Pineal Region Research Articles

Intracranial Off-Midline Mature Teratoma and Pneumosinus Dilatans: A Unique Clinical Report

AbstractTeratomas typically arise as midline lesions in the suprasellar and pineal regions. Pneumosinus dilatans is a rare condition characterized by the expansion of one or more of the paranasal sinuses and thinning of their bony walls with a normal covering mucosa. It usually involves the sphenoid and posterior ethmoid sinuses and has been associated with meningiomas and arachnoid cysts. Off-midline mature teratomas are uncommon, and no reports have described an association with pneumosinus dilatans. We present a rare association between an intracranial off-midline mature teratoma and pneumosinus dilatans in an 18-year-old male patient who presented with a second episode of a left-sided seizure, which has not yet been reported in the literature.

Twelve-year experience of pineal region meningiomas: long-term outcomes of maximal safe resection.

The purpose of this research was to summarize the clinical and prognostic features of pineal region meningiomas, evaluate treatment strategies and long-term prognoses, and improve the management of pineal region meningiomas. We retrospectively studied the data of 37 patients who received surgical resection for pineal region meningiomas at West China Hospital of Sichuan University from 2009 to 2021. Adjuvant gamma knife radiosurgery (GKRS) was conducted according to the extent of resection (EOR). Progression-free survival (PFS), Karnofsky performance status (KPS) scores and recovery of neurological function were adopted to assess a comprehensive management strategy for pineal region meningiomas. The most common symptom was headache associated with intracranial hypertension (75.7%). The occipital transtentorial approach (40.5%) and supracerebellar infratentorial approach (29.7%) were performed in most cases. Gross total resection (GTR) was achieved in 27 patients (73.0%) and the remaining patients were treated with subtotal resection (STR) combined with postoperative GKRS. With a mean follow-up period of 87.0 months, the progression rate was 10.0%, the 5-year PFS rate was 92.9%, and the ΔKPS was 16.3. Multivariate analysis revealed that the STR + GKRS and supracerebellar infratentorial approach were beneficial to the recovery of quality of life of patients. Pineal region meningiomas are sporadic but challenging. It is necessary to select the most appropriate surgical approach, EOR, and hydrocephalus treatment strategy for patients with pineal region meningiomas. Maximal safe resection to protect neurovascular function combined with postoperative GKRS can significantly improve the quality of life of patients.

The Clinical and Molecular Landscape of Rosette-Forming Glioneuronal Tumors.

Rosette-Forming Glioneuronal Tumors (RGNTs) are rare, typically benign central nervous system tumors primarily located in the fourth ventricle and pineal region. Despite being classified as WHO grade I with generally favorable prognoses, RGNTs present complexities in their molecular mechanisms, occasional malignant transformation, and epidemiological characteristics that require further investigation. This study systematically reviews the existing literature to analyze the epidemiological patterns, MRI characteristics, pathological features, diagnostic challenges, and molecular mechanisms associated with RGNTs, aiming to provide a comprehensive theoretical foundation for clinical practice and future research. Through an in-depth review of recent studies, key molecular mechanisms, including mutations in FGFR1, PIK3CA, TERT, and IDH1/2, are highlighted. Additionally, the challenges in accurate diagnosis and the potential for misdiagnosis are discussed, emphasizing the importance of thorough molecular analysis in clinical settings. The literature indicates that RGNTs predominantly affect young adults and adolescents, with a slight female predominance. MRI typically reveals mixed cystic-solid lesions, often accompanied by hydrocephalus. Pathologically, RGNTs are characterized by a combination of neuronal and glial components, with immunohistochemical staining showing positivity for Synaptophysin and GFAP. High frequencies of FGFR1 and PIK3CA mutations underscore the significance of these pathways in RGNT pathogenesis and progression. Although RGNTs generally exhibit low malignancy, the TERT mutations identified in some cases suggest a risk of malignant transformation. This study concludes that while current treatment strategies focus on surgical resection, integrating molecular diagnostics and targeted therapies may be essential for managing recurrent or refractory RGNTs. Future research should explore the impact of various gene mutations on tumor behavior and their correlation with clinical outcomes, to optimize individualized therapeutic strategies and improve patient survival and quality of life.

