Partial Tumor Resection Research Articles

Pediatric Extra-Renal Nephroblastoma (Wilms' Tumor): A Systematic Case-Based Review.

Wilms Tumor (WT) is one of the most common renal tumors in the pediatric population. Occasionally, WT can primarily develop outside the kidneys (Extra-Renal Wilms Tumor, ERWT). Most pediatric ERWTs develop in the abdominal cavity and pelvis, whereas the occurrence of this tumor in other extra-renal sites represents a minor part of ERWT cases. In addition to describing a case of spinal ERWT (associated with spinal dysraphism) in a 4-year boy (to add a further clinical experience on this very rare pediatric tumor), we performed a case-based systematic literature review on pediatric ERWT. We retrieved 72 papers providing enough information on the diagnosis, treatment, and outcomes of 98 ERWT pediatric patients. Our research highlighted that a multimodal approach involving both chemotherapy and radiotherapy, after partial or complete tumor resection in most cases, was typically used, but there is no standardized therapeutic approach for this pediatric malignancy. However, this tumor may be potentially treated with a better success rate if the diagnostic confirmation is not delayed, the mass can be totally resected, and an appropriate and, possibly, tailored multimodal treatment can be promptly established. In this regard, an international agreement on a unique staging system for (pediatric) ERWT is definitely needed, as well as the development of international research, which may be able to gather several children diagnosed with ERWT and, possibly, lead to clinical trials which should also include developing countries.

Clinical analysis of 7 cases of pregnancy with non-Hodgkin lymphoma

Objective: To investigate the clinicopathological features, diagnosis, treatment and outcomes of pregnancy with non-Hodgkin lymphoma (NHL). Methods: The clinicopathological data of 7 patients of pregnancy with NHL admitted to Nanjing Drum Tower Hospital from January 2010 to May 2022 were reviewed. General information, diagnosis, treatment and maternal and child outcomes were retrospectively analyzed. Results: (1) The median age of the 7 patients was 28 years old (range: 26-33 years); 3 cases complained of abdominal pain (2 cases of huge pelvic or abdominal mass with multiple metastases), 2 cases of cough (1 case with superior vena cava syndrome), 1 case of facial swelling and pain, and 1 case of poor appetite. The median time from the onset of symptoms to initial visit was 30 days (range: 15-188 days). (2) Only 3 cases were diagnosed during pregnancy through biopsy, and the biopsy sites including right nasal vestibular mass, left supraclavicular lymph node and lung respectively. One case was suspected to be splenic marginal zone lymphoma through bone marrow puncture during pregnancy, and confirmed by pathological results observed in splenectomy specimens after termination of pregnancy. Three cases were diagnosed as NHL by pathological results of focus biopsy or partial tumor resection during cesarean section. Pathological types: 5 cases of diffuse large B cell lymphoma, one splenic marginal zone lymphoma and one nasal cavity natural killer (NK)/T cell lymphoma. Stages: 1 case of stage Ⅱ, 6 cases of stage Ⅳ. Pathological examination of placentas was performed for 4 patients after delivery, included one case with tumor metastasis to the placenta. (3) Among the 7 patients, 1 case was induced in the second trimester; 5 cases were terminated by cesarean section in the third trimester, all of which were premature; one case of full-term was delivered with forceps. All 6 neonates survived healthy. Treatments: 5 cases received chemotherapy after termination of pregnancy (included 1 case received autologous hematopoietic stem cell retransfusion therapy after chemotherapy), and 1 case received chemotherapy combined nasopharyngeal radiotherapy, and six cases survived without recurrence (follow-up until October 2022). The other case was a patient with hepatitis B virus infection and congenital heart disease who died of multiple organ failure 18 days after cesarean section. Conclusions: It is difficult to diagnose, necessary to pay attention to the complaints of pregnant women, and to actively carry out related examinations during pregnancy. When the lesion involves multiple organs, the possibility of lymphoma should be considered. Pregnancy with NHL is sensitive to chemotherapy. Even for advanced patients, good outcome could still be obtained after standardized treatment.

