OpenMed/multicare-articles huggingface dataset:
Embryonic esophageal rhabdomyosarcoma in an adult male: A case report and literature review
Authors: Xiaoman Zhang, Guang Li
Journal: Front Oncol (2022)
Keywords: chemotherapy, esophageal rhabdomyosarcoma, esophagus, prognosis, radiotherapy
MeSH terms: Case Reports

Basic principle:
There exists a rare aggressive neoplasm called esophageal rhabdomyosarcoma. It originates in cells of the striated muscle or mesenchymal cells which differ from the striated muscle. This tumor has a high degree of malignancy and extensive metastasis. Masses of the sick people are at a high phase when going to hospital. Consequently, the prognosis is exceedingly bad.
Patient concerns:
A 54-year-old male presented with dysphagia as the initial symptom. Gastroscopy showed an irregular protuberant lesion about 18-22 cm from the incisor. The lesion was observed to be pathological under gastroscopy and was diagnosed as an (esophageal) sarcoma.
Diagnosis:
Embryonic esophageal rhabdomyosarcoma.
Interventions:
After receiving two cycles of DP (docetaxel and cisplatin) chemotherapy in the local hospital, the patient received 60 Gy of radiotherapy in 30 fractions combined with chemotherapy at our hospital. Dysphagia was relieved, and the tumor appeared significantly shrunken on imaging after the treatment.
Outcomes:
Lung metastasis occurred 1 month after radiotherapy, and the patient died of pulmonary edema on March 11, 2022.
Lessons:
Previously reported cases of embryonic esophageal rhabdomyosarcoma are few. Theoretically, the disease should occur in adolescents; nevertheless, our case was a man who was in middle-aged; the neoplasm was in an unusual position: the upper part of the esophagus. Moreover, the patient initially had good response to the combination of radiotherapy and chemotherapy. Although he died 8 months after diagnosis, the presented data represent a valuable resource for understanding the survival benefits of treating embryonic esophageal rhabdomyosarcoma patients with radiotherapy combined with chemotherapy. In addition, we reviewed the previously reported literature, and a total of 17 cases of esophageal rhabdomyosarcoma were identified and analyzed.
Abdominal Compartment Syndrome (ACS) With Sigmoid Volvulus (SV): Lost Hours Are Lost Lives
Authors: Andrea C Marin, Sharon Hechter, Ankita Prasad, Dina Alnabwani, Charles Lwoodsky, Pramil Cheriyath
Journal: Cureus (2023)
Keywords: anterior compartment syndrome, gangrene, intraabdominal pressure, ischemia, perforation, peritonitins, prognosis, sepsis, shoch, sigmoid volvulus
MeSH terms: Case Reports

Normal intra-abdominal pressure (IAP) ranges from 0 to 5, and abdominal compartment syndrome (ACS) occurs when a sustained IAP >20 mmHg causes organ dysfunction. ACS mainly occurs in patients who are critically ill. It occurs due to an injury or disease in the abdomen or pelvic area, including trauma, abdominal surgery, acute pancreatitis, pancreatic ileus, volvulus, fecal impaction, and ruptured abdominal aortic aneurysm. If not recognized early, ACS leads to multiorgan dysfunction, shock, and sepsis and has high morbidity and mortality. Our patient was brought to the emergency department (ED) following cardiac arrest and resuscitation and was diagnosed with sigmoid volvulus (SV) and ACS. SV is seen in older men, and its presentation is often insidious and leads to bowel gangrene and ACS. The patient's delay in presenting to the hospital and the severity of his condition leads to a poor outcome despite surgery. A delay in recognizing ACS can lead to a worse outcome.
Case report: Progressive skin rash and lymphadenopathy associated with lamotrigine-valproic acid combination in a bipolar adolescent
Authors: Yingxu Duan, Fangxinrui Qiu, Jingyuan Zhou, Shiyu Liu, Die Zhao, Changjian Qiu
Journal: Front Pharmacol (2023)
Keywords: bipolar disorder, case report, lamotrigine, lymphadenopathy, valproate
MeSH terms: Case Reports

