pineal parenchymal tumor of intermediate differentiation (pptid), a newly-designated tumor:...
DESCRIPTION
Materials and Methods IRB-approved Case material gathered Search of radiology database since 2001 Key words: ‘pineal tumor,’ ‘pineal mass,’ ‘pineocytoma,’ ‘pineoblastoma’ Search hard copy teaching files for atypical- appearing pineal tumors ( ) Gathered pathologically-proven cases from other institutionsTRANSCRIPT
Pineal Parenchymal Tumor of Intermediate Differentiation
(PPTID), a Newly-designated Tumor:
Pathology and Imaging Spectrum in 11 Cases
S. T. Komakula, M. Warmuth-Metz, P. G. Hildenbrand, L. Loevner, R. Hewlett, K. L. Salzman, W. T. Couldwell, C. Lin, A. Osborn
University of Utah
Purpose
Delineate the imaging spectrum of PPTID, new tumor entity recognized in 2007
Identify imaging findings that may suggest the preoperative diagnosis of this newly-recognized neoplasm
Materials and Methods
IRB-approved Case material gathered
Search of radiology database since 2001 Key words: ‘pineal tumor,’ ‘pineal mass,’
‘pineocytoma,’ ‘pineoblastoma’ Search hard copy teaching files for atypical-
appearing pineal tumors (1985-1995) Gathered pathologically-proven cases from
other institutions
Materials and Methods
Inclusion criteria 11 cases of pathology-proven PPTIDs
Data collected included Patient demographics Presenting symptoms Radiological findings Treatment and outcome
Results Demographics:
Age at presentation = 4.5 – 75 yrs (mean = 23 yrs) M:F = 7:4
Presenting symptoms Headache 8/11 Parinaud syndrome 3/11 Gait disturbances 3/11 Other unspecified visual symptoms 2/11 Seizures 1/11
Imaging Findings
General features: Size = 1 - 6 cm (mean = 2.5 cm) Local parenchymal invasion = 9/11 Well-circumscribed = 2/11 Meningeal dissemination = 2/11
CT (7 patients) NECT (3), CECT (3), both (1)
Peripheral ‘exploded’ calcifications 5/7
Gross hemorrhage 1/7 Enhancement
Heterogeneous 3/4 Uniform 1/4
CT (7 patients) NECT (3), CECT (3), both (1)
Peripheral ‘exploded’ calcifications 5/7
Gross hemorrhage 1/7 Enhancement
Heterogeneous 3/4 Uniform 1/4
CT (7 patients) NECT (3), CECT (3), both (1)
Peripheral ‘exploded’ calcifications 5/7
Gross hemorrhage 1/7 Enhancement
Heterogeneous 3/4 Uniform 1/4
MR
All patients had MRI T1 heterogeneously hypointense 11/11 T2 heterogeneously hyperintense 11/11 Strong heterogeneous CE
10/11 Uniform enhancement 1/11 Cystic-appearing foci 8/11
MR
T1WI T2WI
MR
T1+CT1+C
Leptomeningeal seeding
T1+C T1+CT1+C
MRS
1/11 Elevated choline Decreased NAA Lactate doublet
Results: Outcome
Follow up available in 8 patients Surveillance period = 0.5 – 21years 5 patients tumor free Tumor recurrence in 3 patients
Tumor recurrence Partial resection in 1997
(1 year after initial symptoms) Initial histopathology ‘mixed
pineocytoma – pineoblastoma’ Recurrence in 2007
Radiosurgery Continued growth of tumor Review of pathology → PPTID Considering surgery
Initial post-op scan
10 years later
Tumor recurrence Initial diagnosis at 15y → ‘pineal teratoma’ 1st recurrence → ‘pineoblastoma’ Multiple recurrences Died at age 37y Autopsy diagnosis (1991) → ‘Pineoblastoma with transitional
features and retinoblastomatous differentiation’ Pathology reviewed (2010) → PPTID
T1 T1 T2CECT
Discussion: PPTID WHO 2007: New category, PPTID
Describes tumors intermediate in malignancy between pineocytoma (WHO grade I) and pineoblastoma (WHO grade IV)
WHO grade II-III ~ 20% of all pineal parenchymal tumors Usually large, complex pineal masses
T1 T2 T1+C
Macroscopy Hemorrhage Necrosis Local invasion Craniospinal dissemination
Differential Diagnosis Pineocytoma Germinoma Pineoblastoma Papillary tumor of the pineal region
T1+C
Summary PPTID should be considered in an older child
or adult with an atypical or aggressive-appearing, locally-invasive pineal region mass
Histopathology of tumors initially diagnosed as ‘pineocytoma versus pineoblastoma’ or ‘aggressive pineocytoma’ prior to 2007 may also be reviewed as these may be PPTIDs