Ectopic invasive ACTH-secreting pituitary adenoma mimicking chordoma: a case report and literature review

BackgroundEctopic pituitary adenoma (EPA) is defined as a special type of pituitary adenoma that originates outside of the sellar region, is extra- or intra-cranially located, and without connection to normal pituitary tissue. EPA is extremely rare, with most cases presented as case reports or small case series. Due to nonspecific symptoms and laboratory indicators, the preoperative diagnosis, treatment and management for EPA remain challenging.Case presentationHere, we report the imaging phenotype and pathological findings of a case of invasive EPA in a 47-year-old woman. A preoperative non-contrast CT scan revealed a 5.8 × 3.6 × 3.7 cm soft tissue mass located in the sphenoid sinus and clivus. MRI showed an ill-defined solid mass with heterogeneous signals on T1-weighted and T2-weighted images. The mass displayed infiltrative growth pattern, destroying bone of the skull base, invading adjacent muscles and encasing vessels. The patient underwent partial tumor resection via transsphenoidal endoscopic surgery. Pathological examination led to diagnosis of ectopic ACTH-secreting pituitary adenoma. Post-surgery, the patient received external beam radiotherapy.ConclusionEPA with invasive growth pattern has rarely been reported. The imaging phenotype displays its relationship to the pituitary tissue and surrounding structures. Immunohistochemical examination acts as a crucial role in differentiating EPA from other skull base tumors. This case report adds to the literature on EPA by summarizing its characteristics alongside a review of the literature.

Surgical treatment of cervical neurovascular bundle tumors

To determine the main principles of a patient-oriented individual approach to diagnosis and surgical treatment of cervical neurovascular bundle tumors considering the capabilities of neurosurgical hospital. There were 92 patients with cervical soft tissue tumors affecting neurovascular bundle. Age of patients ranged from 9 to 81 years (mean 47). There were 65.1% women and 34.9% men. We found chemodectoma (47.4%), neurofibroma (15.8%), neurinoma (13.2%), papillary thyroid cancer (5.3%), salivary gland heterotopia (5.3%), salivary gland adenocarcinoma (5.3%), Hodgkin lymphoma (2.6%), hemangioendothelioma (2.6%) and cavernous lymphangioma (2.6%). Diagnostic algorithm included neurological examinations, Doppler ultrasound of supra-aortic arteries, transcranial ultrasound of cerebral vessels, MRI of cervical soft tissues, CT-AG, MR-AG, CT-perfusion, direct selective angiography. A total of 94 surgical interventions were performed. All surgeries were performed using surgical optics and neurophysiological monitoring of cranial nerves IX, X, XII. We chose resection technique depending on localization, histological features and blood supply of tumor. En-bloc resection was performed in 46 cases, removal of fragments - in 23 cases, intracapsular resection of tumor followed by resection of the capsule - in 26 cases. Total and subtotal resection was performed in 68 (72%) and 23 (24%) cases, respectively. Three (4%) patients underwent partial resection of infiltrative tumors for carotid artery decompression and histological analysis. In 76% of cases, baseline symptoms of disease regressed after surgery. Persistent moderate bulbar disorders were observed in 16 patients (17%). Ischemic complications with additional surgical interventions were observed in 2 cases. Patients with cervical soft tissue tumors require individual approach regarding choosing the optimal surgical treatment including possible preoperative embolization of tumor, en-bloc or intracapsular resection and carotid artery repair.

Composite hemangioendothelioma in the cervical spine with kaposiform hemangioendothelioma features in an elderly patient: a case report.

Composite hemangioendothelioma (CHE) is an intermediate group of tumors with features between hemangioma and angiosarcoma both histologically and biologically. CHE is predominant in young and middle-aged adults, but very infrequently affects the spine. We describe the case of primary CHE in the cervical spine exhibiting kaposiform hemangioendothelioma (KHE)-like components that was associated with cervical myelopathy with vertebral body destruction in an elderly woman. We retrospectively reviewed the case of a primary cervical spinal tumor, diagnosed as CHE with KHE-like components in pathological findings, associated with cervical myelopathy and cervical vertebral body destruction. An 80-year-old woman presented with progressive cervical myelopathy caused by a cervical spine tumor. Preoperative cervical MRI revealed a neoplastic lesion invading the cervical spine that strongly compressed the spinal cord, causing right upper-limb paralysis. We performed partial tumor resection along with posterior decompression and fixation. Postoperatively, pathological findings showed that the tumor was CHE with KHE-like features. Following radiotherapy, no recurrences have been observed in 21 months. This is the first report of CHE with features of KHE in the spine of an elderly patient. Posterior decompression and fusion of the cervical spine and subsequent radiotherapy resulted in a good outcome.

