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Table of Contents    
Year : 2022  |  Volume : 70  |  Issue : 8  |  Page : 322-325

Meningeal Melanomatosis with a Spinal Meningeal Melanocytoma Trigger by an in vitro Fertilization

1 Department of Neurosurgery, Central University Hospital of Asturias, Oviedo, Spain
2 Hospital University Complex of Albacete, Spain
3 University Institute of Oncology (IUOPA), Spain
4 Department of Neurosurgery, Central University Hospital of Asturias, Oviedo; University Institute of Oncology (IUOPA), Spain

Date of Submission18-Mar-2020
Date of Decision11-Aug-2020
Date of Acceptance14-Dec-2020
Date of Web Publication11-Nov-2022

Correspondence Address:
Marco A Alvarez Vega
Marco A, Av/Roma s/n, 33011, Oviedo
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.360914

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 » Abstract 

Meningeal melanomatosis is an infrequent tumor originating from the melanocytes in the leptomeninges and one of the recognized primary melanocytic tumors of the central nervous system. The average survival has known to be about 5 months. It can be associated with solid tumors, such as meningeal melanocytomas. The patient we present was diagnosed of a meningeal melanomatosis that developed two solid tumors related to an in vitro fertilization. The clinical course was rapidly fatal. Although the use of comprehensive diagnostic procedures, usually the final diagnosis of primary diffuse meningeal melanomatosis is postmortem, it would be advisable for the appropriate management of the patient to make a differential diagnosis and to be aware of the behavior of the tumor.

Keywords: In vitro fertilization, melanocytic tumors-meningeal melanomatosis, meningeal melanocytoma
Key message: The paper demonstrates an uncommon case of meningeal melanomatosis with exceptional figures of the pathology. There are not guides about how to treat this pathology. We consider the possible relationship with in vitro fertilization.

How to cite this article:
Ramos AA, Ferrara P, Fernández VM, Sanchez CR, Alvarez Vega MA. Meningeal Melanomatosis with a Spinal Meningeal Melanocytoma Trigger by an in vitro Fertilization. Neurol India 2022;70, Suppl S2:322-5

How to cite this URL:
Ramos AA, Ferrara P, Fernández VM, Sanchez CR, Alvarez Vega MA. Meningeal Melanomatosis with a Spinal Meningeal Melanocytoma Trigger by an in vitro Fertilization. Neurol India [serial online] 2022 [cited 2022 Dec 3];70, Suppl S2:322-5. Available from: https://www.neurologyindia.com/text.asp?2022/70/8/322/360914

Meningeal melanomatosis is an infrequent tumor[1] originating from the melanocytes in the leptomeninges and is one of the recognized primary melanocytic tumors of the central nervous system (CNS).[1] The average survival is only about 5 months. It can be associated with solid tumors, such as meningeal melanocytomas. Meningeal melanocytoma are low-grade lesions that are considered benign in the World Health Organization classification of CNS tumors.[2],[11],[12] We present a rare case of meningeal melanomatosis with a spinal meningeal melanocytoma that results in a fatal clinical course, trigger by an in vitro fertilization.

 » Case Report Top

A 36-year-old female without known diseases female presented with a 5 days history of acute onset of headache following for vomiting that happened 48 h after an in vitro fertilization. This event requires admission in the intensive care unit, and it was assumed as unspecific meningitis. All the diagnostic work up at this stage was negative. Five months later, she was readmitted with the same symptoms of intracranial hypertension syndrome, trigger again by an in vitro fertilization. Detailed neurological examination did reveal sensory loss in the right leg. There was no history of previous skin lesions or any other melanocytic lesion at any site, and the physical examination was unremarkable. Brain and whole spine 1.5-tesla magnetic resonance imaging revealed pachyleptomeningitis along the spine, the skull base anterior, and the posterior fossa. A 3 cm T11-T12 intradural extramedullary tumor was also identified. This lesion was isointense in T1-weighted and hypointense in T2-weighted MRI, with homogeneous enhancement after contrast administration. The brain study showed only an unspecific meningeal increased enhancement of contrast [Figure 1]. A variation of a Dandy Walker malformation was also described. Lumbar puncture was performed and the cerebrospinal fluid analysis excluded malignant cells. The patient underwent a T11–T12 laminectomy and subtotal resection of an infiltrative intradural extramedullary tumor. We found a thickened, rigid dura, with some hyperpigmented area.
Figure 1: Magnetic resonance imaging. Sagittal T1 of the spine (left), sagittal T2 of de intradural lesión (middle), axial T1 after contrast administration (top right), and T1 axial of the posterior fossa (bottom right)