PDOC-13 ACCESS TO CARE OF CHILD DIAGNOSED WITH PINEOBLASTOMA AND NURSING CHALLENGES TO ADDRESS THE NEEDS ARISE

Abstract Patient Alu (not her real name) was 8 years old girl when diagnosed with Pineoblastoma causing obstructive hydrocephalus. Presented initial at Primary health care with headache,photophobia,lethargic and vomiting for two weeks. CT scan and MRI(Magnetic resonance imaging) were done on arrival to our care and revealed Pineal region tumour. Biopsy were done. Radiotherapy,surgery,chemotherapy including rehabilitation were planned by Multidisciplinary team. This case study focus on the access of care needs of this patient and the family with an emphasis on the Nurses challenges to address those needs. Medical records were retrospectively reviewed for this Patient from June 2021 on the onset of presenting symptoms and initial admission in Redcross War Memorial Children’s Hospital at Cape Town South Africa up to March 2024 last admission in Hospital. Found that were levels of unmet needs some areas of difficulty are not discussed, some areas family feels difficult to explain or mention such as emotional burnout, language, cultural, spritual and schooling etc. In view of all this Nurses may focus on supporting families on discharge and during admission, working better relationship with Primary health care, appropriate referrals for counseling for coping mechanism. Financial constrains is still a major issue throughout.

PADV-01 CHALLENGES OF NEURO-ONCOLOGY CARE IN ZIMBABWE

Abstract Due to the advent of covid 19 coupled with the socio-economic crisis in Zimbabwe, this has resulted in numerous challenges in terms of health care access and resultant diagnostic and treatment barriers for neuro-oncology cases. A retrospective analysis of the burden of neuro-oncology cases which have been seen at a single center in Zimbabwe over the past 5 years and the resultant diagnostic and treatment barriers that have incurred. METHODS An audit of brain tumor cases was done through the analysis of Outpatient department records, Neurosurgery Admission records, and Neurosurgery theatre records at Sally Mugabe Central hospital. RESULTS Total number of cases recorded for the period were 71. Meningiomas 11 (15,5%), Posterior fossa tumors 12 (17%), Gliomas 6 (0.08%), Craniopharyngiomas 6(0.08%), Metastasis 5(0.07%), Pituitary tumors 4(0.06%), choroid plexus papilloma 3(0.04%), Lymphoma 2(0.03%), Pineal region tumor 1(0.01%). 21(30%) tumors were unclassified. Posterior fossa tumors were most common in the pediatric population less than 10years of age. Meningiomas had a female to male ratio of 1.75: 1. 9 (12%) patients were operated on during the same period mostly pediatric tumors. CONCLUSION There is a great deal of institutional as well as patient related factors affecting the diagnosing and treatment of neuro-oncology care in Zimbabwe. This includes lack of operating time, inadequate intensive care facilities, a general unavailability of medical imaging services, delay in pathological reporting, unavailability and costly adjuvant treatment. This is leading to late diagnosis and suboptimal treatment of the various tumors. Although brain tumors are rare, they are not uncommon and there is thus need to improve service delivery to ensure early diagnosis and treatment to have good outcomes.

DNA methylation profiling in a case of papillary tumor of the pineal region.

DNA methylation profiling in a case of papillary tumor of the pineal region.

Letter to the Editor Regarding “Simultaneous Single-Trajectory Endoscopic Biopsy and Third Ventriculostomy in Pineal Region Tumors: A Systematic Review and Single Arm Meta-Analysis”

Letter to the Editor Regarding “Simultaneous Single-Trajectory Endoscopic Biopsy and Third Ventriculostomy in Pineal Region Tumors: A Systematic Review and Single Arm Meta-Analysis”

AB039. Malignant transformation of intracranial germinoma to non-germinomatous germ cell tumors-incidence, treatment strategy and outcome: a case report and literature review.

