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Rare Tumors

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https://www.readbyqxmd.com/read/27746888/primary-hepatic-lymphoma-a-retrospective-multicenter-rare-cancer-network-study
#1
Gamze Ugurluer, Robert C Miller, Yexiong Li, Juliette Thariat, Pirus Ghadjar, Ulrike Schick, Mahmut Ozsahin
Primary hepatic lymphoma (PHL) is a rare malignancy. We aimed to assess the clinical profile, outcome and prognostic factors in PHL through the Rare Cancer Network (RCN). A retrospective analysis of 41 patients was performed. Median age was 62 years (range, 23-86 years) with a male-to-female ratio of 1.9:1.0. Abdominal pain or discomfort was the most common presenting symptom. Regarding B-symptoms, 19.5% of patients had fever, 17.1% weight loss, and 9.8% night sweats. The most common radiological presentation was multiple lesions...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746887/soft-tissue-myoepithelial-carcinoma-of-the-neck-with-spinal-invasion
#2
Elias Moussaly, Bassel Nazha, Shiksha Kedia, Qing Chang, Frank Forte
Soft tissue myoepithelial neoplasms are a rare yet diverse group of tumors, ranging from benign to malignant lesions. Their presentation in the head and neck region is uncommon and represents a challenging diagnosis. Early identification of myoepithelial carcinoma is crucial given its more aggressive course compared to its benign counterpart, although the histopathological distinction between the two can be difficult. EWSR1 gene rearrangement is found in half the cases and has a speculative role in pathogenesis...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746886/unusual-ultrasound-presentation-of-testicular-metastasis-from-renal-clear-cell-carcinoma
#3
Lucio Dell'Atti
Testicular metastases from renal clear cell carcinoma (RCC) are extremely uncommon. To the best of our knowledge, only 32 cases have been reported in the literature. We report a rare case of testicular metastasis from RCC. A 69-year-old patient presented with discomfort and pain in his left testis. He had undergone laparoscopic left radical nephrectomy at another institution. Scrotal ultrasonography revealed a non-palpable lesion at the upper pole of the left testis with hypoechoic aspect, highly suspicious for malignancy...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746885/locally-advanced-stage-high-grade-mucoepidermoid-carcinoma-of-salivary-gland-in-a-9-year-old-girl-the-controversy-of-adjuvant-therapy
#4
Olga Micol Martínez, Elena Daghoum Dorado, María Dolores Amorós García, María Isabel Oviedo Ramírez, Isabel de la Fuente Muñoz, Jose Luis Fuster Soler
Malignant salivary gland tumors are rare in children, mostly represented by low-grade mucoepidermoid carcinomas. For these patients, long-term survival rates above 95% are reported after surgical resection. Here we report a case of a 9-year-old girl with a high grade locally advanced mucoepidermoid carcinoma undergoing adjuvant radiotherapy and chemotherapy after surgery. We emphasize the controversy and lack of evidence-based indication for these highly toxic adjuvant therapy modalities in children.
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746884/erlotinib-induced-fatal-interstitial-lung-disease-in-a-patient-with-metastatic-non-small-cell-lung-cancer-case-report-and-review-of-literature
#5
Ankit Mangla, Nikki Agarwal, Chou Carmel, Thomas Lad
Erlotinib is one of the most widely used tyrosine kinase inhibitor targeting human epidermal growth factor receptor. Since its introduction, it has revolutionized the treatment of advanced non-small cell lung cancer. Skin rashes and diarrhea are the most often reported side effects of erlotinib however it is also associated with interstitial pneumonitis or interstitial lung disease, which often turns out to be fatal complication of using this medicine. Though reported scarcely in the western world, the association of interstitial lung disease with epidermal growth factor receptor has attracted a lot of attention in the recent times...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746883/solitary-fibrous-tumor-of-the-liver-a-rare-tumor-in-a-rarer-location
#6
Biswajit Dey, Debasis Gochhait, Gourav Kaushal, Adarsh Barwad, Biju Pottakkat
Solitary fibrous tumor is an uncommon mesenchymal neoplasm. Liver is a rare location of this tumor. We report a case of hepatic solitary fibrous tumor in a 56-year-old female, who presented with right upper abdominal pain. An extended right hepatectomy was performed. Histopathological and immunohistochemical examination revealed solitary fibrous tumor of the liver.
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746882/primary-retroperitoneal-melanoma-presented-in-a-rare-extracutaneous-site-for-malignant-melanoma
#7
Mohamed Alsharedi, Nadim Bou Zgheib, Yousef Khelfa, Ali Raufi, Nabiha Elmsherghi, Yehuda Lebowicz
Malignant melanoma, as the name implies, is a malignant tumor of melanocytes, found in the skin, eyes, meningeal lining and the mucosal epithelium of the aero-digestive and genitourinary tracts. Malignant melanoma is typically skin malignancy, which rarely presents at extracutaneous site. Here we present a rare case of primary retroperitoneal melanoma and review the findings in comparison with other cases described in literature.
