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International Medical Case Reports... 2022Tumors in the pineal region usually present with acute hydrocephalus. Histologic diagnosis is most important, as it greatly influences treatment, because over 17 tumor...
Tumors in the pineal region usually present with acute hydrocephalus. Histologic diagnosis is most important, as it greatly influences treatment, because over 17 tumor types occur in this area. Biopsies of these lesions play an important role in further management. are benign and rare tumors that typically exhibit a slow progression. However, the appropriate treatment for pineocytoma varies. Surgical excision was considered for good long-term outcomes; however, this may not always be possible. Radiotherapy also appears to be effective in patients with residual pineocytomas. We report a case of pineocytoma with hemorrhagic transformation and complicated hydrocephalus. The patient refused to undergo aggressive tumor excision. Thus, the patient only underwent endoscopic biopsy and external ventricular drain (EVD) implantation, but the outcome was acceptable. In addition, we reviewed the current management strategies for pineocytomas in the literature.
PubMed: 35755243
DOI: 10.2147/IMCRJ.S367293 -
Unedited microneurosurgery of a high-grade pineal parenchymal tumor of intermediate differentiation.Surgical Neurology International 2018WHO Grade II-III pineal parenchymal tumors of intermediate differentiation (PPTIDs) were included in the 2007 World Health Organization Classification of Central Nervous...
BACKGROUND
WHO Grade II-III pineal parenchymal tumors of intermediate differentiation (PPTIDs) were included in the 2007 World Health Organization Classification of Central Nervous System Tumors as pineal parenchymal tumors between pineocytomas and pineoblastomas. PPTIDs comprise more than 20-60% of all pineal parenchymal tumors (PPT) s and are characterized by moderately high cellularity, mild-to-moderate nuclear atypia, and low-to-moderate mitotic activity. Moreover, PPTID includes transitional cases in which pineocytomatous and pineoblastoma features are associated. Synaptophysin and neuron-specific enolase are usually positive, with variable reactivity to neurofilament protein, chromogranin A, retinal S-antigen, and S-100 protein. PPTID Grades II and III can be distinguished on the basis of mitotic activity (higher in high-grade PPTID) and neurofilament protein immunoreactivity (higher in low-grade PPTID). Herein, we present the microsurgical management of a histologically confirmed high-grade PPTID.
CASE DESCRIPTION
A patient with high grade PPTID underwent sitting praying position and right paramedian supracerebellar infratentorial approach. The lesion was identified after lateral opening of the quadrigeminal cistern, followed by removal of its cystic component. Tissue samples were obtained under high microscopic magnification, and internal debulking of the tumor was performed with ring microforceps and bipolar forceps in the right hand and a thumb-regulated suction tube in the left hand. Continuous water irrigation provided us a clean surgical field and the recognition of small bleeding vessels. Moreover, water dissection technique was applied to recognize the cleavage plane of the tumor. Bipolar coagulation forceps were used to shrink the tumor and remove it by piecemeal reduction aiming to identify the anterior and lateral limits of the lesion. The poorly differentiated borders between the tumor and the surrounding parenchyma were determined under microscopic vision. Small vessels feeding the tumor were coagulated and cut. The most critical surgical stage was related with removal of some tumor remnants attached to the internal cerebral veins. A meticulous and skillful dissection was essential aiming to preserve these vascular structures. The final steps included meticulous hemostasis with electrocoagulation, Tachosil, and Surgicel. The postoperative course was uneventful. The patient underwent adjuvant radiotherapy and currently is alive, free of tumor recurrence 4 years after surgery.
CONCLUSION
This unedited video offers all detailed aspects that are, as the senior author JH considers, essential for a neurosurgeon when performing an efficient and safe surgery for a high-grade PPTID.
VIDEOLINK
http://surgicalneurologyint.com/videogallery/pineal-tumor-2.
PubMed: 30603232
DOI: 10.4103/sni.sni_353_18 -
Frontiers in Neurology 2021With the increasing availability and advances in brain imaging, pineal cyst lesions (PCL) are becoming a common finding in the pediatric population. In the absence of...