WITHDRAWN: Thoracic spinal metastasis as recurrence of borderline Brenner tumor without local recurrence: A case report

Brenner tumor is a rare epithelial ovarian neoplasm that accounts for 2-3% of all ovarian neoplasms. Herein, we report the first case of thoracic spinal metastasis of recurrent Brenner tumor without local recurrence.Case Description.A 70-year-old female presented with a feeling of abdominal distension. Computed tomography revealed cystic lesions in her bilateral ovaries. Blood examination revealed high CA-125 [74.9 U/ml]. We excised bilateral ovaries, uterus, and omentum. Borderline Brenner tumor was diagnosed [Ki-67 labeling index: 10%]. Follow-up abdominal echo and CA-125 examination revealed no local recurrence. 26months later she developed paraplegia. Magnetic resonance imaging revealed tumor in the 5th-9th thoracic vertebra and compression of spinal cord at the 6th thoracic vertebra level. Her paraplegia was progressive. We performed semi-urgent partial resection of tumor and release of spinal cord compression. Spinal metastasis from Brenner tumor was diagnosed [Ki-67 labeling index: 50-60%]. She received adjuvant radiation of 30Gy in 10 fractions to the 4th-10th thoracic vertebra. After radiation and rehabilitation, she was discharged home on foot. She received adjuvant radiation and chemotherapy but died 11months after spinal surgery. An autopsy has not been performed on her, and the cause of death is unknown. We report the first case of thoracic metastasis of recurrent Brenner tumor without local recurrence.

RADT-18. PARTICLE BEAM RADIATION THERAPY PLUS TUMOR TREATING FIELDS IN THE TREATMENT OF NEWLY DIAGNOSED WHO GRADE 4 GLIOMAS: AN EARLY RESULT OF PHASE 2 SINGLE-ARM TRIAL

Abstract OBJECTIVES Tumor Treating Fields (TTFields) is increasingly provided to patients with WHO grade 4 gliomas following radiotherapy. Particle beam radiotherapy (PBRT) represents a novel promising radiation approach with improved therapeutic ratio. This study was aimed to report an early result of TTFields in combination with PBRT for treating WHO grade 4 gliomas based on a prospective phase 2 trial from Shanghai Proton and Heavy Ion Center. METHODS This phase 2 trial was designed as a single-arm study to test the outcome of PBRT plus TTFields. Six patients with newly diagnosed WHO grade 4 gliomas receiving surgery, PBRT, temozolomide, and TTFields, from August 2021 to May 2022, were enrolled and analyzed. Tumor histologies based on the WHO CNS5 classification system were IDH-wild glioblastoma (n=4), IDH-mutant astrocytoma (n=1), and H3G34-mutant diffuse hemispheric glioma (n=1). For PBRT, all patients were irradiated with a baseline standard dose of 60GyE/30 utilizing proton. Carbon-ion boost of 15GyE/3 for tumor residual was performed for one patient prior to proton radiation. TTFields treatment was recommended to start soon after PBRT. RESULTS With a median follow-up of 5.5 months, all patients were alive at the time of this analysis; one patient with left thalamic glioblastoma had tumor progression of distant failure outside the radiation field at 4 months after partial tumor resection. The median interval between PBRT and TTFields was 2 days (range 1-22 days). No patient had dermatitis during PBRT. No severe TTField-related skin toxicity (Grade 3 or above) was observed. Five patients (83.3%, 5/6) developed mild skin toxicities (Grade 1) during TTField treatments. CONCLUSIONS Starting TTField early following PBRT is feasible for treating WHO grade 4 gliomas, the overall side effects are mild. Prospective studies with further follow-up are warranted to identify the outcomes of PBRT in combination with TTField in terms of survival and side effects.

NCOG-34. METASTATIC PAPILLARY TUMOR OF THE PINEAL REGION: ROLE OF STEREOTACTIC IRRADIATION AND ADJUVANT TEMOZOLOMIDE

Abstract INTRODUCTION Papillary tumor of the pineal region (PTPR) is a very rare tumor comprising < 1% of all intracranial neoplasms. It was first included as a distinct tumor entity in the 2007 World Health Organization classification. Due to its rarity, only anecdotal data is currently available to guide management. However, with these tumors being recognized more frequently, further molecular observations and therapeutic experiences are necessary. Herein, we report an unusual case of PTPR with a previously treated leptomeningeal metastasis (LM) whom we successfully managed with Gamma Knife Radiosurgery (GKRS) and adjuvant temozolomide at time of distant brain parenchymal tumor progression. CASE REPORT: A 41-year-old man presented with headache and diplopia in 2015, MRI brain revealed a large cystic enhancing lesion in the pineal region protruding into the third ventricle and causing obstructive hydrocephalus. He had a ventriculoperitoneal shunt placed and underwent partial tumor resection. He then transferred care to our institution and a gross total resection was achieved. Pathology confirmed PTPR. A repeat MRI 15 months later showed evidence of radiographic LM in the cerebellum and he underwent craniospinal proton radiation therapy. He remained stable until January 2020 when a new left temporal heterogeneously enhancing multicystic lesion was identified on surveillance MRI. He underwent subtotal resection of this intra-axial lesion. Histological features of the tumor were similar to his initial tumor. Somatic tumor mutation testing using FoundationOne®CDx did not reveal targetable molecular alterations. Following surgery, he received GKRS and six cycles of adjuvant temozolomide. At 21-month follow up, patient continues to do well. CONCLUSION To our knowledge, this is the first case to describe a metastatic brain intraparenchymal PTPR. We also describe a first case of successful therapeutic strategy for a distant recurrence of PTPR.

