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Rare Tumors

Yoshihiro Kawaguchi, Shoichi Kimura, Kosuke Momozono, Tsukasa Igawa, Masanori Noguchi
Lymphoma of the urinary bladder is uncommon, and upper urinary tract obstruction due to lymphoma is rare. Herein, we report a case of malignant lymphoma of the bladder with bilateral hydronephrosis in a 67-year-old female who presented with oliguria. Ultrasonography and computed tomography demonstrated a thickened posterior bladder wall and bilateral hydronephrosis. Whole-body positron emission tomography-computed tomography revealed abnormal accumulation in the right iliac internal lymph nodes. Trans-urethral bladder biopsy led to a histopathological diagnosis of non-Hodgkin diffuse large B-cell malignant lymphoma of the bladder...
2019: Rare Tumors
Bita Geramizadeh, Maedeh Mottavvas, Bijan Zeyaian, Armin Amirian
Hamartoma of lung is a common tumor, majority of which are small and incidentally discovered during plain chest radiography. Our case is a 30-year-old gentleman with an extremely uncommon presentation of a common tumor, that is, a giant hamartoma of lung presenting as massive hemoptysis and intractable cough. To the best of our knowledge, such occurrence of giant pulmonary hamartoma is very uncommon and its presentation with massive hemoptysis is even more uncommon. Less than 20 cases of giant hamartoma of lung have been reported in the English literature so far...
2019: Rare Tumors
Sarra Mestiri, Mohamed Amine Elghali, Rym Bourigua, Nihed Abdessayed, Salsabil Nasri, Ben Abdallah Amine, Nabiha Missaoui, Mahmoud Ben Maitig, Sihem Hmissa, Badreddine Sriha, Moncef Mokni
Background: Soft tissue leiomyosarcomas are rare, accounting for almost 5%-10% of all soft tissue sarcomas; they account for almost 1% of all sarcomas. They are aggressive tumors where location, size, and management require a multidisciplinary approach. Since there are few series published, we here analyze epidemiological pattern, clinical and pathologic features of soft tissue leiomyosarcomas. Methods: We conducted a retrospective study of 29 consecutive cases of histologically proven soft tissue leiomyosarcoma extracted from the database of the Cancer Registry of the Center of Tunisia and the Department of Pathology of Farhat Hached University Hospital of Sousse of Tunisia, during a 10-year period (from January 1996 to December 2005)...
2019: Rare Tumors
Ronak Patel, James Hu, Shefali Chopra, Christopher Lee
Radiation-associated sarcomas are rare and aggressive types of sarcomas that can occur after exposure to ionizing radiation. We examine a case of radiation-associated undifferentiated/unclassified soft-tissue sarcoma with primary disease in the chest wall. The optimal treatment of these patients is surgical resection if possible; however, the role of chemotherapy has not been well defined. The patient described herein had a central tumor in the chest wall. Since many of these patients have borderline resectable tumors, the use of neoadjuvant chemotherapy may be helpful to downstage the tumors for possible surgical resection...
2019: Rare Tumors
Pankaj Shelke, Gargi Sachin Sarode, Sachin Chakradhar Sarode, Rahul Anand, Ghevaram Prajapati, Shankargouda Patil
An alveolar soft-part sarcoma is a malignant neoplasm primarily affecting the soft tissues of head and neck. The aim of the present review is to systematically present the demographic and clinico-pathological data of articles published in the English medical literature. A comprehensive search of the databases (PubMed, Medline, SCOPUS, Web of Science, and Google Scholar) along with cross references to the published articles on alveolar soft-part sarcoma for eligible studies/case reports published since 1957 till date was done to retrieve the data...
2018: Rare Tumors
Muhammad Usman Tariq, Zubair Ahmad, Jamshid Abdul-Ghafar, Nasir Ud Din
Background: Serous cystadenomas of pancreas are rare benign epithelial neoplasms, which predominantly occur in the pancreatic body and tail of elderly females. Majority of these tumors have microcystic appearance. Macrocystic and solid variants have also been described. A number of more aggressive cystic pancreatic lesions are included in the differential diagnosis. Distinction from such lesions is important for optimal management. Objective: Our aim was to study the clinical and histological features of serous cystadenomas which would be helpful in making their correct diagnosis and understanding their behavior...
2018: Rare Tumors
Shinichirou Yoshida, Munenori Watanuki, Kouu Hayashi, Masami Hosaka, Yoshihiro Hagiwara, Eiji Itoi, Masahito Hatori, Shin Hitachi, Mika Watanabe
Osteofibrous dysplasia is a benign fibro-osseous lesion of bone which is most commonly occurred in cortical bone of anterior mid-shaft of the tibia of infancy and childhood. This study reported a case of osteofibrous dysplasia arising in the humerus of adult, resulting in good prognosis after a surgical treatment. A 34-year-old male had felt left upper arm pain and was suspected as having a bone tumor at the humeral shaft by X-ray pictures. The tumor was suspected as the osteofibrous dysplasia of the humerus by a core needle biopsy...
2018: Rare Tumors
Laurence M Briski, Dafydd G Thomas, Rajiv M Patel, Elizabeth R Lawlor, Rashmi Chugh, Jonathan B McHugh, David R Lucas
Background: Previous studies have shown that aberrant activation of the Wnt/β-catenin pathway is associated with many malignant neoplasms. This includes some soft-tissue sarcoma phenotypes, most notably synovial sarcoma, implicating potential targets for novel molecular therapies. Objective: We investigate the level of Wnt/β-catenin pathway activation present in leiomyosarcomas relative to synovial sarcomas, using expression of LEF1 and β-catenin as surrogates...
2018: 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
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