Papillary tumor of the pineal region in a 15-month-old boy

The papillary tumor of the pineal region (PTPR) is a distinct entity that is particularly rare in the pediatric population. The authors document the youngest reported patient with this clinicopathological entity to date. A case of PTPR in a 15-month-old boy is described. Initially thought to be a tectal glioma, the tumor was later identified as a pineal region tumor after demonstrating growth on routine imaging. Diagnosis of PTPR was established by histopathological evaluation of biopsy samples, which revealed papillary, cystic, and solid tumor components. The patient's postoperative course was complicated by tumor growth despite several debulking procedures and chemotherapy, as well as persistent hydrocephalus requiring 2 endoscopic third ventriculostomies and eventual ventriculoperitoneal shunt placement. After a 15-month follow-up period, the patient has received proton-beam therapy and has a stable tumor size. The PTPR is a recently described tumor of the CNS that must be included in the differential diagnosis of pineal region masses. The biological behavior, prognosis, and appropriate treatment of PTPR have yet to be fully defined.

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Solitary Fibrous Tumors in the Central Nervous System

Archives of Pathology & Laboratory Medicine ◽

10.5858/2003-127-0432-sftitc ◽

2003 ◽

Vol 127 (4) ◽

pp. 432-439 ◽

Cited By ~ 10

Author(s):

Tarik Tihan ◽

Michael Viglione ◽

Marc K. Rosenblum ◽

Alessandro Olivi ◽

Peter C. Burger

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Biological Behavior ◽

Clinicopathologic Features ◽

Distinct Entity ◽

Total Resection ◽

Solitary Fibrous Tumors ◽

The Central Nervous System ◽

Extracranial Metastases

Abstract Context.—Solitary fibrous tumors (SFTs) of the central nervous system are rare neoplasms that usually present as dura-based masses and clinically resemble meningiomas. Histologically, they can be similar to fibrous meningioma or hemangiopericytoma (HPC). In particular, densely cellular regions seen in some SFTs can be indistinguishable from HPC. Little is known about the biological behavior of SFTs, although most seem amenable to total resection. Objectives.—To define the clinicopathologic spectrum of SFTs in the central nervous system and to outline their differences from HPC and meningioma. Design.—We present the clinicopathologic features of 18 patients with SFT and compare them with those of an age- and sex-matched cohort of HPCs. Results.—Eleven SFTs were supratentorial, 3 were infratentorial, and 4 were intraspinal. Four of the 18 tumors were intra-axial (2 in the lateral ventricles and 2 within the spinal cord). Histologically, SFTs were similar to their soft tissue counterparts. Six tumors (6/18) had densely cellular regions, and 1 tumor showed frankly anaplastic features. All but 3 patients underwent gross total resection, and there were no metastases or tumor-related mortalities during the median follow-up of 40 months. In contrast, there were 15 local recurrences (83%), 5 extracranial metastases (27%), and 4 tumor-related deaths (22%) in the HPC cohort. Conclusions.—Our study presents the clinicopathologic features of SFT as a distinct entity from both meningioma and HPC. We also present unusual examples of anaplastic, intraventricular, and intramedullary spinal SFTs that expand the clinicopathologic spectrum of these uncommon and sometimes diagnostically difficult neoplasms.

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Papillary Tumor of the Pineal Region: MR Signal Intensity Correlated to Histopathology

Case Reports in Neurological Medicine ◽

10.1155/2015/315095 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4

Author(s):

Marcos Rosa Junior ◽

Antonio Jose da Rocha ◽

Adriano Zanon da Silva ◽

Sergio Rosemberg

Keyword(s):

Subcommissural Organ ◽

Pineal Region ◽

World Health ◽

Pineal Tumor ◽

Imaging Diagnosis ◽

Posterior Commissure ◽

Papillary Tumor ◽

The World ◽

T1 Hyperintensity ◽

Health Organization

Tumors of the pineal region are rare and can be challenging to differentiate by imaging. Papillary tumor of the pineal region (PTPR) was recently recognized as a neoplasm in the World Health Organization (WHO) 2007 classification, arising from specialized ependymocytes in the subcommissural organ, which is located in the pineal region. It is a rare histological type of pineal tumor with only a few cases reported. Here, we describe a case of histologically confirmed PTPR in a 17-year-old man who presented with a headache. A literature review was performed to clarify the clinical, radiological, and pathological features of PTPR. Pineal neoplasms do not have pathognomonic imaging findings; however, we discuss T1 hyperintensity, which is a key for imaging diagnosis according to recent reports. In particular, if the hyperintensity in T1 is not due to fat, calcification, melanin, or hemorrhage in a mass of the posterior commissure or pineal region, the diagnosis of a PTPR may be suggested, as observed in this case.

