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Iranian Journal of Allergy, Asthma, and... Aug 2020Brain tumors are the rarest cause of cerebrospinal fluid rhinorrhea. Non-traumatic cerebrospinal fluid rhinorrhea is also a relatively rare condition. It may be...
Brain tumors are the rarest cause of cerebrospinal fluid rhinorrhea. Non-traumatic cerebrospinal fluid rhinorrhea is also a relatively rare condition. It may be misdiagnosed as allergic rhinitis or chronic sinusitis and lead to unsuitable treatment. We described a 34-year-old man who came to our allergy clinic with a chief complaint of clear rhinorrhea from his left nostril with more than four years of duration. Onlyhypertrophy of left inferior concha was found in the clinical examination. His rhinorrhea aggravated when bending forward. So we were suspicious of CSF rhinorrhea. MRI was done for him and demonstrated a large tumor in the pineal region. The patient underwent surgery with resection of the mass via an infratentorial-supracerebellar approach. This case showed the role of maintaining differential diagnosis for a common complaint; rhinitis which is seen as usual.
Topics: Adult; Carcinoma, Squamous Cell; Cerebrospinal Fluid Rhinorrhea; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Pinealoma; Rhinitis; Symptom Assessment
PubMed: 33463112
DOI: 10.18502/ijaai.v19i4.4121 -
Clinical Neuroradiology Dec 2020
Topics: Adolescent; Brain Neoplasms; Glioma; Headache; Humans; Magnetic Resonance Imaging; Male; Neuropathology; Vision Disorders
PubMed: 33230629
DOI: 10.1007/s00062-020-00973-4 -
Frontiers in Endocrinology 2020Pineal metastasis is an exceedingly rare finding in patients with systemic malignancies. Such lesions are typically the manifestation of a primary lung cancer;...
Pineal metastasis is an exceedingly rare finding in patients with systemic malignancies. Such lesions are typically the manifestation of a primary lung cancer; nonetheless, a variety of malignancies have been reported to disseminate to the pineal gland including gastrointestinal, endocrine, and skin cancers, among others. However, to our knowledge, pineal gland metastasis without a primary origin has yet to be described. Carcinoma of unknown primary origin is a heterogeneous group of cancers characterized by the presence of metastatic disease without an identifiable primary tumor on metastatic workup. Here, we present a case of a 65-year-old male found to have a heterogeneously enhancing lesion of the pineal gland as well as an enhancing lesion of the left cerebellar hemisphere. Comprehensive metastatic workup demonstrated multifocal metastatic adenopathy without an identifiable primary lesion. Stereotactic biopsy of the pineal lesion revealed poorly differentiated carcinoma with an immunophenotype most consistent with gastrointestinal origin. To our knowledge, this is the first case to describe a pineal gland metastasis without a primary origin. We discuss the relevant literature on pineal gland metastases as well as carcinoma of unknown primary origin.
Topics: Aged; Cell Differentiation; Humans; Male; Neoplasms, Unknown Primary; Pinealoma; Prognosis
PubMed: 33193110
DOI: 10.3389/fendo.2020.597773 -
Acta Neuropathologica Oct 2020
Topics: Adolescent; Brain Neoplasms; Cerebellar Neoplasms; Female; Humans; Male; Medulloblastoma; Mutation, Missense; Neoplasms, Germ Cell and Embryonal; Pineal Gland; Pinealoma; Wnt Proteins; beta Catenin
PubMed: 32772175
DOI: 10.1007/s00401-020-02208-9 -
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 -
Frontiers in Endocrinology 2020The purpose of this investigational study was to assess the effects of melatonin replacement therapy on cardiac autonomic modulation in pinealectomized patients. This... (Clinical Trial)
Clinical Trial
The purpose of this investigational study was to assess the effects of melatonin replacement therapy on cardiac autonomic modulation in pinealectomized patients. This was an open-label, single-arm, single-center, proof-of-concept study consisting of a screening period, a 3-month treatment period with melatonin (3 mg/day), and a 6-month washout period. The cardiac autonomic function was determined through heart rate variability (HRV) measures during polysomnography. Pinealectomized patients ( = 5) with confirmed absence of melatonin were included in this study. Melatonin treatment increased vagal-dominated HRV indices including root mean square of the successive R-R interval differences (RMSSD) (39.7 ms, 95% CI 2.0-77.4, = 0.04), percentage of successive R-R intervals that differ by more than 50 ms (pNN50) (17.1%, 95% CI 9.1-25.1, = 0.003), absolute power of the high-frequency band (HF power) (1,390 ms, 95% CI 511.9-2,267, = 0.01), and sympathetic HRV indices like standard deviation of normal R-R wave interval (SDNN) (57.6 ms, 95% CI 15.2-100.0, = 0.02), and absolute power of the low-frequency band (LF power) (4,592 ms, 95% CI 895.6-8,288, = 0.03). These HRV indices returned to pretreatment values when melatonin treatment was discontinued. The HRV entropy-based regularity parameters were not altered in this study, suggesting that there were no significant alterations of the REM-NREM ratios between the time stages of the study. These data show that 3 months of melatonin treatment may induce an improvement in cardiac autonomic modulation in melatonin-non-proficient patients. NCT03885258.