PMON129 Pituitary Adenoma Co-Secreting GH, Prolactin and ACTH

Abstract Background Pituitary adenomas co-secreting GH, Prolactin and ACTH are rare. Clinical Case A 38-year-old male with a past medical history of depression and type 2 diabetes mellitus was seen in the endocrinology clinic for an evaluation of acromegaly. In the preceding four years, the patient had experienced an enlargement of his hands and feet, hyperhidrosis, development of an underbite, snoring, daytime somnolence, erectile dysfunction, headaches, and blurry vision. His visual fields were normal. Physical exam: BP 137/85, BMI 39 kg/m2. He had coarse facial features, acral changes of his hands and feet, and wide spacing of his teeth. There was a notable absence of facial plethora, abdominal obesity with thin extremities, proximal muscle weakness, purple abdominal striae, and galactorrhea. Initial laboratory results: GH 16.9 ng/mL (0.05-3 ng/ml), IGF-1 1,109 ng/mL (82-242 ng/mL), Prolactin (PRL) 96.9 ng/ml (5-20 ng/ml), testosterone 34 ng/dL (200-1000 ng/dL), LH 2.3 miu/ml (1.4–12 miu/ml), FSH 4.8 miu/ml (1.5-12 miu /ml), ACTH 197.6 pg/mL (7.2- 63.3 pg/mL), 0800 cortisol 16.2 ug/dL (5-29 ug/dL), 24-hour urine free cortisol 56.84 mcg (3.5-40 mcg), HS salivary cortisol 0.242 (<0.359 ug/dL), overnight 1 mg Dexamethasone suppression test cortisol 6.9 mcg/dL (< 1.8 mcg/dL), normal Free T4 and TSH, and A1c 9.7%. A pituitary MRI revealed a 3.9 cm lobulated, intra and supra-sellar mass with mass effect on the optic chiasm and 75% encasement of the left supra-clinoid internal carotid artery. Neurosurgery performed an endoscopic, endonasal partial resection of the pituitary tumor. The histology was diagnostic of an adenoma, and immunohistochemical staining was positive for GH and PRL, but not ACTH. Post-op lab results: GH 1.07 ng/ml, IGF-1 353 ng/ml, PRL 9.6 ng/ml, testosterone 41 ng/dL, ACTH 68.2 pg/ml, cortisol 4.5 ug/dL, 24-hr urine free cortisol 8 mcg, HbA1c 5.7%. Discussion The intent of this case presentation is to report the rare finding of a pituitary tumor co-secreting GH, ACTH, and Prolactin. The diagnosis of acromegaly was confirmed by significant elevations of GH and IGF-1. The diagnosis of subclinical Cushing's disease was confirmed by elevated ACTH level, 24-hr urine free cortisol, and failed dexamethasone suppression test. The elevated PRL may have been due to tumor PRL secretion or stalk effect. The reduction of these parameters after surgery corroborated the presence of pleurihormonal secretion. This patient's findings support screening for co-secretion of multiple pituitary hormones in patients with acromegaly. Reference Pleurihormonal ACTH-GH Adenoma: Case Report and Systematic Literature Review World Neurosurg 2018 114: 158-164 Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

ODP334 Must we Wait for the Development of Metastasis to be Able to Distinguish Dopamine Resistant Aggressive Prolactinoma from Prolactin Producing Pituitary Carcinomas? Case Report and Literature Review

