OpenMed/multicare-articles huggingface dataset:
Radiation Therapy Combined With Checkpoint Blockade Immunotherapy for Metastatic Undifferentiated Pleomorphic Sarcoma of the Maxillary Sinus With a Complete Response
Authors: Kripa Guram, Maria Nunez, John Einck, Loren K Mell, Ezra Cohen, P Dominick Sanders, Sayuri Miyauchi, Elizabeth Weihe, Razelle Kurzrock, Sarah Boles, Andrew B Sharabi
Journal: Front Oncol (2018)
Keywords: checkpoint blockade, head and neck cancer, immunotherapy, radiation, sarcoma, stereotactic radiotherapy
MeSH terms: Case Reports

Background: Undifferentiated pleomorphic sarcoma (UPS) of the maxillary sinus is an extremely rare malignancy of the head and neck. Surgery is the mainstay of treatment for UPS; however, proximity to vital structures makes it challenging to achieve negative surgical margins. Adjuvant therapy including radiation therapy with or without chemotherapy is generally indicated. Despite advances in multimodality treatment, objective response rates to available therapies and prognosis of metastatic UPS remain dismal. Immunotherapy has become a fourth cornerstone of cancer therapy and checkpoint blockade immunotherapy is a standard of care for recurrent or metastatic cisplatin-refractory head and neck squamous cell carcinoma. Checkpoint blockade immunotherapy is being studied in metastatic sarcoma, including UPS, and while initial results are promising, objective response rates remain below 20%. However, adding radiation therapy to checkpoint blockade immunotherapy has been shown, in both preclinical and retrospective clinical studies, to have combinatorial effects on both local and metastatic disease. Thus, further investigation into the effects of radiation therapy combined with immunotherapy in head and neck sarcomas is warranted. Case Presentation: We present a case of metastatic, chemotherapy-refractory, UPS of the maxillary sinus in a 55-year-old male treated with checkpoint blockade immunotherapy combined with radiation, which resulted in a complete response. Conclusions: This is the first report to our knowledge of metastatic UPS treated with a combination of radiation and dual agent checkpoint blockade immunotherapy. Further investigation is warranted to study the effects of this combination in patients with metastatic UPS that fail to respond to currently available therapies.
Case report: Abolishing primary resistance to PD-1 blockade by short-term treatment of lenvatinib in a patient with advanced metastatic renal cell carcinoma
Authors: Tingting Tan, Xiaotong Lin, Jing Ling, Rong Wang, Yue Chen, Longmei Cai, Jingyuan Sun, Dehua Wu, Guozhu Xie
Journal: Front Immunol (2023)
Keywords: pd-1, lenvatinib, pembrolizumab, primary resistance, renal cell carcinoma
MeSH terms: Carcinoma, Renal Cell / pathology, Kidney Neoplasms / drug therapy, Kidney Neoplasms / pathology, Case Reports, Humans, Phenylurea Compounds, Prospective Studies, lenvatinib, Phenylurea Compounds

Anti-PD-1 immunotherapy has been extensively used in treatment of patients with advanced metastatic renal cell carcinoma (mRCC). Several prospective clinical trials showed that the combined treatment of anti-PD-1 antibody plus lenvatinib, a potent receptor tyrosine kinase inhibitor (TKI), exhibited high response rate compared with single-agent sunitinib. However, whether the patients with primary resistance to PD-1 blockade could benefit from the addition of lenvatinib is still unclear. Herein, we reported a patient with mRCC who was primary resistant to pembrolizumab and achieved a durable complete response after a short-term treatment with lenvatinib. This case report indicates that the patients with primary resistance to anti-PD-1 therapy could benefit from the short-term lenvatinib in combination with anti-PD-1 therapy, and provides a useful paradigm worthy of establishing a clinical trial for mRCC patients with primary resistance to anti-PD-1 therapy.
Prolonged Remission of Cancer of Unknown Primary following Initiation of Eculizumab Therapy for Paroxysmal Nocturnal Hemoglobinuria
Authors: Ming Y Lim, Keith E Volmar, Nigel S Key
Journal: Case Rep Hematol (2019)
MeSH terms: Case Reports