Background: Lamotrigine may cause severe skin reactions. There is a known interaction between lamotrigine and valproic acid with an increase in lamotrigine levels and lamotrigine toxicity risk. Few cases of severe rash and systemic reactions in bipolar patients using lamotrigine and valproate have been reported. Here, we report a rare case of severe skin rash and lymphadenopathy associated with lamotrigine-valproic acid combination. Case presentation: An 18-year-old female adolescent with bipolar disorder type I was treated with lamotrigine, magnesium valproate, and perospirone for 12 days. After the last dose of lamotrigine, she abruptly developed generalized rash and swollen lymph nodes, which continued to progress over the next 3 days. This finally subsided after stopping valproate and with glucocorticoid treatment. Conclusion: This case suggests that lamotrigine-valproic acid combination may cause not only rash but also lymphadenopathy. Even though the aforementioned reactions appear after the last dose of lamotrigine, it cannot be ruled out as suspicious. We recommend caution during titration of lamotrigine and valproate and early withdrawal of both when signs of hypersensitivity appear.
A Rare Case of Gorlin-Goltz Syndrome in Children
Authors: Fernanda Brasil Daura Jorge Boos Lima, Ana Paula Cota Viana, Luciano Henrique Ferreira Lima, Bruna Campos Ribeiro, Carlos Eduardo Assis Dutra, Glaykon Alex Vitti Stabile, Sergio Monteiro Lima Junior
Journal: Case Rep Dent (2019)
MeSH terms: Case Reports

The Gorlin-Goltz syndrome, nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome is an autosomal dominant condition disorder with high variability expression. It presents a series of relevant clinical manifestations that suggest its diagnosis in cutaneous, bone, dental, soft tissue, nervous, and ocular system disorders. This condition requires a great interaction of several specialists to improve the patient's life. In this case, we presented a 9-year-old male patient referred to the Department of Oral and Maxillofacial Surgery reporting failure in the normal chronology of dental eruption. After evaluation, it was observed that the patient had 13 typical characteristics of the syndrome, including keratocysts, bifid ribs, palmoplantar pits, and 10 other minor characteristics. In conclusion, the expression of so many features of Gorlin-Goltz syndrome is rare in infants, and early diagnosis is important to decrease morbidity and mortality associated with basal cell carcinomas.
A clinically silent tumour of adrenal myelolipoma: A case report
Authors: Nornazirah Azizan, Ohnmar Myint, Aye Aye Wynn, Tin Tin Thein, Firdaus Hayati, Nik Amin Sahid Nik Lah
Journal: Int J Surg Case Rep (2020)
Keywords: benign neoplasms, cat scan, myelolipoma
MeSH terms: Case Reports

Introduction:
Adrenal myelolipoma is a rare, non-functional, benign neoplasm which is constituted of mature haematopoietic elements and adipose tissues in various proportions. It is diagnosed accidentally and frequently with the widespread use of imaging modalities.
Presentation of case:
We report a 63-year-old lady with incidental findings of adrenal tumour on computed tomography (CT) scan during a routine medical check-up. She underwent tumour resection in view of a large tumour of 7 cm in size.
Discussion:
CT scan is sensitive to diagnose adrenal myelolipoma in view of its fat-laden property and useful to monitor the tumour progress. Even previously she opted for conservative management; the decision for surgery was made in view of enlarging tumour and risk of surrounding tissue compression.
Conclusion:
With increased awareness, the detection rate of this tumour is improving, hence able to prevent the complications of a large tumour such as compression, bleeding and tumour necrosis.
Myxoid mesenchymal neoplasm presenting as massive arm and chest wall oedema with pleural effusion
Authors: Vidya B Pai, Rahul Ravilla, Matthew Lindberg, Matthew Steliga, Konstantinos Arnaoutakis
Journal: Ecancermedicalscience (2015)
Keywords: extracellular matrix, malignant pleural effusion, neoplasms, sarcoma
MeSH terms: Case Reports