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Early Treatment Response of a Rare Papillary Tumor of the Pineal Region after Primary Proton-Beam Therapy using the Raster-Scanning Technique at HIT

Tumori Journal ◽

10.1177/030089161209800521 ◽

2012 ◽

Vol 98 (5) ◽

pp. e122-e125 ◽

Cited By ~ 3

Author(s):

Daniel Habermehl ◽

Florian Blachutzik ◽

Swantje Ecker ◽

Jan-Oliver Dittmar ◽

Stefan Rieken ◽

...

Keyword(s):

Treatment Response ◽

Proton Beam ◽

Early Treatment ◽

Proton Beam Therapy ◽

Pineal Region ◽

Primary Proton ◽

Papillary Tumor ◽

Raster Scanning ◽

Scanning Technique ◽

Early Treatment Response

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RARE-48. PAPILLARY TUMOR OF THE PINEAL REGION IN PEDIATRIC POPULATION ASSOCIATED WITH TRISOMY 21: FIRST CASE REPORT AND LITERATURE REVIEW

Neuro-Oncology ◽

10.1093/neuonc/now212.708 ◽

2016 ◽

Vol 18 (suppl_6) ◽

pp. vi169-vi169

Author(s):

Mansour Mathkour ◽

Juanita Garces ◽

Joshua Hanna ◽

Ian Cormier ◽

Olawale A.R. Sulaiman ◽

...

Keyword(s):

Case Report ◽

Literature Review ◽

Trisomy 21 ◽

Pediatric Population ◽

Pineal Region ◽

Papillary Tumor ◽

First Case

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Long-term follow-up of a papillary tumor of the pineal region: addendum to a case report

Journal of Neuro-Oncology ◽

10.1007/s11060-016-2246-2 ◽

2016 ◽

Vol 130 (3) ◽

pp. 601-603 ◽

Cited By ~ 1

Author(s):

Laura Lippa ◽

Anna Maria Di Giacomo ◽

Alfonso Cerase

Keyword(s):

Case Report ◽

Pineal Region ◽

Papillary Tumor ◽

Long Term Follow Up

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Resection of a pineal region papillary tumor using robotic exoscope: improved visualization and ergonomics for deep seeded tumor

Neurosurgical Focus: Video ◽

10.3171/2021.4.focvid2127 ◽

2021 ◽

Vol 5 (1) ◽

pp. V14

Author(s):

Wei X. Huff ◽

Andrew J. Witten ◽

Mitesh V. Shah

Keyword(s):

Marked Improvement ◽

Pineal Region ◽

Operating Microscope ◽

High Definition ◽

Papillary Tumor ◽

Pineal Region Tumor ◽

Pineal Region Tumors ◽

Close Proximity ◽

Supracerebellar Infratentorial Approach ◽

Deep Location

Surgery for pineal region tumors is technically challenging due to their deep location and close proximity to critical deep venous structures, midbrain, and thalamus. A high-definition video exoscope was recently proposed as an alternative to the operating microscope. The authors illustrate a case of the midline supracerebellar infratentorial approach to resect a pineal region tumor using the Modus V exoscope and demonstrate the improved visualization of critical structures in this deep location. Additionally, the marked improvement in surgeon comfort suggests that this system may have significant advantages over traditional microscope-based surgery for tumors of the pineal region. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2127.

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Microsurgical Resection of Posterior Third Ventricular/Pineal Region Papillary Tumor Via Supracerebellar Infratentorial Approach: 3-Dimensional Operative Video

Operative Neurosurgery ◽

10.1093/ons/opy133 ◽

2018 ◽

Vol 15 (6) ◽

pp. E87-E87

Author(s):

Sima Sayyahmelli ◽

Ihsan Dogan ◽

Mustafa K Başkaya

Keyword(s):

Third Ventricle ◽

Pineal Region ◽

Direct Access ◽

Third Ventriculostomy ◽

Papillary Tumor ◽

Pineal Region Tumor ◽

3 Dimensional ◽

History Of ◽

Supracerebellar Infratentorial Approach ◽

Microsurgical Resection

Abstract The posterior third ventricle and pineal region can harbor different pathologies. The supracerebellar infratentorial approach allows a direct access to the pineal region and posterior third ventricle and provides wide exposure of the arachnoid planes and deep venous system. In this 3-dimensional video, we present a patient with posterior third ventricular/pineal region tumor who underwent microsurgical resection via supracerebellar infratentorial approach. The patient is a 28-year-old woman with history of hydrocephalus who underwent endoscopic third ventriculostomy and biopsy at an outside hospital. The histopatology of the tumor was papillary tumor of the pineal region. The patient was referred for further surgical resection due to enlargement of her tumor on follow-up radiological imaging. The surgery and the patient's postoperative course were uneventful and the patient remained unchanged in the postoperative period. The important steps of the surgical approach and microsurgical resection are demonstrated in this 3-dimensional surgical video. The patient consented to publication of her images.

