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Jessica G Labadie, Aleksandra G Florek, Timothy VandenBoom, Pedram Yazdan, Aleksandar L Krunic
We present a 25-year-old male patient with a primary cutaneous primitive neuroectodermal tumor (cPNET) with unusual immunohistochemistry and lack of fusion oncogene generation. The lesion expressed CD99 and WT-1, and the histological features were consistent with cPNET. Differential diagnoses such as rhabdomyosarcoma, desmoplastic small round blue cell tumor, hematolymphoid neoplasm, neuroblastoma, and CIC-DUX round cell sarcoma were ruled out based on immunohistochemistry, genetic studies, and histology. Previous cPNET cases have been published detailing abnormal immunochemistry and genetic expression...
July 31, 2018: American Journal of Dermatopathology
Angel Fernandez-Flores, José M Suarez Peñaranda, Gonzalo De Toro, Cesar C Alvarez Cuesta, Maria T Fernández-Figueras, Werner Kempf, Carlos Monteagudo
High-endothelial venules are a common feature of 3 types of cutaneous pseudolymphomas: pretibial lymphoplasmacytic plaque (PLP) of children, acral pseudolymphomatous angiokeratoma of children (APACHE), and T-cell rich angiomatoid polypoid pseudolymphoma (TRAPP). In addition, primary cutaneous angioplasmacellular hyperplasia (PCAH) overlaps with these other 3 conditions. We intend to study the expression of peripheral node addressins in PLP, APACHE, TRAPP, and PCAH. We studied 1 case of PLP, 2 cases of APACHE, 2 cases of TRAPP, and 2 cases of PCAH...
July 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
David A Jacobsohn, Michael R Loken, Mingwei Fei, Alexia Adams, Lisa Eidenschink Brodersen, Brent R Logan, Kwang Woo Ahn, Bronwen E Shaw, Morris Kletzel, Marie Olszewski, Sana Khan, Soheil Meshinchi, Amy Keating, Andrew Harris, Pierre Teira, Reggie E Duerst, Steven P Margossian, Paul L Martin, Aleksandra Petrovic, Christopher C Dvorak, Eneida R Nemecek, Michael W Boyer, Allen R Chen, Jeffrey H Davis, Shalini Shenoy, Sureyya Savasan, Michelle P Hudspeth, Roberta H Adams, Victor A Lewis, Albert Kheradpour, Kimberly A Kasow, Alfred P Gillio, Ann E Haight, Monica Bhatia, Barbara J Bambach, Hilary L Haines, Troy C Quigg, Robert J Greiner, Julie-An Talano, David C Delgado, Alexandra Cheerva, Madhu Gowda, Sanjay Ahuja, Mehmet Ozkaynak, David Mitchell, Kirk R Schultz, Terry J Fry, David M Loeb, Michael A Pulsipher
We enrolled 150 patients in a prospective multicenter study of children with acute myeloid leukemia undergoing hematopoietic stem cell transplantation (HSCT) to compare the detection of measurable residual disease (MRD) by a "difference from normal" flow cytometry (ΔN) approach with assessment of Wilms tumor 1 (WT1) gene expression without access to the diagnostic specimen. Prospective analysis of the specimens using this approach showed that 23% of patients screened for HSCT had detectable residual disease by ΔN (...
June 19, 2018: Biology of Blood and Marrow Transplantation
John K Chan, William Brady, Bradley J Monk, Jubilee Brown, Mark S Shahin, Peter G Rose, Jae-Hoon Kim, Angeles Alvarez Secord, Joan L Walker, David M Gershenson
OBJECTIVES: To determine the efficacy and tolerability of sunitinib in recurrent or persistent clear cell ovarian cancer patients. METHODS: All patients had one or two prior regimens with measurable disease. Tumors were at least 50% clear cell histomorphology and negative for WT-1 antigen and estrogen receptor expression by immunohistochemistry. Sunitinib 50 mg per day for 4 weeks was administered in repeated 6-week cycles until disease progression or prohibitive toxicity...
