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Very And Rare And Tumors

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https://www.readbyqxmd.com/read/30317238/low-grade-endometrial-stromal-sarcoma-a-review
#1
Falk C Thiel, Sonja Halmen
Like other uterine sarcomas, low-grade endometrial stromal sarcomas (LG-ESS) are a very rare tumor entity. In the past, research studies therefore discussed the various different types of the disease in combination. In addition, the classification of endometrial stromal tumors presented difficulties for quite some time so that in earlier studies it was not always possible to precisely distinguish between LG-ESS, high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. For LG-ESS, surgery with hysterectomy and adnexectomy is the first-line treatment...
October 13, 2018: Oncology Research and Treatment
https://www.readbyqxmd.com/read/30314832/ewing-s-sarcoma-and-or-peripheral-primitive-neuroectodermal-tumorsin-bronchus
#2
REVIEW
Jun Chen, Tao Yuan, Xiao Liu, Bei Hua, Chenfeng Dong, Yawu Liu, Guanmin Quan
Ewing sarcoma/peripheral primitive neuroectodermal tumors (ES/pPNET), a member of the Ewing sarcoma family of tumors, is a malignant soft tissue tumor with small undifferentiated neuroectodermal cells. Primary trachea-bronchial ES/pPNET is very rare. The most common pulmonary ES is due to a metastasis. We describe a case of ES/pPNET which originated in the left basal trunk bronchus. The patient was a 30-year-old male, presenting with irritable cough and fever for 10 days. A tumor of 60 mm in diameter was found in the left basal trunk bronchus, extending to the left lower lobe...
August 22, 2018: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/30314491/wide-excision-and-reconstruction-surgery-for-recurrent-sweat-gland-umbilical-adenocarcinoma-followed-by-chemotherapy-can-prevent-the-risk-of-recurrences
#3
Adeodatus Yuda Handaya, Nova Yuli Prasetyo Budi, Guntur Marganing Adi Nugroho, Aditya Rifqi Fauzi
BACKGROUND: Adenocarcinoma derived from umbilicus is very rare. Most adenocarcinomas in umbilicus are secondary events. Carcinoma derived from sweat glands is sporadic, highly radioresistant and has a clinical appearance that is difficult to predict. CASE PRESENTATION: A 37-year-old woman presented with recurrent umbilicus adenocarcinoma after a history of umbilicus tumor surgery 14 months earlier and Capecitabine chemotherapy six times. Malignant cells were found in Fine Needle Aspiration Biopsy (FNAB) examination...
October 12, 2018: BMC Surgery
https://www.readbyqxmd.com/read/30313044/pathological-features-of-paraganglioma-in-the-lumbar-spinal-canal-a-case-report
#4
Zhe-Hui Wang, Yuan-Tao Wang, Fei Cheng, Yu Hu
INTRODUCTION: This study aims to investigate the pathological features of a patient with paraganglioma in the lumbar spinal canal. CASE PRESENTATION: The patient was 36 years old male with electrical pain in the waist and buttock which occurred intermittently, and was not relieved under oral use of analgesics. Immunohistochemical staining was performed to diagnose the disease. The results revealed that the size of the tumor was 3.4 × 1.6 × 1.4 cm. The hematoxylin and eosin (H&E)-stained tumor cells mainly presented with an organ-like arrangement under low power microscope, showing prominent chrysanthemum-like, pseudo glandular or pseudo papillary arrangements...
October 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/30310176/aberrant-receptor-tyrosine-kinase-signaling-in-lipofibromatosis-a-clinicopathological-and-molecular-genetic-study-of-20-cases
#5
Alyaa Al-Ibraheemi, Andrew L Folpe, Antonio R Perez-Atayde, Kyle Perry, Jakob Hofvander, Elsa Arbajian, Linda Magnusson, Jenny Nilsson, Fredrik Mertens
Lipofibromatosis is a rare pediatric soft tissue tumor with predilection for the hands and feet. Previously considered to represent "infantile fibromatosis", lipofibromatosis has distinctive morphological features, with mature adipose tissue, short fascicles of bland fibroblastic cells, and lipoblast-like cells. Very little is known about the genetic underpinnings of lipofibromatosis. Prompted by our finding of the FN1-EGF gene fusion, previously shown to be a characteristic feature of calcifying aponeurotic fibroma (CAF), in a morphologically typical case of lipofibromatosis that recurred showing features of CAF, we studied a cohort of 20 cases of lipofibromatosis for this and other genetic events...
