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Neurorradiologia

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Case History

  • White male
  • 49 years old
  • From São Paulo
  • Previously healthy
  • The patient reported severe holocranial headaches. In the same month, he began experiencing spasms in the right foot.

December 2023

  • Severe, bilateral headaches.
  • The first seizure involvingright foot spasms.

February 2024

  • New episode of severe headache with a hypertensive peak.

March 2024

  • Generalized tonic-clonic seizures;
  • Right hemibody weakness.

Physical Examination

  • Dysarthric.
  • Complete right-sided hemiparesis
  • Laboratory: normal

Diagnostic Hypothesis

February 2024 – CT

  • Extra-axial hemorrhage

February 2024 – MRI

  • Diagnostic Hypothesis:  Subarachnoid hemorrhage? Expansive lesion?

March 2024 – CT

  • Intraparenchymal hemorrhage: Vascular malformation? Expansive lesion?

Differential diagnosis

Arteriovenous malformation

Congenital abnormal connections between cerebral arteries and veins (arteriovenous shunting).

Imaging features:  

  • Nidus (*) within the brain parenchyma(CT, MRI or conventional angiography);
  • Early venous drainage (conventional catheter angiography).

Given the initial suspicion of an arteriovenous malformation, the patient underwent cerebral angiography, which proved to be normal.

Diagnostic Hypothesis

March 2024 – MRI

  • The evolutionary aspect of the content is not typical for hematic content.
February 2024 -> Late March 2024
  • Extra axial lesion
  • Apparently expansive
  • Stability
  • Enhancement
  • T1WI: Hyperintensity
  • High protein content of a nonhematic nature -> Melanin – Metastatic melanoma?

Clinical Case Report

Radiological Diagnosis: Expansive lesion containing melanin

Most common sites of melanoma metastasis: skin and subcutaneous tissue, followed by the lungs, liver, bones and brain.

  • Normal thoracic, abdominal and pelvic CT scans.

 

Differential diagnosis

Primary melanocytic neoplasms of the CNS

Meningeal melanocytoma:

  • Slow-growing,
  • Typically benign neoplasms;
  • 5th decade of life.

Imaging features 

  • CT:

Extra-axial

Iso- to hyperattenuating lesions;

Post-contrast enhancement.

  • MRI:

Iso to hyperintensity on T1-weighted;

Hypo to isointensity on T2-weighted sequences;

Homogeneous enhancement post-gadolinium.

Pathologic and Histologic Features

  • Grossly: mostly black / reddish-brown.
  • Microscopically: cellular nests or whorls.
  • Immunohistochemistry: expression of  melanocytic markers.

Primary leptomeningeal melanomatosis:

  • Aggressive;
  • Diffuse growth pattern;
  • 4th decade of life.
Primary leptomeningeal melanomatosis in a 32-year-old man. Sagittal postcontrast T1-weighted image of the spine.

Imaging Appearance

  • Diffuse leptomeningeal thickening
  • Abnormal enhancement post-contrast.
  • T1: iso to hyperintense. areas (melanin’s paramagnetic properties)
  • T2: hypointensity.
*: Nerve Roots

Pathologic and Histologic Features

  • Grossly: diffuse darkening and thickening of leptomeninges.
  • Histologically: Polygonal neoplastic cells with cytoplasmic pigment and prominent nucleoli.

Primary leptomeningeal melanoma

Matheus Tonholo Ikedo
Instituto de Radiologia – Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (INRAD – HCFMUSP)
Orientador: Dr. Izaely Ramos Prates
 
  • Surgical excision of the leptomeningeal lesion
  • Removal of the intraparenchymal hematoma
 
  • Radiological: expansive lesion containing melanin
  • Exclusion of an occult primary lesion outside the CNS
  • Macroscopic aspect: leptomeningeal dark pigmented lesion
  • H&E: pigmented, highly cellular tumor, with pleomorphism and atypical mitoses
  • Positive staining for HMB 45 (human melanoma black Ab)
  • More frequent in men (~50 years);
  • Develops within the leptomeninges;
  • Absence of melanoma outside the CNS.
Prognosis:
  • Better prognosis than does metastatic melanoma.
Melanoblasts:
  • Neural crest origin
  • Migrate to the skin, uvea, mucous membranes and leptomeninges of the CNS.
Pathologic and Histologic Features:
  • Darkly pigmented neoplasm.
  • Highly cellular tumor.
  • Pleomorphic cells: Abundant cytoplasm / Dark pigmentation / Nuclear pleomorphism / Mitoses / Prominent nucleoli
Following surgery: the patient underwent radiotherapy and is currently receiving a chemotherapy regimen. The patient reported an improvement in neurological symptoms, with a residual minor degree of right hemiparesis. There were no other complications.​  

References

  1. Smith AB, Rushing EJ, Smirniotopoulos JG. Pigmented Lesions of the Central Nervous System: Radiologic-Pathologic Correlation. RadioGraphics. 2009 Sep;29(5):1503–24.
  2. Xie ZY, Hsieh KLC, Tsang YM, Cheung WK, Hsieh CH. Primary leptomeningeal melanoma. Journal of Clinical Neuroscience. 2014 Jun;21(6):1051–2.
  3. Qazi SS, Shah SMI, Baqai MWS, Enam SA. Primary leptomeningeal melanoma in association with neurocutaneous melanosis: A case report. Surgical Neurology International. 2022 Nov 25;13:547.
  4. Pan Z, Yang G, Wang Y, Yuan T, Gao Y, Dong L. Leptomeningeal metastases from a primary central nervous system melanoma: a case report and literature review. World Journal of Surgical Oncology. 2014 Aug 20;12(1).