We report the case of a 64-year-old woman who presented with cancer of unknown primary treated with carboplatin and paclitaxel, followed by maintenance erlotinib. Her chemotherapy regimen was discontinued due to the development of profound hemolysis that was later identified to be due to paroxysmal nocturnal hemoglobinuria (PNH). She was started on a complement inhibitory antibody, eculizumab 900 mg every 2 weeks, with marked suppression of hemolysis. Eight years after diagnosis of cancer, the patient remains on eculizumab with no signs of cancer recurrence on regular imaging. Regardless of whether the co-occurrence of cancer and PNH was any more than coincidental in this patient, the uniqueness of the case is emphasized by the remarkable and sustained response of not only PNH but also possibly the associated cancer to eculizumab.
Case report: Chronological symptom profile after cessation of overdose zolpidem in a patient with comorbid bipolar disorder-from anxiety, craving, paresthesia and influenza-like symptoms to seizures and hallucinations
Authors: Zi-Xin Mao, Xia Yang, Hui-Yao Wang, Wan-Jun Guo
Journal: Front Psychiatry (2022)
Keywords: bipolar disorder, chronological feature, overdose, withdrawal symptoms, zolpidem
MeSH terms: Case Reports

Introduction:
Insomnia is a major public health problem that determines the quality of life. Among the many causes of insomnia, psychological factors have an important influence on the process, duration of insomnia, help-seeking behavior, and treatment choice. Regarding medical treatment, zolpidem is always chosen to treat acute and transient insomnia due to its few side effects. Although some randomized controlled trials have verified its safety, zolpidem abuse and withdrawal reactions have been reported in recent years.
Case report:
A 25-year-old unmarried man with a college degree who worked as a graphic designer was referred and admitted to the inpatient ward for a chief complaint of "alternative episodes of lowering and elevation of mood for 10 years, overdosage use of zolpidem for two years." He underwent a time-dependent withdrawal reaction after admission. It was characterized by rebound insomnia, anxiety, craving, skin paresthesia, influenza-like symptoms, tonic-clonic-type seizures, and hallucinations. At the 1-year follow-up, he did not exhibit any remaining withdrawal symptoms.
Discussion:
The acute cessation of overdosage zolpidem use causes a series of withdrawal symptoms that manifest in chronological order. Additionally, long-term benzodiazepine exposure has potential influences on zolpidem dependence/tolerance. However, patients with a history of abuse or dependence, or mental disorders seem to be at risk of drug abuse. Clinicians should be alert to the potential for zolpidem dependence and addiction. Once the acute cessation of overdosage zolpidem use occurs, the potential of the withdrawal reaction needs to be considered and addressed properly.
Case report: Successful treatment of acute generalized pustular psoriasis of puerperium with secukinumab
Authors: Gao Xue, Ma Lili, Fang Yimiao, Wu Miao, Yang Xiaohong, Wang Dongmei
Journal: Front Med (Lausanne) (2022)
Keywords: agep, gpp, biologic therapy, interleukin-17a inhibition, secukinumab
MeSH terms: Case Reports

