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Rare Tumors

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https://www.readbyqxmd.com/read/29760872/giant-morphea-form-basal-cell-carcinoma-of-the-umbilicus-successful-debulking-with-vismodegib
#1
Mariana Orduz Robledo, Eve Lebas, Marie-Annick Reginster, Mahmoud Baghaie, Sabine Groves, Arjen F Nikkels
Basal cell carcinoma of the umbilicus is very rare. The nodular subtype is the main representative. Giant basal cell carcinomas represent around 1% of all basal cell carcinomas. The hedgehog pathway inhibitor vismodegib is indicated for advanced basal cell carcinoma and CD56-negative immunostaining seems indicative for successful treatment. A 54-year-old man presented a 10 cm × 14 cm large and 4.5 cm deep morphea-form basal cell carcinoma with faint immunohistochemical CD56 expression arising from the umbilicus...
2018: Rare Tumors
https://www.readbyqxmd.com/read/29760871/presacral-myelolipoma-as-a-possible-parasymptom-of-cancer-a-case-report
#2
Akio Sakamoto, Iori Nagamatsu, Eisuke Shiba, Takeshi Okamoto, Masanori Hisaoka, Shuichi Matsuda
Presacral myelolipomas are rare, benign, asymptomatic tumors composed of mature adipose tissue and hematopoietic elements. Presacral myelolipomas can occur in patients with a medical history of malignancy, steroid use, and/or endocrine disorders including diabetes mellitus. A 65-year-old man with no specific medical history experienced temporal abdominal pain without bowel symptoms that lasted a few hours. By the time he visited a hospital, the pain had diminished. Computed tomography failed to detect any abnormality in the abdominal or pelvic organs that would have caused the abdominal pain but revealed a lesion 4 cm in diameter in the frontal sacrum...
2018: Rare Tumors
https://www.readbyqxmd.com/read/29760870/a-multicenter-phase-ii-study-of-q3-week-or-weekly-paclitaxel-in-combination-with-bevacizumab-for-the-treatment-of-metastatic-or-unresectable-angiosarcoma
#3
Nam Bui, Nikhil Kamat, Vinod Ravi, Sant Chawla, Marti Lohman, Kristen N Ganjoo
Paclitaxel (P) and bevacizumab (B) are agents that provide clinical benefit in advanced angiosarcoma (AS). The objective of this study was to assess the efficacy and safety of P-B in two different scheduled regimens. Patients were to receive P 200mg/m2 IV with B 15mg/kg IV every 21 days (Regimen A) or P 90mg/m2 IV weekly D1, 8, 15 with B 15mg/kg IV D1 of a 28 day cycle (Regimen B) x6 cycles. Maintenance B followed at a dose of 15 mg/kg intravenously once every 21 days. The primary end point was 4 month non-progression rate (NPR)...
2018: Rare Tumors
https://www.readbyqxmd.com/read/29218135/langerhans-cell-sarcoma-of-the-skin-in-association-with-superficial-atypical-langerhans-cell-proliferation
#4
Alejandro Peralta Soler, Michael Miller, Thomas L Smith
No abstract text is available yet for this article.
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/29142658/magnetic-resonance-imaging-of-pineal-tumors-and-drop-metastases-a-review-approach
#5
Aikaterini G Solomou
No abstract text is available yet for this article.
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/29085618/squamous-cell-lung-carcinoma-presenting-as-melena-a-case-report-and-review-of-the-literature
#6
Ibrahim Azar, Efstratios Koutroumpakis, Raina Patel, Syed Mehdi
Lung cancer has a predilection to widely metastasize to the liver, bone, brain and adrenal glands. Metastasis of primary lung tumors to the stomach is infrequent, with only sporadic cases reported. Most cases are asymptomatic and diagnosed post-mortem on autopsy. The incidence of symptomatic gastrointestinal metastases is extremely rare. Herein, we describe a case of gastric metastasis by squamous cell lung carcinoma, presenting as melena and diagnosed by esophagogastroduodenoscopy. To the best of our knowledge, only twenty other cases in the English literature have reported symptomatic gastric metastasis of lung cancer diagnosed by endoscopic biopsy...