Myxoid mesenchymal tumours are a heterogeneous group of neoplasms characterised histologically by their abundant mucoid and myxoid extracellular matrix (ECM). Encompassing a broad spectrum of clinical behaviour ranging from benign to malignant, there are more than 60 reactive and neoplastic entities currently classified under its domain. Its varied clinical and histopathologic features continue to pose a diagnostic challenge to clinicians and pathologists. Here, we describe a rare case of myxoid mesenchymal tumour presenting as oedema of the upper extremity with pleural metastasis and partial response to chemotherapy, which to the best of our knowledge has not yet been described in the literature.
Concurrent Diagnosis of Chronic Myeloid Leukemia and Follicular Lymphoma: An Unreported Presentation
Authors: Amy G Starr, Sushma R Jonna, Joeffrey J Chahine, Bhaskar V Kallakury, Chaitra S Ujjani
Journal: Case Rep Hematol (2018)
MeSH terms: Case Reports

Lymphadenopathy in chronic myeloid leukemia (CML) is usually due to extramedullary involvement with accelerated or blast phases of the disease. The occurrence of non-Hodgkin lymphoma (NHL) as a synchronous malignancy with CML is rare. We report a case of a 73-year-old male who presented with dyspnea and right-sided lower extremity edema in the setting of leukocytosis. Bone marrow evaluation indicated a chronic phase chronic myeloid leukemia (CML), confirmed by molecular testing. Imaging of the chest for persistent dyspnea revealed supraclavicular and mediastinal lymphadenopathy. Biopsy of the cervical node showed expanded lymphoid follicles with atypical germinal centers that were positive for CD10, BCL-2, and BCL-6, consistent with follicular lymphoma (FL). Nodal PCR demonstrated clonal IGH and IGK gene rearrangements, and FISH analysis was positive for IGH-BCL-2 fusion. Together, these tests supported the diagnosis of FL. Additionally, the lymph node showed paracortical expansion by maturing pan-hematopoietic elements, no blastic groups, and positive RT-PCR analysis for BCR-ABL1, indicating concomitant involvement by chronic phase-CML. To our knowledge, this is the first reported case of a patient with a concurrent diagnosis of CML and FL.
Biopsy Proven Tumefactive Multiple Sclerosis with Concomitant Glioma: Case Report and Review of the Literature
Authors: Esteban E Golombievski, Matthew A McCoyd, John M Lee, Michael J Schneck
Journal: Front Neurol (2015)
Keywords: demyelination, glial cell tumors, multiple sclerosis, oligoastrocytoma, tumefactive

We report a case of pathologically confirmed tumefactive multiple sclerosis (MS) followed shortly thereafter by the diagnosis of an oligoastrocytoma. The complexity of diagnosis and management of concomitant presence of tumefactive MS and glial cell tumors is discussed.
Physiological and Pathophysiological Consequences of a 25-Day Ultra-Endurance Exercise Challenge
Authors: Nicholas B Tiller, Scott T Chiesa, Justin D Roberts, Louise A Turner, Siana Jones, Lee M Romer
Journal: Front Physiol (2019)
Keywords: cardiovascular, nutrition, respiratory, ultra-endurance, ultra-marathon