Intracranial germ cell tumors are rare neoplasms seen mainly in children and adolescents. Compared to non-germinomatous germ cell tumors (NGGCTs), germinomas typically respond well to chemo-radiotherapy and portend a better prognosis. We report here a patient with intracranial germinoma which showed complete remission following chemo-radiotherapy but re-presented with malignant transformation to NGGCT thirteen years later. We then performed a literature review to understand the treatment strategy and outcome of this rare phenomenon. Review of the patient's case note, followed by a literature review of the topic. A 13-year-old male presented with 6 weeks of polyuria and polydipsia. Imaging showed bifocal (suprasellar and pineal) lesions. Histology from an open biopsy diagnosed a germinoma. He underwent extended whole ventricular irradiation with local boost and chemotherapy, which led to complete resolution on imaging. Thirteen years later, he re-presented with headaches and diabetes insipidus. Imaging showed a new right frontal lesion with elevated serum beta-human chorionic gonadotropin (β-hCG) and alpha-fetoprotein (AFP). The tumor was excised with histology confirming a mixed tumor comprising germinoma, yolk sac, and choriocarcinoma. A literature review returned eight case reports of malignant transformation of intracranial germinoma to NGGCTs, covering eight patients (seven males, and one female; aged 5-23 years old). Four had the primary tumor located in the pineal region, three in the suprasellar region, and one at both sites. Recurrence with malignant transformation occurred at a median of 24.5 months after initial diagnosis (range, 5 months to 14 years). Five patients had recurrences intra-abdominally, all of whom had a ventriculoperitoneal shunt (VPS) inserted during the treatment of the initial tumor. Of the remaining three intracranial recurrences, two were at the same site while one at a distant site. The most common histology was yolk sac tumor (five patients), followed by two each of immature teratoma, choriocarcinoma, and embryonal carcinoma (some were mixed germ cell tumors). Of those with intra-abdominal recurrence, four died within 2 months of diagnosis. Those with intracranial recurrences survived longer, with a median survival of 15 months, and one longer than 27 months. Malignant transformation to NGGCTs is rare. Relapse can occur intracranially, or in cases where VPS was present, intraabdominally. Outcomes following transformation were poor despite aggressive treatment, with those intra-abdominal recurrences faring much worse.

SNO-EANO-EURACAN consensus on management of pineal parenchymal tumors.

Pineal parenchymal tumors are rare neoplasms for which evidence-based treatment recommendations are lacking. These tumors vary in biology, clinical characteristics, and prognosis, requiring treatment that ranges from surgical resection alone to intensive multimodal antineoplastic therapy. Recently, international collaborative studies have shed light on the genomic landscape of these tumors, leading to refinement in molecular-based disease classification in the 5th edition of the World Health Organization (WHO) classification of tumors of the central nervous system. In this review, we summarize the literature on diagnostic and therapeutic approaches, and suggest pragmatic recommendations for the clinical management of patients presenting with intrinsic pineal region masses including parenchymal tumors (pineocytoma, pineal parenchymal tumor of intermediate differentiation, and pineoblastoma), pineal cyst, and papillary tumors of the pineal region.

Papillary tumor of the pineal region: analysis of DNA methylation profiles and clinical outcomes in 76 cases

Papillary tumor of the pineal region (PTPR) is an uncommon tumor of the pineal region with distinctive histopathologic and molecular characteristics. Experience is limited with respect to its molecular heterogeneity and clinical characteristics. Here, we describe 39 new cases and combine these with 37 previously published cases for a cohort of 76 PTPR’s, all confirmed by methylation profiling. As previously reported, two main methylation groups were identified (PTPR-A and PTPR-B). In our analysis we extended the subtyping into three subtypes: PTPR-A, PTPR-B1 and PTPR-B2 supported by DNA methylation profile and genomic copy number variations. Frequent loss of chromosome 3 or 14 was found in PTPR-B1 tumors but not in PTPR-B2. Examination of clinical outcome showed that nearly half (14/30, 47%) of examined patients experienced tumor progression with significant difference among the subtypes (p value = 0.046). Our analysis extends the understanding of this uncommon but distinct neuroepithelial tumor by describing its molecular heterogeneity and clinical outcomes, including its tendency towards tumor recurrence.

A rare case of pineoblastoma – Case report

Pineoblastoma is a very rare and aggressive malignant tumor of pineal gland. We report a case of 12-year-old male patient presented to S.V.P. tertiary care hospital with complain of progressive loss of vision in both eyes with nausea and headache. On examination bilateral upward gaze palsy was present. The magnetic resonance imaging report revealed possibility of pineal Germinoma. The patient underwent ventriculoperitoneal shunt insertion surgery for obstructive hydrocephalus and suboccipital craniectomy for tumor resection. The Histopathological examination report stated the tumor as Pineoblastoma, Grade IV. Immunohistochemistry studies were positive for synaptophysin and Integrase interactor-1 (INI-1) markers with high ki-67 labelling index and negative for CD-117 and Glial fibrillary acid protein (GFAP) markers. Microscopic and Immuno histochemisty findings are important to differentiate pineoblastoma from other pineal region tumors.