August 2018: Gynecologic Oncology
Di Zhou, Zhiwei Quan, Jiandong Wang
Primary and secondary intrahepatic malignant mesothelioma (PIHMM & SIHMM) caused by Peritoneal mesothelioma (PM) are extremely rare tumors and their clinicopathological characteristics remain unclear. The current study presented a case of a 63-year-old female with PIHMM and a literature review of Chinese case reports of SIHMM and PIHMM was performed. The patient received curative left hemihepatectomy because of a 5.5 × 5.0 × 4.0 cm mass occupying the II, III and the lateral portion of the IV segments and meanwhile tightly infiltrating the diaphragm (yellow arrow) was also observed...
May 2018: Intractable & Rare Diseases Research
Nguyen V Son, James K Chambers, Takanori Shiga, Takuya E Kishimoto, Shotaro Kikuhara, Kohei Saeki, Reina Fujiwara, Masaya Tsuboi, Ryohei Nishimura, Kazuyuki Uchida, Hiroyuki Nakayama
A 12-year-old intact male Welsh Corgi was presented with enlargement of the right scrotum. Both testicles were surgically removed and histopathologically examined. On gross examination, white nodules were found in the epididymis and ductus deferens. Histopathologically, the nodules developed continuously from the tunica vaginalis testis of the right scrotum and consisted of spindle-shaped neoplastic cells that invaded the surrounding tissue. Immunohistochemically, the neoplastic cells were diffusely positive for vimentin, cytokeratin and Wilms tumor-1 (WT-1)...
July 12, 2018: Journal of Veterinary Medical Science
S Wu, D Xia, H X Liu, H P Zhao, X H Wang, Y F Gao, Z Q Zhao, C Xiao, X M Xing, Y He
Objective: To investigate the effects of cerebral cavernous malformation 3 (CCM3) gene knockout on the lead exposure-induced blood-brain barrier malfunction in mice brain, and the relationship between CCM3 knockout and the Alzheimer's disease (AD). Methods: Wide type (WT) mice and CCM3(+)/- mice were divided into 4 groups, control group and lead exposed group in WT as well as CCM3(+/-) mice. Lead exposed groups were treated with 0.05% lead acetate in drinking water for 12 weeks, while control group drink deionized water freely...
May 6, 2018: Zhonghua Yu Fang Yi Xue za Zhi [Chinese Journal of Preventive Medicine]
Ji-Yeong Byeon, Yun Jeong Lee, Young-Hoon Kim, Se-Hyung Kim, Choong-Min Lee, Jung-Woo Bae, Choon-Gon Jang, Seok-Yong Lee, Chang-Ik Choi
Tamsulosin, a selective antagonist of the α1 -adrenoceptor, is primarily metabolized by CYP3A4 and CYP2D6, and tamsulosin exposure is significantly increased according to the genetic polymorphism of CYP2D6. In this study, we investigated the effects of diltiazem, a moderate inhibitor of CYP3A4, on the pharmacokinetics of tamsulosin in subjects with different CYP2D6 genotypes. Twenty-three healthy Korean male subjects with CYP2D6*wt/*wt (*wt = *1 or *2) and CYP2D6*10/*10 were enrolled in the prospective, open-label, two-phase parallel pharmacokinetic study...
May 2018: Archives of Pharmacal Research
David L Kolin, Fei Dong, Michele Baltay, Neal Lindeman, Laura MacConaill, Marisa R Nucci, Christopher P Crum, Brooke E Howitt
Small cell carcinoma of the ovary, hypercalcemic type is a rare, aggressive malignancy which usually occurs in young women and is characterized by mutations in SMARCA4, with few other alterations. We recently encountered uterine tumors with morphologic, immunohistochemical, and genetic similarities to small cell carcinoma of the ovary, hypercalcemic type. Herein we report the clinicopathologic and molecular features (using a targeted massively parallel sequencing [MPS] assay) of these tumors. The cases were diagnosed on cervical and endometrial biopsies (n = 2, 34, and 29 years) or hysterectomy and bilateral salpingo-oophorectomy (n = 3, 25, 33, and 58 years)...
