OpenMed/multicare-articles huggingface dataset:
Combination of Sorafenib, Camrelizumab, Transcatheter Arterial Chemoembolization, and Stereotactic Body Radiation Therapy as a Novel Downstaging Strategy in Advanced Hepatocellular Carcinoma With Portal Vein Tumor Thrombus: A Case Series Study
Authors: Yun Huang, Zeyu Zhang, Weijun Liao, Kuan Hu, Zhiming Wang
Journal: Front Oncol (2021)
Keywords: camrelizumab, downstaging strategy, hepatocellular carcinoma, sorafenib, stereotactic body radiation therapy, transcatheter arterial chemoembolization

Background and aim:
Although the treatment effect and availability of therapeutic options for advanced hepatocellular carcinoma (HCC) are limited, the downstaging strategy may improve patient prognosis. This study aimed to investigate the potential of combination therapy as a downstaging strategy for treating advanced HCC with portal vein tumor thrombus (PVTT).
Methods:
This retrospective case series included patients having advanced HCC with PVTT, who received the combination therapy of sorafenib, camrelizumab, transcatheter arterial chemoembolization (TACE), and stereotactic body radiation therapy (SBRT) from January 2019 to December 2019 in Xiangya Hospital, Central South University. The downstaging rate, treatment responses, progression-free survival (PFS), overall survival (OS), disease control rate, and toxicities were evaluated.
Results:
Of the 13 patients, HCC downstaging was achieved in 4 (33.3%) patients who later received hepatectomy. The overall response rate was 41.7%, and the disease control rate was 50.0%. The median PFS time was 15.7 months, with a 1-year PFS rate of 58.3%, whereas the median OS was not reached after 1 year (1-year OS, 83.3%). No severe adverse events or grade 3-4 adverse effect was observed in 12 of the 13 enrolled patients; therapy had to be discontinued in only one patient due to adverse events, who was excluded from the study. The most common adverse effect was fever (n = 4, 33.3%), followed by skin reaction (n = 3, 25%).
Conclusion:
A combination therapy comprising sorafenib, camrelizumab, TACE, and SBRT is an effective downstaging strategy for advanced HCC with PVTT and is associated with few adverse events.
Pulmonary Combined Large Cell Neuroendocrine Carcinoma
Authors: Meihui Li, Lan Yang, Hongyang Lu
Journal: Pathol Oncol Res (2022)
Keywords: diagnosis, pathological characteristics, pulmonary combined large cell neuroendocrine carcinoma (clcnec), targeted therapy, therapy
MeSH terms: Carcinoma, Large Cell / genetics, Carcinoma, Neuroendocrine / therapy, Carcinoma, Non-Small-Cell Lung / drug therapy, Lung Neoplasms / genetics, Review, Humans, Retrospective Studies

Pulmonary combined large-cell neuroendocrine carcinoma (CLCNEC) is a rare neuroendocrine tumor pertained to lung large cell neuroendocrine carcinoma (LCNEC) with aggressive behavior and poor prognosis generally. The clinical features of CLCNEC are not specific including cough, expectoration, chest distress, chest pain, etc., which are prone to have different manifestations of the mixed components. Owing to the low incidence, there are few related small-scale retrospective studies and case reports. Currently, the treatment regimen of CLCNEC mainly refers to LCNEC that complete surgical resection is preferred in the early stage and according to previous researches, platinum-based small cell lung cancer (SCLC) standard treatment regimen showed promising results in postoperative and advanced CLCNEC as compared to that of non-small cell lung cancer (NSCLC). Adenocarcinoma-CLCNEC more likely harbor driver gene mutation, and may benefit from targeted therapy. As for immunotherapy, more clinical trial data are needed to support its benefits. This article will fill the gap and will provide new insight into the clinical characteristics, pathological diagnosis and treatment endeavors of CLCNEC.
Adult pancreatic hemangioma: case report and literature review
Authors: Gerhard S Mundinger, Shannon Gust, Shien T Micchelli, Elliot K Fishman, Ralph H Hruban, Christopher L Wolfgang
Journal: Gastroenterol Res Pract (2009)
MeSH terms: Case Reports

