CASE REPORT

Tumor Medular Melanocítico: relato de caso

Rare Melanocytic Medullar Tumor: a case report study

  • Ricardo Ribeiro de Lima (1)    Ricardo Ribeiro de Lima (1)
  • Oscar Nelson Reimann Junior (2,3)    Oscar Nelson Reimann Junior (2,3)
  • Stephanie Lindner (3,4)    Stephanie Lindner (3,4)
  • Felipe Negrão (3,5)    Felipe Negrão (3,5)
  • Stefan Moreira (2,3)    Stefan Moreira (2,3)
  • Diogo Pasquali Nones (2,3)    Diogo Pasquali Nones (2,3)
  • Daniel Alves Branco Valli (3,6)    Daniel Alves Branco Valli (3,6)
  • Arlindo Américo de Oliveira (3,6)    Arlindo Américo de Oliveira (3,6)
  • Chelin Auswaldt Steclan (3,7,8,9)    Chelin Auswaldt Steclan (3,7,8,9)
  • Samya Hamad Mehanna (10,11,12,13,14)    Samya Hamad Mehanna (10,11,12,13,14)
  • Michael Ricardo Lang (3,8,15,16)    Michael Ricardo Lang (3,8,15,16)
  Views: 283
  Downloads: 27

Resumo

Tumores melanocíticos medulares são extremamente raros e representam um desafio tanto para o diagnóstico quanto ao tratamento. Originados comumente dos melanócitos presentes nas leptomeninges, estand Grupo de lesões inclui: melanocitose difusa, melanocitoma e melanoma. A morbidade associada a esses tumores é significativa e os pacientes afetados podem experimentar dor intensa na região afetada da coluna vertebral e déficits neurológicos como fraqueza, alterações sensoriais e disfunção dos movimentos. Os sintomas podem comprometer significativamente a qualidade de vida dos indivíduos. O diagnóstico é frequentemente realizado por meio de exames de imagem, como ressonância magnética, que auxiliam na identificação da área afetada e sua localização. A confirmação do diagnóstico é feita por meio de análise histopatológica de amostras de tecido obtidas por biópsia. O tratamento geralmente envolve abordagem multidisciplinar. A cirurgia é frequentemente realizada para remover o tumor, aliviar a compressão da medula espinhal e reduzir os sintomas neurológicos. Neste contexto, o presente estudo relata o caso de paciente masculino, 61 anos, com achados clínicos, cirúrgicos e histopatológicos de tumor melanocítico medular. Estudos e pesquisas contínuas sobre tumores na coluna vertebral são necessários para aprimorar a compreensão dessas lesões melanocíticas e desenvolver estratégias mais eficazes de diagnóstico e tratamento. A busca por terapias inovadoras e abordagens personalizadas pode melhorar os resultados clínicos e a qualidade de vida dos pacientes afetados por essa condição incomum.

Palavras-chave

Coluna vertebral; Microneurocirurgia; Tumor medular melanocítico

Abstract

Medullary melanocytic tumors are extremely rare and pose a challenge for both diagnosis and treatment. Commonly originating from the melanocytes present in the leptomeninges, this group of lesions includes diffuse melanocytosis, melanocytoma, and melanoma. The morbidity associated with these tumors is significant and affected patients may experience severe pain in the affected region of the spine and neurological deficits such as weakness, sensory changes, and movement dysfunction. Symptoms can significantly compromise the quality of life of individuals. Diagnosis is often made through imaging tests, such as magnetic resonance imaging, which aid in identifying the affected area and its location. Confirmation of the diagnosis is made by histopathological analysis of tissue samples obtained by biopsy. Treatment usually involves a multidisciplinary approach. Surgery is often performed to remove the tumor, relieve spinal cord compression, and reduce neurological symptoms. In this context, the present study reports the case of a 61-year-old male patient with clinical, surgical and histopathological findings of medullary melanocytic tumor. Ongoing studies and research on spinal tumors are needed to enhance understanding of these melanocytic lesions and develop more effective diagnostic and treatment strategies. The search for innovative therapies and personalized approaches can improve clinical outcomes and quality of life for patients affected by this unusual condition.