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Histologically, the tumor was composed of tightly paced spindle cells; some of them contained variable amounts of melanin. Microscopic examination revealed a hyperchromatic and elongated lesion, formed by fusiform cells without atypia, low mitotic account, and low proliferative index. However, some early phases of malignant evolution could be observed, like cell size and pigment increase. Immunohistochemically studies showed strong and diffuse staining for the melanocytic marker human melanoma black-45 (HMB-45). S100, vimentine, and Melan A were also positive [Figure 2].
Figure 2: Microscopic examination of surgical specimen of infiltrative intradural extramedulary tumor. Photomicrographs of a spinal meningeal melanocytoma. We can observe that the lesion produce nerve compression and invasion of the spinal cord. Hematoxylin and eosin; original magnification X 4; X 10; and X 20 (left to right)

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During postoperative course, the patient remained without any new neurological deficits. Adjuvant treatment with whole brain radiotherapy was started, but in the second session, the patient developed a malignant intracranial hypertension and died. The postmortem examination developed two dark brown masses on the cerebellum and spinal cord, and also dark brown lesion with blurred boundaries along the spine, in the posterior fossa and the skull base, indicating a meningeal melanomatosis of the leptomeninges that affected all CNS. There was not macroscopic invasion of the nervous tissue [Figure 3].
Figure 3: Photographs of longitudinal and axial section of the spine during the autopsy. Macroscopic segment of the spinal cord covered by brownish meninges that affect all central nervous system. In axial section, the thick and attached meninges are noticeable. This image corresponded to menalocytoma

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 » Discussion Top

Melanocytic tumors of the CNS are derivated from melanocytes.[13],[14] These cells migrate during embryiogenesis to the leptomeninges (the inner two meningeal layers—arachnoid and pia). Lesions can be a solid mass or can be present as diffuse disseminations in the subarachnoid space.[3],[15],[16] The current spectrum of melanocytic tumors ranges from most benign to most malignat forms. Meningeal melanocytoma (benign mass-forming proliferation), meningeal melanoma (malignant mass-forming proliferation), meningeal melanocytosis (diffuse meningeal benign melanocytic proliferation), and meningeal melanomatosis (diffuse meningeal malignant melanocytic proliferation).[2],[4],[5] The patient we present was diagnosed of meningeal melanomatosis that also developed two solid tumors, one in the spinal cord and the other one in the cerebellum. We performed a surgery of the first one and result in a meningeal melanocytoma.

Limas and Tio first proposed the term “meningeal melanocytoma” in 1972 based on ultrastructural features to differentiate these benign tumors from those originating from meningothelial fibroblasts. Meningeal melanocytoma is a rare, benign but locally aggressive tumor. They are usually located intradural extramedullary and less commonly extradural. Gross total resection is the gold standard of all the therapeutic options. According to the literature, adjuvant radiotherapy should be performed postoperatively in patients whom resection was incomplete and in multiple lesions where surgery is not indicated. It has been shown to have 40% success rate in preventing local recurrence. Recent successes with BRAF inhibitors and immune checkpoint inhibitors in metastatic CNS melanoma suggest the potential of these drugs to treat CNS melanoma.[5] Anyway, the role and efficacy of radiotherapy in these tumors remain unclear. The main point in our case was the invasion of the CNS. There are few many reports concerning meningeal melanomatosis.[4],[6] In all of them, a rapidly fatal course was described.[7] The average survival is about 5 months. Early diagnosis is important in order to get treatment before neurological deterioration appears.

On the other hand, whether or not IVF predisposed or promoted cancer development is hard to demonstrate. There are many studies that have analyzed the risk of cancer in infertile women with hormonal treatment. Most of the results have little evidence or are contradictory although this possibility has been pointed out.[8],[9]

Another interesting point is that an association is exist between neurocutaneous melanosis and Dandy Walker malformation. Even though our patient did not have a neurocutaneous melanosis, it could be found some relationship in between the cerebellar development and the melanocytosis.[10]

Malignant melanocytic lesions have a poor prognosis despite available treatments.

 » Conclusion Top

Meningeal melanomatosis is a very rare form of brain tumor. Although the use of comprehensive diagnostic procedures, usually the final diagnosis of primary diffuse meningeal melanomatosis, is postmortem, it would be advisable for the appropriate management of the patient to make a differential diagnosis and to be aware of the behavior of the tumor.