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746881/gastric-metastasis-of-breast-cancer-a-case-series
#8
Gustavo Dos Santos Fernandes, Luiza D Batista Bugiato Faria, Isadora de Assis Pereira, Natália C Moreira Neves, Yasmine Oliveira Vieira, Alessandro I Cavalcanti Leal
Gastric metastasis is rare but it can be the initial symptom of cancer. The second leading cause of this type of metastasis is breast cancer. A lack of clinical signs and nonspecific side effects of the treatment of primary tumors can lead to the misdiagnosis of metastatic gastric cancer. Upper gastrointestinal endoscopy with biopsy and immunohistochemistry should be used for diagnosis. Treatment is palliative; it includes chemo, endocrine, and radiation therapies. Four patients with breast cancer and gastric metastasis were identified...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746880/a-giant-phyllodes-tumor-of-the-breast
#9
Charlotte Schillebeeckx, Guy Verbeeck, Geert Daenen, Dirk Servaes, Marc Bronckaers
Phyllodes tumors of the breast are rare, accounting for less than 1% of the breast tumors. They are mostly seen in women between 45 and 49 years old. These are fast growing tumors with a large spectrum of behavior (from benign to metastatic) and can resemble fibroadenomas. Correct diagnosis mostly through core needle biopsy is important to decide whether a surgical excision has to be done. Here we report a case of a 57-year-old woman with a fast growing, ulcerated tumor in the left breast. Core needle biopsy suggested a malignant phyllodes tumor with heterologous liposarcomatous differentiation...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746879/rare-aggressive-behavior-of-mdm2-amplified-retroperitoneal-dedifferentiated-liposarcoma-with-brain-lung-and-subcutaneous-metastases
#10
Imen Ben Salha, Shane Zaidi, Jonathan Noujaim, Aisha B Miah, Cyril Fisher, Robin L Jones, Khin Thway
Dedifferentiated liposarcoma (DDL) is a histologically pleomorphic sarcoma, traditionally defined as well-differentiated liposarcoma with abrupt transition to high grade, non-lipogenic sarcoma. It can occur as part of recurrent well-differentiated liposarcoma, or may arise de novo. DDL most frequently occurs within the retroperitoneum, and while it is prone to local recurrence, it usually has a lower rate of metastasis than other pleomorphic sarcomas. We describe a case of retroperitoneal dedifferentiated liposarcoma in a 63-year-old male, who showed MDM2 amplification with fluorescence in situ hybridization, which displayed unusually aggressive behavior, with brain, lung and subcutaneous soft tissue metastases...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746878/national-incidence-management-and-survival-of-urachal-carcinoma
#11
Dearbhaile C Collins, Kyra Velázquez-Kennedy, Sandra Deady, Adrian P Brady, Paul Sweeney, Derek G Power
Urachal carcinoma is an uncommon cancer whose rarity has precluded its study and evidence-based management strategies are lacking. This study assessed all urachal carcinomas in Ireland and clinical parameters in order to improve understanding. Urachal carcinomas diagnosed from 1994 to 2011 were identified from the National Cancer Registry in Ireland. Data obtained included patient age, gender, diagnostic year, pathology, tumor stage, patient treatment strategies and survival. Twenty-six urachal carcinomas were identified, the majority being adenocarcinoma...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746877/cerebellar-liponeurocytoma-a-novel-report-from-nigeria-in-a-6-year-old-girl-and-review-of-literature
#12
Martin A Nzegwu, Samuel Ohegbulam, Chika Ndubuisi, Okwuoma Okwunodulu, Emeka Okorie, Sunday Nkwerem, Onyiye Okonkwo, Onyeka Aniume, Sunday Nnamani, Onyekachi Nwokoro, Anthony Eni, Isaiah Nwideyi, Victor Nzegwu
Liponeurocytoma is a newly defined clinical entity predominantly seen in the cerebellum as a slow-growing tumor. In this report, we present the case of a 6-year-old Nigerian girl with a liponeurocytoma, and review of literature.
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746876/post-transplant-lymphoproliferative-disorder-presenting-as-cd20-negative-plasmablastic-lymphoma-in-the-lung
#13
Tasneem Kaleem, Jennifer A Crozier, David M Menke, Taimur Sher
Post-transplant lymphoproliferative disorders (PTLD) are a serious complication of transplantation with a high mortality. Most PTLD present within the first year of transplantation and are associated with Epstein-Barr virus (EBV) infection. Plasmablastic lymphoma (PBL) is a rare but aggressive disease originally described in patients with HIV, presenting most commonly in the jaw and oral mucosa. To our knowledge, this is the first case of PBL presenting as PTLD of the lung in a HIV and EBV negative patient...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746875/angiosarcoma-a-case-report-of-gingival-disease-with-both-palatine-tonsils-localization
#14
Frédéric Chamberland, Tristan Maurina, Séverine Degano-Valmary, Thierry Spicarolen, Loïc Chaigneau
Angiosarcomas are one of the rarest subtypes of sarcomas; those are malignant vascular tumors arising from vascular endothelial cells. Occurrence of intra-oral angiosarcoma is extremely rare (0.0077% of all cancers in Europe). We present here, to our knowledge, the first case of a 83-year-old man with gingival and both palatine tonsils localization of a grade-two angiosarcoma discovered after a two months history of a painful lesion followed by hematoma and spontaneous bleeding. Chemotherapy with paclitaxel and hemostatic radiotherapy were inefficient and he died seven months after the first symptoms...