Abstract Background Prolactinomas, the most prevalent type of neuroendocrine disease, account for approximately 40% of all pituitary adenomas. In the vast majority of cases, these are benign tumors with excellent clinical response to medical therapy with dopamine agonist agents such as bromocriptine and cabergoline. Rarely medical treatment fails to normalize prolactin levels and/or decrease the size of the tumor, posing a challenge to the clinician. Case presentation 40-year-old man who underwent resection of a 7.4 cm pituitary prolactinoma via frontal craniotomy followed by radiotherapy. He developed panhypopituitarism and was placed on chronic hormonal replacement therapy plus high dose cabergoline. Two years later he presented with recurrence of his disease and was diagnosed with an unresectable 4.9 cm pituitary adenoma showing cortical breakthrough of the sellar floor, invasion into the optic chiasm, erosion of the posterior sphenoid walls, extension into the sphenoid sinuses and posteriorly into the pons, midbrain and third ventricle, as well as complete encasement of the distal, superior cerebellar and posterior cerebral arteries. The patient underwent endoscopic trans sphenoidal partial tumor resection and CSF leak repair. Pathology revealed a pituitary prolactinoma with Ki-67 of up to 8% and positive p53 immunostaining. His cabergoline dose was adjusted to 0.5 mg PO daily and he was also started on Sandostatin 20 mg IM monthly as well as appropriate hormonal replacement of the other deficient pituitary hormonal axes. Follow up imaging revealed stability of the lesion. The patient continues to do clinically well and actively follows with endocrinology and neurosurgery with serial brain MRIs and laboratory workup. Discussion The histological and biochemical characteristics of aggressive prolactin secreting tumors has been reported to be of minimal utility in distinguishing benign from malignant lesions. Tumors can only be diagnosed as carcinomas after they have metastasized and they can only be classified as aggressive after treatment failure or unusual growth, which creates a disadvantage for the prognosis and management of these patients. Clinicians are in need of better guidelines that can help them identify the rare cases that might warrant more aggressive clinical interventions beyond dopamine agonists such as surgery, radiation, temozolomide, somatostatin receptor agonists and others, with the hope of delaying or preventing the development of metastasis. Case reports of atypical presentations of aggressive prolactinomas such as the one here presented, can help broaden our understanding of the predictable signs of poor prognosis. Presentation: No date and time listed

Pigmented villonodular synovitis of the cervical spine: a case report

Pigmented villonodular synovitis (PVNS) is a proliferative disease of the synovial membranes. Large joints are most commonly affected, while PVNS rarely affects the spine. We present a 70-year-old female patient with PVNS in the cervical spine who experienced both radiculopathy and myelopathy. An osteolytic lesion was observed over the sixth and seventh intervertebral foramina on the right cervical vertebrae on the radiological examination, and surgery was performed to remove the tumor and stabilize the cervical spine. Partial tumor resection was performed to preserve the right seventh cervical root. She was pathologically diagnosed with PVNS. After surgery, the patient's symptoms improved, and no recurrence was observed at outpatient follow-up 1 year later. In consideration of the recurrence rate, complete resection of the tumor is considered the standard treatment, but additional research is needed because there is no standard for adjuvant treatment in cases of incomplete resection or for methods to reduce the recurrence rate.

Adaptive Hybrid Surgery Experiences in Benign Skull Base Tumors.

Background: The treatment of benign skull base tumors remains challenging. These tumors are often located in close relationship to critical structures. Therefore, radical resection of these tumors can be associated with high morbidity. Multimodal treatment concepts, including controlled partial tumor resection followed by radiosurgery, should be considered. Methods: Adaptive hybrid surgery analysis (AHSA) is an intraoperative tool that has been introduced for the automatic assessment of tumor properties, and virtual real-time radiosurgical treatment simulation and continuous feasibility analysis of adjuvant radiosurgery. The AHSA method (Brainlab®, Munich, Germany) was applied to five patients who underwent partial resection of a benign skull base tumor. Tumor volumetry was obtained on pre- and postoperative MR scans. Organs at risk were, preoperative, automatically delineated with atlas mapping software (Elements® Segmentation Cranial), and adaptations were made if necessary. Results: Five patients with benign skull base lesions underwent planned partial tumor resection in a multimodal therapeutic surgery followed by radiosurgery. The preoperative tumor volumes ranged between 8.52 and 25.2 cm3. The intraoperative residual tumor volume measured with the AHSA® software ranged between 2.13–12.17 cm3 (25–52% of the preoperative tumor volume). The intraoperative automatic AHSA plans of the remaining tumor volume suggested, in all five patients, that safe hypofractionated radiation was feasible. Patients were followed for 69.6 ± 1.04 months, and no complications occurred after the patients were treated with radiation. Conclusions: Intraoperative SRS planning based on volumetric assessments during resection of skull base tumors using AHSA® is feasible and safe. The AHSA method allows the neurosurgeon to continuously evaluate the feasibility of adjuvant radiosurgery while planning and performing a surgical resection. This method supports the treatment strategy of a complementary approach during surgical resection of complex skull base tumors and might contribute to preventing surgical and radiosurgical complications.