We report an adult pancreatic hemangioma diagnosed on pathological specimen review following pylorus preserving pancreaticoduodenectomy for a symptomatic cystic mass in the head of the pancreas. Eight cases of adult pancreatic hemangioma have been reported in literature since 1939. Presenting symptoms, radiographic diagnosis, pathologic characteristics, and treatment of adult pancreatic hemagiomas are discussed following review of all published cases.
TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report
Authors: Marie Nagai, Takahiro Uchida, Muneharu Yamada, Shuuhei Komatsu, Kohei Ota, Mitsuya Mukae, Hitoshi Iwamoto, Hiroshi Hirano, Miho Karube, Shinya Kaname, Takashi Oda
Journal: Front Med (Lausanne) (2021)
Keywords: tafro syndrome, autopsy, kidney transplant, membranoproliferative glomerulonephritis, thrombotic microangiopathy
MeSH terms: Case Reports

A 57-year-old man who received a kidney transplant 4 years previously owing to unknown underlying disease presented with thrombocytopenia and fever. Hepatosplenomegaly and lymphadenopathy were observed, and development of prominent anasarca and worsening of renal function yielded the diagnosis of TAFRO syndrome. He was treated with high-dose steroids and plasmapheresis, and a thrombopoietin receptor agonist was administered for refractory thrombocytopenia. However, his general condition worsened, and he died on day 92. Histopathological analysis of a kidney autopsy specimen showed thrombotic microangiopathy characterized by glomerular endothelial swelling, mesangiolysis, and double contours of the glomerular capillary walls. His bone marrow showed megakaryocytic hyperplasia with mild reticulin fibrosis. Interestingly, these clinical and pathological features were remarkably similar to those the patient demonstrated before the kidney transplant, suggesting the recurrence of TAFRO syndrome. TAFRO syndrome is a rare systemic disorder whose concept has recently been established, but information on its long-term outcome is scarce. To our knowledge, this is the first case of TAFRO syndrome developing in a kidney transplant recipient, which suggests that disease recurrence occurs many years after the kidney transplant.
Actinomycosis presenting as an abdominal mass in a child
Authors: Rahsan Ozcan, Emil Mammadov, Emrah Aydin, Ibrahim Adaletli, Tugçe Esen, Sergülen Dervisoglu, Sinan Celayir
Journal: APSP J Case Rep (2011)
Keywords: abdominal actinomycosis, abdominal trauma, actinomycosis, mass abdomen
MeSH terms: Case Reports

Abdominal actinomycosis in childhood period is very rare and a relation to trauma is not well established. Herein we report a case that appeared subsequent to abdominal trauma. A 17 years old boy presented with left lower quadrant abdominal mass and signs of acute abdomen. The symptoms of abdominal discomfort began after a fall from height 3 months before admission. There were signs of acute abdomen at physical examination. Ultrasound of abdomen demonstrated a mass; CT scan findings pointed to a suspicious "internal hernia". An emergency laparotomy was performed. During surgery, a mass located over sigmoid colon and infiltrating the lateral abdominal wall was found. It was removed en bloc with the adjacent omentum. Except for the thickened sigmoid colon, no other pathologies were present at laparotomy. The pathology specimen revealed the actinomyces infection. The patient was treated with oral penicillin after discharge and the follow-up was uneventful. We advocate, keeping the actinomyces infection in mind in cases presenting with abdominal mass of unknown origin in childhood period.
Facial Emphysema following Closure of Oroantral Fistulae
Authors: Muhammad Aiman Mohd Nizar, Syed Nabil
Journal: Case Rep Dent (2021)
MeSH terms: Case Reports

Subcutaneous emphysema (SE) is a swelling which develops due to air entrapped underneath the subcutaneous tissue and facial planes causing distention of the overlying skin. SE can develop due to trauma, surgery, or infection. The diagnosis of SE is mostly based on clinical findings of crepitation upon palpation of the swelling. Once diagnosed, SE is usually managed by close observation and in some cases may require surgical decompression and antibiotic prophylaxis. We report a rare case of SE of the left malar which developed following closure of oroantral communication using the buccal fat pad.
Traumatic Lens Dislocation in an Eye with Anterior Megalophthalmos
Authors: Huda AlGhadeer
Journal: Case Rep Ophthalmol Med (2022)
MeSH terms: Case Reports

