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Rare Tumors

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https://www.readbyqxmd.com/read/28058101/secretory-carcinoma-in-a-79-year-old-woman-an-exceptionally-rare-type-of-breast-carcinoma
#1
Nelson Montalvo, Veronica Posso, Ligia Redrobán
Secretory breast carcinoma is an exceptionally rare mammary gland neoplasia described mainly in adult females and children of both sexes, and very rarely in the elderly. It has particular histopathological and immunohistochemical features and a favorable prognosis. We report the case of a 79-year-old Hispanic woman with a palpable breast mass. Currently, the patient is disease free after a followup period of 6 years without local recurrence or axillary lymph-nodes nor distant metastases.
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28058100/non-mucinous-lepidic-predominant-adenocarcinoma-presenting-with-extensive-aerogenous-spread
#2
Yusuke Takanashi, Shogo Tajima, Masaru Tsukui, Kazuya Shinmura, Takamitsu Hayakawa, Tsuyoshi Takahashi, Hiroshi Neyatani, Kazuhito Funai
An extremely rare case of non-mucinous lepidic-predominant invasive adenocarcinoma (LPA) showing extensive aerogenous spread with a pneumonic presentation is reported. A 73-year-old woman was referred to our hospital because of an infiltrative shadow on chest xray. Chest computed tomography revealed extensive ground glass opacities in the right lower lobe, which was accompanied by infiltrative shadow with a pneumonic presentation. Invasive mucinous adenocarcinoma was presumed, and a partial resection of the right lower lobe was done...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28058099/adenomatoid-tumor-of-the-adrenal-gland-in-young-woman-from-clinical-and-radiological-to-pathological-study
#3
Brankica Krstevska, Sasha Jovanovska Mishevska, Rubens Jovanovic
Adenomatoid tumors are neoplasms of mesothelial origin, usually occurring in the male and female genital tracts. Extragenital localization sites such as adrenal glands are rare but have been reported. When found in the adrenals, they represent great clinical, radiological and pathological diagnostic challenge, with wide range of differential diagnoses to be considered. We present a case of a 30 years old female, with incidental ultrasound finding of unilateral tumor in the right adrenal gland. Multi slices CT scan was of value in localizing this tumor, but not in the precise diagnosis...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27994832/mayo-clinic-cancer-center-experience-of-metastatic-extramammary-paget-disease-1998-2012
#4
Leslie Padrnos, Nina Karlin, Thorvardur R Halfdanarson
Extramammary Paget disease (EMPD) is a rare cutaneous malignancy. The most common presentation of EMPD is the vulva followed by perianal involvement. Most cases are localized to the dermis with treatment focused on surgery, topical treatment or radiotherapy. Recurrence is frequent despite therapies utilized. Metastatic extramammary Paget disease is uncommon and, as such, standard treatment guidelines do not exist. This study sought to evaluate the treatment regimens and outcomes of patients treated at a Mayo Clinic Center from 1998-2012...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27994831/primary-intraosseous-smooth-muscle-tumor-of-uncertain-malignant-potential-original-report-and-molecular-characterization
#5
Lauren Kropp, Gene P Siegal, Garrett M Frampton, Michael G Rodriguez, Svetlana McKee, Robert M Conry
We report the first case of primary intraosseous smooth muscle tumor of uncertain malignant potential (STUMP) which is analogous to borderline malignant uterine smooth muscle tumors so designated. The tumor presented in the femur of an otherwise healthy 30-year-old woman. Over a 3-year period, the patient underwent 11 biopsies or resections and 2 cytologic procedures. Multiple pathologists reviewed the histologic material including musculoskeletal pathologists but could not reach a definitive diagnosis. However, metastases eventually developed and were rapidly progressive and responsive to gemcitabine and docetaxel...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27994830/primary-intimal-sarcoma-of-the-left-atrium-an-incidental-finding-on-routine-echocardiography
#6
Gautam Valecha, Dhaval Pau, Nikhil Nalluri, Ying Liu, Farhan Mohammad, Jean Paul Atallah
Cardiac sarcomas are extremely rare primary malignant tumors of the heart. In this article, we present the case of a 70-year-old female, who was found to have a left atrial mass during a routine outpatient transthoracic echocardiography. Further investigation with cardiac magnetic resonance imaging confirmed the presence of a bilobulated mass with heterogeneous enhancement. Left atrial myxoma was the first diagnostic consideration, followed by other primary cardiac tumors, and thrombus. The patient subsequently underwent resection of the mass, utilizing cardiopulmonary bypass...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/27746888/primary-hepatic-lymphoma-a-retrospective-multicenter-rare-cancer-network-study
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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