Reproduced with permission from Albano et al. Primary sellar melanocytomas report of two cases treated at the same institution and their long-term outcome, British Journal of Neurosurgery, 2019 [12]

Fig. 2

Reproduced with permission from Albano et al. Primary sellar melanocytomas report of two cases treated at the same institution and their long-term outcome, British Journal of Neurosurgery, 2019 [12]

Similar content being viewed by others

References

  1. 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

    Article  CAS  Google Scholar 

  2. 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

    Article  PubMed  Google Scholar 

  3. 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

    CAS  PubMed  Google Scholar 

  4. 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

    Article  Google Scholar 

  5. 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)

    Article  Google Scholar 

  6. 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

    Article  PubMed  Google Scholar 

  7. 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

    Article  PubMed  Google Scholar 

  8. 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

    Article  CAS  PubMed  Google Scholar 

  9. 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

    Article  PubMed  Google Scholar 

  10. 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

    Article  PubMed  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. 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

    Article  Google Scholar 

  13. Nmkc LD (1999) The neural crest. Cambridge University Press, Cambridge

    Google Scholar 

  14. 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

    Article  Google Scholar 

  15. 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

    Article  PubMed  Google Scholar 

  16. Wjkt M (2007) Melanin, melanocytes, skin. In: Shaw P (ed) Pathology of melanocytic disorders. Hodder Arnold, London, p 6

    Google Scholar 

  17. 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

    Article  CAS  Google Scholar 

  18. Wjkt M (2007) Melanin, melanocytes, skin. In: Shaw P (ed) Pathology of melanocytic disorders. Hodder Arnold, London, p 4

    Google Scholar 

  19. 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

    Article  PubMed  Google Scholar 

  20. WHO (2016) WHO classification of tumours of the central nervous system, 4th edn. IARC, Lyon

    Google Scholar 

  21. 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

    Article  CAS  PubMed  Google Scholar 

  22. 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

    Google Scholar 

  23. Litofsky NS, Zee CS, Breeze RE, Chandrasoma PT (1992) Meningeal melanocytoma: diagnostic criteria for a rare lesion. Neurosurgery 31(5):945–948

    Article  CAS  Google Scholar 

  24. 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

    CAS  PubMed  Google Scholar 

  25. 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

    Article  PubMed  Google Scholar 

  26. 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

    Article  CAS  Google Scholar 

  27. 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

    Article  CAS  Google Scholar 

  28. 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

    Article  CAS  Google Scholar 

  29. 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

    Article  PubMed  Google Scholar 

  30. 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

    Article  CAS  PubMed  Google Scholar 

  31. 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

    Article  CAS  PubMed  Google Scholar 

  32. 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

    Article  CAS  PubMed  Google Scholar 

  33. 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

    Article  CAS  PubMed  Google Scholar 

  34. 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

    Article  CAS  PubMed  Google Scholar 

  35. 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

    Article  PubMed  PubMed Central  Google Scholar 

  36. 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

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. 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

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. 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

    Article  CAS  PubMed  Google Scholar 

  39. 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

    Article  CAS  PubMed  Google Scholar 

  40. Czarnecki EJ, Silbergleit R, Gutierrez JA (1997) MR of spinal meningeal melanocytoma. Am J Neuroradiol 18(1):180–182

    CAS  PubMed  Google Scholar 

  41. Naul LG, Hise JH, Bauserman SC, Todd FD (1991) CT and MR of meningeal melanocytoma. Am J Neuroradiol 12(2):315–316

    CAS  PubMed  Google Scholar 

  42. 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

    Article  CAS  PubMed  Google Scholar 

  43. 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

    PubMed  Google Scholar 

  44. Hamasaki O, Nakahara T, Sakamoto S, Kutsuna M, Sakoda K (2002) Intracranial meningeal melanocytoma. Neurol Med Chir (Tokyo) 42(11):504–509

    Article  Google Scholar 

  45. Barkovich AJ, Frieden IJ, Williams ML (1994) MR of neurocutaneous melanosis. Am J Neuroradiol 15(5):859–867

    CAS  PubMed  Google Scholar 

  46. Chen CJ, Hsu YI, Ho YS, Hsu YH, Wang LJ, Wong YC (1997) Intracranial meningeal melanocytoma: CT and MRI. Neuroradiology 39(11):811–814

    Article  CAS  Google Scholar 

  47. Stevens G, Firth I, Coates A (1992) Cerebral metastases from malignant melanoma. Radiother Oncol 23(3):185–191

    Article  CAS  Google Scholar 

  48. 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

    Article  CAS  PubMed  Google Scholar 

  49. 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

    Article  CAS  PubMed  Google Scholar 

  50. Post KD, McCormick PC, Bello JA (1987) Differential diagnosis of pituitary tumors. Endocrinol Metab Clin N Am 16(3):609–645

    Article  CAS  Google Scholar 

  51. 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

    Article  PubMed  Google Scholar 

  52. 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

    Article  CAS  Google Scholar 

  53. 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

    Google Scholar 

  54. 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

    Article  CAS  PubMed  Google Scholar 

  55. 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)

    Article  PubMed  Google Scholar 

  56. 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

    Article  CAS  Google Scholar 

  57. Bydon A, Gutierrez JA, Mahmood A (2003) Meningeal melanocytoma: an aggressive course for a benign tumor. J Neurooncol 64(3):259–263

    Article  Google Scholar 

  58. 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

    Article  CAS  PubMed  Google Scholar 

  59. 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

    CAS  PubMed  Google Scholar 

  60. 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

    Article  CAS  Google Scholar 

  61. 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

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  62. 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

    Article  PubMed  PubMed Central  Google Scholar 

  63. 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

    Article  PubMed  Google Scholar 

Download references

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.

Funding

No funding was received for this research.

Author information

Authors and Affiliations

  1. Department of Neurosurgery and Gamma Knife Radiosurgery, I.R.C.C.S. San Raffaele Scientific Institute, Vita-Salute University, Via Olgettina 60, 20132, Milan, Italy

    L. Albano, M. Losa, L. R. Barzaghi, P. Panni & P. Mortini

  2. Department of Neurosurgery, Assistance Publique Hopitaux de Marseille, Marseille, France

    G. Spatola

  3. Department of Pathology, I.R.C.C.S. San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy

    M. R. Terreni

Authors
  1. L. Albano
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. Losa
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. L. R. Barzaghi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. G. Spatola
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. P. Panni
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. M. R. Terreni
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. P. Mortini
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

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.

Corresponding author

Correspondence to L. Albano.

Ethics declarations

Conflict of interest

The authors report no conflict of interest.

Ethical approval 

 This article does not contain any studies with human participants or animals performed by any of the authors.

Informed consent

For this type of study, formal consent is not required.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40618-019-01158-8

Keywords

Search

Navigation