Long-term follow-up of surgical intervention pattern in pediatric low-grade gliomas: report from the German SIOP-LGG 2004 cohort.

Neurosurgical treatment is an integral part of the treatment algorithms for pediatric low-grade glioma (LGG), yet patterns of surgical procedures are rarely challenged. The objective of this study was to evaluate surgical treatment patterns in pediatric LGG. The German Societé Internationale d'Oncologie Pédiatrique (SIOP)-LGG 2004 cohort was analyzed to identify relevant patient and tumor characteristics associated with time to death, next surgery, number of resections, and radiological outcome. A total of 1271 patients underwent 1713 neurosurgical interventions (1 intervention in 947, 2 in 230, 3 in 70, and 4-6 in 24). The median age of the study population was 8.57 years at first surgery, and 46.1% were female. Neurofibromatosis type 1 (NF1) was found in 4.4%, and 5.4% had tumor dissemination. Three hundred fifty-four patients (27.9%) had chemotherapy and/or radiotherapy. The cumulative incidence of second surgery at 10 years was 26%, and was higher for infants, those with spinal and supratentorial midline (SML) tumors, and those with pilomyxoid astrocytomas. The hazard ratio for subsequent surgery was higher given dissemination and noncomplete initial resection, and lower for caudal brainstem and SML tumors. Among 1225 patients with fully documented surgical records and radiological outcome, 613 reached complete remission during the observation period, and 50 patients died. Patients with pilocytic astrocytoma had higher chances for a final complete remission, whereas patients with initial partial or subtotal tumor resection, dissemination, NF1, or primary tumor sites in the spinal cord and SML had lower chances. Neurosurgery is a key element of pediatric LGG treatment. In almost 50% of the patients, however, at least some tumor burden will remain during long-term follow-up. This study found that most of these patients reached a stable disease status without further surgeries. Multidisciplinary team decisions must balance the goal of complete resection, risk factors, repeated surgeries, and possible treatment alternatives in a wide range of heterogeneous entities. Procedural details and neurological outcome should be recorded to better assess their impact on long-term outcome.

Phase I/II trial of local interstitial chemotherapy with arsenic trioxide in patients with newly diagnosed glioma

BackgroundGlioma is the most common primary brain tumor in adults with poor prognosis. The glioma patients benefit from STUPP strategy, including maximum and safe resection and adjuvant radiotherapy and chemotherapy. Arsenic trioxide could inhibit various tumors. However, it is a challenge to evaluate the efficiency and safety of srsenic trioxide in glioma patients.ObjectiveThe arsenic trioxide has the potent therapeutic effect on glioma. However, the safety and efficacy of local interstitial chemotherapy with arsenic trioxide in newly diagnosed glioma patients is unclear.MethodsAll patients received partial or complete tumor resection and intraoperative implantation of Ommaya reservoirs followed by standard radiotherapy. Arsenic trioxide with the starting dose 0.3 mg was administered via an Ommaya reservoir catheter inserted into the tumor cavity for 5 consecutive days every 3 months for a total of eight cycles unless tumor progression or excessive toxicity was observed.ResultsNo hematological or grade 4 non-hematological toxicity was observed in any patient during arsenic trioxide treatment. The maximum tolerated dose of 1.5 mg of arsenic trioxide was safe and well tolerated. The median overall survival for WHO grade 3 glioma was 33.6 months, and for glioblastoma was 13.9 months. The median progression-free survival for WHO grade 2 glioma was 40.3 months, for grade 3 glioma was 21.5 months, and for glioblastoma was 9.5 months.ConclusionThese results suggest that arsenic trioxide is safe and well tolerated with local delivery into the tumor cavity of the brain, and the dose recommended for a phase II trial is 1.5 mg.

