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Rare Tumors

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https://www.readbyqxmd.com/read/28458793/osteosarcoma-of-the-larynx-treatment-outcomes-and-patterns-of-failure-analysis
#1
Nathan R Bennion, Michael J Baine, Timothy Malouff, Weining Zhen
The incidence of laryngeal sarcoma is exceedingly low with osteosarcomas of the larynx being rarer still, comprising less than 1% of all associated malignancies. To date, only 32 cases have been reported since this pathologic entity was first described in 1942. In this article, we discuss the most recent case of laryngeal osteosarcoma in a patient presenting with respiratory distress found to be due to a tumor mass arising from her cricoid cartilage. We further summarize current knowledge regarding the epidemiology, presentation, and diagnosis of this uncommon disease...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28458792/tracheal-glomus-tumor-a-case-report-and-review-of-the-literature
#2
Ollin Venegas, Andrew Newton, Norge Vergara, Sunil Singhal, Jarrod D Predina
Glomus tumors are rare neoplasms that typically occur within the dermis or subcutis of the subungual space. Primary glomus tumors of the thorax are exceedingly uncommon, thus standard-of-care management is lacking. In this report we describe the management of a patient presenting with a symptomatic glomus tumor of the posterior trachea, and provide a comprehensive review including all documented tracheal glomus tumor reports.
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28458791/adamantinoma-arising-in-the-distal-end-of-the-fibula
#3
Shinichirou Yoshida, Takashi Murakami, Kentarou Suzuki, Shigemi Itou, Munenori Watanuki, Masami Hosaka, Yoshihiro Hagiwara
Adamantinoma is a rare, low-grade, malignant bone tumor. It frequently occurs in the tibia but rarely arise in the distal end of the fibula. This study reported a case of adamantinoma arising in the distal end of the fibula, resulting in good prognosis. A 38-year old female felt left ankle pain, and was suspected as having a bone tumor at the distal end of the fibula by X-ray. She was diagnosed as the classical adamantinoma of the fibula by open biopsy. En bloc wide resection of the tumor, primary arthrodesis of the ankle was performed...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28458790/renal-cancer-in-recipients-of-kidney-transplant
#4
REVIEW
Prajwal Dhakal, Smith Giri, Krishmita Siwakoti, Supratik Rayamajhi, Vijaya Raj Bhatt
The aim of our study is to determine characteristics and outcomes of kidney cancer in renal transplant recipients. MEDLINE® database was searched in June 2015 to identify cases of kidney cancer in renal transplant recipients. We include also a new case. Descriptive statistics were used for analysis. Forty-eight (48) recipients reported in 25 papers met the eligibility criteria. The median age was 47 years (range 9-66); 27% were females. Chronic glomerulonephritis, cystic kidney disease and hypertension were common indications for renal transplant...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28458789/microsatellite-instability-in-medullary-carcinoma-of-the-colon
#5
Mario Martinotti, Fernando Cirillo, Marco Ungari, Giulia Tanzi, Giovanni Rolando, Antonio Tarasconi, Valerio Ranieri, Paolo Aulisa, Marco Vismarra, Massimo Rovatti, Monica Trombatore
Medullary carcinoma (MC) of the large intestine is a relatively new histological type of adenocarcinoma characterized by poor glandular differentiation and an intraepithelial lymphocytic infiltrate. MC can be associated to a defective mechanism for DNA mismatch repair, caused by the so-called microsatellite instability (MSI). We present the case of a 44 years old Caucasian woman, who referred to the Emergency Room with symptoms mimicking an acute appendicitis. Computed tomography and colonoscopy demonstrated an ulcerated and stenotic lesion of the caecum without signs of metastasis and peritoneal carcinosis...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28458788/hairy-cell-lymphoma-a-potentially-under-recognized-entity
