Aggressive primary pediatric intracranial malignant melanoma: Sphinx of the tissue diagnosis

Sivaraju, Laxminadh; Ghosal, Nandita; Mahadevan, Anita; Krishna, AS Uday; Rao, Shilpa; Hegde, Alangar S.

doi:10.4103/ajns.ajns_253_17

Cited by 3 publications

(7 citation statements)

References 18 publications

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“…Overall survival is reported to be between 9 and 24 months [22]. At the time of this writing, up to 250 cases of PIMM have been reported [23–26], with less than a handful cases of PIMM in children [5, 7, 8, 24]. Data from a population-based study using the National Cancer Institute SEER program in USA collected from 1973 to 2015 observes that the incidence of PIMM peaks during the fourth and fifth decades of life [23–25].…”

Section: Discussionmentioning

confidence: 99%

“…The mean age for PIMM is 45.8 years (minimum 12 years, maximum 82 years), and patients are predominantly male (33; 61.1%) [25]. Put together, it should be emphasized that cases of PIMM in children are very rare [5, 7, 8, 12–15]. A list of these patients from contemporary literature is summarized in Table 2.…”

Section: Discussionmentioning

confidence: 99%

“…This aggressive neoplasm is thought to arise from the melanocytes of leptomeninges and has a purported incidence of less than 3% of all pediatric cancers [3, 4]. In children, there is often a strong association with neurocutaneous melanosis [5]. At the time of this writing, there is a paucity of publications on pediatric PIMM [6, 7] as it has been mostly reported in the adult population [6].…”

Section: Introductionmentioning

confidence: 99%

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Pediatric Primary Intracranial Malignant Melanoma: Case Report and Literature Review

Lim¹,

Tan²,

Wong³

et al. 2023

Pediatr Neurosurg

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Introduction: Primary intracranial malignant melanoma (PIMM) is an extremely rare primary brain tumor with most cases diagnosed in adults. To date, there are only a few cases reported in the pediatric population. Owing to its infrequency, there are no established guidelines to treat this aggressive neoplasm. Recent insights suggest that PIMM are molecularly different between adults and children, whereby NRAS mutations drives tumor growth in the latter group. We present a unique case of PIMM in a pediatric patient and discuss the case in corroboration with current literature. Case Presentation: A previously well 15-year-old male presented with progressive symptoms of raised intracranial pressure. Neuroimaging reported a large solid-cystic lesion with significant mass effect. He underwent gross total resection of the lesion that was reported to be a PIMM with pathogenic single nucleotide variant NRAS p.Gln61Lys. Further workup for cutaneous, uveal and visceral malignant melanoma was negative. A trial of whole brain radiotherapy followed by dual immune checkpoint inhibitors was commenced. Despite concerted efforts, the patient had aggressive tumor progression and eventually demised from his disease. Conclusion: We therein report a case of pediatric PIMM, in the context of the patient’s clinical, radiological, histopathological and molecular findings. This case highlights the therapeutic difficulties faced in disease management and contributes to the very limited pool of medical literature for this devastating primary brain tumor.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pediatric Primary Intracranial Malignant Melanoma: Case Report and Literature Review

Lim¹,

Tan²,

Wong³

et al. 2023

Pediatr Neurosurg

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“…CT findings can be indeterminate and inconclusive if there is no clear history of melanosis, neurocutaneous disorder, or primary cutaneous melanoma. In the pediatric population, most cases are associated with cutaneous melanosis and giant congenital nevus [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…It is important to note that melanocytic tumors also have a propensity to bleed, and often, the features of melanin and hemosiderin can be found in the same tumor. Despite their peculiar imaging characteristics and specific immunological features, primary brain melanomas are indistinguishable from melanotic metastasis to the brain from an extra-CNS primary tumor; therefore, a thorough clinical analysis must be done [ 3 ]. Often, this is a diagnosis of exclusion and is made when skin or uveal melanocytic lesions are not found to account for a primary malignancy.…”

Section: Introductionmentioning

confidence: 99%

Primary Brain Melanoma in a Pediatric Patient: A Case Report

2021

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Patient: Male, 17-year-old Final Diagnosis: Primary malignant melanoma of brain Symptoms: Tonic-clonic seizure Medication: — Clinical Procedure: Resection of brain tumor Specialty: Neurosurgery • Pediatrics and Neonatology • Radiology Objective: Rare disease Background: Primary malignant melanoma of the brain is a challenging radiological diagnosis and a high index of suspicion is required about patients with the condition. In the pediatric population, only a few cases have been reported in the literature. The purpose of this report was to describe the expected imaging characteristics and the importance of a multidisciplinary approach in the diagnosis of this rare entity. Case Report: A 17-year-old Hispanic male who presented with new-onset tonic-clonic seizures had no focal neurologic deficits on physical examination. An initial computed tomography scan showed a hyperdense, right frontal, parafalcine mass. Brain magnetic resonance imaging was performed and revealed a T1 hyperintense and T2 hypointense, right-frontal-lobe, extra-axial mass with foci of susceptibility. Resection of the mass revealed a lesion that had a dark, pigmented macroscopic appearance. Histopathologic analysis confirmed that it was a primary intracranial malignant melanoma after no primary site was identified on dermatologic and ophthalmologic evaluations. Conclusions: Diagnosing a primary intracranial melanoma with imaging alone is virtually impossible if clinical data and findings from a thorough physical examination are unavailable. Intracranial primary malignant melanoma remains a complex radiological diagnosis that relies on the exclusion of other potentially more common entities and an optimal multidisciplinary approach.

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Primary intracranial malignant melanoma in an adolescent female: a case report

Vishwajeet,

Saraf,

Elhence

et al. 2024

Egypt J Neurosurg

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Background Primary central nervous system melanoma is an extremely rare entity and even rarer in children and adolescents as compared to adults. It is often difficult to consider a diagnosis of intracranial melanoma pre-operatively without any clinical evidence of neurocutaneous melanosis. Case presentation Herein, a case of primary melanoma of the brain is reported in a 17-year-old female who presented with headache, vomiting, and focal neurological deficit in the form of left-sided facial palsy and limb weakness. A contrast-enhanced computed tomography of head was performed which revealed a heterogeneously hyperattenuating mass lesion at left gangliocapsular region showing peripheral enhancement with internal non-enhancing cystic component. The patient underwent left frontotemporal craniotomy. The diagnosis was made on histopathological examination, which showed an invasive tumor comprising of epithelioid to spindled cells arranged in sheets, nests, and singly scattered. The special stains and immunohistochemical markers proved very helpful in establishing the diagnosis. Conclusions The case highlights the uncommon occurrence of primary intracranial melanoma in the pediatric age group, the perplexing histological features, and the rapid and fatal course.

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Aggressive primary pediatric intracranial malignant melanoma: Sphinx of the tissue diagnosis

Cited by 3 publications

References 18 publications

Pediatric Primary Intracranial Malignant Melanoma: Case Report and Literature Review

Pediatric Primary Intracranial Malignant Melanoma: Case Report and Literature Review

Primary Brain Melanoma in a Pediatric Patient: A Case Report

Primary intracranial malignant melanoma in an adolescent female: a case report

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