✓ Nine original cases of intradural spinal cord lipomas have been examined from a clinical and pathological standpoint. These tumors occur more commonly in men in the second to fourth decade and are found most frequently in the thoracic spinal cord. Paraparesis, sensory changes, urinary incontinence, and pain are frequent presenting complaints. Myelography is the diagnostic study of choice. All lipomas in this series were located primarily within the cord; four of these also presented an extramedullary extension. Admixed nerve bundles were present in five cases with associated hypertrophic onion-bulb formations in three. Decompression with biopsy or subtotal resection is the operative procedure of choice.
A clinicopathological correlation
Bruce J. Ammerman, James M. Henry, Umberto De Girolami and Kenneth M. Earle
Michael P. Catalino, David M. Meredith, Umberto De Girolami, Sherwin Tavakol, Le Min and Edward R. Laws Jr.
This study was done to compare corticotroph hyperplasia and histopathologically proven adenomas in patients with Cushing disease by analyzing diagnostic features, surgical management, and clinical outcomes.
Patients with suspected pituitary Cushing disease were included in a retrospective cohort study and were excluded if results of pathological analysis of the surgical specimen were nondiagnostic or normal. Cases were reviewed by two experienced neuropathologists. Total lesion removal was used as a dichotomized surgical variable; it was defined as an extracapsular resection (including a rim of normal gland) in patients with an adenoma, and for hyperplasia patients it was defined as removal of the presumed lesion plus a rim of surrounding normal gland. Bivariate and multivariate analyses were performed. Recurrence-free survival was compared between the two groups.
The final cohort consisted of 63 patients (15 with hyperplasia and 48 with adenoma). Normal pituitary acinar architecture was highly variable. Corticotroph hyperplasia was diagnosed based on the presence of expanded acini showing retained reticulin architecture and predominant staining for adrenocorticotropic hormone. Crooke’s hyaline change was seen in 46.7% of specimens, and its frequency was equal in nonlesional tissue of both groups. The two groups differed only by MRI findings (equivocal/diffuse lesion in 46% of hyperplasia and 17% of adenoma; p = 0.03). Diagnostic uncertainty in the hyperplasia group resulted in additional confirmatory testing by 24-hour urinary free cortisol. Total lesion removal was infrequent in patients with hyperplasia compared to those with adenoma (33% vs 65%; p = 0.03). Initial biochemical remission was similar (67% in hyperplasia and 85% in adenoma; p = 0.11). There was no difference in hypothalamic-pituitary-adrenal axis recovery or disease recurrence. The median follow-up was 1.9 years (IQR 0.7–7.6 years) for the hyperplasia group and 1.2 years (IQR 0.4–2.4 years) for the adenoma group. Lack of a discrete lesion and diagnostic uncertainty were the only significant predictors of hyperplasia (sensitivity 53.3%, specificity 97.7%, positive predictive value 88.9%, negative predictive value 85.7%). An adjusted Cox proportional hazards model showed similar recurrence-free survival in the two groups.
This study suggests an association between biochemically proven Cushing disease and histopathologically proven corticotroph hyperplasia. Imaging and operative findings can be ambiguous, and, compared to typical adenomas with a pseudocapsule, the surgical approach is more nuanced. Nevertheless, if treated appropriately, biochemical outcomes may be similar.
Sandro Santagata, Sagun Tuli, Don E. Wiese II, Arthur Day and Umberto De Girolami
✓ Neuromas typically arise in the peripheral nervous system in response to traumatic injury at the site of partial or complete nerve transection as new axons from the proximal nerve stump sprout to reinnervate the distal segment. In rare cases neuromas have also been described as intramedullary spinal cord lesions. These lesions have been identified as incidental autopsy findings in association with prior trauma and cervical spondylosis, multiple sclerosis, spinal tumors, and syringomyelia.
The authors report the case of a 50-year-old man who had been involved in a motor vehicle accident, during which his car was struck from behind as it was stationary at an intersection, more than 5 years before presentation. A workup for syncopal and presyncopal episodes involved magnetic resonance imaging that revealed a 1.1-cm lesion at the cervicomedullary junction (CMJ). The imaging features of the lesion raised the question of an ependymoma or subependymoma. The lesion was excised, and examination of the tissue demonstrated a neuroma with haphazardly arranged interlacing bundles of axons ensheathed by Schwann cells with interfascicular regions of reactive glial cells and Rosenthal fibers, consistent with those present after traumatic injury. This case may represent the first true traumatic intramedullary neuroma of the CMJ diagnosed in a living patient and treated surgically.
Demetrios C. Nikas, Umberto De Girolami, Amir A. Zamani, Geraldine S. Pinkus, Lorenzo Bello, Matthias Kirsch and Peter McL. Black
✓ This 63-year-old man presented with complaints of “having a feeling of falling backward” over a 3-month period. Results of his general physical examination, laboratory studies, and neurological examination were unremarkable. A magnetic resonance image revealed a 1.8 × 1.4 × 1.2—cm enhancing mass in the posterior third ventricle just above the corpora quadrigemina. The pineal gland was found to be diffusely enlarged at operation and separable from the posterior thalamus and was totally resected. The patient had an uneventful postoperative course but continues to be somewhat confused. The lesion consisted of a remarkable chronic inflammatory cell infiltrate permeating the pineal lobules and was composed of T and B lymphocytes, macrophages, eosinophils, and mast cells. Immunoperoxidase studies did not demonstrate Langerhans cells, and a search for microorganisms was unrevealing. There was no evidence of neoplasia; results of immunostaining for germ cell markers and other tumor-associated antigens were negative.