A CASE OF RENAL CELL CARCINOMA WITH SMALL INTESTINE METASTASIS DURING NIVOLUMAB PLUS IPILIMUMAB

A 71-year-old man with a history of hoarseness and right upper extremity numbness was referred to our department for evaluation of an intrathoracic mass that was detected on chest radiography and a right kidney tumor observed on computed tomography (CT). Histopathological examination of percutaneous kidney biopsy and bronchoscopic lung biopsy specimens revealed renal clear cell carcinoma with multiple lung metastases. The patient showed a poor risk based on the International Metastatic renal cell carcinoma Database Consortium score, and nivolumab plus ipilimumab were initiated as first-line therapy. His symptoms gradually improved, following four courses of nivolumab plus ipilimumab treatment, and CT revealed shrinkage of all lesions. However, he developed diarrhea, rash, anemia, and elevated serum C-reactive protein levels (CRP) following this therapy. Diarrhea and rash were considered immune-related adverse events, and he was treated with oral prednisolone and topical corticosteroid. Nivolumab administration was discontinued because anemia worsened together with elevated serum CRP levels despite improvement in diarrhea. He subsequently developed constipation and abdominal bloating, following further treatment for 4 months. CT revealed intestinal tumor-induced intussusception, necessitating partial resection of the small intestinal tumor, which was histopathologically diagnosed as metastases. Both anemia and elevated CRP improved postoperatively. Currently, all metastatic lesions other than the resected intestine have continued to respond to treatment over 12 months after initiation of nivolumab plus ipilimumab therapy.

A homogeneous treatment for non-DIPG diffuse midline glioma.

The H3K27M-mutant diffuse midline glioma (DMG) was first included in the World Health Organization (WHO) Classification of central nervous system (CNS) tumors in 2016, and confirmed in its fifth edition. The biological behavior and dismal prognosis of this tumor resemble diffuse intrinsic pontine gliomas (DIPG). Homogeneously-treated series are rarely reported. From 2016 onwards, we treated patients with DMG with radiotherapy and concomitant/adjuvant nimotuzumab/vinorelbine, plus re-irradiation at relapse, as already done for DIPG. We treated nine patients, seven females, with a median age at diagnosis of 13 years. Tumor sites were: thalamic in five cases, pontocerebellar in two, pineal in one, and paratrigonal with nodular/leptomeningeal dissemination in one. Three patients were biopsied, and six had partial tumor resections. Central pathological review was always performed. The median time to local progression was 12.7 months, and the median overall survival was 17.8 months. Six patients died of tumor progression, one of cerebral bleeding at progression. Two were alive, one in continuous remission, the other after relapsing, at 38.6 and 46.3 months after diagnosis. Progression-free survival was 33.3% at one year. Overall survival was 88.9%, 33.3% and 22.2% at 1, 2 and 3 years, respectively. This is a small series of homogeneously-treated DMG patients. The results obtained are comparable with those of DIPG patients. Given the phenotypically- and molecularly-defined setting of DMG and severe outcome in this orphan population, they should be treated and included in registries and protocols of DIPG.

Surgical Treatment of Spinal Metastatic Pheochromocytoma and Paraganglioma: A Single Institutional Cohort of 18 Patients.

Retrospective Cohort Study. To describe surgeries and treatment outcomes of metastatic pheochromocytomas and paragangliomas (PPGLs) on the spine. We reviewed a cohort of 18 patients with spinal PPGLs who were consecutively treated in our spinal center. Their clinical data was reviewed. The survival period and its relevant factors was then analyzed. The cohort included ten cases of pheochromocytomas and eight paragangliomas. The local pain and neurological deficits were the two most common symptoms. One third of the spinal PPGLs were diagnosed as functional tumors, arousing secondary hypertension. The imaging features were consistent with those of osteolytic lesions. The surgical strategies for the cohort included percutaneous vertebroplasty, neurological decompression and partial tumor resection, and total en-bloc resection. The postoperative courses were uneventful except 1 patient developed heart failure. The adjuvant therapies were implemented in 6 patients with 131I-MIBG, five with radiotherapy, two with chemotherapy, and 1 with target therapy. The median survival period was 39months, and the overall survival rate of 1year was 77.8% (14/18). The patients' Karnofsky performance scores were positively correlated with the survival period (P < .05). Surgery is indicated for intractable local pain and neurological impairment in the patients with spinal PPGLs. Palliative surgical strategies, including neurological decompression and partial tumor resection, could bring fair outcomes, especially for the patients in poor physical conditions.

Brachytherapy for Pediatric Patients at Gustave Roussy Cancer Campus: A Model of International Cooperation for Highly Specialized Treatments

