BACKGROUND AND IMPORTANCE: Aggressive pituitary adenomas (APAs) are pituitary tumors that are refractory to standard treatments and carry a poor prognosis. Current treatment guidelines are not standardized but combine surgical resection, radiation therapy, and chemotherapy. Temozolomide is the only chemotherapeutic agent with documented effectiveness and is recommended for APA in European Society of Endocrinology clinical guidelines. CLINICAL PRESENTATION: A 57-year-old man presented with visual deterioration and bitemporal hemianopsia. MRI of the brain demonstrated a sellar mass suspected to be pituitary macroadenoma with displacement of the stalk and optic nerve impingement. The patient underwent stereotactic endoscopic transsphenoidal resection of the mass. Postoperative MRI demonstrated gross total resection. Pathology revealed a sparsely granulated corticotroph adenoma with malignant transformation. Immunohistochemistry showed loss of expression of MLH1 and PMS2 in the tumor cells. Proton therapy was recommended given an elevated Ki67 index and p53 positivity. Before radiotherapy, there was no radiographic evidence of residual tumor. Temozolomide therapy was initiated after surveillance MRI showed recurrence at 16 months postoperatively. However, MRI demonstrated marked progression after 3 cycles. Next-generation sequencing using the MSK-IMPACT platform identified somatic mutations in MLH1 Y548lfs*9 and TP53 R337C. Immunotherapy with ipilimumab/nivolumab was initiated, and MRI demonstrated no residual tumor burden 34 months postoperatively. CONCLUSION: APA is a tumor with frequent recurrence and a short median expected length of survival. Here, we demonstrate the utility of immunotherapy in a single case report of APA, with complete resolution of recurrent APA and improved survival compared with life expectancy.
Chemotherapy-induced thrombocytopenia (CIT) is a critical condition in which platelet counts are abnormally reduced following the administration of chemotherapeutic compounds. CIT poses a treatment conundrum to clinicians given the increased risk of spontaneous bleeding, obstacles to surgical management of tumors, and exclusion from clinical trials. Treatment of CIT involves the removal of the offending agent combined with platelet infusion or thrombopoietin agonist treatment. However, due to the autoimmune and infection risks associated with infusions, this treatment is only reserved for patients with critically low platelet counts. One potential solution for patients in the mid to low platelet count range is Carica papaya leaf extract (CPLE). In this case, we report the novel use of CPLE as a method of bolstering platelet counts in a patient presenting with CIT. The patient was initiated on CPLE therapy consisting of 1 tablespoon twice daily with meals. Following CPLE treatment, the patient’s platelet counts rebounded from less than 10,000/µL to 113,000/µL. This clinical vignette supports the use of CPLE in the clinical context of CIT when thrombopoietin agonists are not a viable option. The potential benefits of CPLE as a method for increasing platelet count deserve further exploration, especially as a treatment option for refractory patients or those ill-suited for other traditional thrombocytopenia therapies.
INTRODUCTION Pituitary carcinoma (PC) accounts for just 0.1% of all pituitary tumors, often recurs following resection, and has a median reported survival of 1 year. Current treatment guidelines are not standardized but combine surgical resection, radiation therapy, and chemotherapy [1]. Temozolomide is the only chemotherapeutic with documented effectiveness, and the only recommended agent for aggressive pituitary carcinomas in ESE clinical guidelines [3]. CASE: A 57-year-old male presented with visual deterioration over a three-month period. Ophthalmologic evaluation revealed bitemporal visual field deficits. MRI brain W/WO demonstrated a sellar mass suspected to be pituitary macroadenoma with displacement of the stalk and optic nerve impingement (Figure 1a). The patient underwent stereotactic endoscopic transsphenoidal resection of the mass [2]. Postoperative MRI demonstrated gross total resection (Figure 1b). Pathology revealed a sparsely granulated corticotroph-adenoma with malignant transformation (early in-situ PC). Immunohistochemistry showed LOE of MLH1 and PMS2 in the tumor cells; Genetic analysis revealed MGMT methylation. Proton therapy was recommended given the elevated Ki67 index (75%) and p53 positivity. Before radiotherapy, there was no evidence of residual tumor or metastasis radiographically. He received 6600cGy of radiation over 33 fractions. Surveillance MRI showed recurrence at 21 months postoperatively, and temozolomide was initiated. However, MRI demonstrated marked progression after 3 cycles, and at 44 months, he developed a new 6th nerve palsy (Figure1c). Next-generation sequencing using the MSK-IMPACT platform identified somatic mutations in MLH1 Y548lfs*9 and TP53 R337C[4]. Immunotherapy with ipilimumab/nivolumab was initiated [5], and the patient noted resolution of his third nerve palsy soon after. MRI demonstrated a dramatic response with only minimal residual tumor burden (Figure1d). CONCLUSION PC is a rare tumor with frequent recurrence and a short median expected length of survival. Here we demonstrate the utility of immunotherapy in a single case report of PC. This treatment helped our patient survive well beyond the expected median life expectancy of this aggressive disease.
