Pituicytoma with Gelsolin Amyloid Deposition
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Pituicytoma with Gelsolin Amyloid Deposition Cristiane M. Ida & Xiaoling Yan & Mark E. Jentoft & N. Sertac Kip & Bernd W. Scheithauer & Jonathan M. Morris & Ahmet Dogan & Joseph E. Parisi & Kalman Kovacs
# Springer Science+Business Media New York 2013
Abstract Pituicytoma is a rare low-grade (WHO grade I) sellar region glioma. Among sellar tumors, pituitary adenomas, mainly prolactinomas, may show amyloid deposits. Gelsolin is a ubiquitous calcium-dependent protein that regulates actin filament dynamics. Two known gene point mutations result in gelsolin amyloid deposition, a characteristic feature of a rare type of familial amyloid polyneuropathy (FAP), the Finnish-type FAP, or hereditary gelsolin amyloidosis (HGA). HGA is an autosomal-dominant systemic amyloidosis, characterized by slowly progressive neurological deterioration with corneal lattice dystrophy, cranial neuropathy, and cutis laxa. A unique case of pituicytoma with marked gelsolin amyloid deposition in a 67year-old Chinese woman is described. MRI revealed a 2.6-cm well-circumscribed, uniformly contrast-enhancing solid sellar mass with suprasellar extension. Histologically, the lesion was characterized by solid sheets and fascicles of spindle cells with slightly fibrillary cytoplasm and oval nuclei with pinpoint nucleoli. Surrounding brain parenchyma showed marked reactive piloid gliosis. Remarkably, conspicuous amyloid deposits were identified as pink homogeneous spherules on light microscopy that showed apple-green birefringence on Congo red with polarization. Mass spectrometric-based proteomic analysis identified the amyloid as gelsolin type. Immunohistochemically, Bernd W. Scheithauer passed away during the course of the study. C. M. Ida : M. E. Jentoft (*) : N. S. Kip : B. W. Scheithauer : A. Dogan : J. E. Parisi Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA e-mail: [email protected] J. M. Morris Department of Radiology, Mayo Clinic, Rochester, MN, USA X. Yan Department of Pathology, Tianjin HuanHu Hospital, Tianjin, China K. Kovacs Department of Pathology, St. Michael’s Hospital, Toronto, ON, Canada
diffuse reactivity to S100 protein and TTF1, focal reactivity for GFAP, and no reactivity to EMA, synaptophysin, and chromogranin were observed. HGA-related mutations were not identified in the tumor. No recurrence was noted 14 months after surgery. To the knowledge of the authors, amyloid deposition in pituicytoma or tumor-associated gelsolin amyloidosis has not been previously described. This novel finding expands the spectrum of sellar tumors that may be associated with amyloid deposition. Keywords Pituicytoma . Gelsolin . Amyloid . Pituitary . Adenoma
Introduction Pituicytoma is a distinct low-grade (WHO grade I) glioma of the sellar region that was recognized as an entity by the WHO Classification of Tumours of the Central Nervous System in 2007 [1]. It is a very rare tumor. The largest series included only nine cases [2]; and according to the 2009 report on the German Society
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