Background: This case-report characterized the respiratory, cardiovascular, and nutritional/gastrointestinal (GI) responses of a trained individual to a novel ultra-endurance exercise challenge. Case Presentation: A male athlete (age 45 years; V ˙ O2max 54.0 mL⋅kg-1⋅min-1) summited 100 mountains on foot in 25 consecutive days (all elevations >600 m). Measures: Laboratory measures of pulmonary function (spirometry, whole-body plethysmography, and single-breath rebreathe), respiratory muscle function (maximum static mouth-pressures), and cardiovascular structure and function (echocardiography, electrocardiography, large vessel ultrasound, and flow-mediated dilatation) were made at baseline and 48 h post-challenge. Dietary intake (four-day food diary), self-reported GI symptoms and plasma endotoxin concentrations were assessed at baseline, pre/post mid-point, pre/post end-point, and 48 h post-challenge. Results: The challenge was completed in a total exercise time of 142 h (5.3 ± 2.8 h⋅d-1), with a distance of 1141 km (42.3 ± 43.9 km⋅d-1), and energy expenditure of 80460 kcal (2980 ± 1451 kcal⋅d-1). Relative to baseline, there were post-challenge decreases in pulmonary capacities and expiratory flows (≤34%), maximum expiratory mouth-pressure (19%), and maximum voluntary ventilation (29%). Heart rate variability deteriorated, manifesting as a 48% decrease in the root mean square of successive differences and a 70% increase in the low-frequency/high-frequency ratio. Pre- to post-challenge endotoxin concentrations were elevated by 60%, with a maximum increase of 130% after a given stage, congruent with an increased frequency and severity of GI symptoms. Conclusion: The challenge resulted in pulmonary and autonomic dysfunction, endotoxaemia, and GI distress. The findings extend our understanding of the limits of physiological function and may inform medical best-practice for personnel supporting ultra-endurance events.
Breaking the Thumb Sucking Habit: When Compliance Is Essential
Authors: Orlando Tanaka, Wagner Oliveira, Melissa Galarza, Vanessa Aoki, Bruno Bertaiolli
Journal: Case Rep Dent (2016)

The anterior open bite (AOB) and posterior cross bite are the most frequent malocclusions associated with prolonged sucking habits. This clinical case illustrates and discusses the use of a Haas-type palatal expander for stopping a thumb sucking habit. The improvement in closing the open bite with discontinuation of the habit was observed. But with the return of the habit and lack of cooperation, the relapse of anterior open bite occurred. Therefore, different approaches are necessary. The need of a multidisciplinary approach, consent, and cooperation are keys to a good prognosis.
Refractory IgD Multiple Myeloma Treated with Daratumumab: A Case Report and Literature Review
Authors: Muhammad Husnain, Sandra Kurtin, Nikki Barkett, Irbaz Bin Riaz, Amit Agarwal
Journal: Case Rep Oncol Med (2016)
MeSH terms: Case Reports

Patients with relapsed and refractory multiple myeloma have poor prognosis. A recent analysis of patients with relapsed and refractory multiple myeloma who were refractory to both proteasome inhibitors and immunomodulatory drugs showed the median overall survival of 9 months only. Daratumumab is the first-in-class human monoclonal antibody against CD38 cells which was studied in phase I/II trials for treatment of these patients with relapsed refractory multiple myeloma. It showed an overall response rate of 36% and a median overall survival (OS) of 17 months in these patients. We report a case of 40-year-old man with immunoglobulin D (IgD) multiple myeloma whose disease was refractory to at least 5 different chemotherapy regimens including proteasome inhibitors and immunomodulatory drugs. The clinical studies assessing daratumumab did not include any patients with IgD myeloma which is a rare form of multiple myeloma and to our knowledge is the first study reporting use of daratumumab in IgD myeloma.
Primary Small Bowel GIST Presenting as a Life-Threatening Emergency: A Report of Two Cases
Authors: Safi Khuri, Hayim Gilshtein, Abd-Alkarim Darawshy, Hany Bahouth, Yoram Kluger
Journal: Case Rep Surg (2017)
MeSH terms: Case Reports