April 26, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Hishaam Ismael, Steven Cox
INTRODUCTION: Primary Intrahepatic mesotheliomas are malignant tumors arising from the mesothelial cell layer covering Glisson's capsule of the liver. They are exceedingly rare with only fourteen cases reported in the literature. They have nonspecific signs and symptoms and need a high index of suspicion and an extensive workup prior to surgery. Surgery remains the mainstay of treatment. PRESENTATION OF CASE: 48 year old male presented with a 3 months history of abdominal pain, productive cough, anemia and weight loss...
2018: International Journal of Surgery Case Reports
Sara Mangosing, Ekaterina Perminov, Olga Gonzalez, Erica K Barkei, Emily M Corbin, Shyamesh Kumar, Edward J Dick
Uterine tumors resembling ovarian sex cord tumors (UTROSCTs) are rare uterine neoplasms that exhibit prominent sex cord-like differentiation. The authors describe 4 cases of UTROSCTs that were identified as incidental lesions in female baboons. All baboons were in good body condition. One animal had a 2-mm-diameter yellow-tan mass in the uterine body along the attachment of the left broad ligament; the other 3 did not have any gross lesions in the uterus. Histologically, the myometrium contained multifocal well-demarcated neoplasms composed of cuboidal to columnar cells arranged in variable arrangements of sheets, nests, cords, trabecular, and retiform patterns that occasionally formed Call-Exner-like bodies...
January 1, 2018: Veterinary Pathology
Emma F Johnson, Dawn M Davis, Megha M Tollefson, Karen Fritchie, Lawrence E Gibson
Vascular tumors in infants present a diagnostic and treatment dilemma for both clinicians and pathologists. Infantile hemangioma, the most common vascular tumor in infants, can be confused for other less common vascular tumors in infants. Correct and timely diagnosis is important, as some vascular tumors can be associated with life-threatening coagulopathy. We present the cases of 5 vascular tumors that have clinical and histologic overlap: infantile hemangioma, pyogenic granuloma, noninvoluting congenital hemangioma, tufted angioma, and kaposiform hemangioendothelioma...
April 2018: American Journal of Dermatopathology
Amanda Przespolewski, Andras Szeles, Eunice S Wang
Evasion of the host immune system is a key mechanism to promote malignant progression. Therapeutically targeting immune pathways has radically changed the treatment paradigm for solid and lymphoid tumors but has yet to be approved for myeloid malignancies. Here, we summarize the most recent advances in immunotherapy for acute myeloid leukemia. Topics reviewed here include adoptive cellular approaches (chimeric antigen receptor-T cells, natural killer and other immune cells), checkpoint inhibitors (anti-PD-1/PD-L1, anti-CTLA-4 and TIM-3) and vaccines (WT-1, HLA-A2 and hTERT)...
April 2018: Future Oncology
Mi-Jung Kim, Ji-Yeong Byeon, Young-Hoon Kim, Se-Hyung Kim, Choong-Min Lee, Eui Hyun Jung, Won Ki Chae, Yun Jeong Lee, Choon-Gon Jang, Seok-Yong Lee, Chang-Ik Choi
Clomiphene citrate, a selective estrogen receptor modulator, is metabolized into its 4-hydroxylated active metabolites, primarily by CYP2D6. In this study, we investigated the effects of the most common CYP2D6 variant allele in Asians, CYP2D6*10, on the pharmacokinetics of clomiphene and its two active metabolites (4-OH-CLO and 4-OH-DE-CLO) in healthy Korean subjects. A single 50-mg oral dose of clomiphene citrate was given to 22 Korean subjects divided into three genotype groups according to CYP2D6 genotypes, CYP2D6*wt/*wt (n = 8; *wt = *1 or *2), CYP2D6*wt/*10 (n = 8) and CYP2D6*10/*10 (n = 6)...
