Pediatr Radiol (2009) 39:188–190 DOI 10.1007/s00247-008-1060-1
CASE REPORT
Papillary tumor of the pineal region: report of a rapidly progressive tumor with possible multicentric origin Takashi S. Sato & Patricia A. Kirby & John M. Buatti & Toshio Moritani
Received: 15 August 2008 / Revised: 21 September 2008 / Accepted: 22 October 2008 / Published online: 27 November 2008 # Springer-Verlag 2008
Abstract Papillary tumor of the pineal region (PTPR) is an uncommon tumor recently added to the WHO classification of CNS tumors. We report a case of PTPR in a young boy that was noteworthy for early CSF dissemination and relentless progression. In spite of intensive chemotherapy and comprehensive radiotherapy, the boy died. The neuroimaging appearance is unique with possible multicentric origin of the tumor and intense uptake of 111In-DTPA-pentetreotide.
Classification of Brain Tumors [2]. Additional cases have been reported, but this is still a rare tumor and the full spectrum of diagnostic features, clinical course and standardized therapy are not yet established [3, 4]. We present a case of PTPR in an 18-year-old boy, noteworthy for its rapid progression and fatal outcome in spite of aggressive chemotherapy and radiotherapy.
Keywords Pineal region tumors . Papillary pineal tumor . Multicentric . Somatostatin receptor
Case report
Introduction Primary pineal tumors with a papillary growth pattern are rare. In 2003, Jouvet et al. [1] reported an unusual papillary tumor of the pineal region (PTPR), which was formally recognized as a distinct entity by the 2007 WHO T. S. Sato Carver College of Medicine, University of Iowa, Iowa City, IA, USA P. A. Kirby Department of Pathology, University of Iowa, Iowa City, IA, USA J. M. Buatti Department of Radiation Oncology, University of Iowa, Iowa City, IA, USA T. Moritani (*) Department of Radiology, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, IA 52242, USA e-mail:
[email protected]
An 18-year-old boy presented to his primary physician with headaches diagnosed as migraines. An MRI scan at an outside hospital (Fig. 1) showed a pineal region mass with cystic and solid components, measuring 15×27×17 mm. The solid component was slightly hyperintense to gray matter on both T1- and T2-W images and avidly and uniformly enhanced with intravenous gadolinium. This lesion was diagnosed initially as a “pineal cyst” and observed. However, in retrospect there was an additional small contrast-enhancing suprasellar mass and mild ventricular enlargement. No leptomeningeal enhancement was present. The patient presented to our institution 4 months after the initial presentation when physical examination revealed ataxia, anisocoria, and papilledema. CT-guided stereotactic biopsy of the pineal mass was performed with simultaneous VP shunt placement. Biopsy revealed a PTPR and tumor cells were present in the CSF obtained during the procedure. Repeat MRI 2 weeks later (Fig. 2) revealed a progressively enlarging pineal region mass measuring 19×32×20 mm. The floor of the anterior third ventricle had become thicker and nodular. The MR signal characteristics of the suprasellar mass were identical to those of the pineal mass with uniform contrast enhancement. Extensive leptomeningeal enhancement was now evident in the perimesencephalic and
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Fig. 1 Initial MRI. Sagittal T1-W (a), axial FLAIR (b), post-contrast T1-W (c) and coronal post-contrast T1-W (d) images. A solid and cystic pineal region mass is present with minimal ventricular enlargement. The solid component of the mass is slightly hyperintense
to the gray matter on both T1-W and FLAIR images, enhancing with gadolinium. Note subtle thickening of the floor of the third ventricle (a, arrow) with contrast enhancement (d, arrow)
prepontine cistern, cerebellar folia and over the cervical cord. MRI of the spine (not shown) demonstrated extensive leptomeningeal enhancement of the surface of the cord and abnormal contrast enhancement of the dural sac. Pathology revealed tumor with a papillary growth pattern of plump epithelial-like cells lining thin vascular cores. Nuclei were uniform, vesicular with fine chromatin with rare mitoses. On immunohistochemistry, the tumor cells were immunoreactive for cytokeratin, synaptophysin, neuronspecific enolase, and chromogranin A. S-100 and vimentin were variably positive. The pathology was consistent with PTPR. A 111In-DTPA-pentetreotide scan (Fig. 2) revealed intense radiotracer uptake within the pineal and suprasellar masses with diffuse uptake along the spinal canal. The patient was initially treated with chemotherapy consisting of two cycles of ifosfamide, cisplatin and etoposide. However, because of poor tumor response, radiation was initiated with craniospinal fields using 6 MV photons. The boy received 36 Gy in 20 fractions to the spinal axis and 39.6 Gy in 22 fractions to the whole brain. An additional boost was given to the posterior fossa to bring the total to 52.2 Gy in 29 fractions. A final boost was given to the pineal
region and tumor-coated brainstem to bring this volume to 55.8 Gy in 31 fractions. Despite this high dose and comprehensive treatment, the patient showed no significant response and he died 12 months after initial presentation.