October 11, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/30306789/a-unique-model-for-sdh-deficient-gist-an-endocrine-related-cancer
#6
James F Powers, Brent Cochran, James D Baleja, Hadley D Sikes, Xue Zhang, Inna Lomakin, Troy Langford, Kassi Taylor Stein, Arthur S Tischler
We describe a unique patient-derived xenograft (PDX) and cell culture model of succinate dehydrogenase-deficient gastrointestinal stromal tumor (SDH-deficient GIST), a rare mesenchymal tumor that can occur in association with paragangliomas in hereditary and non-hereditary syndromes. This model is potentially important for what it might reveal specifically pertinent to this rare tumor type and, more broadly, to other types of SDH-deficient tumors. The primary tumor and xenografts show a very high proliferative fraction, and distinctive morphology characterized by tiny cells with marked autophagic activity...
November 1, 2018: Endocrine-related Cancer
https://www.readbyqxmd.com/read/30306232/-genetics-of-pheochromocytoma-and-the-relevance-in-surgery
#7
REVIEW
E von Dobschütz, H P H Neumann
Chromaffin tumors, e.g. pheochromocytomas and paragangliomas are caused by germline mutations of several susceptibility genes in 30-40% of the patients. The corresponding syndromes are multiple endocrine neoplasia type 2 (MEN2, RET gene), von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF1), paraganglioma syndrome types 1-5 (PGL1-5, SDHx gene) and familial pheochromocytoma due to mutations in the MAX and TMEM127 genes. Clinically, screening for such diseases should be carried out by clinical symptoms and mutation analyses...
October 10, 2018: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
https://www.readbyqxmd.com/read/30305848/primary-central-nervous-system-lymphoma
#8
REVIEW
Sarah Löw, Catherine H Han, Tracy T Batchelor
Primary central nervous system lymphoma (PCNSL) is a rare and aggressive extranodal non-Hodgkin lymphoma (NHL), confined to the brain, eyes, spinal cord or leptomeninges without systemic involvement. Overall prognosis, diagnosis and management of PCNSL differ from other types of NHL. Prompt diagnosis and initiation of treatment are vital to improving clinical outcomes. PCNSL is responsive to radiation therapy, however whole-brain radiotherapy (WBRT) inadequately controls the disease when used alone and its delayed neurotoxicity causes neurocognitive impairment, especially in elderly patients...
2018: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/30305573/-a-case-of-obstructive-pancreatitis-induced-by-extramedullary-pancreatic-metastasis-in-a-patient-with-immunoglobulin-d-multiple-myeloma
#9
Masahiro Umemura, Takeshi Chida, Erika Matsunaga, Jun Ito, Kazuyoshi Ohta, Shin Shimoyama, Satoru Yamazaki, Kazuhito Kawata, Takaaki Ono, Yoshimasa Kobayashi
A 51-year-old Brazilian female who had IgD-lambda type multiple myeloma presented with epigastralgia and obstructive jaundice during her follow-up. Contrast-enhanced computed tomography (CT) showed an enhanced mass of 25mm in the pancreatic head, and endoscopic retrograde cholangiopancreatography revealed smooth stenoses in the lower bile duct and main pancreatic duct (MPD) of the head. We diagnosed the patient with extramedullary pancreatic metastasis of multiple myelomas. Plastic stents were endoscopically placed into both the common bile duct and MPD...
2018: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/30304733/challenging-diagnosis-of-a-solitary-retroperitoneal-mass-a-case-report-of-castleman-s-disease-and-review-of-the-literature
#10
Diego M Carrion, Mario Alvarez-Maestro, Juan Gómez Rivas, Yaroslav Brygadyr, Eugenia García-Fernandez, Luis Martínez-Piñeiro
Castleman's disease (CD) is an uncommon type of lymphoproliferative disorder. Its etiology and prevalence are unclear. The retroperitoneum is a very rare site for presentation of the unicentric variant, where it mimics malignant tumors. A 59-year-old man is referred to the urology outpatient clinic for the study of microhematuria found in a routine analysis. CT scan of the abdomen identified a solid, circumscribed mass, measuring 28 × 30 × 31 mm in the left para-aortic zone, with homogeneous contrast enhancement...