Atypical teratoid rhabdoid tumor (ATRT) is a highly malignant embryonal tumor of the CNS, largely affecting pediatric patients, with exceedingly rare cases in adults at an estimated annual incidence of 1/1,000,000. We report a unique case of ATRT in a 43-year-old female patient who first presented with progressive focal headaches. Imaging revealed a sellar mass with suprasellar extensions, which was partially removed via a transsphenoidal resection. The tumor aggressively recurred just 1 month postoperatively. Her care team pursued a novel treatment plan by using a slightly modified COG ACNS 0332 regimen, which involved radiation, followed by 4 cycles of monthly chemotherapy including vincristine, cyclophosphamide, and cisplatin. Hematopoietic stem cells were collected between radiation and chemotherapy in the event that the patient required stem cell salvage therapy postadjuvant chemotherapy. The MRIs taken at 2 and 4 months postrecurrence indicated a substantial decrease in tumor volume, with corresponding clinical improvements to cranial nerve deficits. Given the scarcity of literature on adult cases of ATRT and the lack of a standard of care for these cases, discussing the efficacy of our patient's treatment plan may aid clinical decision making for adult ATRT cases.
Cancer survivorship is an important area of medicine as it focuses on the quality of life of patients after experiencing cancer and cancer treatment. Thankfully, many patients survive cancers and other benign tumor diagnoses but must live with the side effects and subsequent consequences of their treatment. These, sometimes lifelong, effects can diminish quality of life and interfere with day-to-day activities. A number of therapy tools have been developed to aid in survivorship care, but how can we advance new digital therapy tools for cancer survivorship and brain-related diseases, specifically quality of life? The University of Cincinnati has curated an innovative team of faculty and students across disciplines to develop digital applications to emphasize cancer and brain tumor survivorship and create new methods for health and wellbeing at the hands of people. This includes the CAN Quality of Life applications which use novel technologies to administer digital pre-existing therapies. The first, ARMCan, is a digital music therapy instrument used by breast cancer patients with post-chemotherapy brain fog. The second, ARTCan, is a digital art therapy platform in which vestibular schwannoma and NF2 patients with mood issues can complete self-guided art activities. ARTCan is partnered with ARCCan, animal robotic-like companions. A narrative application for cancer survivors and caregivers is being developed. These CAN applications hope to improve the quality of life for the people they serve by improving executive function and mood, respectfully. ARMCan and ARTCan/ARCCan pilot feasibility studies are currently accruing subjects at our institution.
e13619 Background: Breast cancer is the most diagnosed cancer in women with approximately 2 million women diagnosed in 2018. The 10-year survival rate is 78%. Breast cancer patients who undergo chemotherapy treatment can suffer neurocognitive impairment resulting in significant effects on their cognitive functioning. Chemotherapy-related dysfunction is known as “chemobrain”. Chemobrain is the basis of significant neurological morbidities in the breast cancer population. It causes difficulty in people being able to carry out activities of daily living and impacts people’s livelihoods and well-being. Studies have shown that music-based intervention can improve cognitive function. Methods: Since music-based interventions to specifically address chemo-related cognitive deficits have not been explored, we are conducting a pilot feasibility study to beta test the novel Active Receptive Music for Cancer patients application (ARMCan) which allows its user to have an interactive musical experience. Patients are randomized into two arms. In receptive listening, users listen to a genre of their choice, and in active listening, users add sounds over a base trace. At the beginning of the study, patients will be given a FACT-Cog questionnaire and undergo MRI imaging. The study invention will be conducted for six months where patients will use the ARMCan app for 15 minutes daily. They will be followed for one year post-study intervention with FACT-Cog questionnaires given directly after the cessation of the study intervention and at months six and 12 of follow up. An additional MRI will be taken at month 12 of follow up. Results: FACT-Cog and MRI data will be used to analyze the results of active versus receptive music therapy on chemotherapy-related cognition dysfunction. Conclusions: We are currently accruing patients to beta test this interactive application, which may promote executive function recovery in breast cancer patients with chemobrain and advance clinical care in breast cancer survivorship.
Secondary brain tumors and neurocognitive damage from radiation or chemotherapy are often the commonest neuro-oncological problems in cancer. Breast cancer is the most commonly diagnosed cancer in women, with approximately 2 million women diagnosed in 2018.1 The 10-year survival rate for women diagnosed with breast cancer is 78% (World Cancer Research Fund, 2018). Although the 10-year survival rate is high, women who undergo chemotherapy can experience neurocognitive impairment resulting in significant effects of their cognitive functioning.2 Chemo related dysfunction is known as “chemobrain” or “chemofog.” Chemobrain can result in difficulty with attention, daily activities of living, and memory. This impacts people’s livelihoods and affects their general well-being. Current research on the topic of chemobrain in breast cancer survivors is minimal. However, this study aims to reduce the post-chemotherapy outcomes of chemobrain through the use of interactive versus receptive music. “Brain Fog” or chemobrain is the basis of significant neurological morbidities in the breast cancer population. It causes difficulty in people being able to even carry out activities of daily living. We have developed a prototype “ARMCan—a music software application to help breast cancer patients with “brain fog.” We are conducting a pilot feasibility study to beta test this interactive application which will promote executive function recovery in breast cancer patients with chemobrain.
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