Gastrointestinal stromal tumor (GIST) is a rare stromal neoplasm, which represents the most common mesenchymal tumor of the gastrointestinal tract. It is characterized by indolent clinical symptoms, although it can present as a life-threatening emergency. Herein, we present two cases of primary small bowel GIST treated at our department. A 68-year-old female patient presented to our emergency department with a diffuse abdominal pain of acute onset. Imaging studies revealed a mass at proximal jejunum, with a nearby free air and fluid. At surgery, a mass of 9 cm was found at proximal jejunum, 3 cm distal to the treitz ligament, with perforation on the lateral wall of the mass. En bloc resection was performed. Pathology report was positive for gastrointestinal stromal tumor. A 70-year-old male patient presented to our emergency department with 3 days of dark tarry stool and few hours of hematochezia. Computed tomography angiography revealed a mass at the pelvis, with calcifications, attached to the distal ileum, with intraluminal blush of intravenous iodine. At surgery, a mass of 8 cm at the distal ileum was found. Resection of the mass along with a 20 cm of ileum was completed. Histopathology report was positive for malignant gastrointestinal stromal tumor.
Macular findings in Spectral Domain Optical Coherence Tomography and OCT Angiography in a patient with Kearns-Sayre syndrome
Authors: Alvaro Ortiz, Juan Arias, Pedro Cárdenas, John Villamil, Marcela Peralta, Luis C Escaf, Jacobo Ortiz
Journal: Int J Retina Vitreous (2017)
Keywords: kearns–sayre syndrome, mitochondrial disease, optical coherence tomography, retinal pigmentary epithelium
MeSH terms: Case Reports

Background:
To report the clinical, electrophysiological and the anatomical findings in a patient with Kearns-Sayre syndrome (KSS).
Case presentation:
We present the case of a 55-year-old female with KSS, who developed systemic features and ocular manifestations as ophthalmoplegia and retinal dysfunction, that were corroborated by electrophysiological test and High Definition Spectral Domain Optical Coherence Tomography (HD SD OCT) and OCT-Angiography (OCT-A).
Conclusion:
We report a patient with KSS, accompanied by some alterations of the RPE and photoreceptors observed in the external HD SD OCT and OCT-A. In the best of our knowledge, this is the first report in the literature of HD SD OCT findings in a patient with KSS.
Acute Splenic Artery Thrombosis and Infarction Associated with COVID-19 Disease
Authors: Osama Qasim Agha, Ryan Berryman
Journal: Case Rep Crit Care (2020)
MeSH terms: Case Reports

Coronavirus 2019 disease (COVID-19) is a viral illness caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). It emerged in Wuhan, China, in December 2019 and has caused a widespread global pandemic. The symptoms of COVID-19 can vary from mild upper respiratory symptoms to severe pneumonia with hypoxemic respiratory failure. Multiple studies and reports have reported a hypercoagulable state associated with this disease, and various recommendations have emerged to guide the use of anticoagulants for prophylaxis. We are reporting a case of symptomatic acute splenic thrombosis causing splenic infarction in a patient suffering from a severe case of COVID-19 and despite the use of an intermediate dose of low-molecular-weight heparin (LMWH). The patient was treated with full-dose anticoagulation and was eventually discharged home on a direct oral anticoagulant.
Coexistence of Myelolipoma and Primary Bilateral Macronodular Adrenal Hyperplasia With GIP-Dependent Cushing's Syndrome
Authors: Stéphanie Larose, Louis Bondaz, Livia M Mermejo, Mathieu Latour, Odile Prosmanne, Isabelle Bourdeau, André Lacroix
Journal: Front Endocrinol (Lausanne) (2019)
Keywords: bmah, cushing's syndrome, aberrant ligands, ectopic receptor, myelolipoma
MeSH terms: Case Reports