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Conflicts of interest

There are no conflicts of interest.

 » References Top

Gempt J, Buchmann N, Grams A, Zoubaa S, Schlegel J, Meyer B, et al. Black brain: transformation of a melanocytoma with diffuse melanocytosis into a primary cerebral melanoma. J Neurooncol 2011;102:323-8.  Back to cited text no. 1
Louis D, Perry A, Refenberger G, von Deimling A, Figarella-Branger D, Cavenee W, et al. The 2016 World health organization classification of the central nervous system: A summary. Acta Neuropathol 2016;27:430-8.  Back to cited text no. 2
Lepreux S, Sagnier S, Perez JT, Léger F, Sibon I, Vital A. Primary diffuse leptomeningeal gliomatosis: do we miss the diagnosis?. Clin Neuropathol 2017;36:222-6.  Back to cited text no. 3
Eichberg DG, Achua JK, Locatelli E, Shah AH, Komotar RJ, Ghods AJ. Primary diffuse leptomeningeal melanomatosis: case report and review of the literature. World Neurosurg 2019;122:648-55.  Back to cited text no. 4
Noronha C, Rocha L. Meningeal melanocytosis: A challenging diagnosis. Lancet Oncol 2019;20:e343.  Back to cited text no. 5
Saadeh YS, Hollon TC, Fisher-Hubbard A, Savastano LE, McKeever PE, Orringer DA. Primary diffuse leptomeningeal melanomatosis: Description and recommendations: J Clin Neurosci 2018;50:139-43.  Back to cited text no. 6
Fujimori K, Sakai K, Higashiyama F, Oya F, Maejima T, Miyake T. Primary central nervous system malignant melanoma with leptomeningeal melanomatosis: a case report and review of the literature: Neurosurg Rev 2018;41:333-9.  Back to cited text no. 7
Cohen PR, Erickson CP, Sateesh BR, Uebelhoer NS, Calame A. Melanoma following in vitro fertilization: co-incident or coincidence?. Cureus 2019;7:11:e4857.  Back to cited text no. 8
Berk-Krauss J, Bieber AK, Criscito MC, Grant-Kels JM, Driscoll MS, Keltz M, et al. Melanoma risk after in vitro fertilization: A review of the literature. J Am Acad Dermatol 2018;79:1133-40.  Back to cited text no. 9
Seok-Gu K, Do Sung Y, Kyoung Suok C, Dal Soo K, Ean o H, Moon Chan K. Coexisting intracraneal meningeal melanocytoma, dermoid tumor, and Dandy- Walker cyst in a patient with neurocutaneus melanosis. Case report. J Neurosurg 2006;104:444-7.  Back to cited text no. 10
Nandeesh BN, Rao S, Sadashiva N, Mahadevan A, Yasha TC, Santosh V. Clinicopathological Study of Extra-Axial Small Round Cell Tumors of the Cranium. Neurol India 2020;68:1175-82.  Back to cited text no. 11
[PUBMED]  [Full text]  
Meena R, Doddamani RS, K Gour SS, Mahajan S, Agrawal D, Chandra PS. Malignant Melanoma of the Brainstem: A Unicorn Not a Popcorn. Neurol India 2022;70:340-4.  Back to cited text no. 12
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Ramachandran H, Radhakrishnan A, Radhakrishnan SE. Neurocutaneous Melanosis: Cutaneous and Neuroimaging Findings. Neurol India 2020;68:1508.  Back to cited text no. 13
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Zagrajek M, Belowska-Bień K, Bladowska J, Hałoń A, Paradowski B. Neurocutaneous Melanocytosis in a 24-year-old Woman. Neurol India 2021;69:1103-4.  Back to cited text no. 14
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Gupta A, Chacko G, Chacko AG. Prevalence and Pattern of Leptomeningeal Pigmentation in the Human Brain and Its Role in the Safe Surgical Excision of Extra-Axial Brain Tumors. Neurol India 2021;69:1204-9.  Back to cited text no. 15
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Kumar A, Sardhara JC, Singh G, Kanjilal S, Maurya VP, Behari S. Malignant Meningitis Associated with Hydrocephalus. Neurol India 2021;69(Supplement):S443-S455.  Back to cited text no. 16


  [Figure 1], [Figure 2], [Figure 3]


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