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Rare Tumors

Koichiro Wasano, Kouhei Sakurai, Taiji Kawasaki, Kimihide Kusafuka, Masao Kasahara, Naoki Kondo, Ken-Ichi Inada, Kaoru Ogawa
Salivary duct carcinoma is a relatively rare salivary cancer, and most cases are androgen receptor -positive. Salivary duct carcinoma growth is suggested to be androgen dependent, which can reportedly be controlled by androgen deprivation therapy. However, the effectiveness and underlying molecular mechanisms of androgen deprivation therapy for salivary duct carcinoma remain unknown. We report a salivary duct carcinoma case (65-year-old man) arising from the parotid gland with metastasis to the neck lymph nodes and lungs...
2018: Rare Tumors
Alan Todd Blank, Mazdak Khalighi, R Lor Randall, Kevin B Jones
Soft tissue sarcomas are a rare group of mesenchymal malignancies which can range from low to high grade. These tumors have different clinical, radiographic, and histopathological characteristics. Beta human chorionic gonadotropin is a naturally secreted hormone by placental syncytiotrophoblast cells during pregnancy. On very rare occasions, sarcomas can develop the ability to ectopically produce human chorionic gonadotropin. Very few cases exist in the literature of soft tissue sarcomas expressing this hormone...
2018: Rare Tumors
Sean James Judge, Trevor A Plescia, Cyrus P Bateni, Morgan A Darrow, Christopher P Evans, Robert J Canter
Ataxia-telangiectasia confers a significant increase in the development of several cancer types, most commonly leukemia and lymphoma. However, as the natural history for these patients is evolving and their lifespan is increasing, there is the potential for the development of additional uncommon tumors in an already rare patient population. We report the first case, to our knowledge, of an incidental retroperitoneal tumor in a 26-year-old woman undergoing evaluation for hepatic dysfunction. The mass was suspicious for retroperitoneal sarcoma, but proved to be an extramedullary hematopoietic pseudotumor after extensive pathologic evaluation...
2018: Rare Tumors
Andrea P Espejo, Jeremy L Ramdial, Breelyn A Wilky, Darcy A Kerr, Jonathan C Trent
In patients with sarcoma, concomitant malignancy is found in 1.2% -2.5% of cases. Previous studies have demonstrated conflicting results in terms of positive or negative effects on cancer prognosis with comorbid sarcoidosis. Additionally, there are no data determining whether an association between sarcoidosis and sarcomas exists. Finding an association between the two entities could prevent inadvertent upstaging of a primary sarcoma based on pulmonary nodularity mistaken for metastatic disease. Here, we will describe eight sarcoma patients with concomitant occurrence of sarcoidosis identified since 2007...
2018: Rare Tumors
Magnus Hallin, Yurina Miki, Andrew J Hayes, Robin L Jones, Cyril Fisher, Khin Thway
Myxoinflammatory fibroblastic sarcoma is a rare malignant soft tissue neoplasm that typically arises on the distal extremities of adults. It usually behaves in a low-grade manner and its characteristic histology is of a lobulated proliferation of moderately atypical spindled to epithelioid cells, vacuolated cells, and enlarged or bizarre cells with prominent nucleoli, dispersed within myxoid stroma containing a mixed inflammatory cell infiltrate. The etiology of myxoinflammatory fibroblastic sarcoma remains unknown with no definite causal factors identified...
2018: Rare Tumors
Obianuju Ugwuoke, Marios Hadjipavlou, Thelma Pinto, Ajay Arora, Mohamed Y Hammadeh
Villous adenoma is a rare pathology seen in the urinary tract; it is mostly found in the large bowel. When encountered in the urinary tract, it mainly originates from intestinal segments of urinary reconstruction. Villous adenoma is commonly seen in patients more than 50 years and has a male predominance. In the urinary tract, it has been reported in the urachus, bladder dome and trigone. A few cases of villous adenoma have been described in the literature. We report a case of primary villous adenoma in the remnant of the urachus and discuss the pathophysiology, investigation and management...
2018: Rare Tumors
Robbert Wylleman, Maria Debiec-Rychter, Raf Sciot
We report the exceptional occurrence of murine double minute clone 2 amplification in an atypical meningioma and its recurrent anaplastic meningioma arising in the right frontal lobe of a 75-year-old man. Murine double minute clone 2 amplification was shown by array comparative genomic hybridization and confirmed by fluorescence in situ hybridization. This is a rare finding with only one similar report in the literature. Awareness of this finding is indicated and should not lead to misdiagnosis of other entities that more commonly show this feature...
2018: Rare Tumors
Naoki Sakata, So-Ichi Suenobu, Munehiro Okano, Satoshi Ueda, Masatomo Kimura, Tsukasa Takemura
We describe our experience with a 12 year-old girl with kaposiform hemangioendothelioma accompanied by Kasabach-Merritt phenomenon with exacerbation of the disease 10 years after the initial diagnosis. Kaposiform hemangioendothelioma infiltrated into the subcutaneous tissue of the facial skin with deterioration of coagulopathy despite conventional therapies including corticosteroid, vincristine, and propranolol. Sirolimus, a mammalian target of rapamycin inhibitor, produced rapid and dramatic improvement of the Kasabach-Merritt phenomenon and kaposiform hemangioendothelioma shrinkage...
2018: Rare Tumors
Bita Geramizadeh, Zahra Zare, Amir Reza Dehghanian, Shahram Bolandparvaz, Mahsa Marzban
Low-grade fibromyxoid sarcoma is a rare soft tissue tumor which has been mostly reported in lower extremities; however, it can also occur in other parts of the body such as head and neck and abdominal wall, but its occurrence in the abdominal cavity and mesentery of bowel is an extremely rare event and has very rarely been reported. Herein, we report our experience with a 24-year-old lady with a huge mesenteric mass, turned out to be low-grade fibromyxoid sarcoma. This case is the largest one reported in the English literature...
2018: Rare Tumors
Kazuhiko Hashimoto, Shunji Nishimura, Kousuke Fujii, Ryosuke Kakinoki, Masao Akagi
Synovial sarcomas are malignant soft tissue tumors that carry a metastatic potential and portend poor prognoses. We report a very rare synovial sarcoma arising from the tibial nerve and describe its successful treatment including limb preservation. To the best of our knowledge, this is the 40th reported incident of intraneural synovial sarcoma, and only the fourth arising from the tibial nerve. Extensive resection of synovial sarcoma arising in the popliteal region is difficult, particularly when limb preservation is desired...
2018: Rare Tumors
Osamu Imataki, Akihiro Takeuchi, Shumpei Uchida, Shigeyuki Yokokura, Makiko Uemura, Norimitsu Kadowaki
Acute erythroid leukemia, also known as acute myeloid leukemia-M6, may be associated with previous chemotherapy or immunosuppressive therapy. For 10 years, a 69-year-old Japanese female patient with pure erythroid leukemia (or acute myeloid leukemia-M6b) was treated for polymyositis with 50-100 mg/day azathioprine. She complained of dyspnea with low-grade fever and was diagnosed as having pure erythroid leukemia. Chromosomal analysis revealed a complex karyotype abnormality, with the deletion of 5q, -6, -7 and addition of 11q13...
2018: Rare Tumors
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
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
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
Alejandro Peralta Soler, Michael Miller, Thomas L Smith
No abstract text is available yet for this article.
October 3, 2017: Rare Tumors
Aikaterini G Solomou
No abstract text is available yet for this article.
October 3, 2017: Rare Tumors
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
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
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
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
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