Sugar-Coating or Zuckerguss Appearance on Magnetic Resonance Imaging in Leptomeningeal Carcinomatosis in a Patient with Acute Myeloid Leukemia

A 65-year-old woman with a known past medical history of Bell’s palsy, hypothyroidism, migraines, acute myeloid leukemia (AML) with mutations of NPM1 and FLT3, currently being treated with azacitidine and venetoclax, who also had leukemia cutis as a past complication of her AML, presented to the hospital with worsening daily headaches, dizziness, right arm weakness, and slurred speech. Since her headache was different from her usual migraines, along with new neurologic deficits, a magnetic resonance imaging (MRI) of the brain with and without contrast was performed. It showed contrast-enhancing lesions in a “sugar coating,” “caking,” or “zuckerguss” appearance that is classically seen in leptomeningeal carcinomatosis, as seen in Figure 1. MRI of the complete spinal cord with and without contrast showed leptomeningeal enhancement of the upper spinal cord; enhancement of the pia arachnoid/intrathecal contents of the distal thoracic spinal cord involving the pia arachnoid, with extension to the conus; and “caking” of enhancement of the descending nerve roots with clustering of the descending nerve roots, as seen in Figure 2. A lumbar puncture (LP) was then performed, with normal glucose, a slightly elevated protein (106 mg/dL), and a total nucleated cell count of 218 per mm3. All cerebrospinal fluid (CSF) infectious testing was negative, and CSF flow cytometry confirmed relapsed AML. Pathology also showed atypical cells that were suspicious for blasts.Figure 1: (a-d) MRI brain with and without contrast showing contrast-enhancing lesions in a “sugar coating,” “caking,” or “zuckerguss” appearance in leptomeningeal carcinomatosis. MRI: magnetic resonance imagingFigure 2: (a-d) MRI of the complete spinal axis with and without contrast showing leptomeningeal enhancement of the upper spinal cord; enhancement of the pia arachnoid/intrathecal contents of the distal thoracic spinal cord involving the pia arachnoid with extension to the conus; and “caking” of enhancement of the descending nerve roots with clustering of the descending nerve roots. MRI: magnetic resonance imagingThe main differential diagnosis of leptomeningeal carcinomatosis is infectious causes, and thus, MRI of the brain and spine and LP are essential to make the diagnosis and exclude other causes.[1] Clinical signs of leptomeningeal carcinomatosis can include focal neurologic deficits, cranial nerve palsies, seizures, encephalopathy, and signs of increased intracranial pressure such as headache.[1,2] Other manifestations in the central nervous system (CNS) of AML can include leukemic vasculitis, CNS hemorrhage, and spinal cord compression.[3] The incidence of AML involving the CNS is unknown, however, one study found that 3.3% of patients had CNS involvement during the course of their disease.[4] It is also thought to be less common than that found in adult cases of acute lymphoblastic leukemia (ALL). There is some belief that the incidence of CNS involvement could approach 20% if a LP was performed in all AML patients at the time of diagnosis.[4] Due to the suspected low incidence of AML and CNS involvement, a LP is not done unless patients exhibit neurological deficits. Five to ten percent of adult AML cases can also have leukemia cutis, which is when there are cutaneous manifestations in any type of leukemia[5,6] In those with AML and leukemia cutis, extramedullary leukemic involvement in the CNS is seen in about 17% of cases and is the most common site of extramedullary leukemic involvement in this population.[7] Declaration of patient consent Written informed consent was obtained from the patient for publication of this case report and accompanying figures. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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