title: Pineal parenchymal tumor of intermediate differentiation: diagnostic pitfalls and discussion of treatment options of a rare tumor entity.
abstract: Tumors of the pineal region are uncommon, comprising approximately 0.4-1% of all intracranial tumors in adults in European and American series. Histopathologically, they are a very heterogeneous group of tumors. Of genuine pineal tumors, pineal parenchymal tumors of intermediate differentiation (PPTIDs) are the least frequently found type. In this paper, we report on the case of a patient with an unexpected and difficult-to-diagnose PPTID. A 2.2 x 2.2-cm midline mass within the posterior part of the third ventricle with consecutive obstructive hydrocephalus was found in a 44-year-old man presenting with diplopia and gait disturbances. There was no clear connection of the tumor to the pineal gland. Differential diagnosis included all intraventricular and midline tumors, therefore a biopsy was taken. Preliminary histopathological diagnosis was germinoma or primitive neuroectodermal tumor, and the tissue sample was reexamined by a referential neuropathological institute. Final diagnosis was PPTID. The tumor was then resected through a transventricular/transchoroidal approach. Histopathological examination of tumor specimen confirmed the diagnosis of a PPTID. Postoperatively, the patient received gamma-knife radiosurgery. At 1-year follow-up, there are no signs of tumor regrowth. Diagnosis of pineal parenchymal tumors in general and PPTIDs in particular can be troublesome. Their histopathological features are still being defined, as is the biological behavior of the different tumor entities. Thus, treatment options including surgery, radiation therapy, and chemotherapy remain controversial. We recommend surgical removal of PPTID, preferably in toto whenever the size of the tumor permits that kind of excision.
authors.affiliation: Department of Neurosurgery-ZNN, Johann Wolfgang Goethe-University Clinics, Schleusenweg 2-16, 60528 Frankfurt, Germany. c.senft@med.uni-frankfurt.de
authors.forename: Christian
authors.initials: C
authors.lastname: Senft
authors.forename: Andreas
authors.initials: A
authors.lastname: Raabe
authors.forename: Elke
authors.initials: E
authors.lastname: Hattingen
authors.forename: Daniel
authors.initials: D
authors.lastname: Sommerlad
authors.forename: Volker
authors.initials: V
authors.lastname: Seifert
authors.forename: Kea
authors.initials: K
authors.lastname: Franz
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor; D018997:Chorionic Gonadotropin, beta Subunit, Human / Q000378:metabolism; D020233:Gait Disorders, Neurologic / Q000209:etiology; D018237:Germinoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D006261:Headache / Q000209:etiology; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D018242:Neuroectodermal Tumors, Primitive / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D019635:Neurosurgical Procedures; D016612:Paraffin Embedding; D010871:Pinealoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D011877:Radionuclide Imaging; D016634:Radiosurgery; D020542:Third Ventricle / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed; D000509:alpha-Fetoproteins / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor; D018997:Chorionic Gonadotropin, beta Subunit, Human; D000509:alpha-Fetoproteins