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Papillary tumor of the pineal region: Is stereotactic radiosurgery efficient for this rare entity?

Surgical Neurology International ◽

10.25259/sni_613_2020 ◽

2021 ◽

Vol 12 ◽

pp. 386

Author(s):

Hajar Bechri ◽

Mohammed Yassaad Oudrhiri ◽

Sidi Mamoun Louraoui ◽

Adyl Melhaoui ◽

Sanae Sefiani ◽

...

Keyword(s):

Stereotactic Radiosurgery ◽

Tumor Volume ◽

Therapeutic Option ◽

Pineal Region ◽

Rare Entity ◽

Recurrence Rates ◽

Papillary Tumor ◽

Classification Management ◽

Endoscopic Third Ventriculocisternostomy

Background: Papillary tumors of the pineal region are rare neuroepithelial lesions that were described for the 1st time in the WHO 2007 classification. Management of such lesions remains controversial. Case Description: We describe the case of a 26-year-old female who presented with intracranial hypertension syndrome secondary to a 1.9 cm3 lesion of the pineal region causing hydrocephalus. The patient benefited from an endoscopic third ventriculocisternostomy and a biopsy of her lesion in favor of a papillary tumor of the pineal region. After discussion of the surgical risks, the patient refused the surgical option and a stereotactic radiosurgery (SRS) was performed. She improved both clinically (allowing her to regain autonomy) and radiologically (reduction of 60% of tumor volume) at 1 year follow-up. Conclusion: Because of the rarity of the lesion, literature is yet not able to find consensus concerning management of such lesion, but SRS has proven efficiency for these Grades II or III lesions with high recurrence rates. Therefore, it should be considered as a primary therapeutic option allowing good outcome with low risks for the patient.

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Our Experience with Infratentorial Supracerebellar (ITSC) Approach for Pineal Region Tumor Excision

Nepal Journal of Neuroscience ◽

10.3126/njn.v12i1.15923 ◽

2016 ◽

Vol 12 (1) ◽

pp. 38-39

Author(s):

Gopal R Sharma ◽

Prakash Bista ◽

Nilam Khadka ◽

Rajiv Jha ◽

Maya Bhattachan ◽

...

Keyword(s):

Obstructive Hydrocephalus ◽

Air Embolism ◽

Pineal Region ◽

Treatment Modalities ◽

Surgical Approaches ◽

Good Recovery ◽

Total Resection ◽

Pineal Region Tumor ◽

Pineal Region Tumors

Pineal region tumors are rare and their incidence is less than 1% of primary CNS tumors. Varieties of tumors can be found in pineal region ranging from benign to malignant pathology. Treatment modalities ranged from biopsy to total excision of tumor. Many surgical approaches have been described in the literature, however, infratentorial supracerebellar ( ITSC ) approach is versatile and widely used by many neurosurgeons worldwide.The aim of this study is to analyze and discuss the surgical outcome of pineal region tumor using ITSC approach. Advantages and limitations of this approach will be discussed.This is a retrospective study of 10 patients who underwent microsurgical excision of pineal region masses using ITSC approach in sitting position under general anesthesia at our institute between April 2009 and March 2014. The follow up period ranged from 7 months to 5 years and outcome was measured with GOS (Glasgow Outcome Scale).There were 9 male and 1 female and age ranged from 10 to 50 years.Gross total resection was performed in all 10 cases using ITSC approach. 9 patients required VP shunt for obstructive hydrocephalus. 60% (6) had benign and 40% (4) had malignant pathology.Six patients suffered from postoperative complications which included air embolism ,pneumocephalus, pseudomeningocele and seizure.Eighty percent (8) have good recovery, 1 was severely disabled and 1 died.Advanced neurological techniques combined with neuroanesthesia, neuroimaging and postoperative critical care methods have made aggressive surgical resection a mainstay of management of pineal region tumors with excellent results.Nepal Journal of Neuroscience 12:31-37, 2015

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