Sertoli cell tumor of the testis causing intracranial metastasis two years after orchiectomy. Report of a rare case and review of the literature

Sertoli cell tumor of the testis (SCTT) accounts for less than 1% of all testicular tumors with only 10% of cases exhibiting malignant behavior. In the present report, a case of malignant SCTT causing multiple metastases in a 32-year-old man is described. After being diagnosed and treated for bone and lymph nodes metastases, the patient presented with a brief history of worsening headaches and visual impairment. A head MRI demonstrated an extra-axial tumor located in the right fronto-parietal junction exhibiting avid contrast enhancement and leptomeningeal involvement. To the best of the authors’ knowledge, this represents the second case of intracranial metastasis from SCTT described to date.

RADT-03. OUTCOMES OF PATIENTS WITH HER2-POSITIVE BREAST CANCER METASTATIC TO BRAIN TREATED WITH HER2-TARGETED SYSTEMIC THERAPY AND STEREOTACTIC RADIOSURGERY

OBJECTIVE For patients with HER2-positive breast cancer metastatic to brain, HER2-directed systemic therapies are increasingly used with stereotactic radiosurgery (SRS). These include monoclonal antibodies such as trastuzumab (H) and pertuzumab (P), antibody-drug conjugates such as ado-trastuzumab emtansine (T-DM1), and tyrosine kinase inhibitors such as lapatinib. Limited data exist regarding appropriate timing with SRS and outcomes of this treatment regimen. METHODS A single-institution retrospective review collected clinical data on patients with breast cancer metastatic to brain who were treated with SRS from 2009-2020. Statistical analyses were performed using the Kaplan-Meier method and chi-square statistic. RESULTS Of 82 patients with breast cancer metastatic to brain treated with SRS, 33 (40%) were HER2-positive, 18 of whom were hormone receptor-positive. At brain metastasis diagnosis, 15 patients (45%) had >1 intracranial metastasis (range 2-7), and the median brain metastasis maximal dimension was 2.0 cm. Fifteen patients had uncontrolled extracranial disease. After brain metastasis diagnosis, 9 patients (27%) were treated with systemic therapy first (T-DM1+/-HP, lapatinib+HP, chemotherapy+/-HP) followed by SRS at a median of 18.6 months after starting systemic therapy. Seven patients (21%) were treated with SRS first, followed by systemic therapy in 6 of these patients (multi-agent regimens, 4 including T-DM1 or lapatinib). Seventeen (52%) received concurrent systemic therapy and SRS (T-DM1+/-chemotherapy, lapatinib, HP, hormone therapy, chemotherapy). Median follow-up time was 21.1 months. Median overall survival was 24.8 months and not statistically different between treatment groups. Four patients (12%) developed symptomatic radionecrosis; 3 were on T-DM1 concurrent with SRS. CONCLUSION In this small patient sample, we noted favorable survival outcomes for patients with HER2-positive breast cancer metastatic to brain when treated with HER2-targeted therapies together with SRS. The sequence of systemic therapy and SRS does not appear to impact survival outcomes. Concurrent treatment with T-DM1 and SRS may be associated with higher rates of radionecrosis.

PATH-12. CLINICAL AND PATHOLOGICAL ANALYSIS OF 43 PATIENTS WITH EPITHELIOID GLIOBLASTOMA

BACKGROUND AND PURPOSE Epithelioid glioblastoma (eGBM) is a rare and aggressive subtype of glioblastoma. The clinical characteristics, pathological features and radiological findingsare still not well characterized. METHODS The clinical data of 43 patients with eGBM confirmed pathologically after surgery in Guangdong Sanjiu Brain Hospital from June 2015 to January 2021 were reviewed, and to investigate the clinical, pathological and imaging characteristics of the eGBM. RESULTS The range of patients' age was from 5 to 70 years (median 39 years). There were 24 males and 19 females. The median karnofsky performance score (KPS）at diagnosis was 70 (40-90). The most common symptom were headache (81.4%), and other common symptoms included nausea or vomiting (44.1%), limb weakness (30.2%), disturbance of consciousness (20.9%) and seizures (9.3%). Most of the tumors (97.6%) were located in the cerebral hemisphere ,except for one case located in the cerebellum,and with an average diameter of 5.3 cm (2.5-9.4 cm). There were 2 cases of intracranial metastasis and 7 cases of spinal cord metastasis in MRI. In pathologic examination, immunohistochemistry showed all the patients were IDH1 wild-type (43/43) and H3K27M wild-type(34/34). 25 (58.1%) of cases harbored BRAF mutation and 19 (44.2%) cases of MGMT positive. Most of patients were positive for GFAP, P53, Olig-2 and ATRX. CONCLUSION Epithelioid glioblastoma is more common in adults, most of which are located on the supratentorial, intracranial and spinal cord dissemination may occur. Half of patients showed BRFA mutation and maybe benefit from targeted therapy.These findings may help clinicians understand and treat epithelioid glioblastoma better.