With the increasing availability and advances in brain imaging, pineal cyst lesions (PCL) are becoming a common finding in the pediatric population. In the absence of evidence-based guidelines, optimal diagnostic and therapeutic approaches have not been established, and there is a risk of under- or overtreatment of these patients. The aim of our study was to evaluate the clinical presentation and radiological features of PCL in a cohort of pediatric patients and to identify clinical parameters more commonly associated with neoplasms in the pineal region. In addition, the prevalence of PCL in the pediatric population of Slovenia was estimated. In this observational, cohort study, children treated at University Children's Hospital, Ljubljana, Slovenia in the period 1997-2016 were included if PCL was found on brain imaging. We analyzed indications for referral to a neurologist, clinical signs and symptoms, radiological features, treatment and outcome. The cohort consisted of 143 children with PCL. Pineocytoma was suspected in 31 children (21.7%). Six children underwent surgery - pineocytoma was confirmed in two cases and germinoma in one (2/3 of these children had signs of increased intracranial pressure (ICP), while PCL was benign in the remaining 4 cases. Only 2 PCL enlarged during the study period, both <2mm, none of these children developed neoplasm. Two children had PCL >20mm in diameter; both showed signs of increased ICP, one patient was found to have a germinoma of the pineal region, while the other had no neoplasm. Most PCL do not change their features during radiological follow-up and even atypical PCL are very rarely associated with a malignant neoplasm of the pineal region. A PCL larger than 20 mm and signs of increased ICP were identified as potential markers for selecting patients at risk.
PubMed: 34512533
DOI: 10.3389/fneur.2021.722696 -
Neurology India 2019
Topics: Brain Neoplasms; Humans; Pineal Gland; Pinealoma
PubMed: 31085867
DOI: 10.4103/0028-3886.258023 -
Neurology India 2019Pineal gland tumors range from the well-differentiated "pineocytoma" [World Health Organization (WHO) grade I], which have a very good prognosis, to the aggressive and...
BACKGROUND
Pineal gland tumors range from the well-differentiated "pineocytoma" [World Health Organization (WHO) grade I], which have a very good prognosis, to the aggressive and poorly differentiated "pineoblastoma" (WHO grade IV) with "pineal parenchymal tumor of intermediate differentiation" (PPTID; WHO grades II and III) occupying intermediary differentiation and prognosis. Papillary tumor of the pineal region (PTPR; WHO grades II and III) is a distinct entity with propensity for recurrence and spinal dissemination. However, the diagnostic criteria to differentiate these entities, especially between WHO grades II and III of both PPTID and PTPR, remain nebulous.
OBJECTIVE
To evaluate the relative frequency of the individual entities and histomorphological (including the proliferation indices) features across the spectrum of pineal parenchymal tumors (PPTs) [including PTPRs] along their course.
DESIGN
All cases of PPTs were retrieved, reviewed, and graded based on the histological criteria defined in the literature.
RESULTS
PPTID, more commonly seen in young adults, was the most common subtype of PPT. This was followed by pineoblastoma which was more commonly seen in children. Clinical progression was seen in both grades II and III of PPTID; however, it was more commonly seen in cases with a MIB1 labeling index of >10%. PTPRs (both grades II and III) showed an aggressive histological transformation and also intraparenchymal metastasis.
CONCLUSION
PPTIDs are the most common adult primary PPTs and have the potential to progress and disseminate in both grades II and III. Both grades of PTPRs have a metastatic potential. These findings suggest the need for postoperative adjuvant therapy in both grades of PPTID and PTPR.
Topics: Brain Neoplasms; Child; Combined Modality Therapy; Female; Humans; Male; Neoplasm Recurrence, Local; Pineal Gland; Pinealoma; Prognosis; Young Adult
PubMed: 31085866
DOI: 10.4103/0028-3886.258045 -
Asian Journal of Neurosurgery 2021Primary tumors of the pineal gland occur infrequently with a preponderance of either parenchymal tumors or germ cells tumors. Papillary tumor of the pineal region is a...