Generalized pustular psoriasis (GPP) is a rare and severe form of psoriasis presenting with erythematous, aseptic pustules. Common systemic symptoms include fever and myalgias. The presentation of GPP resembles acute generalized exanthematous pustulosis (AGEP). However, the treatment of these two pathologies differs. While AGEP is self-limiting and treated with topical corticosteroids and constrain of systemic steroids. GPP treatment avoids corticosteroid, choosing acitretin, methotrexate, and cyclosporine as first-line agents. In this case report, a 27-year-old female with a medical history of AGEP presented to the hospital with extensive erythema and pustules. Complete blood count acute phase reactant analysis revealed an elevated white blood cell count and C-reactive protein (CRP). Two histopathological examinations revealed psoriatic hyperplasia of the epidermis with keratosis, along with Kogoj and Munro micro abscesses above the spina layer. Lymphocytic and neutrophilic infiltrate was present in the superficial derma layer along with vasodilation. The patient was diagnosed with GPP according to pathological and clinical criteria. Treatment was initiated with secukinumab because of the patient's failure to respond to systemic treatment with Acitretin, methotrexate, and cyclosporin. Following 2 weeks of therapy with 300 mg of secukinumab, the pustular lesions had resolved. This study indicates the potential efficacy of secukinumab as an effective therapy that can rapidly improve the clinical symptoms of GPP.
Postoperative and Recurrent Hematuria after Pretransplant Core Needle Biopsy in Living Donor Kidney Transplant
Authors: Yazan Al-Adwan, Navdeep Singh, Pranit N Chotai, Farjad Siddiqui, Ashley Limkemann, Austin Schenk, Jayanthan Subramanian, W Kenneth Washburn, Musab Alebrahim, Amer Rajab
Journal: Case Rep Transplant (2022)
MeSH terms: Case Reports

Background:
Core needle and wedge biopsies are the two main pathologic ways to determine the suitability of a kidney allograft and to have a baseline allograft biopsy in case of future rejection. Case Presentation. A 57-year-old patient developed a renal arteriovenous fistula causing postoperative and recurrent hematuria after allograft pretransplant renal core needle biopsy and treated with selective Interventional radiology coil embolization.
Conclusion:
Delayed profound hematuria can be seen after pretransplant core needle renal biopsies and can recur again even after complete resolution, due to arteriovenous fistula formation in the renal calyceal system.
Pulmonary-Renal Syndrome with Negative ANCAs and Anti-GBM Antibody
Authors: Hiroshi Yamaguchi, Atsuhisa Shirakami, Takashi Haku, Takashige Taoka, Yoshikazu Nakanishi, Toru Inai, Takanori Hirose
Journal: Case Rep Nephrol (2013)

We report the case of a 76-year-old woman who was referred to our hospital for a gradually worsening cough and renal dysfunction. Although pneumonia was initially suspected, imaging findings of the lungs revealed diffuse alveolar hemorrhage at a later date. Renal failure developed and hemodiafiltration was performed on the 9th day. Rapidly progressive glomerulonephritis with crescent formation was diagnosed by renal biopsy. This case presentation has important clinical implications because uncategorizable pulmonary-renal syndrome (PRS) without the presence of ANCAs and anti-GBM antibody is extremely rare and has high rates of morbidity and mortality. No treatment has been established.
46,XX male disorder of sexual development:a case report
Authors: Ahmet Anık, Gönül Çatlı, Ayhan Abacı, Ece Böber
Journal: J Clin Res Pediatr Endocrinol (2013)
MeSH terms: 46, XX Disorders of Sex Development / diagnosis, 46, XX Disorders of Sex Development / genetics, Case Reports, Adolescent, Chromosomes, Human, X / genetics, Genes, sry / genetics, Humans, In Situ Hybridization, Fluorescence, Karyotype, Karyotyping, Male, Organ Size / genetics, Testis / metabolism, Testis / pathology, Translocation, Genetic