Clinical features and prognosis of paediatric rhabdomyosarcoma with bone marrow metastasis: a single Centre experiences in China

Background The aim of this study was to summarize the clinical characteristics, therapeutic effects and prognosis of patients with rhabdomyosarcoma (RMS) and bone marrow metastasis, improve the understanding of this disease. Method This was a single-institution retrospective study involving the children with RMS, who presented with bone marrow metastasis at initial presentation to our hospital between 1st, Jan, 2006 and 31st, Dec,2019. Follow-up concluded on 31st, Dec, 2020 and the clinical data were collected and analysed. Result Between 1st Jan 2006 and 31st Dec 2019, 13 eligible patients presented to our hospital, including 10 males and 3 females, these eligible patients accounted for 4.5% of all RMS patients. The median age at onset was 5.6 years (range 1.7-14 years). The patients not only had unfavourable primary sites, but also had multiple metastases. The bone marrow aspirate samples of the patients comprised 8-95% blast-like cells. Nine of 13 patients were misdiagnosed with haematological malignancies or other solid tumours. With respect to histology, four of 13 children were classified as embryonal RMS and nine as alveolar RMS. Eleven patients underwent PAX-FOXO1 fusion testing; eight had the POX- FOXO1 fusion gene. Immunohistochemically(IHC) analysis revealed that the tumour cells were positive for Desmin, Vimentin, Myo-D1 and Myogenin. More importantly, the patients had extremely poor prognoses, the median EFS was 12.0 months (range 3-28.3 months) and the median OS was 27.0 months (range6-46.2 months). Conclusion This study demonstrates that children with RMS and bone marrow metastasis usually exhibit atypical primary sites and multiple metastases, with presentation mimicking haematological malignancies or other solid tumors at initial presentation. Pathology and IHC analysis combined with POX-FOXO1 fusion gene detections can effectively confirm the diagnosis. These patients are more likely to relapse or progress during early treatment and are prone to intracranial metastasis. While multidisciplinary therapy combined with Temozolomide may prevent it, further prospective research is required to evaluate the therapeutic effects.

OTHR-06. PACS Lesion Tracking Tool provides real time automatic information on brain tumor metastasis growth curves and RECIST criteria

Objective Communicating metastatic brain treatment response can be complicated. A widely used method to assess clinical response is called response evaluation criteria in solid tumors or RECIST. In our study, we use a PACS Lesion Tracking Tool (TT) to assess intracranial metastasis using RECIST criteria. We predict that the TT will be superior to the standard radiology reports. Methods Nuance ® mPowerTM was used to identify 30 patients with brain metastasis who received brain MRI from 4/2020–4/2021. Patient’s first brain MRI with metastasis was set as baseline and subsequent 3 brain MRI studies were examined. All lesions were measured on post-gadolinium sequence and defined as target lesions or new lesions. The TT was used to measure lesion size over time with creation of growth curves and RECIST outcomes, which include stable disease, progressive disease, partial response, or complete response. Subsequently, RECIST evaluations were compared with radiologic impressions for discrepancy, and further evaluations were made to see if it made a clinical difference in patient management and/or provide additional useful information. These evaluations were given a rating of agree/yes, equivocal, or disagree/no. They were assessed by 3 neuroradiologists. Results Number of lesions ranged from 1–27. The assessments from 3 neuroradiologists were averaged. Comparing impression versus RECIST evaluation, the results demonstrated the following: 8/30 disagreement, 4/30 equivocal, and 18/30 agreement. Using more stringent criteria, assessing whether the TT would result in either change in patient management or provide additional useful information, the results were the following: 6/30 yes, 4/30 equivocal, and 20/30 no. Discussion In addition to providing real time RECIST criteria evaluations and visually descriptive lesion growth tables, the TT was easy to use. Interpretation of these additional data provided more clarity and was found to be superior to standard radiology report.