Topics: Adolescent; Adult; Autonomic Nervous System; Central Nervous System Depressants; Child; Female; Follow-Up Studies; Heart; Heart Rate; Humans; Male; Melatonin; Pinealectomy; Pinealoma; Prognosis; Sleep Wake Disorders; Young Adult
PubMed: 32431667
DOI: 10.3389/fendo.2020.00239 -
Surgical Neurology International 2020Biphasic tumors of the central nervous system are rarely described and mainly consisted out of the glial and mesenchymal component. The tumor originated out of both...
BACKGROUND
Biphasic tumors of the central nervous system are rarely described and mainly consisted out of the glial and mesenchymal component. The tumor originated out of both astrocytes and pinealocytes, best to our knowledge, has not been described. We present a case of a brain tumor consisted out of pilocytic astrocytoma (PA) and pineocytoma as components situated in the pineal region and posterior cranial fossa in young adult.
CASE DESCRIPTION
We present a 21-year-old patient with a history of intermittent headache, followed by nausea and vomiting, double vision, and dextropulsion. Magnetic resonance imaging revealed an extensive cystic-solid expansive formation in the posterior cranial fossa with a solid part in the area of the pineal gland. The patient underwent surgical resection. The pathohistological analysis showed two types of tumor cells; the major part of tumor showed features of PA, while minor part corresponded to pineocytoma.
CONCLUSION
PA accounts for 5% of all gliomas and is most common in children and young adults. It usually occurs in the cerebellum, the optic pathway, third ventricular region, etc. Pineocytomas are rare, accounting up to 1% of all intracranial tumors. Since tumors origin is different, there must be complex molecular events or mutations that can lead to cell rearrangements and generation of two histologically different tissues in the same tumor mass. The course of treatment options is different for PA and pineocytoma; therefore, the case of brain mass consisted out of two different tissues can be helpful when deciding about the treatment of tumors in posterior cranial fossa and pineal region.
PubMed: 32363059
DOI: 10.25259/SNI_288_2019 -
Nature Communications Apr 2020Pineoblastoma is a rare pediatric cancer induced by germline mutations in the tumor suppressors RB1 or DICER1. Presence of leptomeningeal metastases is indicative of...
Pineoblastoma is a rare pediatric cancer induced by germline mutations in the tumor suppressors RB1 or DICER1. Presence of leptomeningeal metastases is indicative of poor prognosis. Here we report that inactivation of Rb plus p53 via a WAP-Cre transgene, commonly used to target the mammary gland during pregnancy, induces metastatic pineoblastoma resembling the human disease with 100% penetrance. A stabilizing mutation rather than deletion of p53 accelerates metastatic dissemination. Deletion of Dicer1 plus p53 via WAP-Cre also predisposes to pineoblastoma, albeit with lower penetrance. In silico analysis predicts tricyclic antidepressants such as nortriptyline as potential therapeutics for both pineoblastoma models. Nortriptyline disrupts the lysosome, leading to accumulation of non-functional autophagosome, cathepsin B release and pineoblastoma cell death. Nortriptyline further synergizes with the antineoplastic drug gemcitabine to effectively suppress pineoblastoma in our preclinical models, offering new modality for this lethal childhood malignancy.
