Contributed by MacLean P. Nasrallah, MD PhD1; Ilya M. Nasrallah, MD PhD2; Marisa S. Prelack, MD3; Margaret O. Johnson, MD MPH3; Travis B. Lewis, MD PhD3; Michael Rubenstein, MD3; Jane E. Minturn, MD PhD4; Arati Desai, MD5; Paul Marcotte, MD6; Mariarita Santi, MD PhD7; Maria Martinez-Lage, MD1
1Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania; 2Department of Radiology, Hospital of the University of Pennsylvania; 3Department of Neurology, Hospital of the University of Pennsylvania; 4Division of Oncology/Neuro-Oncology, Children's Hospital of Philadelphia; 5Department of Medical Oncology, Hospital of the University of Pennsylvania; 6Department of Neurosurgery, Hospital of the University of Pennsylvania; 7Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia
CCLINICAL HISTORY AND NEUROIMAGING
A 19-year-old male with history of narcolepsy, but otherwise healthy with normal development and cognition, presented with one month of daily headache and a single unprovoked transient confusional episode consistent with a seizure. During the episode, the patient experienced right upper extremity incoordination, orolingual automatisms and aphasia. Physical examination was notable only for macrocephaly. MRI of the brain revealed multiple heterogeneously enhancing dural-based masses and dural nodularity with mild parenchymal volume loss, thinning and remodeling of the calvarium, remodeling of the skull base, and sagging appearance of brainstem (Figures 1a, 1b). There was no lesion in the spinal canal. Cerebrospinal fluid analysis was normal except for elevated protein content. Electroencephalography (EEG) showed left temporal focal slowing with sharp transients. Extensive serologic testing was within normal limits, notable for normal ANA, ANCA, RF, RPR, Quantiferon Gold, FSH, LH, prolactin, TSH, SPEP, antigliadin antibody, and IgG4, as well as negative HIV. CT scans of the chest, abdomen, and pelvis did not identify any visceral lesions and ophthalmologic and dermatologic examinations were essentially normal. A biopsy of the left parietal dural-based nodule was performed, but did not yield a definitive diagnosis. The patient was treated with levetiracetam and corticosteroid therapy and discharged home with planned outpatient follow up. Approximately four weeks later, he presented with recurrence of severe retro-orbital headache and emesis. A second biopsy, this time of a left frontal dural-based nodule was performed.
GROSS AND MICROSCOPIC PATHOLOGY
Grossly, the left parietal biopsy (first biopsy) consisted of a firm spherical nodule of tan soft tissue attached to a portion of thickened, white dura. Microscopically, the biopsy was remarkable for a florid meningothelial proliferation (Figure 1c) with granulomatous changes, including multinucleated giant cells with refractile crystalline inclusions (Figure 1d) and vessel wall abnormalities. The meningothelial proliferation had cystic areas and infiltrating cells with clear and foamy cytoplasm and was adherent to thickened dura, which was infiltrated by cords and nests of atypical cells with clear cytoplasm. EMA was positive in the meningothelial proliferation. The histiocytic foamy and clear cells were positive for CD68 (Figure 1e) and the infiltrating clear cells were positive for S100 (Figures 1f, 1g) and synaptophysin; CD1a, HMB45 and melanA showed no staining. The second biopsy of the frontal nodule showed infiltrating cords and nests of cells with clear cytoplasm on a continuum with sheets of cells with a higher nuclear/cytoplasmic ratio, involving the meninges and dura (Figures 1h, 1i). At higher magnification, up to 8 mitoses per 10 high power fields as well as numerous apoptotic bodies were seen (Figure 1j). The clear and round cells were positive for S100, synaptophysin and Olig2 (Figure 1k). What is your diagnosis?What is your diagnosis?