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Rare Tumors

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https://www.readbyqxmd.com/read/28191296/dedifferentiated-liposarcoma-of-the-esophagus-a-case-report-and-selected-review-of-the-literature
#1
Christopher L Brett, Daniel H Miller, Liuyan Jiang, Herbert C Wolfsen, Steven Attia, Lauren Hintenlang, Niveditha Jagadesh, Robert C Miller
Soft tissue sarcomas of the esophagus represent an extremely rare cause of esophageal masses, and an even smaller proportion of these tumors represent dedifferentiated liposarcomas. We present a case of a 75-year-old gentleman presenting with dysphagia found to have a 5 cm pedunculated mass in the cervical esophagus, originating at the cricopharyngeus. This was found to have involvement limited to the superficial mucosa by endoscopic ultrasound, and the lesion was subsequently resected endoscopically. Pathology demonstrated an undifferentiated pleomorphic sarcoma later determined to represent dedifferentiated liposarcoma after fluorescence in situ hybridization analysis...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191295/small-cell-carcinoma-of-the-prostate-in-an-elderly-patient-a-case-report-and-review-of-the-literature
#2
Dale Alan Whitaker, Daniel H Miller, Niveditha Jagadesh, Gerald W Strong, Lauren Hintenlang, William B Schenk, Gregory A Broderick, Katherine S Tzou, Steven J Buskirk
Prostate cancer is the most common malignancy of men in the United States. Small-cell carcinoma (SCC), which typically presents as an aggressive lung malignancy, is a rare diagnosis within the setting of prostate cancer pathology. Due to its limited prevalence, little information regarding the treatment and prognosis of this disease in large populations is available. To date our current knowledge base is largely limited to case reports and retrospective case reviews. The mainstay of treatment for this particular histology most often involves a multimodality approach utilizing chemotherapy in conjunction with radiation therapy, androgen deprivation therapy, or prostatectomy...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191294/complete-remission-of-locally-advanced-penile-squamous-cell-carcinoma-after-multimodality-treatment
#3
Yifan Meng, Helen Levey Bernie, Tzu-Hua Weng, Dean-An Ling, Edward M Messing, Elizabeth Guancial
Treatment of locally advanced penile squamous cell carcinoma (pSCC) remains highly controversial secondary to disease rarity and lack of prospective randomized controlled trials. The current mainstays of care are multi-modality treatment with neoadjuvant chemotherapy and surgery. However, clinicians often have difficulty making recommendations for patients unable to tolerate chemotherapy or surgery due to scarcity of data to guide clinical decision-making. We report two cases of locally advanced pSCC that achieved complete remission after treatment with cisplatin-based neoadjuvant chemotherapy and surgery in one case, and concurrent cisplatin chemoradiation in a second, supporting the use of chemotherapy as part of first-line multimodal therapy...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191293/the-imaging-and-pathological-features-of-metastatic-leiomyosarcoma-in-the-gallbladder
#4
Yi Guo, Eleanor Chen, Darin J Davidson, Venu G Pillarisetty, Robin L Jones, Seth M Pollack
Uterine leiomyosarcoma is a rare and aggressive malignancy with poor overall prognosis. There have been few reports of metastatic leiomyosarcoma in the gallbladder. We report a case of a 41-year-old female who underwent total abdominal hysterectomy due to presumed uterine fibroids. The postoperative pathology revealed high-grade pleomorphic leiomyosarcoma, with involvement of the uterine serosal surface. She subsequently underwent exploratory laparotomy, followed by pelvic radiation and chemotherapy. Since initial management she has developed metastatic disease and has been under treatment and surveillance for 11 years...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191292/oncocytic-variant-of-medullary-thyroid-carcinoma-a-rare-case-of-sporadic-multifocal-and-bilateral-ret-wild-type-neoplasm-with-revision-of-the-literature
#5
Gian Luca Rampioni Vinciguerra, Niccolò Noccioli, Claudia Cippitelli, Angelo Minucci, Ettore Capoluongo, Armando Bartolazzi
Oncocytic variant of medullary thyroid carcinoma (OV-MTC) is a very unusual entity, up to date only 17 cases have been reported in the literature. MTC is a neuro-endocrine malignancy arising from the para-follicular C cells of the thyroid gland. It generally has a slight female predominance and appears as a single lesion. However in the Multiple Endocrine Neoplasia Syndrome 2, linked to the point mutation of RET oncogene, multifocal MTCs may also occur. Herein, we report the case of a 75 years old man with a rare form of sporadic multifocal and bilateral OV-MTC expressing wild-type RET gene...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191291/diffuse-follicular-variant-of-papillary-thyroid-carcinoma-a-case-report-with-a-revision-of-literature
#6
Gian Luca Rampioni Vinciguerra, Niccolò Noccioli, Armando Bartolazzi
The diffuse follicular variant of papillary thyroid carcinoma (DFV-PTC) is a rare malignant thyroid condition. It represents an uncommon variant of papillary carcinoma characterized by a diffuse involvement of thyroid parenchyma, follicular architecture and nuclear features of PTC in absence of a surrounding capsule. Up to date few data have been collected about this entity and, at the best of our knowledge, only 24 cases have been reported in the literature. According to these reports DFV-PTC seems to occur preferentially in young women and shows more aggressive behavior than other papillary thyroid tumors...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191290/loss-of-efficacy-of-pasireotide-after-its-re-administration-is-there-a-reason-why
#7
Stylianos Mandanas, Lemonia Mathiopoulou, Maria Boudina, Alexandra Chrisoulidou, Kalliopi Pazaitou-Panayiotou
Pasireotide is a recently approved medical treatment for persistent or recurrent Cushing's disease (CD). However, an escape from the initial successful response has not yet been described. A 42-year-old female presented with several symptoms indicative of hypercortisolism. Biochemical evaluation and imaging were consistent with CD due to a pituitary adenoma. Surgical excision of the adenoma was unsuccessful and gamma-knife radiosurgery was followed. Our patient remained hypercortisolemic thus treatment with pasireotide (900 mg subcutaneously twice daily) was decided...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28191289/renal-cell-carcinoma-metastatic-to-the-scalp
#8
Mounir Errami, Vitali Margulis, Sergio Huerta
Because of the asymptomatic natural history of renal cell carcinoma (RCC), by the time a diagnosis is made, metastatic disease is present in about one third of the cases. Thus, the overall survival of patients with RCC remains poor. Ultimately up to 50% of patients with RCC will develop metastases. Metastatic lesions from RCC are usually observed in the lungs, liver or bone. Metastases to the brain or the skin from RCC are rare. Here we present a patient diagnosed with RCC, found to have no evidence of metastases at the time of nephrectomy, who presented two years later with metastases to the scalp...
November 17, 2016: Rare Tumors
https://www.readbyqxmd.com/read/28058101/secretory-carcinoma-in-a-79-year-old-woman-an-exceptionally-rare-type-of-breast-carcinoma
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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