Pituitary germinoma after resection of a mature third ventricular teratoma: illustrative case.

Metachronous intracranial germ cell tumors (iGCTs)-unrelated, histologically different iGCTs occurring at different time points-occurring within the same patient remain a rarity. Herein, the authors report such a case and discuss the literature and potential pathophysiological mechanisms leading to this phenomenon. A 9-year-old boy presented with new-onset impaired balance, headaches, nausea, visual disturbances, and left facial paresis. Magnetic resonance imaging (MRI) scans revealed a suspected pineal region teratoma originating from the pineal gland with consecutive obstructive hydrocephalus. A mature teratoma was diagnosed and resected. Postoperative recovery was good, and the patient could return to his normal daily activities. However, a new, slowly progressive lesion in the sellar region with an enlarged infundibular stalk was detected on follow-up MRI 3.5 years after initial pineal region teratoma resection. Biopsy revealed a newly developed pure germinoma. The patient was treated with radiotherapy plus chemotherapy and remained relapse free at the last follow-up. Sixteen other cases have reported a surgically resected primary mature teratoma, wherein patients developed metachronous germinomas during follow-up. Different theories try to elaborate this phenomenon, yet none can completely account for it. Although rare, metachronous iGCT is a phenomenon neurosurgeons should be aware of. In patients treated for iGCT, close long-term clinical, imaging, and laboratory follow-up is recommended. https://thejns.org/doi/10.3171/CASE2443.

The Prognostic Value of Immunonutritional Indexes in Pineal Region Tumor

The Prognostic Value of Immunonutritional Indexes in Pineal Region Tumor

Microsurgical Management of Pineal Region Tumors

BackgroundPineal tumours are rare and the pineal region is a challenging surgical location for neurosurgeons. The present study aimed to investigate the effects of microsurgical management in patients with pineal region tumours and explore probable factors associated with preoperative hydrocephalus, postoperative hydrocephalus remission, and prolonged length of hospital stay (LoS). MethodsA retrospective study of patients with pineal region tumours, who underwent microsurgical management at the First Affiliated Hospital of Soochow University(Jiangsu, China) between 1 January 2010 and 31 October, 2022, was conducted. ResultsData from 36 patients were included in this study. The top five common symptoms included headache (58%), dizziness (44%), double vision (22%), vomiting (19%), and nausea (14%). Patients with a lower Karnofsky Performance Score (KPS), younger age, and larger maximum tumour diameters were more vulnerable to preoperative hydrocephalus. Preoperative hydrocephalus also led to a prolonged postoperative LoS (P=0.021). Patients with a lower KPS score (P=0.020) or larger maximum tumour diameters (P=0.045) were more likely to achieve postoperative remission of hydrocephalus. Most postoperative complications led to increased hospital LoS. ConclusionMicrosurgical resection of pineal tumours yielded favourable long-term outcomes. Patients with a lower KPS score, younger age, and larger maximum tumour diameters were more vulnerable to preoperative hydrocephalus and prolonged LoS. Patients with a lower KPS score or larger tumour diameter were likely to achieve significant remission of hydrocephalus after tumour resection.

The occipital interhemispheric transtentorial approach: historical perspective and evolution over time.

Access to the pineal region has always represented a fascinating challenge to the neurosurgeons. Almost equally thrilling is the historical evolution from the hard beginnings with extremely high mortality rates to the current surgical outcomes, with excellent resection rates without long-term morbidity for most of the patients. The purpose of this paper is to provide an overview of the historical evolution of the occipital interhemispheric transtentorial (OITT) approach and its role in the development of access to the pineal region. Review of the literature highlights the occipital transtentorial approach from the historical context prior its description and the beginning to the current modifications and new recent insights. The occipital transtentorial approach described initially by Poppen in 1966 has played a key role in the progress and success accessing the pineal area. This historical review aims to highlight the extraordinary effort of those neurosurgeons who guided and led the development of one of the most important approaches to the pineal region.