September 5, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27441080/paratesticular-fibrous-pseudotumors
#15
Sadi Turkan, Mehmet Kalkan, Ozan Ekmekcioglu, Hacer Haltas, Coskun Sahin
Paratesticular fibrous pseudotumors (PFPs) are rare pathologies with quite wide and variable topographic-morphological features. It is difficult to distinguish PFPs from malignant masses. Treatment can be done by resection of the mass. We reported a young patient's findings about this rare pathology.
June 28, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27441079/three-years-sustained-complete-remission-achieved-in-a-primary-refractory-alk-positive-anaplastic-t-large-cell-lymphoma-treated-with-crizotinib
#16
Carolina Valeria Mahuad, María de Los Ángeles Vicente Repáraz, Marta E Zerga, María Florencia Aizpurua, Claudia Casali, Gonzalo Garate
The prognosis of the primary refractory anaplastic lymphoma kinase (ALK+) anaplastic T large cell lymphoma is ominous. The identification of molecular targets with potential to drive oncogenesis remains a cornerstone for the designing of new selective cancer therapies. Crizotinib is a selective ATP-competitive inhibitor for ALK, approved for its use in lung cancer with rearrangements on ALK gene. The reported cases describe the use of crizotinib as a bridging strategy prior to allotransplantation; there are no reported prolonged survivals under monotherapy with Crizotinib...
June 28, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27441078/nuclear-protein-of-the-testis-midline-carcinoma-masquerading-as-a-primary-mediastinal-seminoma
#17
Maria S Sayapina, Nikita A Savelov, Apollon I Karseladze, Anatoly A Bulanov, Alexey A Tryakin, Dmitry A Nosov, Avgust M Garin, Sergey A Tjulandin
Nuclear protein of the testis (NUT) midline carcinomas are rare aggressive carcinomas characterized by chromosomal rearrangements that involve the gene encoding the NUT. This article reviews the clinicopathologic features and the differential diagnosis of these malignancies.
June 28, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27441077/appendiceal-hemangioma-mimicking-acute-appendicitis-in-a-17-year-old-girl
#18
Bita Geramizadeh, Amin Niakan, Nadjmeh Zolmadjdi, Mahsa Marzban
Epithelial tumors of appendix are not so common, and mesenchymal tumors of the appendix are even less common. Capillary hemangioma of the appendix is an extremely rare event and to the best of our knowledge only 4 cases have been reported in the English literature so far. In this case report we want to explain our experience with an extremely rare occurrence of capillary hemangioma of appendix in a 17-year-old girl presented with right lower quadrant pain that was operated with the clinical impression of acute appendicitis...
June 28, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27441076/recurrent-high-grade-invasive-mucoepidermoid-carcinoma-of-larynx-a-case-report-and-review-of-the-literature
#19
Whitney King, Stephen Ko, Daniel Miller
Recurrent invasive high-grade mucoepidermoid carcinoma of the larynx and hypopharynx is a rare occurrence. These tumors have been commonly associated with salivary gland tumors, most commonly the parotid gland. The patient usually presents with the following symptoms: hoarseness (if larynx is involved), or changes in voice character, sore throat, cough, odynophagia, dysphagia, otalgia, difficulty breathing, weight loss, lymphadenopathy. Here we present a case of a recurrent invasive high-grade mucoepidermoid carcinoma of larynx and hypopharynx...
June 28, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27441075/symptomatic-ovarian-steroid-cell-tumor-not-otherwise-specified-in-a-post-menopausal-woman
#20
Neha Sood, Kaniksha Desai, Ana-Maria Chindris, Jason Lewis, Tri A Dinh
Steroid cell tumor not otherwise specified (NOS) is a rare subtype of sex cord stromal tumor of the ovary and contributes less than 0.1% of all ovarian neoplasms. The majority of tumors occur in pre-menopausal women (mean age: 43 years), in which 56-77% of patients present with virilization due to excess testosterone. An 80-year-old woman with worsening alopecia and excessive growth of coarse hair on abdomen and genital area was found to have elevated serum testosterone level (462 ng/mL). Radiologic studies were consistent with bilateral adrenal adenomas...
June 28, 2016: Rare Tumors
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