October 10, 2018: Urologia Internationalis
https://www.readbyqxmd.com/read/30303161/malignant-sertoli-cell-tumor-of-the-testis-masquerading-as-seminoma-with-bone-metastasis
#11
Chaturbhuj Ramanand Agrawal, Venkata Pradeep Babu Koyyala, Vineet Talwar, Juhi Tayal, Dharma Ram Poonia
Sex cord-stromal tumors of the testes are rare malignancies as compared to germ cell tumors. Pure Sertoli cell tumors are still rare representing <1% of testicular cancers and the malignant forms are too rare. Furthermore, the occurrence of metastasis in such cases is extremely rare with <30 cases reported in literature so far to the best of our knowledge. We present herein a case of malignant Sertoli cell tumor in a 48-year-old male who was initially misdiagnosed as seminoma based on histology and clinical presentation...
October 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/30303159/primary-intrauterine-dysgerminoma-in-a-pregnant-woman-a-rare-case-report
#12
Mojgan Akbarzadeh-Jahromi, Fatemeh Sari Aslani, Fatemesadat Najib, Shahla Hosseini
Primary extraovarian dysgerminoma is very rare. Nearly all reported uterine germ cell tumors are nondysgerminoma. Herein, we reported a primary intrauterine dysgerminoma. A 21-year-old pregnant woman G2 L1 with a gestational age of 33 weeks referred to an obstetric ward with a chief complaint of labor pain and membrane rupture. Ultrasonography showed a large hypoechoic lobulated area adjacent to the lower part of her uterus. She underwent an operation and a huge mass was detected in her uterus, which was extended to her pelvic floor...
October 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/30300686/genetic-hypercalcemia
#13
Catherine Cormier
A genetic disorder should be suspected in patients with hypercalcemia, notably those who are young; have family members with hypercalcemia; or have had a tumor of the endocrine pancreas, thyroid, pituitary, adrenal gland, or jaw bone. All forms of hypercalcemia should be interpreted according to the serum level of parathyroid hormone (PTH). Genetic forms are thus classified as related or unrelated to a parathyroid gland disorder. When the PTH level is elevated or is not depressed despite the hypercalcemia, findings that suggest hyperparathyroidism due to a genetic cause include syndromic manifestations in the patient or family members, parathyroid cancer (either suspected before surgery or confirmed during parathyroidectomy), multiple or recurrent parathyroid tumors, a family history of primary hyperparathyroidism, and the onset of primary hyperthyroidism before 50 years of age...
October 6, 2018: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/30297384/clinical-benefit-to-an-aurora-a-kinase-inhibitor-in-a-patient-with-metastatic-integrase-interactor-1-deficient-carcinoma
#14
Theodoros Karantanos, Lisa Rooper, Youme Kang, Cheng Ting Lin, Pawla Wenga, Sarah Sagorsky, Josh Lauring, Hyunseok Kang
Integrase interactor 1 (INI-1)-deficient carcinoma is a rare cancer characterized by the loss of the SWItch/Sucrose Non-Fermentable-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 gene ( SMARCB1 ) and tends to follow an aggressive clinical course. There is no currently available standard therapy option, although a few promising treatment strategies, including enhancer of zeste homolog 2 (EZH2) inhibition, are under active investigation. This report describes a 30-year-old woman with INI-1-deficient carcinoma who progressed on combination chemotherapy and an EZH2 inhibitor...
October 8, 2018: Oncologist
https://www.readbyqxmd.com/read/30291889/anaplastic-large-cell-lymphoma-of-the-palate-a-case-report
#15
P Lapthanasupkul, K Songkampol, K Boonsiriseth, N Kitkamthorn
Anaplastic large cell lymphoma (ALCL) is a very rare subtype of T-cell non-Hodgkin's lymphoma (NHL). Similar to other types of NHL, ALCL primarily involves the nodal areas and sometimes it can involve several extranodal sites such as skin, lung and soft tissue. Primary oral involvement of systemic ALCL is very rare. We report a 55-year-old Thai female with anaplastic lymphoma kinase (ALK)-negative ALCL primarily occurring at the hard palate. The patient was referred to the Department of Oral and Maxillofacial Surgery, Mahidol University complaining of a swelling on her left palate...