Topics: Animals; Autophagosomes; Autophagy; Cluster Analysis; Disease Models, Animal; Gene Deletion; Germ-Line Mutation; Humans; Integrases; Kaplan-Meier Estimate; Lysosomes; Mice; Neoplasm Metastasis; Nortriptyline; Pinealoma; Retinoblastoma Protein; Tumor Suppressor Protein p53
PubMed: 32286280
DOI: 10.1038/s41467-020-15585-2 -
Cancer Biology & Therapy Jun 2020Metastasis in the pineal region is a rare condition. To best of our knowledge, there is no case report of isolated pineal metastasis secondary to acute lymphocytic...
Metastasis in the pineal region is a rare condition. To best of our knowledge, there is no case report of isolated pineal metastasis secondary to acute lymphocytic leukemia (ALL). The aim of this study is to show the pineal gland involvement of ALL in a case for the first time in the literature. A 25-year-old male patient diagnosed with ALL 2 years ago presented with headache and visual impairment. Brain magnetic resonance imaging (MRI) revealed a well-defined solid lesion which was revealed intensive enhancement after contrast. On diffusion-weighted images, the lesion showed significant diffusion restriction. Three months after therapy, control MRI demonstrated a completely resorbed pineal lesion. The pineal region may be a possible site of metastasis and involvement due to the absence of a blood-brain barrier, and should not be overlooked in patients with not only solid cancers but also ALL.
Topics: Adult; Humans; Male; Pinealoma; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Prognosis
PubMed: 32208886
DOI: 10.1080/15384047.2020.1735605 -
Pediatric Blood & Cancer Jun 2020We report the outcomes of patients with pineoblastoma and trilateral retinoblastoma syndrome enrolled on the Head Start (HS) I-III trials.
BACKGROUND
We report the outcomes of patients with pineoblastoma and trilateral retinoblastoma syndrome enrolled on the Head Start (HS) I-III trials.
METHODS
Twenty-three children were enrolled prospectively between 1991 and 2009. Treatment included maximal surgical resection followed by five cycles of intensive chemotherapy and consolidation with marrow-ablative chemotherapy and autologous hematopoietic cell rescue (HDCx/AuHCR). Irradiation following consolidation was reserved for children over six years of age or those with residual tumor at the end of induction.
RESULTS
Median age was 3.12 years (range, 0.44-5.72). Three patients withdrew from the study treatment and two patients experienced chemotherapy-related death. Eight patients experienced progressive disease (PD) during induction chemotherapy and did not proceed to HDCx/AuHCR. Ten patients received HDCx/AuHCR; eight experienced PD post-consolidation. Seven patients received craniospinal irradiation (CSI) with a median dose of 20.7 Gy (range, 18-36 Gy) with boost(s) (median dose 27 Gy; range, 18-36 Gy); three received CSI as adjuvant therapy (two post-HDCx/AuHCR) and four upon progression/recurrence. The five-year progression-free survival (PFS) and overall survival (OS) were 9.7% (95% confidence intervals [CI]: 2.6%-36.0%) and 13% (95% CI: 4.5%-37.5%), respectively. Only three patients survived beyond five years. Favorable OS prognostic factors were CSI (hazard ratio [HR] = 0.30 [0.11-0.86], P = 0.025) and HDCx/AuHCR (HR = 0.40 [0.16-0.99], P = 0.047).
CONCLUSIONS
Within the HS I-III trials, CSI and HDCx/AuHCR were statistically associated with improved survival. The high PD rate during later induction cycles and following consolidation chemotherapy warrants consideration of fewer induction cycles prior to consolidation and the potential intensification of consolidation with multiple cycles of marrow-ablative chemotherapy and irradiation.
Topics: Brain Neoplasms; Child; Child, Preschool; Combined Modality Therapy; Female; Follow-Up Studies; Humans; Infant; Male; Pineal Gland; Pinealoma; Prognosis; Prospective Studies; Survival Rate
PubMed: 32187454
DOI: 10.1002/pbc.28252