Childhood cancer is rare, and treatment is frequently associated with long-term morbidity. Disparities in survival and long-term side effects encourage the establishment of networks to increase access to complex organ-conservative strategies, such as brachytherapy. We report our experience of an international cooperation model in childhood cancers. We examined the outcome of all children referred to our center from national or international networks to be treated according to a multimodal organ-conservative approach, including brachytherapy. We identified 305 patients whose median age at diagnosis was 2.2 years (range, 1.4 months to 17.2 years). Among these patients, 99 (32.4%) were treated between 2015 and 2020; 172 (56.4%) were referred from national centers; and 133 (43.6%) were international patients from 31 countries (mainly Europe). Also, 263 patients were referred for primary treatment and 42 patients were referred for salvage treatment. Genitourinary tumors were the most frequent sites, with 56.4% bladder/prostate rhabdomyosarcoma and 28.5% gynecologic tumors. In addition to brachytherapy, local treatment consisted of partial tumor resection in 207 patients (67.9%), and 39 patients (13%) had additional external radiation therapy. Median follow-up was 58 months (range, 1 month to 48 years), 93 months for national patients, and 37 months for international patients (P < .0001). Five-year local control, disease-free survival, and overall survival rates were 90.8% (95% confidence interval [CI], 87.3%-94.4%), 84.4% (95% CI, 80.1%-89.0%), and 93.3% (95% CI, 90.1%-96.5%), respectively. Patients referred for salvage treatment had poorer disease-free survival (P < .01). Implementation of image guided pulse-dose-rate brachytherapy was associated with better local control among patients with rhabdomyosarcoma referred for primary treatment (hazard ratio, 9.72; 95% CI, 1.24-71.0). At last follow-up, 16.7% patients had long-term severe treatment-related complications, and 2 patients (0.7%) had developed second malignancy. This retrospective series shows the feasibility of a multinational referral network for brachytherapy allowing high patient numbers in rare pediatric cancers. High local control probability and acceptable late severe complication probability could be achieved despite very challenging situations. This cooperation model could serve as a basis for generating international reference networks for high-tech radiation such as brachytherapy to increase treatment care opportunities and cure probability.

Endoscopic Transcortical-Transventricular Approach in Treating Third Ventricular Craniopharyngiomas-Case Series With Technical Note and Literature Review.

A paradigm shift toward less radicality in the treatment of craniopharyngioma (CP) has increasingly gained recognition. In selected patients, a less invasive approach aiming at cyst decompression may be suitable. To present our experience with the endoscopic transcortical-transventricular approach (ETTA) for the treatment of CP located in the third ventricle, describe our surgical technique, and explore the results reported in the literature. Data for third ventricular CPs treated using ETTA in 2 tertiary medical centers between 2006 and 2020 were retrospectively reviewed. Cystic, mixed lesion, new, and recurrent cases were included. ETTA aimed at cyst fenestration, subtotal or partial tumor resection, managing the hydrocephalus, or a combination of these modalities. Fractionated radiotherapy (RT) was performed (54 Gy in 30 fractions). The described approach was performed in 13 patients with a mean age of 30.2 years. Tumor excision was attempted in 10 patients. Cystic fenestration alone was conducted in 3 patients, followed by intracystic catheter implantation in 2 patients. Permanent diabetes insipidus developed in 3 patients, and 1 patient experienced a surgical site infection. The recurrence rate for ETTA + RT (pre-ETTA in 33.3%) was 2/9 (22.2%) after a mean follow-up of 21.3 months. Four patients did not undergo RT after ETTA, and all these patients experienced recurrences after a mean follow-up of 15.5 months. Hydrocephalus resolved in all 6 patients who presented with accompanying hydrocephalus. ETTA combined with adjuvant RT is a safe and effective option in the management of cystic or predominantly cystic CP extending into the third ventricle. This procedure also effectively treats the accompanying hydrocephalus.

GLIOMA DE LA VÍA ÓPTICA E HIPOTÁLAMO EN UN NIÑO: A PROPÓSITO DE UN CASO

Introduction: Gliomas of the optic nerve, visual pathway, and hypothalamus are treated as a single entity, being considered benign neoplasms in pediatric age, grade I according to the WHO. 25% of them are confined to the optic nerve, 40-75% involve the optic chiasm, and 33-60% are posterior lesions. Most do not present symptoms, but in the case of presenting, the most frequent is loss of vision. The gold standard for diagnosis is magnetic resonance imaging (MRI) with contrast. The first line of treatment is chemotherapy, with surgery used for nerve decompression, if necessary. Clinical case: A 4-year-old woman with a 3-month illness characterized by headache, vomiting, and seizures. The MRI showed a heterogeneous, solid cystic tumor with a solid component that captures contrast. A craniotomy and partial tumor resection were performed, finding both nerves and the optic chiasm thickened. Pathology was reported as pilomyxoid astrocytoma. The patient presented a favorable evolution and was discharged on postoperative day 9. Conclusion: Gliomas of the optic pathway and hypothalamus are tumors with a benign course in childhood, and their main form of treatment is chemotherapy. Surgery only plays an important role if decompression is required to preserve the patient's visual function. Keywords: Astrocytoma, Optic Chiasm, Decompression, Craniotomy, Visual Pathways. (source: MeSH NLM)