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/29081930/mammary-analogue-secretory-carcinoma-of-salivary-glands-diagnostic-pitfall-with-distinct-immunohistochemical-profile-and-molecular-features
#7
Oliver Bissinger, Carolin Götz, Andreas Kolk, Henning A Bier, Abbas Agaimy, Henning Frenzel, Sven Perner, Julika Ribbat-Idel, Klaus Dietrich Wolff, Wilko Weichert, Caroline Mogler
Mammary analogue secretory carcinoma (MASC) is a newly defined entity among salivary gland malignancies which has just been established in the 4th edition of the WHO classification of head and neck tumors. MASC (synonym: secretory carcinoma) are characterized by a specific rearangement of the ETV6 gene locus. Here, we present a series of 3 MASC cases including clinical data with follow-up for up to 26 months. All tumours immunhistochemically displayed strong positivity for cytokeratin 7, and mammaglobin, focal positivity for S100, cytokeratin 5/6 and muc-4...
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/29081929/mucinous-cystic-neoplasms-in-male-patients-two-cases
#8
Shunsuke Tamura, Hirotaka Yamamoto, Shinichiro Ushida, Kazufumi Suzuki
Mucinous cystic neoplasms (MCNs) of the pancreas in male patients are very rare. Though few cases of MCNs in male patients have been reported, it is difficult to reveal them preoperatively. The first patient was a 50-year-old man and was incidentally found to have a cystic mass of 51 mm in the tail of the pancreas. We performed a distal pancreatectomy. The second patient was a 73-year-old man, incidentally found to have a cystic mass of 25 mm in diameter in the tail of the pancreas and a carcinoma of the bile duct...
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/29081928/primary-adenocarcinoma-of-the-seminal-vesicle
#9
Safae Terrisse, Maria Eugenia Comblor, Jérôme Vérine, Hélène Gauthier, Pierre Mongiat-Artus, Stéphane Culine
Primary adenocarcinoma of the seminal vesicle is a rare condition with only about 60 cases described in the literature. The unusual characteristics of this disease makes diagnosis difficult and treatment strategies differ as there are no specific guidelines available. This report presents a case of adenocarcinoma of the seminal vesicle with lung metastases in which surgical and chemotherapeutic treatments have been carried out. The MVAC dose dense regimen following local resection seems effective in this scenario and may be used in the treatment of this disease...
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/29081927/atelectasis-in-pediatrics-a-case-of-carcinoid-tumor
#10
Cláudia Vaz Guerreiro, Paula Ornelas, Luísa Pereira, Nuno Abecasis, Maria Teresa Almodovar
Carcinoid pulmonary tumors occur in the fourth to sixth decades of life. Usually, typical carcinoid arise a decade earlier when compared to atypical carcinoid (45 years and 55 years, respectively). Typical carcinoid tumors are the most common primary lung neoplasm in children and late adolescents, but there are less than 40 cases described in the literature. The clinical presentation is nonspecific and usually the symptoms are due to bronchial obstruction, sometimes with recurrent pneumonia. Its rarity may delay diagnosis but in most cases a favorable course after treatment is observed...
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/29081926/mucinous-cystadenocarcinoma-of-the-breast-the-challenge-of-diagnosing-a-rare-entity
#11
Nektarios Koufopoulos, Christina Goudeli, John Syrios, Evangelos Filopoulos, Lubna Khaldi
Mucinous cystadenocarcinoma is an extremely rare variant of primary breast tumor which is histologically similar to mucinous cystadenocarcinoma of the ovary and pancreas. Herein we report a case of a 63 years old woman diagnosed with diverse histological types of non-synchronous rare primary breast tumors, a medullary carcinoma of the right breast and a mucinous cystadenocarcinoma of the left breast. Macroscopically the neoplasm appeared multilocular filled with mucoid material. Under light microscopy the cystic areas were lined by columnar cells with abundant intracellular and extracellular mucin...
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/29081925/sertoli-leydig-cell-tumor-of-the-ovary-masquerading-as-a-mucinous-adenocarcinoma-a-frozen-section-pitfall
#12
Jonathan E Zuckerman, Neda A Moatamed
Sertoli-Leydig cells tumors are rare ovarian neoplasms that can be managed with conservative resection given their generally excellent prognosis. Here we report a case of Sertoli-Leydig cell tumor mistakenly diagnosed as an invasive mucinous adenocarcinoma at time of intraoperative consultation because of its blue-mucinous appearance in the frozen section material. The patient subsequently underwent an extensive staging procedure revealing unilateral, ovarian confined disease. The mucinous features seen on frozen section were lost on the slides prepared from formalin fixed tissues...