Primary tumors of the pineal gland occur infrequently with a preponderance of either parenchymal tumors or germ cells tumors. Papillary tumor of the pineal region is a rare neuroepithelial lesion that arises exclusively in the pineal region. They have been designated as either Grade II or Grade III lesions as per the 2016 WHO classification of central nervous system tumors. Clinically, they usually present with obstructive hydrocephalus and visual disturbance. On imaging, these tumors are solid-cystic, heterogeneously enhancing, and show T2 hyperintensity. Pathologically, they can closely resemble a Grade I pineocytoma and immunohistochemistry is essential to differentiate the two. No definite guidelines exist to confirm the ideal protocol of treatment. Evidence regarding the role of radiation after surgery is limited to case reports and series. Adjuvant therapy is usually recommended for tumors with subtotal excision, high proliferative/mitotic index, or proven metastasis. We describe a case of a 29-year-old male with a recurrent papillary tumor of the pineal region, 9 years after primary surgery where it was misdiagnosed as a pineocytoma. The tumor was effectively controlled with surgical excision, cerebrospinal fluid diversion, and adjuvant radiation for 8 years before showing two recurrences within a span of 6 months with a rising proliferation index.
PubMed: 34268174
DOI: 10.4103/ajns.AJNS_439_20 -
Archives of Pathology & Laboratory... Aug 2006Pineal anlage tumor is an extremely rare tumor that is not listed in the 2000 World Health Organization Classification of nervous system tumors. It has been defined as a... (Review)
Review
Pineal anlage tumor is an extremely rare tumor that is not listed in the 2000 World Health Organization Classification of nervous system tumors. It has been defined as a primary pineal tumor with both neuroepithelial and ectomesenchymal differentiation and without endodermal differentiation. We review the literature on this tumor, including the clinical presentation, gross pathology, histopathology, immunohistochemistry, differential diagnosis, and prognosis.
Topics: Biomarkers, Tumor; Brain Neoplasms; Diagnosis, Differential; Humans; Immunohistochemistry; Medulloblastoma; Mesenchymoma; Neuroectodermal Tumor, Melanotic; Pineal Gland; Pinealoma; Rhabdomyosarcoma; Teratoma
PubMed: 16879032
DOI: 10.5858/2006-130-1233-ROPATW -
Neurosurgical Review Jun 2021Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous histological entities require different therapeutic regimens. We...
Pineal region tumors commonly present with non-communicating hydrocephalus. These heterogeneous histological entities require different therapeutic regimens. We evaluated our surgical experience concerning procurance of a histological diagnosis, management of hydrocephalus, and choice of antitumoral treatment. We analyzed the efficacy of neuroendoscopic biopsy and endoscopic third ventriculocisternostomy (ETV) in patients with pineal region tumors between 2006 and 2019 in a single-center retrospective cross-sectional study with regard to diagnostic yield, hydrocephalus treatment, as well as impact on further antitumoral management. Out of 28 identified patients, 23 patients presented with untreated hydrocephalus and 25 without histological diagnosis. One patient underwent open biopsy, and 24 received a neuroendoscopic biopsy with concomitant hydrocephalus treatment if necessary. Eighteen primary ETVs, 2 secondary ETVs, and 2 ventriculoperitoneal shunts (VPSs) were performed. Endoscopic biopsy had a diagnostic yield of 95.8% (23/24) and complication rates of 12.5% (transient) and 4.2% (permanent), respectively. ETV for hydrocephalus management was successful in 89.5% (17/19) with a median follow-up of more than 3 years. Following histological diagnosis, 8 patients (28.6%) underwent primary resection of their tumor. Another 9 patients underwent later-stage resection after either adjuvant treatment (n = 5) or for progressive disease during observation (n = 4). Eventually, 20 patients received adjuvant treatment and 7 were observed after primary management. One patient was lost to follow-up. Heterogeneity of pineal region tumor requires histological confirmation. Primary biopsy of pineal lesions should precede surgical resection since less than a third of patients needed primary surgical resection according to the German pediatric brain tumor protocols. Interdisciplinary decision making upfront any treatment is warranted in order to adequately guide treatment.