The main factor influencing sex determination of an embryo is the sex-determining region Y (SRY), a master regulatory gene located on the Y chromosome. The presence of SRY causes the bipotential gonad to differentiate into a testis. However, some individuals carry a Y chromosome but are phenotypically female (46,XY females) or have a female karyotype but are phenotypically male (46,XX males). 46, XX male is rare (1:20 000 in newborn males), and SRY positivity is responsible for this condition in approximately 90% of these subjects. External genitalia of 46,XX SRY-positive males appear as normal male external genitalia, and such cases are diagnosed when they present with small testes and/or infertility after puberty. Herein, we report an adolescent who presented with low testicular volume and who was diagnosed as a 46,XX male. SRY positivity was demonstrated in the patient by fluorescence in situ hybridization method.
Extramedullary Plasmacytoma Diagnosed in an HIV-Positive Patient by an Unusual Clinical Presentation
Authors: Paulo de Camargo Moraes, Luiz Alexandre Thomaz, Victor Angelo Martins Montalli, José Luiz Cintra Junqueira, Camila Maria Beder Ribeiro, Luciana Butini Oliveira
Journal: Case Rep Dent (2016)

The aim of this paper is to describe a case report of EMP in an HIV-positive patient. A 44-year-old, dark-skinned HIV-infected woman was referred to the Oral Diseases Treatment Center with a swelling at palate and left gingival fornix in the maxilla. Biopsy was taken and the oral lesion was diagnosed as EMP with well-differentiated plasma cells and restriction of the lambda light-chain. Skeletal survey was performed and no radiograph alterations were observed, thus supporting the diagnosis of EMP. Patient was referred to treatment and after two months of chemo and radiotherapy, an expanding lesion was observed in L5/S1 patient's vertebrae. Biopsy of the spinal lesion was consistent with lymphoma with plasmocitary differentiation, supporting the diagnosis of multiple myeloma (MM). Regarding the medical history, the final diagnostic was an oral extramedullary plasmacytoma with rapid progression into multiple myeloma. It is crucial to emphasize the relevance of HIV infection as a risk factor for both aggressive clinical behavior and unusual clinical presentation of extramedullary plasmacytoma cases.
MRI of giant cell tumour of larynx: marked T 2 hypointensity due to abundant haemosiderin deposition
Authors: Etsushi Iida, Matakazu Furukawa, Naofumi Matsunaga, Yoshimi Anzai
Journal: BJR Case Rep (2016)
MeSH terms: Case Reports

Giant cell tumours (GCTs) are benign tumours commonly found in the long bones. Rarely, they may occur in the larynx, often resulting in hoarseness and anterior neck swelling. Since Wessely reported the first case of laryngeal GCT in 1940, 35 cases have been identified. Herein, we present a case of a 53-year-old male with GCT of the larynx that showed T 1 and T 2 hypointense signal on MRI, presumably owing to abundant haemosiderin deposition. We also discuss the imaging findings of CT and 18F-fludeoxyglucose positron emission tomography scans, as well as the pathological correlation.
Isolated gastric myeloid sarcoma: a case report and review of the literature
Authors: Pankit Vachhani, Prithviraj Bose
Journal: Case Rep Hematol (2014)

Myeloid sarcoma represents the proliferation of myeloblasts of acute myeloid leukemia (AML) at extramedullary sites. While extramedullary involvement in AML is uncommon in itself, isolated myeloid sarcomas, that is, myeloid sarcomas without any bone marrow involvement, are extremely rare and pose a diagnostic and therapeutic challenge. Here, we present the case of a middle-aged woman with isolated myeloid sarcoma in the stomach-an organ seldom involved by this disease. Additionally, the literature on the epidemiology, diagnosis, pathology, prognosis, and therapeutic options in myeloid sarcomas has been reviewed.
Abnormal B-Cell Maturation and Increased Transitional B Cells in CBL Syndrome
Authors: Francesco Saettini, Tiziana Angela Coliva, Francesca Vendemini, Marta Galbiati, Cristina Bugarin, Riccardo Masetti, Daniele Moratto, Marco Chiarini, Fabiola Guerra, Maria Iascone, Raffaele Badolato, Giovanni Cazzaniga, Charlotte Niemeyer, Christian Flotho, Andrea Biondi
Journal: Front Pediatr (2022)
Keywords: alps, benta, card11, cbl, rald, rasopathies, lymphocytosis, splenomegaly
MeSH terms: Case Reports