March 2018: Archives of Pharmacal Research
Yasunori Enomoto, Sayomi Matsushima, Shiori Meguro, Hideya Kawasaki, Isao Kosugi, Tomoyuki Fujisawa, Noriyuki Enomoto, Naoki Inui, Yutaro Nakamura, Takafumi Suda, Toshihide Iwashita
Pathological differential diagnoses of pleuroparenchymal fibroelastosis (PPFE) include usual interstitial pneumonia (UIP) and pulmonary apical cap (PAC); however, there are no specific immunostaining makers to distinguish between these diseases. We performed immunohistochemistry using several pleural mesothelial cell-related markers, including cytokeratin-5/6, CAM5.2, WT-1, calretinin, desmin and podoplanin, for PPFE (n = 4), UIP (n = 10) and PAC (n = 3) lung sections. Among the examined markers, in PPFE and PAC lungs podoplanin commonly showed positivity for spindle cells both in thickened pleura and subpleural fibroelastosis lesions; these cells were also stained with α-smooth muscle actin, a marker of myofibroblasts...
June 2018: Histopathology
Ying Zhang, Lin Tao, Can Yin, Weiwei Wang, Hong Zou, Yan Ren, Weihua Liang, Jinfang Jiang, Wenjie Zhang, Wei Jia, Feng Li
Ovarian microcystic stromal tumor is a relatively rare tumor type. This tumor is characterized by a unique microcyst structure and essentially all tumors show benign biological behavior. Here, we report a case with a primary ovarian microcystic stromal tumor that experienced recurrence. Pathological findings showed that the original tumor, relapsed tumor in the ovary, and the recurrent tumor in the iliac fossa presented similar histological features. The tumor mainly consisted of microcysts, solid cellular regions, and a fibrous stroma...
February 16, 2018: Human Pathology
Fatma Tokat, Julia S Lehman, Engin Sezer, Emel Dikicioglu Cetin, Umit Ince, Emel Ozturk Durmaz
Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular proliferative disorder mainly located in the periauricular region. The etiopathogenesis of ALHE is unknown, and it is still controversial as to whether the entity represents a benign vascular neoplasm or an inflammatory process. Aim: Recently, the intracytoplasmic staining pattern of Wilms tumor 1 (WT1) on immunohistochemistry has highlighted true vascular neoplasms, such as microvenular hemangioma, tufted angioma, and spindle cell hemangioma, which has made it helpful to distinguish ALHE from vascular malformations, as there is a negative staining pattern in the other entities...
January 2018: Dermatology Practical & Conceptual
Keita Kanzaki, Daiki Watanabe, Chihiro Aibara, Yuki Kawakami, Takashi Yamada, Yoshitaka Takahashi, Masanobu Wada
It has been shown that calpains are involved in the proteolysis of muscle proteins that occurs with eccentric contraction (ECC) and that exogenously applied nitric oxide decreases the calpain-mediated proteolysis. The aim of this study was to examine the effects of ingestion of l-arginine (ARG), a nitric oxide precursor, on ECC-related calpain activation. In the first and second experiments, male Wistar rats were given ARG in water for 7 days starting from 3 days before the ECC protocol (average ingestion, ~600 mg kg-body wt-1  day-1 )...
January 2018: Physiological Reports
Ashutosh Kumar Rai, Rupesh Chaturvedi, Ashok Kumar
This study deals with the isolation and purification of an important variant of microcystins namely microcystin-RR (MCYST-RR) from Microcystis aeruginosa and reports its effects on mice liver protein profile and cellular functions. Protein profiling by 2-dimensional gel electrophoresis revealed changes in the number and accumulation of protein spots in liver of mice treated with different concentrations of MCYST-RR. Untreated (control) mice liver showed 368 protein spots while the number was 355, 348 and 332 in liver of mice treated with 200, 300 and 400 µg kg body wt-1 of MCYST-RR respectively...
January 22, 2018: Scientific Reports
Noriko Kimura, Masaru Hasegawa, Kenzo Hiroshima
Malignant mesothelioma (MM) with rhabdoid features is an MM variant. Fifteen cases have been reported previously, all of which were combined with other types of MM. Herein, we report an autopsy case of pleural MM with monomorphic rhabdoid features. The patient was a 62-year-old male without a history of asbestos exposure. An autopsy revealed a soft, granular tumor that replaced the entire left pleura and had invaded to the diaphragm and lower lobe of the lung. The tumor cells, which had eosinophilic plump cytoplasm and eccentric nuclei, were loosely cohesive...
February 2018: Pathology International
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