Topics: Adolescent; Brain Neoplasms; Child; Child, Preschool; Cross-Sectional Studies; Disease Management; Female; Follow-Up Studies; Humans; Hydrocephalus; Infant; Male; Neuroendoscopy; Pineal Gland; Pinealoma; Retrospective Studies; Ventriculoperitoneal Shunt; Ventriculostomy
PubMed: 32504201
DOI: 10.1007/s10143-020-01323-1 -
Rare Tumors Oct 2011Pineal parenchymal tumors are rare. Of the three types of pineal parenchymal tumors, pineocytomas are the least aggressive and are not known to diffusely disseminate. In...
Pineal parenchymal tumors are rare. Of the three types of pineal parenchymal tumors, pineocytomas are the least aggressive and are not known to diffusely disseminate. In this paper, we report the successful treatment of a case of pineocytoma with diffuse leptomeningeal relapse following initial stereotactic radiotherapy. A 39-year-old female presented with headaches, balance impairment, urinary incontinence, and blunted affect. A pineal mass was discovered on magnetic resonance imaging (MRI). A diagnosis of pineocytoma was established with an endoscopic pineal gland biopsy, and the patient received stereotactic radiotherapy. Ten years later, she developed diffuse leptomeningeal dissemination. The patient was then successfully treated with craniospinal radiation therapy. Leptomeningeal spread may develop as late as 10 years after initial presentation of pineocytoma. Our case demonstrates the importance of long-term follow-up of patients with pineal parenchymal tumors following radiation therapy, and the efficacy of craniospinal radiation in the treatment of leptomeningeal dissemination.
PubMed: 22355508
DOI: 10.4081/rt.2011.e53 -
The Neuroradiology Journal Apr 2015Diffusion weighted imaging (DWI) has been shown to be helpful in characterizing tumor cellularity, and predicting histology. Several works have evaluated this technique...
BACKGROUND
Diffusion weighted imaging (DWI) has been shown to be helpful in characterizing tumor cellularity, and predicting histology. Several works have evaluated this technique for pineal tumors; however studies to date have not focused on pediatric pineal tumors.
OBJECTIVE
We evaluated the diffusion characteristics of pediatric pineal tumors to confirm if patterns seen in studies using mixed pediatric and adult populations remain valid.
MATERIALS AND METHODS
This retrospective study was performed after Institutional Review Board approval. We retrospectively evaluated all patients 18 years of age and younger with pineal tumors from a single institution where preoperative diffusion weighted imaging as well as histologic characterization was available.
RESULTS
Twenty patients (13 male, 7 female) with pineal tumors were identified: seven with pineoblastoma, four with Primitive Neuroectodermal Tumor (PNET), two with other pineal tumors, and seven with germ cell tumors including two germinomas, three teratomas, and one mixed germinoma-teratoma. The mean apparent diffusion coefficient (ADC) values in pineoblastoma (544 ± 65 × 10⁻⁶ mm²/s) and pineoblastoma/PNET (595 ± 144 × 10⁻⁶ mm²/s) was lower than that of the germ cell tumors (1284 ± 334 × 10⁻⁶ mm²/s; p < 0.0001 vs pineoblastoma). One highly cellular germinoma had an ADC value of 694 × 10⁻⁶ mm²/s.
CONCLUSION
ADC values can aid in differentiation of pineoblastoma/PNET from germ cell tumors in a population of children with pineal masses.
Topics: Adolescent; Brain Neoplasms; Child; Child, Preschool; Diffusion Magnetic Resonance Imaging; Female; Humans; Infant; Male; Pineal Gland; Pinealoma; Reproducibility of Results; Retrospective Studies; Sensitivity and Specificity
PubMed: 25963154
DOI: 10.1177/1971400915581741