Spontaneous Intraventricular Tension Pneumocephalus

Here, we report a very rare case of spontaneous intraventricular tension pneumocephalus. This case concerns a 40-year-old patient with medical history of a tumor of the pineal region and a secondary hydrocephalus treated by multiple ventriculoperitoneal shunts. He presented in the emergency room because of unusual headaches, nausea, and visual loss. In addition, he reported slight rhinorrhea in the past few weeks. The initial brain computed tomography scan revealed a spontaneous intraventricular tension pneumocephalus. There was no history of recent head trauma and no sign of disconnection of the shunt system. A complementary radiologic assessment including a thin-slice bone computed tomography scan and a radioisotope cisternography revealed an osseous defect and an isotope leakage at the junction between the tegmen tympani and the squamous part of the left temporal bone. A middle cranial fossa surgery was performed to repair the osteo-meningeal breach.

Clinical effects of neuroendoscopic infratentorial supracerebellar approach surgical technique for resecting pineal tumors: a retrospective study

ObjectivePineal tumors are relatively rare central nervous system lesions with a predilection for the pediatric population. This article aims to explore the clinical effects of neuroendoscopic infratentorial supracerebellar approach for resecting tumors in the pineal area. MethodsThis is a retrospective study that included patients who underwent neuroendoscopic infratentorial supracerebellar approach to resect nine tumors in the pineal area at the Department of Neurosurgery of the Second Hospital of Lanzhou University from December 2017 to October 2023. ResultsThe results of postoperative MRI revealed that all tumors were resected. Five patients received postoperative radiotherapy, three patients received radiotherapy along with chemotherapy, and one patient received neither radiotherapy nor chemotherapy. The pathological results showed that four patients were diagnosed with germinoma, two patients with teratoma, two patients with mixed germ cell tumors, and one patient with central neurocytoma. After surgery, one patient developed psychiatric symptoms, two patients developed binocular upward vision and diplopia, and one patient developed unstable walking and diplopia. With a follow-up of 1.7–4.8 years, all nine patients lived normally. Furthermore, none of them had tumor recurrence or death. ConclusionThe simple neuroendoscopic infratentorial supracerebellar approach has some safety and efficacy. It is suitable for tumors in the pineal region where the disease is mainly located below the Galen vein complex.

ETMR-03. REPORT OF 9 CASES OF EMBRYONAL TUMORS OF THE CENTRAL NERVOUS SYSTEM WITH MULTILAYERED ROSETTES (ETMR)

Abstract OBJECTIVE Embryonal Tumors of the Central Nervous System with Multilayered Rosettes (ETMR) are rare, and there’s no standard treatment protocol. This article reports on the current treatment status and prognosis of ETMR in our hospital. METHODS We retrospectively collected patients with ETMR who received treatment in the Oncology Department of Guangdong sanjiu Brain Hospital from January 2017 to January 2021, analyzed their clinical data. RESULTS Among 31 cases of central nervous system embryonic tumors (excluding medulloblastoma and ATRT), 9 (29.03%) were ETMR. The median age at diagnosis was 3 years old (1-15), and the male to female ratio was 4:5. Among them, 5 cases were accompanied by C19MC gene variation. Tumor location:6 was in supratentorial, 1 in the pineal region, 1 in the pons, 1 in the cerebellopontine. Eight patients underwent surgical resection at the first diagnosis, with 6 cases of total resection, 1 case of partial resection, and 1 case of biopsy. One case was misdiagnosed as a germinoma in the pineal region at the initial. After recurrence, surgical resection was performed to confirm the pathology as ETMR. One case was misdiagnosed as glioblastoma during the first surgery. The initial treatment for 4 patients after surgery was radiotherapy combined with concurrent chemotherapy. Recurrence patterns: 2 cases were intracranial dissemination, 2 cases were local recurrence, 1 was spinal cord dissemination. One patient did not receive treatment after surgery and tumor recurrence 3 months later. Survival analysis: The median survival time was 20 months. The 1-year progression-free survival rate (PFS) and overall survival rate (OS) were 22% and 61%, and the 2-year overall survival rate (OS) was 31%. CONCLUSION ETMR has a low incidence and poor prognosis. It is easy to misdiagnose and NGS is necessary to assist diagnosis when needed. More effective treatment methods need to be explored.