October 3, 2018: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/30288189/intramuscular-myxoma-in-the-supinator-muscle-with-transient-postoperative-posterior-interosseous-nerve-palsy-a-case-report
#16
Toshihiro Nonaka, Motoki Sonohata, Shuhei Takeshita, Yosuke Oba, Yoshimasa Fujii, Masaaki Mawatari
Background: Intramuscular myxomas are rare, benign mesenchymal tumors in the musculoskeletal system, and usually, the tumors arise in the large muscles of the thigh, buttocks, shoulder, and upper arm. However, a tumor of the forearm is very rare. Herein, we describe the case of an intramuscular myxoma in the supinator muscle of a 56-year-old female patient. Case Presentation: Magnetic resonance imaging showed a well-defined mass that was hypointense with the peritumoral fat ring sign...
2018: Open Orthopaedics Journal
https://www.readbyqxmd.com/read/30288003/malignant-spindle-cell-tumor-breast-a-diagnostic-dilemma
#17
Sheetal Arora, Deepshikha Rana, Mukta Pujani, Varsha Chauhan
Primary malignant spindle cell tumors are rare constituting 1.0% of breast malignancies. Spindle cell lesions occurring in soft tissues can occur in breast with overlapping morphologies. It can present as benign lesion and have inconclusive cytological findings, so easily missed if not properly dealt with. Stromal sarcoma should be diagnosed only after thorough sectioning and negative staining for p63, broad spectrum, and high molecular weight keratin. We present a case of right breast lump. Cytological features revealed fibro histiocytic lesion...
September 2018: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/30287803/primary-intrahepatic-squamous-cell-carcinoma-with-histological-collision-of-adenocarcinoma-and-squamous-cell-carcinoma-a-case-report
#18
Kohei Tamaoka, Masahiro Tanemura, Kenta Furukawa, Manabu Mikamori, Takuro Saito, Masahisa Ohtsuka, Yozo Suzuki, Mitsuyoshi Tei, Kentaro Kishi, Hironao Yasuoka, Masahiko Tsujimoto, Hiroki Akamatsu
BACKGROUND Cholangiocarcinoma is a rare, aggressive biliary tract malignancy. On histopathology, most tumors are adenocarcinomas, while squamous cell carcinoma of the biliary tract is extremely rare. CASE REPORT An 82-year-old male was admitted due to the detection of a space-occupying lesion at S6 of the liver. On abdominal dynamic computed tomography, there was an irregular mass with inhomogeneous density associated with mild delayed enhancement in the tumor's peripheral zone, measuring approximately 22×25 mm, at S6, with secondary dilated biliary ducts of B6...
October 5, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/30286282/nucleus-of-circulating-tumor-cell-determines-its-translocation-through-biomimetic-microconstrictions-and-its-physical-enrichment-by-microfiltration
#19
Yiqiu Xia, Yuan Wan, Sijie Hao, Merisa Nisic, Ramdane A Harouaka, Yizhu Chen, Xin Zou, Si-Yang Zheng
The mechanism of cells passing through microconstrictions, such as capillaries and endothelial junctions, influences metastasis of circulating tumor cells (CTCs) in vivo, as well as size-based enrichment of CTCs in vitro. However, very few studies observe such translocation of microconstrictions in real time, and thus the inherent biophysical mechanism is poorly understood. In this study, a multiplexed microfluidic device is fabricated for real-time tracking of cell translocation under physiological pressure and recording deformation of the whole cell and nucleus, respectively...
October 4, 2018: Small
https://www.readbyqxmd.com/read/30283584/primary-intracranial-manifestation-of-a-carcinosarcoma
#20
Reza Akhavan-Sigari, Hesam Abdolhoseinpour, Werner Meyer, Veit Rohde, Walter Schulz-Schaeffer
Carcinosarcomas are tumors comprising part adenocarcinoma and part sarcoma; the presence of carcinosarcoma in the head-and-neck region is very rare. These tumors are typically highly aggressive (G3) and arise most frequently from the salivary gland. Here, we present a case report on a brain metastasis as the primary manifestation of a carcinosarcoma. Magnetic resonance imaging showed a tumor of the pineal region with infiltration of the brainstem and the corpus. The staging following the histopathological diagnosis revealed the origin of the tumor in the left parotid gland...
July 2018: Asian Journal of Neurosurgery
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