CBL syndrome is a Noonan-like RASopathy with heterogeneous clinical phenotype and predisposition to juvenile myelomonocytic leukemia (JMML). Here we describe two patients with identical germline CBL mutation and clinical and immune-hematological overlapping features with autoimmune lymphoproliferative syndrome (ALPS) and B-cell expansion with NF-κB and T-cell anergy (BENTA) syndrome. Increased immature/transitional B cells can be depicted in CBL syndrome, ALPS, and BENTA. Nonetheless, our patients here described showed peculiar B-cell phenotype due to increased immature/transitional CD34+ B cells. This feature differentiates CBL syndrome from BENTA, pointing toward an abnormal proliferation of B-cell early precursors.
Pipeline Embolization Device for Salvage Treatment of a Willis Covered Stent Prolapse Into the Aneurysmal Sac
Authors: Zeng-Bao Wu, Sheng Wang, Li-Gang Song, Xin-Jian Yang, Shi-Qing Mu
Journal: Front Neurol (2019)
Keywords: willis covered stent, endoleak, migration, pipeline embolization device, prolapse
MeSH terms: Case Reports

The Willis covered stent (WCS) may prolapse into the aneurysmal sac due to device migration or foreshortening. We present a useful salvage strategy that can reorient a prolapsed WCS into a more suitable alignment. An intra-procedural prolapse of a WCS into a large cavernous aneurysm occurred in a 70-year-old female patient. A pipeline embolization device (PED) was used to retrieve the WCS and successfully accomplish flow diversion. Maintaining proximal access and ensuring that the microwire is securely held within the central axis of the herniated stent are critical until the entire parent vessel can be reconstructed. This salvage technique may help to regain proximal access and reposition the flow diversion constructs following WCS prolapse.
Not all the bowlegs is rickets! (a case report)
Authors: Keta Vagha, Patel Zeeshan Jameel, Jayant Vagha, Ashish Varma, Siddhartha Murhekar, Parameshwar Reddy, Spandana Madirala
Journal: Pan Afr Med J (2022)
Keywords: blount’s disease, bowing, case report, tibial intorsion
MeSH terms: Genu Varum / complications, Rickets / diagnosis, Rickets / drug therapy, Rickets / etiology, Case Reports, Bone Diseases, Developmental, Calcium, Child, Child, Preschool, Cholecalciferol, Humans, Male, Osteochondrosis / congenital, Tibia, Cholecalciferol, Calcium, Blount disease

Bowing of the legs is common in childhood. Most times it is considered to be rickets without considering other possibilities. Blount´s disease is a close differential diagnosis which is developmental deformity characterized by intorsion of tibia leading to varus angulation. This case report aims to encourage pediatricians to expand their vision and consider other possibilities when a case of bowing of legs is encountered. Here we report a case of a four-year-old boy with bowing of both legs noticed first at 2.5 years of age. There was no history suggestive of trauma. Development of the child was age appropriate in all domains. He was receiving treatment for rickets for 1.5 years in form of oral vitamin D3 and calcium supplementations. He had no other clinical signs of rickets like frontal bossing, widening of wrists, and rachitic rosary except bowing of legs. His biochemical parameters did not show any alterations that would support the diagnosis of rickets. Weight-bearing radiographs of lower limbs showed medial intorsion of bilateral tibia with metaphyseo-diaphysial angle to be 25º on the right side and 20º on the left side, which was beyond the physiological normal angulation, therefore he was diagnosed as a case of Blount´s disease, stage III as per Langenskiöld classification. All the bow legs is not always rickets in pediatric practice. Therefore, various differential diagnoses should be kept in mind as early diagnosis and intervention can change a child´s life.
Food fear, quick satiety and vomiting in a 16 years old girl: It's bulimia, or maybe not…? A case report of Wilkie's syndrome (superior mesenteric artery syndrome)
Authors: Giovanni Frongia, Jens-Peter Schenk, Anja Schaible, Peter Sauer, Arianeb Mehrabi, Patrick Günther
Journal: Int J Surg Case Rep (2019)
Keywords: case report, duodenal stenosis, duodeno-jejunostomy, superior mesenteric artery syndrome, vomiting, wilkie syndrome