Isolated Progressive Enlargement of Head Mass as the First Symptom: A Rare Case Report of Cranial Metastasis of Hepatocellular Carcinoma and Literature Review

Background: This article presents a rare case of skull metastasis of primary hepatocellular carcinoma that manifests the isolated progressive enlargement of the head mass as the first symptom. Case presentation:A 65-year-old female patient presented an isolated painless mass in the head, which grew rapidly over the last month. Head CT revealed a 6.4 cm×5.6cm osteolytic destruction in the right parietal bone. MRI further revealed that the occupation was significantly enhanced in the T1 reinforced phase. The patient underwent total surgical resection. Postoperative pathology confirmed that the head mass was the skull metastasis from hepatocellular carcinoma (HCC). Conclusion: The case of skull metastasis from primary hepatocellular carcinoma is rare, and HCC patient with intracranial metastasis tends to have a rather poor prognosis. Surgical excision of the metastatic mass and radiotherapy can improve the life quality and prolong the survival time of the patient.

Evolving role of entrectinib in treatment of NTRK-positive tumors

Targeted therapy has shown to be a very effective treatment in tumors with specific genomic drivers. Trk has proven to be one such target. Efforts to target the Trk fusion with specific inhibitors have shown remarkable responses in a tumor agnostic fashion, with responses seen even in patients with intracranial metastasis. Entrectinib is a first-generation Trk inhibitor with impressive activity in early phase trials performed in patients with NTRK fusion positive solid tumors and ROS1 positive non-small-cell lung cancers with subsequent approval for those indications. Entrectinib was also found to be effective in treatment of brain metastasis and generally well tolerated.

Intracranial Metastasis from Prostate Cancer: Investigation, Incidence, and Imaging Findings in a Large Cohort of Australian Men

Objectives: Prostate cancer metastasizing to the brain is remarkably uncommon, with the incidence never having been described in the modern setting. The objective of this study was to determine the incidence and imaging pattern of intracranial metastasis from prostate cancer in a large cohort of Australian men with prostate cancer. Material and Methods: Retrospective review was undertaken of imaging reports for all known prostate cancer patients, who underwent an imaging examination inclusive of the brain, between July 1, 2014, and July 1, 2020. Once an intracranial lesion was identified, all available imaging and clinical notes were reviewed. Results: A total of 5644 imaging examinations which included the brain were identified in 4341 prostate cancer patients. The majority (92.1%) of examinations were 68-Gallium-labeled prostate-specific membrane antigen (68Ga-PSMA) positron emission tomography/computed tomography (PET/CT). Eight patients were identified as having an intracranial metastasis from prostate cancer, yielding an incidence of 0.18%. All patients had a Gleason score of 9 (where known), and the majority of patients (5/8) had a non-acinar variant of prostate cancer. At the time of diagnosis of intracranial metastasis, all patients had extensive metastatic disease. Imaging characteristics of the intracranial lesions were highly variable. Conclusion: The incidence of intracranial metastasis in prostate cancer patients has never been well-established. In this study, we determined the incidence as being 0.18%. Given the majority of metastasis constituted unexpected findings on routine restaging 68Ga-PSMA PET/CT, the incidence determined in our study is arguably the most accurate and clinically relevant described to date.

Gamma Knife for Recurrent Ameloblastoma with Cavernous Sinus Metastasis: A Case Report

Ameloblastoma (AME) is a rare, benign intraosseous progressively growing odontogenic tumor. Due to its invasive behavior, the rate of recurrence is high. Recurrent AME tends to transform malignantly and metastatic. Lung is the most common sites of AME metastasis, followed by lymph nodes. Here we present a case of AME with intracranial metastasis. A 26-year-old woman who had recurrent AME in the left jaw. After the second resection, AME metastasis to the cavernous sinus, sellar and suprasellar regions. Because the metastatic tumor was unresectable, she received Gamma Knife instead. After 3 years follow-up, the tumor was well controlled. In conclusion, Gamma Knife can be a feasible option for unresectable Oligometastatic AME.