Keywords

Spinal column; Microneurosurgery; Melanocytic spinal cord tumor

References

1. Varela-Poblete J, Vidal-Tellez A, Cruz-Quiroga JP, Montoya- Salvadores F, Medina-Escobar J. Melanocytic lesions of the central nervous system: a case series. Arq Neuropsiquiatr. 2022;80(2):153-60. http://dx.doi.org/10.1590/0004-282x-anp-2021-0082. PMid:35352754.

2. Küsters-Vandevelde HV, Küsters B, van Engen-van Grunsven AC, Groenen PJ, Wesseling P, Blokx WA. Primary melanocytic tumors of the central nervous system: a review with focus on molecular aspects. Brain Pathol. 2015;25(2):209-26. http://dx.doi.org/10.1111/bpa.12241. PMid:25534128.

3. van Goethem JW, van den Hauwe L, Ozsarlak O, De Schepper AM, Parizel PM. Spinal tumors. Eur J Radiol. 2004;50(2):159-76. http://dx.doi.org/10.1016/j.ejrad.2003.10.021. PMid:15081130.

4. Spirig J, Fournier JY, Hildebrandt G, Gautschi OP. Spinal tumors - part 1: extradural tumors. Epidemiology, clinical aspects and therapy. Praxis. 2011;100(14):839-48. http://dx.doi.org/10.1024/1661-8157/ a000591. PMid:21732296.

5. Spirig J, Fournier JY, Hildebrandt G, Gautschi OP. Spinal tumors - part 2: intradural tumors. Epidemiology, clinical aspects and therapy. Praxis. 2011;100(14):849-56. http://dx.doi.org/10.1024/1661-8157/ a000592. PMid:21732297.

6. Thakur NA, Daniels AH, Schiller J, et al. Benign tumors of the spine. J Am Acad Orthop Surg. 2012;20(11):715-24. http://dx.doi. org/10.5435/JAAOS-21-02-65. PMid:23118137.

7. Lyu L, Zou L, Jiang Y, et al. Clinical characteristics and treatment outcomes of pigmented tumors in central nervous system: focusing on melanocytic tumors. J Clin Neurosci. 2020;81:83-9. http://dx.doi. org/10.1016/j.jocn.2020.09.009. PMid:33222976.

8. Jaiswal S, Vij M, Tungria A, Jaiswal AK, Srivastava AK, Behari S. Primary melanocytic tumors of the central nervous system: a neuroradiological and clinicopathological study of five cases and brief review of literature. Neurol India. 2011;59(3):413-9. http://dx.doi. org/10.4103/0028-3886.82758. PMid:21743173.

9. Alektoroff K, Moulopoulos LA, Papanagiotou P. Spinal tumors. Radiologe. 2021;61(3):267-74. http://dx.doi.org/10.1007/s00117-021- 00815-5. PMid:33570679.

10. Litak J, Czyżewski W, Szymoniuk M, et al. Biological and clinical aspects of metastatic spinal tumors. Cancers. 2022;14(19):4599. http://dx.doi.org/10.3390/cancers14194599. PMid:36230523.

11. Furtado AD, Panigrahy A, Fitz CR. CNS and spinal tumors. Handb Clin Neurol. 2016;136:1139-58. http://dx.doi.org/10.1016/B978-0-444- 53486-6.00059-4. PMid:27430462.

12. Okamoto K, Natsumeda M, Oishi M, Fujii Y. Melanocytic tumors. No Shinkei Geka. 2021;49(2):389-94. PMid:33762462.

13. Brat DJ, Giannini C, Scheithauer BW, Burger PC. Primary melanocytic neoplasms of the central nervous systems. Am J Surg Pathol. 1999;23(7):745-54. http://dx.doi.org/10.1097/00000478-199907000- 00001. PMid:10403296.

14. Liubinas SV, Maartens N, Drummond KJ. Primary melanocytic neoplasms of the central nervous system. J Clin Neurosci. 2010;17(10):1227-32. http://dx.doi.org/10.1016/j.jocn.2010.01.017. PMid:20558070.

15. Trinh VA, Davis JE, Anderson JE, Kim KB. Dabrafenib therapy for advanced melanoma. Ann Pharmacother. 2014;48(4):519-29. http:// dx.doi.org/10.1177/1060028013513009. PMid:24259661.