#6
William A Hammond, Abhisek Swaika, David Menke, Han W Tun
Hairy cell leukemia (HCL) is a low grade B-cell lymphoproliferative disorder that typically presents with splenomegaly, cytopenias, and diffuse bone marrow infiltration. There have been few cases in the literature of HCL presenting as lymphomas in extra-nodal locations, such as soft tissues and bones without circulating leukemic cells, splenomegaly, or iliac crest bone marrow involvement. We present an additional case presenting as a thoracic mass, and discuss potential diagnostic pitfalls and management of these rare cases...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28458787/myoepithelial-carcinoma-of-the-paracecal-mesentery-aggressive-behavior-of-a-rare-neoplasm-at-an-unusual-anatomic-site
#7
Khin Thway, Jonathan Noujaim, D Michael Thomas, Cyril Fisher, Robin L Jones
Myoepithelial tumors of the soft tissues represent a rare group of neoplasms that vary in their clinical behavior, pathologic features and genetics. They are histopathologically typified by a myoepithelial immunohistochemical phenotype, of expression of one or more epithelial markers, S100 protein and smooth muscle actin. Because of their rarity and occurrence over a wide age range and at a variety of anatomic sites, they can be difficult to diagnose due to the lack of familiarity by physicians, which is compounded by their spectrum of histologic features and morphologic overlap with several other neoplasms...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28458786/large-plasmacytoma-occupying-the-upper-limb-in-a-myeloma-patient
#8
Junichiro Takano, Kensuke Ohikata, Daisuke Nagase, Toru Igari, Kazuhiko Natori, Shotaro Hagiwara
Extramedullary disease (EMD) is an issue for patients with multiple myeloma (MM), since extramedullary spread of MM is associated with an aggressive course and a poor prognosis. Moreover, the mechanism of EMD development is uncertain. Here, we present extensive extramedullary plasmacytoma occupying the left upper limb of a 66-year-old female patient with MM with an extremely aggressive course and multiple visceral organ involvement without bone marrow infiltration or plasma cell leukemia. EMD of this large size is extremely rare and this case may provide a clue for better understanding of clinical features of EMD in MM...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28435647/helical-tomotherapy%C3%A2-is-a-safe-and-feasible-technique-for-total-scalp-irradiation
#9
Francesco Cuccia, Vanessa Figlia, Antonella Palmeri, Francesco Verderame, Antonio Lo Casto, Mariella Mannino, Giuseppe Ferrera
Angiosarcoma of the scalp is a rare aggressive tumor that affects elderly patients. Chemoradiation is the treatment of choice for multicentric and extensive disease. The shape of the scalp represents a dosimetric challenge in terms of achieving a homogeneous concave dose distribution with coverage of the entire target volume and an acceptable organs-at-risk sparing. We report a case of an 81-year-old man with a multifocal angiosarcoma of the scalp treated with Helical TomoTherapy® (Accuray Inc., Sunnyvale, CA, USA) intensity modulated radiotherapy...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28435646/osteosclerosis-secondary-to-metastatic-oligodendroglioma
#10
Patrick R Maloney, Vitor Nagai Yamaki, Ravi Kumar, Derek Johnson, Christopher Hunt, Mark E Jentoft, Michelle Clarke
This paper reviews a case of metastatic 1p/19q codeleted oligodendrioglioma causing diffuse osteosclerosis and pain. Primary central nervous system (CNS) tumors rarely metastasize outside the CNS, and metastatic oligodendroglioma is rarer still. The patient in this study had relief of pain after being treated with temozolomide. We discuss this rare presentation and potential treatment options, and review the literature in regards to metastatic oligodendrogliomas.