Anterior megalophthalmos is a rare, bilateral, nonprogressive, hereditary, congenital disorder characterized by the enlargement of all anterior segment structures of the eye, with megalocornea, iris atrophy, and zonular abnormalities. We report a case of an 8-year-old male who presented to the emergency department with a history of visual loss after a blunt ocular trauma to the left eye. The patient presented with markedly enlarged corneas and deepened anterior chambers bilaterally. Best-corrected visual acuity (BCVA) was hand motion in the left eye. An additional examination revealed multiple anterior segment abnormalities, leading to the diagnosis of megalophthalmos and lens dislocation in the anterior chamber. The patient underwent a lensectomy and anterior vitrectomy in the left eye. At six months postoperatively, the BCVA was 20/200 in the left eye. Lens dislocation in patients with megalocornea is rare. Cataract surgery in these patients requires attention to the zonular abnormalities and lens enlargement, resulting in increased rates of intraoperative and postoperative complications. Ophthalmologists should be able to diagnose this rare disorder and manage the associations and complications.
Atrial Fibrillation and Resistant Stroke: Does Left Atrial Appendage Morphology Matter? A Case Report
Authors: Cristina Sarti, Miroslava Stolcova, Giulia Domna Scrima, Fabio Mori, Ylenia Failli, Donatella Accavone, Silvia Biagini, Costanza Maria Rapillo, Patrizia Nencini, Alessio Mattesini, Carlo Di Mario, Francesco Meucci
Journal: Front Neurol (2020)
Keywords: anticoagulants, atrial fibrillation, case-report, left atrial appendage occlusion, recurrent stroke, resistant stroke
MeSH terms: Case Reports

Introduction: Patients with atrial fibrillation (AF) can experience ischemic stroke despite adequate anticoagulant therapy. The secondary prevention strategy of these so-called "resistant strokes" is empirical. Since about 90% of patients with ischemic stroke due to atrial fibrillation have thrombus in left atrial appendage (LAA) we sought to explore the possibility that resistant stroke could have a LAA morphology resistant to anticoagulants. Case Report: A 77 years old man affected by AF experienced two cardioembolic ischemic stroke while on anticoagulants. The study of LAA showed a windsock-like morphology in the proximal part while distally the LAA presented a cauliflower morphology with a large amount of pectinate muscles and blood stagnation. The precise characteristics of LAA were properly understood integrating images obtained by cardiac CT, transesophageal echocardiography, and selective angiography. A high risky LAA for thrombus formation was diagnosed and its occlusion (LAAO) as an add-on therapy to anticoagulants was proposed and performed. Six month follow-up was uneventfully. Conclusion: The systematic study of LAA in patients with resistant-stroke could help to identify LAA malignant morphology. The efficacy on stroke recurrence of the combined therapy (anticoagulants plus LAAO) is worthy to be tested in randomized trials.
Case report: A rare presentation of high risk epithelioid hemangioendothelioma on leg treated with surgical excision
Authors: Joshua Kazdan, Victoria Sharp, John Pui
Journal: Int J Surg Case Rep (2019)
Keywords: epithelial hemangioendothelioma, epithelioid, high risk, invasive, pathology, surgical excision, vascular tumor

Introduction:
A rare vascular tumor, epithelioid hemangioendothelioma (EHE), can be difficult to diagnose for physicians. Although uncommon, EHE has the potential to become malignant and cause patient death. The five year mortality rate with the diagnosis of the high risk type has been reported to be as high as 41. Thus this finding requires aggressive treatment to prevent amputation or death (Deyrup et al., 2008).
Presentation of case:
In this case, a 60-year-old male patient was evaluated for a suspicious subepidermal nodule on the upper lateral aspect of the lower right leg just distal to the knee. It was excised to the level of muscle with appropriate margins of 1 cm circumferentially. The specimen underwent appropriate pathological testing and did reveal a high-risk-type epithelioid hemangioendothelioma with remaining tumor present at the deep margin. The patient had additional surgery by an orthopedic surgeon in order to obtain clear margins of the lesion and retain maximum function of leg. Despite surgical excision with ultimately clean margins, the lesion did metastasize to the right groin area seven months after surgery and the secondary metastatic site also required surgical excision.
Discussion:
There is very little information available to the identification and treatment of a finding of EHE. The only definitive treatments to prevent malignancy is excision or amputation, possibly in conjunction with radiation therapy. The role of oncology intervention should be considered because the finding may be either a cause or an effect of malignancy.
Conclusion:
The goal of this paper is to raise awareness of the importance of pathology for soft issue lesions even if there is initially a low clinical index of suspicion. Unique characteristics in the pathology is the cornerstone to the identification and treatment. Although surgical excision can appear to be a curative treatment, EHE may inevitably metastasize, so aggressive and definitive treatment is best.
Burkitt's Lymphoma in a Pregnant Woman: Case Report and Review of the Literature
Authors: Carlos Zagalo, Francesca Pierdomenico, José Cabeçadas, Pedro David Santos
Journal: Case Rep Oncol Med (2013)