Introduction: Adrenal myelolipomas are usually isolated benign adrenal lesions, but can be adjacent to steroid-secreting adrenocortical tumors. We studied the aberrant regulation of cortisol secretion in a 61 year-old woman with combined bilateral myelolipomas and primary bilateral macronodular adrenal hyperplasia (BMAH) causing Cushing's syndrome. Materials and Methods: Cortisol response was measured during in vivo tests that transiently modulated the levels of ligands for potential aberrant receptors, including GIP. Response to medical therapies decreasing GIP was monitored. Expression of ACTH and of GIP receptors were examined in resected adrenal tissues by immunohistochemistry and reverse transcription polymerase chain reaction (RT-PCR). Results: In vivo, cortisol increased in response to mixed meals (+353%), oral 75 g glucose (+71%), GIP infusion (+416%), and hLH IV (+243%). Suppression of GIP by pasireotide improved cortisol secretion but produced hyperglycemia. The left adrenal was predominantly composed of myelolipoma and strands of BMAH, while the right was mainly composed of BMAH with some foci of myelolipoma on pathology. No ACTH was detectable by immunohistochemistry in BMAH or myelolipomas tissue. Ectopic GIP receptor was confirmed by RT-PCR and immunohistochemistry in BMAH tissues but not in the myelolipomas. No germline mutations were identified in the ARMC5 gene of the patient's leucocyte DNA. Conclusion: This is the first report of interspersed myelolipoma and BMAH with GIP-dependent Cushing's syndrome. In contrast with the BMAH tissues, myelolipoma tissue did not express specific GIP receptors. The potential mechanisms responsible for the interspersed growth of those two lesions remain to be identified.
Novel Therapies for Myocardial Irritability following Extreme Hydroxychloroquine Toxicity
Authors: Paul B McBeth, Perseus I Missirlis, Harry Brar, Vinay Dhingra
Journal: Case Rep Emerg Med (2015)

Introduction. Hydroxychloroquine (HCQ) overdose is rare and potentially deadly when consumed in large doses. Management of severe HCQ toxicity is limited and infrequently reported. This report presents the case of a massive ingestion of HCQ. Case Report. A 23-year-old female presents following an intentional ingestion of approximately 40 g of HCQ. Within six hours after ingestion, she developed severe hemodynamic instability resulting from myocardial irritability with frequent ventricular ectopic activity leading to runs of polymorphic ventricular tachycardia (PMVT) and ventricular fibrillation (VF) requiring multiple defibrillations. Additional treatments included intravenous diazepam, epinephrine, norepinephrine, sodium bicarbonate, and magnesium sulfate. Despite the ongoing hemodynamic instability, the patient was also treated with Intralipid (ILE) and received hemodialysis. Improvements in her hemodynamics were observed after 18 hours. She survived her massive overdose of HCQ. Conclusion. HCQ poisoning is rare but serious because of its rapid progression to life-threatening symptoms. Hemodynamic support, gastric decontamination, electrolyte monitoring and replacement, and management of arrhythmias are the mainstays of treatment. The combined role of dialysis and ILE in the setting of massive HCQ overdose may improve outcomes.
Emergency Laparoscopic Repair of Giant Left Diaphragmatic Hernia following Minimally Invasive Esophagectomy: Description of a Case and Review of the Literature
Authors: Enrico Erdas, Gian Luigi Canu, Luca Gordini, Paolo Mura, Giulia Laconi, Giuseppe Pisano, Fabio Medas, Pietro Giorgio Calò
Journal: Case Rep Surg (2018)
MeSH terms: Case Reports

Postoperative diaphragmatic hernia (PDH) is an increasingly reported complication of esophageal cancer surgery. PDH occurs more frequently when minimally invasive techniques are employed, but very little is known about its pathogenesis. Currently, no consensus exists concerning preventive measures and its management. A 71-year-old man underwent minimally invasive esophagectomy for esophageal cancer. Three months later, he developed a giant PDH, which was repaired by direct suture via laparoscopic approach. A hypertensive pneumothorax occurred during surgery. This complication was managed by the anaesthesiologist through a high fraction of inspired O2 and several recruitment manoeuvres. The patient remained free of hernia recurrence until he died of neoplastic cachexia 5 months later. Laparoscopic repair of PDH may be safe and effective even in the acute setting and in the case of massive herniation. However, surgeons and anaesthesiologists should be aware of the risk of intraoperative pneumothorax and be prepared to treat it promptly.