October 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28975020/a-case-of-neuroendocrine-carcinoma-developing-from-the-broad-ligament-of-the-uterus
#13
Yui Itonaga, Masakazu Nishida, Harunobu Matsumoto, Kaei Nasu, Hisashi Narahara
Neuroendocrine carcinoma (NEC), also called small cell carcinoma or large cell carcinoma, is a rare and aggressive tumor that develops mainly in the lung and intestine. More rarely, NEC develops in gynecologic organs, with poor prognoses. We experienced a case of NEC in the broad ligament of the uterus. The patient was a 74-year-old woman with symptoms of abdominal distension and constipation. Ultrasound sonography detected an abdominal tumor larger than 10 cm. She was then admitted to our hospital. She underwent surgery under the diagnosis of ovarian cancer, but the bilateral ovaries and uterus were normal in appearance, and a tumor was developing instead from the broad ligament of the uterus...
July 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28975019/novel-therapies-in-platinum-refractory-metastatic-germ-cell-tumor-a-case-report-with-a-focus-on-a-pd-1-inhibitor
#14
Kah Poh Loh, Chunkit Fung
Testicular germ cell tumor (GCT) is the most common malignancy in young males between the ages of 15 to 35 years. Although the overall cure rate of GCTs approaches 95%, almost 25% of patients with distant metastases die from the cancer. Active investigations on novel treatment options for platinum-refractory GCTs include immunotherapies such as program-death 1 (PD-1)/program death-ligand 1 (PD-L1) inhibitors. In this case, we report a patient with metastatic GCTs who was treated with pembrolizumab, a PD-L1 inhibitor, in a phase II study after failing several lines of chemotherapy...
July 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28975018/comprehensive-genomic-profiling-of-a-rare-thyroid-follicular-dendritic-cell-sarcoma
#15
Jaime I Davila, Jason S Starr, Steven Attia, Chen Wang, Ryan A Knudson, Brian M Necela, Vivekananda Sarangi, Zhifu Sun, Yingxue Ren, John D Casler, David M Menke, Gavin R Oliver, Richard W Joseph, John A Copland, Alexander S Parker, Jean-Pierre A Kocher, E Aubrey Thompson, Robert C Smallridge, Yan W Asmann
We previously reported an extremely rare case of follicular dendritic cell sarcoma (FDCS) presented as a thyroid mass. Given the rarity of this disease, there are no personalized and molecularly targeted treatment options due to the lack of knowledge in the genomic makeup of the tumor. A 44-year-old white woman was diagnosed with an extranodal FDCS in thyroid. The patient underwent a total thyroidectomy, central compartment dissection, parathyroid re-implantation, and adjuvant radiation therapy. Tumor DNA sequencing of 236 genes by FoundationOne panel found truncating mutations in PTEN and missense mutations in RET and TP53...
July 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28975017/variegated-colors-of-pediatric-glioblastoma-multiforme-what-to-expect
#16
Vivek Immanuel, Pamela A Kingsley, Preety Negi, Roma Isaacs, Sarvpreet S Grewal
Malignant gliomas account for 35-45% of primary brain tumors; among these glioblastoma multiforme (GBM) is the most common adult brain tumor constituting approximately 85%. Its incidence is quite less in the pediatric population and treatment of these patients is particularly challenging. Exposure to ionizing radiation is the only environmental factor found to have any significant association with GBM. Several genetic alterations associated with GBM in adults have been well documented such as epidermal growth factor receptor amplification, overexpression of mouse double minute 2 homolog also known as E3 ubiquitin-protein ligase, Phosphatase and tensin homolog gene mutation, loss of heterozygosity of chromosome 10p and isocitrate dehydrogenase-1 mutation...
July 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28975016/granular-cell-tumor-of-cecum-a-common-tumor-in-a-rare-site-with-diagnostic-challenge
#17
Meyyappa Devan Rajagopal, Debasis Gochhait, Dasarathan Shanmugan, Adarsh Wamanrao Barwad
Granular cell tumor (GCT) also known as Abrikossoff's tumor is a benign neoplasm that is usually seen in the fourth to sixth decades of life with slight female preponderance. It is most frequently seen in the oral cavity, skin, and subcutaneous tissue. Gastrointestinal tract involvement is uncommon, in which esophagus is the most commonly affected site. There are case reports of GCT in stomach, appendix, colon and rectum. In this article, we report a case of GCT involving cecum. The cell of origin in GCT is controversial...
July 3, 2017: Rare Tumors
https://www.readbyqxmd.com/read/28458793/osteosarcoma-of-the-larynx-treatment-outcomes-and-patterns-of-failure-analysis
#18
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
#19
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
#20
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
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