Introduction:
Wilkie's syndrome (WS), also known as superior mesenteric artery syndrome, is a rare clinical entity caused by compression of the horizontal (third) part of the duodenum between the superior mesenteric artery and the abdominal aorta leading to duodenal obstruction.
Presentation of case:
We report a case of a 16 years girl with long-term history of spontaneous vomiting and self-induced vomiting, also suffering from recurrent retrosternal pain, weight loss and thus reduced quality of life. Contrast intestinal series showed a large axial hernia which was laparoscopically reduced and treated by hiatoplasty and anterior hemifundoplication. After initial relief, recurrent postprandial nausea and vomiting reoccurred 4 weeks postoperatively. Abdominal MRI study revealed findings compatible with WS. After endoscopic exclusion of an anatomical duodenal lumen stenosis, she was successfully treated by duodeno-jejunostomy with a favorable outcome.
Discussion:
WS might be hidden behind presumably more evident diagnoses such as bulimia, significant axial hernia and gastro-esophageal reflux disease in patients with recurred vomiting, abdominal pain and weight loss.
Conclusion:
The rare clinical entity of a WS necessitates a targeted diagnostic evaluation and therapy. Clinical details, diagnostic studies and treatment are discussed here.
Intraductal Papillary Mucinous Neoplasm of the Pancreas Arising in the Setting of an Intermixed Acinar Cell Cystadenoma of the Pancreas: Report of a Rare Case
Authors: Benjamin B Scott, Thea P Price, Zachary M Callahan, Justin S Poling, Harish Lavu
Journal: Case Rep Pancreat Cancer (2016)
Keywords: acinar cell cystadenoma, cystic lesions of the pancreas, intraductal papillary mucinous neoplasm, pancreas
MeSH terms: Case Reports

Background: Synchronous cystic lesions of the pancreas with different pathophysiology in the same patient are a rare occurrence.. Case Presentation: We report the incidental finding of a multicystic lesion within the pancreatic head in a morbidly obese woman during workup for bariatric surgery. The lesion contained an intraductal papillary mucinous neoplasm (IPMN) with high-grade dysplasia within an acinar cell cystadenoma (ACA). ACAs are rare tumors first described in 2002. Conclusion: To date, there have been no published reports of synchronous IPMN within an ACA. This case report intends to increase provider awareness of these lesions as well as highlight the importance of surveillance and careful histological examination of heterogeneous cystic lesions of the pancreas.
Case Report: A Primary Right Ventricular Vascular Malformation Presenting as a Mass
Authors: Hongduan Liu, Xin Li, Cuiwei Zhang, Chengming Fan, Liming Liu, Juyi Wan
Journal: Front Cardiovasc Med (2021)
Keywords: cardiac surgery, cardiac tumor, echocardiography, hemangioma, vascular malformation
MeSH terms: Case Reports

Primary right ventricular vascular malformation is a rare primary benign anomaly in heart in nature. Due to the extremely low incidence and the progress on the classification of vascular malformation, a few cases were reported in the literatures. In the current case study, a 55-year-old women presented with a cardiac mass that was identified in right ventricle during a routine medical checkup. Magnetic resonance imaging demonstrated a well-circumscribed mass attached to the interventricular septum. Median sternotomy for the surgical resection of the mass and a cardiopulmonary bypass were performed. The intraoperative transesophageal echocardiogram showed that the mass had been successfully removed. The patient recovered well and was discharged from hospital 9 days after the surgery. The pathological diagnosis was primary cardiac arteriovenous malformation. No mass recurrence was shown by echocardiography during the 13 months' follow-up.