Fig. 2 Imaging at 5 months after presentation. a–c MRI: sagittal T1W (a), sagittal post-contrast T1-W (b) and coronal port-contrast T1-W (c) images. The pineal mass has enlarged and the floor of the third ventricle is thicker and more nodular (a, arrow), and enhancing with contrast (c, arrow). Extensive leptomeningeal enhancement is present.
d 111In-DTPA-pentetreotide scan, midsagittal image (A anterior). Intense radiotracer uptake is apparent in the pineal region (large arrow), and also in the suprasellar region (small arrow) and along the spinal cord (dashed arrow)
Discussion PTPR is a rare tumor originally described by Jouvet et al. [1] and formally recognized as a distinct entity in the 2007 WHO classification. Immunohistochemistry is essential for differentiating PTPR from other pineal region tumors with papillary histology [2]. PTPR occurs both in children and in adults. In a review of 31 cases by Fèvre-Montange et al. [3], patients’ ages ranged from 5 years to 66 years with a mean age of 31.5 years [3]. On imaging, PTPRs are well-circumscribed pineal region masses usually measuring 0.5–5.0 cm in diameter, and commonly have a cystic component [3]. Chang et al. [5] reported unique intrinsic T1 hyperintensity in four patients with PTPR and hypothesized that protein, glycoprotein or other T1-shortening substances produced by
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tumor cells with well-differentiated secretory functions were the cause. The T1 signal in the present patient was not as hyperintense as in those reported by Chang et al. [5], and suggests variability of T1 hyperintensity among PTPRs. One of the unique features in our patient was the simultaneous presence of a contrast-enhancing suprasellar mass with the pineal mass, clearly preceding the development of widespread leptomeningeal metastasis. Simultaneous multicentric tumors in pineal and suprasellar regions have been observed in germinoma [6], but have not been reported in PTPR. The proposed cell of origin for PTPR is specialized ependymocytes located in the subcommissural organ (SCO) in the pineal region [1, 3]. The SCO may be present in extrapineal locations. Chordoid glioma of the third ventricle, another tumor of possible SCO cell origin, characteristically originates in the anterior third ventricle [7]. We hypothesize that, due to the possible presence of the SCO in multiple locations adjoining the third ventricle, simultaneous development of PTPR in the pineal and hypothalamic regions may occur in a manner similar to that seen in germinomas. Because of his inoperable, widespread disease at the time of biopsy, the patient underwent 111In-DTPA-pentetreotide imaging as a possible candidate for radiolabeled octreotide treatment. Pentetreotide is a somatostatin analog and, when given intravenously, 111In-DTPA-pentetreotide acts as a somatostatin receptor-specific tracer. OctreoScan has previously been used for the diagnosis of neuroendocrine tumors such as carcinoid [8]. Strong tracer uptake in PTPR suggests a high density of somatostatin receptors. This may have future implications for this molecular-specific imaging technique not only in the diagnosis of PTPR but also in targeted treatment using such agents as 90Y-DOTA-tyr3-pentetreotide, a beta-ray emitter, for inoperable cases. To date there is no consensus as to the best therapy for PTPR [3]. Aggressive local therapy with maximal surgical resection and adjuvant radiotherapy has been advocated. In a multiinstitutional retrospective review of 31 cases, Fèvre-Montange
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et al. [3] reported recurrence in 70% and CSF dissemination in 7%. Gross total resection was the only clinical factor that tended to be associated with overall survival and decreased recurrence. A single patient in their series who did not undergo surgical resection had early CSF dissemination and succumbed to the disease in 21 months [3]. In our patient, surgical resection was not performed due to CSF dissemination at the time of presentation to our facility. Early diagnosis and aggressive local therapy with maximal surgical resection is emphasized. In conclusion, we report a case of PTPR in a young male, noteworthy for early CSF dissemination. Surgical resection could not be performed and relentless progression, in spite of intense chemotherapy and radiotherapy, resulted in an early fatal outcome. The neuroimaging appearance is unique with possible multicentric origin of the tumor and intense uptake of 111In-DTPA-pentetreotide.
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