16. Griewank KG, Koelsche C, van de Nes JAP, et al. Integrated genomic classification of melanocytic tumors of the central nervous system using mutation analysis, copy number alterations, and DNA methylation profiling. Clin Cancer Res. 2018;24(18):4494-504. http://dx.doi.org/10.1158/1078-0432.CCR-18-0763. PMid:29891723.

17. Escott EJ. A variety of appearances of malignant melanoma in the head: a review. Radiographics. 2001;21(3):625-39. http://dx.doi. org/10.1148/radiographics.21.3.g01ma19625. PMid:11353111.

18. Smith AB, Rushing EJ, Smirniotopoulos JG. Pigmented lesions of the central nervous system: radiologic-pathologic correlation. Radiographics. 2009;29(5):1503-24. http://dx.doi.org/10.1148/ rg.295095109. PMid:19755608.

19. Pirini MG, Mascalchi M, Salvi F, et al. Primary diffuse meningeal melanomatosis: radiologic-pathologic correlation. AJNR Am J Neuroradiol. 2003;24(1):115-8. PMid:12533338.

20. Tang K, Kong X, Mao G, et al. Primary cerebral malignant melanoma: a case report with literature review. Medicine. 2017;96(4):e5805. http://dx.doi.org/10.1097/MD.0000000000005805. PMid:28121927.

21. Shang JX, Zhang D, Zhang JT, Zhao JZ, Zhang Y. Melanocytic neoplasms of central nervous system analysis]. Zhonghua Yi Xue Za Zhi. 2013;93(1):34-6. PMid:23578451.

22. Kinnen F, Fleck SK, Baldauf J, et al. Primary leptomeningeal melanocytic tumors of the spine: report of two cases and review of the literature. World Neurosurg. 2019;124:228-36. http://dx.doi. org/10.1016/j.wneu.2019.01.015. PMid:30660881.

23. Wadasadawala T, Trivedi S, Gupta T, Epari S, Jalali R. The diagnostic dilemma of primary central nervous system melanoma. J Clin Neurosci. 2010;17(8):1014-7. http://dx.doi.org/10.1016/j.jocn.2009.12.020. PMid:20627582.



1MS, Medical student, Universidade do Contestado, Mafra, SC, Brasil.

2MD, Neurologist, Hospital São Vicente de Paulo, Mafra, SC, Brasil.

3Grupo Neuromax, Mafra, SC, Brasil.

4MD, Neurosurgeon, Hospital São Vicente de Paulo, Mafra, SC, Brasil.

5MD, Neuroradiologist, Hospital São Vicente de Paulo, Mafra, SC, Brasil.

6MD, Neurosurgeon, Hospital São Universitário Santa Terezinha, Joaçaba, SC, Brasil.

7PhD, Cellular and Molecular Biologist, Professor, Medicine Course, Universidade do Contestado, Mafra, SC, Brasil.

8Researcher, NUPESC, Universidade do Contestado, Mafra, SC, Brasil.

9Master Program of Desenvolvimento Regional, Universidade do Contestado, Mafra, SC, Brasil.

10MD, MSc, Doctorate student, Hospital São Vicente de Paulo, Mafra, SC, Brasil.

11Pathologist Doctor, Centro de Patologia de Curitiba – CPC, Curitiba, PR, Brasil.

12Professor, Medicine Course, Faculdade Evangélica Mackenzie do Paraná, Curitiba, PR, Brasil.

13Professor, Medicine Course, Fundação Escola do Ministério Público do Estado do Paraná – FEMPAR, Curitiba, PR, Brasil.

14Professor at Medicine Course, Faculdades Pequeno Príncipe – FPP, Curitiba, PR, Brasil.

15MD, Neurosurgeon, Hospital São Vicente de Paulo, Mafra, SC, Brasil.

16Professor, Medicine Course, Universidade do Contestado, Mafra, SC, Brasil.

 

Received Dec 1, 2023

Accepted Jan 18, 2024

JBNC  Brazilian Journal of Neurosurgery

  •   ISSN (print version): 0103-5118
  •   e-ISSN (online version): 2446-6786

Contact

Social Media

   

ABNc  Academia Brasileira de Neurocirurgia

  •   Rua da Quitanda 159 – 10º andar - Centro - CEP 20091-005 - Rio de Janeiro - RJ
  •   +55 21 2233.0323
  •    abnc@abnc.org.br

Sponsor

  • Brain4Care
  • Hospital INC
  • Strattner