Burkitt's lymphoma (BL) is an aggressive B-cell malignancy with very high proliferation rate, more common in males than females. Here, we describe a case of Burkitt's lymphoma in a 24-week pregnant woman with cervical and abdominal involvement. The common genetic event of virtually all BL is a reciprocal chromosomal translocation involving the proto-oncogene MYC and one of the Ig gene heavy or light chain loci. Supportive treatment was administered until early delivery, after which the patient was treated according to protocol LMB96. Pregnancy and tumorogenesis share some important events such as immunologic tolerance, angiogenesis, and editing the host immune response. Little is known about the relationship between these events in pregnancy and in tumorogenesis.
Synchronous eyelid oncocytoma and conjunctival intraepithelial neoplasia
Authors: Zachariah Chowdhury, Benjamin Nongrum, Chandana Rajbongshi
Journal: Autops Case Rep (2021)
Keywords: eyelid neoplasms, papilloma, pterygium
MeSH terms: Case Reports

Oncocytoma of the eyelid is a rare neoplasm. Oncocytoma associated with an ocular surface squamous neoplasm, namely conjunctival intraepithelial neoplasia, is very hard to find in the literature. Herein we report a case of a 53-year-old male who presented with a swelling in the right lower lid over the last 6 years, along with a growth in the conjunctiva of the same eye for the last 2 years and encroaching upon the cornea for the last 4 months. Excision biopsy of the lower lid mass showed histopathological features consistent with oncocytoma. The conjunctival tissue revealed conjunctival intraepithelial neoplasia 3 (severe dysplasia). This case documents a rare synchronous dual ocular neoplasia, a very unlikely coexistence of oncocytoma with conjunctival intraepithelial neoplasia.
Plasmablastic Lymphoma Associated with Adjacent Mature Plasma Cell Population Exhibiting Opposite Light Chain Restriction
Authors: Karina Furlan, Ira Miller, Brett Mahon, Fernando A Ocampo Gonzalez, Nicholas Ward
Journal: Case Rep Pathol (2020)
MeSH terms: Case Reports

Plasmablastic lymphoma (PBL) is an aggressive high-grade B cell lymphoma, considered a variant of diffuse large B cell lymphoma with approximately 75% mortality within 6-7 months. We describe an unusual case of PBL arising as a maxillary mass in an HIV-negative, nontransplanted 78-year-old female. Histologic examination revealed a diffuse infiltrate of anaplastic appearing cells exhibiting plasmablastic morphology with an adjacent contiguous infiltrate of mature appearing plasma cells. The PBL and mature plasma cell components both demonstrated an immunophenotype of CD20(-), CD38(+), and CD138(+). The two populations differed by the PBL featuring a high proliferation rate by Ki-67 (~95%) with coexpression of both c-MYC and EBV, while the mature plasma cell component featured a low proliferation rate by Ki-67 (~5%) without coexpression of c-MYC or EBV. Kappa/lambda staining demonstrated lambda light chain restriction involving the PBL, while the mature plasma cell infiltrate revealed kappa light chain restriction. Our findings describe the rare association of PBL with a synchronous distinct population of mature plasma cells exhibiting opposite light chain restriction.
A case report of concurrent embryonal rhabdomyosarcoma and diffuse large B-cell lymphoma in an adult without identifiable cancer predisposition
Authors: M D Mathias, M V Ortiz, H Magnan, S R Ambati, E K Slotkin, A J Chou, M F Walsh, K Offit, C Moskowitz, A Kentsis, L H Wexler
Journal: Biomark Res (2017)
Keywords: case report, diffuse large b cell lymphoma, molecular analysis, rhabdomyosarcoma
MeSH terms: Case Reports