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28435645/angiofibrolipoma-of-posterior-mediastinum-with-transforaminal-extension
#11
Monia Attia, Imen Megdiche, Henda Neji, Ameur Belhadj, Ines Baccouche, Soussen Hantous Zannad, Khaoula Ben Miled M'rad
Angiofibrolipoma is a histological variant of lipoma, which commonly occurs in subcutaneous tissues. In the present report we illustrate the case of an angiofibrolipoma of the posterior upper mediastinum in a 75-year-old man presented with progressive chest pain. Xray chest showed a homogeneous opacity vertically oriented along the right lateral aspect of thoracic vertebrae with an obtuse angle to the mediastinum. The upper extremity of the mass extended above the superior clavicle, suggestive of a posterior mediastinal lesion...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28435644/immunohistochemical-diagnosis-of-primary-cardiac-leiomyosarcoma-in-a-latin-american-patient
#12
Ruben Blachman-Braun, Carlos Manuel Aboitiz-Rivera, Alberto Aranda-Fraustro, Adrián Ransom-Rodríguez, Mario Enrique Baltazares-Lipp, Jorge Manuel Catrip-Torres, Jesús Octavio Martínez-Reding
Primary cardiac malignancies are rare entities. Although sarcomas enclosed the main group of malignant heart neoplasms, primary cardiac leiomyosarcomas are extremely rare and constitutes less than 8% of cardiac tumors. Leiomyosarcoma usually originates from the pulmonary veins and have a worm-like shaped structure. In this article, we present a case of a 40-year-old Hispanic man diagnosed with a cardiac tumor who underwent surgical resection, during pathological examination the tissue samples were consistent with a malignancy of mesenchymal origin that contained irregular bundles of spindle cells...
March 24, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28191296/dedifferentiated-liposarcoma-of-the-esophagus-a-case-report-and-selected-review-of-the-literature
#13
Christopher L Brett, Daniel H Miller, Liuyan Jiang, Herbert C Wolfsen, Steven Attia, Lauren Hintenlang, Niveditha Jagadesh, Robert C Miller
Soft tissue sarcomas of the esophagus represent an extremely rare cause of esophageal masses, and an even smaller proportion of these tumors represent dedifferentiated liposarcomas. We present a case of a 75-year-old gentleman presenting with dysphagia found to have a 5 cm pedunculated mass in the cervical esophagus, originating at the cricopharyngeus. This was found to have involvement limited to the superficial mucosa by endoscopic ultrasound, and the lesion was subsequently resected endoscopically. Pathology demonstrated an undifferentiated pleomorphic sarcoma later determined to represent dedifferentiated liposarcoma after fluorescence in situ hybridization analysis...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191295/small-cell-carcinoma-of-the-prostate-in-an-elderly-patient-a-case-report-and-review-of-the-literature
#14
Dale Alan Whitaker, Daniel H Miller, Niveditha Jagadesh, Gerald W Strong, Lauren Hintenlang, William B Schenk, Gregory A Broderick, Katherine S Tzou, Steven J Buskirk
Prostate cancer is the most common malignancy of men in the United States. Small-cell carcinoma (SCC), which typically presents as an aggressive lung malignancy, is a rare diagnosis within the setting of prostate cancer pathology. Due to its limited prevalence, little information regarding the treatment and prognosis of this disease in large populations is available. To date our current knowledge base is largely limited to case reports and retrospective case reviews. The mainstay of treatment for this particular histology most often involves a multimodality approach utilizing chemotherapy in conjunction with radiation therapy, androgen deprivation therapy, or prostatectomy...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191294/complete-remission-of-locally-advanced-penile-squamous-cell-carcinoma-after-multimodality-treatment
#15
Yifan Meng, Helen Levey Bernie, Tzu-Hua Weng, Dean-An Ling, Edward M Messing, Elizabeth Guancial
Treatment of locally advanced penile squamous cell carcinoma (pSCC) remains highly controversial secondary to disease rarity and lack of prospective randomized controlled trials. The current mainstays of care are multi-modality treatment with neoadjuvant chemotherapy and surgery. However, clinicians often have difficulty making recommendations for patients unable to tolerate chemotherapy or surgery due to scarcity of data to guide clinical decision-making. We report two cases of locally advanced pSCC that achieved complete remission after treatment with cisplatin-based neoadjuvant chemotherapy and surgery in one case, and concurrent cisplatin chemoradiation in a second, supporting the use of chemotherapy as part of first-line multimodal therapy...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191293/the-imaging-and-pathological-features-of-metastatic-leiomyosarcoma-in-the-gallbladder