RADT-08. BASAL GANGLIA TOXICITY FOLLOWING PROTON THERAPY OF THE PINEAL REGION WITH BILATERAL ACTION-INDUCED MYOCLONUS

Abstract INTRODUCTION We sought to evaluate the toxicity profile for children receiving 54Gy to the pineal region with a significant dose delivered to posterior thalamus and basal ganglia. METHODS We performed a retrospective review of patients treated at a single institution with intensity modulated proton therapy (IMPT) receiving 54Gy to the pineal region. Multiple beam angles were utilized for planning. Patient characteristics and dosimetric data were correlated with late-toxicity and imaging. RESULTS From 2021-2023, six children were treated, and at a median follow-up of 13 months [9-26], all patients remain disease-free. Three patients had non-germinomatous germ cell tumors, 1 low-grade glioma, 1 atypical teratoid rhabdoid tumor (ATRT), and 1 embryonal tumor with multilayered rosettes. Age range was 2.4 to 16.8 years. All patients received chemotherapy prior to IMPT. Neuropsychological evaluation indicated range of intellectual functioning (mean = 98.3 [83-118]). One patient, a 4-year-old female with ATRT, developed T2 changes within the globus pallidus and thalamus on 3 month follow-up. At 5 months, she developed bilateral action-induced myoclonus with corresponding enhancing lesions in the globus pallidus bilaterally. Neuropsychological evaluation at 5 months post-treatment indicated memory impairments. She was treated with dexamethasone without improvement. Between 912 months post-treatment, tremors improved with physical therapy and imaging showed near complete resolution of enhancement within globus pallidus. CONCLUSION One of six children developed grade 3 toxicity manifesting as bilateral action-induced myoclonus with imaging changes in the globus pallidus bilaterally following IMPT and exhibited related neuropsychological deficits. While brainstem toxicity from IMPT is well documented, globus pallidus toxicity is not well characterized. Given the contiguous neuronal tracts with the brainstem, it is logical to apply similar treatment planning techniques, i.e. minimizing hotspots within the globus pallidus and thalamic “core” structures, and being cognizant of LET/RBE effects for treatment involving the pineal region in young children.

NFS-26.RB1-ALTERED SUBTYPE (GROUP A) PINEOBLASTOMA AS A PART OF «TRILATERAL RETINOBLASTOMA» PHENOMENON: NEURO-ONCOLOGICAL AND OPHTHALMIC-ONCOLOGICAL VIEW

Abstract BACKGROUND RB1-altered subtype (Group A) pineoblastoma as a part of «Trilateral retinoblastoma» is an extremely rare phenomenon. But over the past 10 years, the incidence of this disease has been increasing. Optimal therapeutic strategy is unknown. The aim of this study was to estimate the incidence of this phenomenon among patients with retinoblastoma, age at onset of retinoblastoma/RB1-altered pineoblastoma and genetic, gender, age predisposition. DESIGN/METHODS From 2019 to 2023 years 256 pediatric patients with de novo retinoblastoma were treated in the FSBI «N.N.Blokhin National Medical Research Center of Oncology». RB1-altered pineoblastoma was revealed in 9 cases (3,5% of all patients, 9,5% among patients with bilateral retinoblastoma, 6,6% for patient with the onset of retinoblastoma under the age of 12 months). Diagnosis was determined according to familial history, retinal camera and MRI data, histology, immunohistochemistry and Illumina Infinium methylation EPIC Bead Chip method (FFPE tumor samples), NGS and MLPA methods for detection of RB1 germline mutation status (blood samples). Therapeutic options for patients with RB1-altered subtype pineoblastoma were presented as like–COGARET0321, like–HIT–MED2017/2020, selective intra-arterial chemotherapy, intravitreal chemotherapy, transpupillary thermotherapy, brachytherapy. RESULTS Gender ratio was 1:2 (3 male and 6 female). In all cases, intraocular retinoblastoma was bilateral. Family history was revealed in two cases. Age at onset of retinoblastoma: less than 6 months in 8 cases, all cases less 2 years, median 3±1,9 months [1,5-20,5]. Median age at onset of RB1-altered subtype pineoblastoma was 20,5±7,3 months [1,5-57,5]. A synchronous variant of the disease was identified in 5 cases, metachronous in 4. Pineoblastoma of pineal region was detected in 7 cases, suprasellar region in 2. Six out of 9 patients are alive, median follow-up 16,5±5,1 months [1,5-43,0]. CONCLUSION It is necessary to expand both regional and international interdisciplinary cooperation to develop an optimal algorithm for the diagnosis and treatment of this disease.