Background:
Diffuse large B-cell lymphoma (DLBCL) is the most common form of non-Hodgkin lymphoma. Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood. makes up less than 1% of solid malignancies in adults with around 400 new cases each year in the United States. They have not previously been reported concurrently.
Case presentation:
A 37 year old woman presented with painful enlarging leg mass. Biopsy of the mass was consistent with embryonal rhabdomyosarcoma. Staging imaging revealed a PET avid anterior mediastinal lymph node. Excisional biopsy of this mass was consistent with diffuse large B-cell lymphoma. Hybridization capture-based next-generation DNA sequencing did not reveal shared somatic tumor mutations. Germline analysis did not show identifiable aberrations of TP53 or other heritable cancer susceptibility genes. She was treated with a personalized chemotherapy regimen combining features of R-CHOP and Children's Oncology Group ARST 0331.
Conclusions:
This case illustrates a unique clinical entity successfully treated with a personalized chemotherapeutic regimen.
A family with Wagner syndrome with uveitis and a new versican mutation
Authors: Pierre-Raphaël Rothschild, Antoine P Brézin, Brigitte Nedelec, Cyril Burin des Roziers, Tiffany Ghiotti, Lucie Orhant, Mathieu Boimard, Sophie Valleix
Journal: Mol Vis (2013)
MeSH terms: Mutation / genetics, Retinal Degeneration / complications, Retinal Degeneration / genetics, Uveitis / complications, Uveitis / genetics, Versicans / deficiency, Case Reports, Research Support, Non-U.S. Gov't, Adolescent, Adult, Aged, Base Sequence, Computer Simulation, Family, Female, Fundus Oculi, Humans, Male, Middle Aged, Molecular Sequence Data, Phenotype, RNA Processing, Post-Transcriptional / genetics, RNA Splice Sites / genetics, RNA, Messenger / genetics, RNA, Messenger / metabolism, Versicans / genetics, Young Adult, RNA Splice Sites, RNA, Messenger, VCAN protein, human, Versicans, Hyaloideoretinal degeneration of Wagner

Purpose:
To report the clinical and molecular findings of a kindred with Wagner syndrome (WS) revealed by intraocular inflammatory features.
Methods:
Eight available family members underwent complete ophthalmologic examination, including laser flare cell meter measurements. Collagen, type II, alpha 1, versican (VCAN), frizzled family receptor 4, low density lipoprotein receptor-related protein 5, tetraspanin 12, and Norrie disease (pseudoglioma) genes were screened with direct sequencing.
Results:
The index case was initially referred for unexplained severe and chronic postoperative bilateral uveitis following a standard cataract surgery procedure. Clinical examination of the proband revealed an optically empty vitreous with avascular vitreous strands and veils, features highly suggestive of WS. The systematic familial ophthalmologic examination identified three additional unsuspected affected family members who also presented with the WS phenotype, including uveitis for one of them. We identified a novel c.4004-6T>A nucleotide substitution at the acceptor splice site of intron 7 of the VCAN gene that segregated with the disease phenotype.
Conclusions:
We present a family with WS with typical WS features and intraocular inflammatory manifestations associated with a novel splice site VCAN mutation. Beyond the structural role in the retinal-vitreous architecture, versican is also emerging as a pivotal mediator of the inflammatory response, supporting uveitis predisposition as a clinical manifestation of WS.
Ifosfamide may be safely used in patients with end stage renal disease on hemodialysis
Authors: Sheron Latcha, Robert G Maki, Gary K Schwartz, Carlos D Flombaum
Journal: Sarcoma (2009)
MeSH terms: Case Reports

Background. Pharmacokinetic data on clearance of ifosfamide in hemodialysis patients are limited. Consequently, these patients are excluded from therapy with this agent. We review the outcomes for patients at our institution with end stage renal disease on dialysis who received ifosfamide for metastatic sarcoma. Patients and Methods. We treated three patients with end stage renal disease on hemodialysis with escalating doses of ifosfamide. Data on radiographic response to therapy, WBC and platelet counts, signs or symptoms of infection, neuropathy and bladder toxicity are reported. Starting doses of ifosfamide were based on review of the literature available with subsequent modifications based on each patient's prior exposure to myelosuppressive agents and on symptoms of neurotoxicity and the degree of myelosuppression following each cycle of chemotherapy. Results. Myelosuppression was the most common side effect from therapy, but no patient developed a life threatening infection, neurotoxicity, or hematuria. One patient developed epistaxis in the setting of thrombocytopenia while on warfarin therapy. All patients had clinical evidence for therapeutic response and two had documented radiographic improvement following ifosfamide administration. Conclusion. Ifosfamide can be used safely in combination with hemodialysis in patients with end stage renal disease.