#16
Yi Guo, Eleanor Chen, Darin J Davidson, Venu G Pillarisetty, Robin L Jones, Seth M Pollack
Uterine leiomyosarcoma is a rare and aggressive malignancy with poor overall prognosis. There have been few reports of metastatic leiomyosarcoma in the gallbladder. We report a case of a 41-year-old female who underwent total abdominal hysterectomy due to presumed uterine fibroids. The postoperative pathology revealed high-grade pleomorphic leiomyosarcoma, with involvement of the uterine serosal surface. She subsequently underwent exploratory laparotomy, followed by pelvic radiation and chemotherapy. Since initial management she has developed metastatic disease and has been under treatment and surveillance for 11 years...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191292/oncocytic-variant-of-medullary-thyroid-carcinoma-a-rare-case-of-sporadic-multifocal-and-bilateral-ret-wild-type-neoplasm-with-revision-of-the-literature
#17
Gian Luca Rampioni Vinciguerra, Niccolò Noccioli, Claudia Cippitelli, Angelo Minucci, Ettore Capoluongo, Armando Bartolazzi
Oncocytic variant of medullary thyroid carcinoma (OV-MTC) is a very unusual entity, up to date only 17 cases have been reported in the literature. MTC is a neuro-endocrine malignancy arising from the para-follicular C cells of the thyroid gland. It generally has a slight female predominance and appears as a single lesion. However in the Multiple Endocrine Neoplasia Syndrome 2, linked to the point mutation of RET oncogene, multifocal MTCs may also occur. Herein, we report the case of a 75 years old man with a rare form of sporadic multifocal and bilateral OV-MTC expressing wild-type RET gene...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191291/diffuse-follicular-variant-of-papillary-thyroid-carcinoma-a-case-report-with-a-revision-of-literature
#18
Gian Luca Rampioni Vinciguerra, Niccolò Noccioli, Armando Bartolazzi
The diffuse follicular variant of papillary thyroid carcinoma (DFV-PTC) is a rare malignant thyroid condition. It represents an uncommon variant of papillary carcinoma characterized by a diffuse involvement of thyroid parenchyma, follicular architecture and nuclear features of PTC in absence of a surrounding capsule. Up to date few data have been collected about this entity and, at the best of our knowledge, only 24 cases have been reported in the literature. According to these reports DFV-PTC seems to occur preferentially in young women and shows more aggressive behavior than other papillary thyroid tumors...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191290/loss-of-efficacy-of-pasireotide-after-its-re-administration-is-there-a-reason-why
#19
Stylianos Mandanas, Lemonia Mathiopoulou, Maria Boudina, Alexandra Chrisoulidou, Kalliopi Pazaitou-Panayiotou
Pasireotide is a recently approved medical treatment for persistent or recurrent Cushing's disease (CD). However, an escape from the initial successful response has not yet been described. A 42-year-old female presented with several symptoms indicative of hypercortisolism. Biochemical evaluation and imaging were consistent with CD due to a pituitary adenoma. Surgical excision of the adenoma was unsuccessful and gamma-knife radiosurgery was followed. Our patient remained hypercortisolemic thus treatment with pasireotide (900 mg subcutaneously twice daily) was decided...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191289/renal-cell-carcinoma-metastatic-to-the-scalp
#20
Mounir Errami, Vitali Margulis, Sergio Huerta
Because of the asymptomatic natural history of renal cell carcinoma (RCC), by the time a diagnosis is made, metastatic disease is present in about one third of the cases. Thus, the overall survival of patients with RCC remains poor. Ultimately up to 50% of patients with RCC will develop metastases. Metastatic lesions from RCC are usually observed in the lungs, liver or bone. Metastases to the brain or the skin from RCC are rare. Here we present a patient diagnosed with RCC, found to have no evidence of metastases at the time of nephrectomy, who presented two years later with metastases to the scalp...
November 17, 2016: Rare Tumors
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