Cerebellar liponeurocytoma: a novel report from Nigeria in a 6-year-old girl, and review of literature
In this report, we present the case of a 6-year-old Nigerian girl with a liponeurocytoma, and review of literature. (Source: Rare Tumors)
Source: Rare Tumors - September 4, 2016 Category: Cancer & Oncology Source Type: research
Post-transplant lymphoproliferative disorder presenting as CD20-negative plasmablastic lymphoma in the lung
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. Given the increasing number of transplants performed, we would like to share this uncommon presentation of PTLD as PB...
Source: Rare Tumors - September 4, 2016 Category: Cancer & Oncology Source Type: research
National incidence, management and survival of urachal carcinoma
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. This comprised 0.3% of all invasive bladder tumors. Patients were predominantly male (62%) and over 50 years of age (58%). Twenty-two patients (85%) underwent surgery, with only six (23%) undergoing...
Source: Rare Tumors - September 4, 2016 Category: Cancer & Oncology Source Type: research
Outcome and predictive factors in uterine carcinosarcoma using postoperative radiotherapy: a Rare Cancer Network study
This study reports on outcome and predictive factors when using postoperative radiotherapy. We analyzed a retrospective analysis in 124 women treated between 1987- 2007 in the framework of the Rare-Cancer- Network. Median follow-up was 27 months. Postoperative pelvic EBRT was administered in 105 women (85%) and 92 patients (74%) received exclusive or additional vaginal brachytherapy. Five-year overall survival (OS), disease-free survival (DFS), cancer specific survival (CSS) and locoregional control (LRC) were 51.6% (95% CI 35-73%), 53.7% (39-71%), 58.6% (38-74%) and 48% (38-67%). Multivariate analysis showed that external...
Source: Rare Tumors - June 29, 2016 Category: Cancer & Oncology Source Type: research
Symptomatic ovarian steroid cell tumor not otherwise specified in a post-menopausal woman
Steroid cell tumor not otherwise specified (NOS) is a rare subtype of sex cord stromal tumor of the ovary and contributes less than 0.1% of all ovarian neoplasms. The majority of tumors occur in pre-menopausal women (mean age: 43 years), in which 56-77% of patients present with virilization due to excess testosterone. An 80-year-old woman with worsening alopecia and excessive growth of coarse hair on abdomen and genital area was found to have elevated serum testosterone level (462 ng/mL). Radiologic studies were consistent with bilateral adrenal adenomas. Bilateral adrenal venous sampling ruled out the adrenal gland as ori...
Source: Rare Tumors - June 29, 2016 Category: Cancer & Oncology Source Type: research
Recurrent high-grade invasive mucoepidermoid carcinoma of larynx: a case report and review of the literature
Recurrent invasive high-grade mucoepidermoid carcinoma of the larynx and hypopharynx is a rare occurrence. These tumors have been commonly associated with salivary gland tumors, most commonly the parotid gland. The patient usually presents with the following symptoms: hoarseness (if larynx is involved), or changes in voice character, sore throat, cough, odynophagia, dysphagia, otalgia, difficulty breathing, weight loss, lymphadenopathy. Here we present a case of a recurrent invasive high-grade mucoepidermoid carcinoma of larynx and hypopharynx. The patient was a 67- year-old male that originally presented in 2006. At that ...
Source: Rare Tumors - June 29, 2016 Category: Cancer & Oncology Source Type: research
Colorectal carcinomas in Uyo City, Southern geopolitical zone of Nigeria: a review of clinicopathological characteristics and literature
This study is aimed to identify the clinical and pathological characteristics of CRC diagnosed between July 2006 and June 2015 in the University of Uyo Teaching Hospital, and a Private Specialist Laboratory, Uyo, Akwa Ibom State, Nigeria. All histological diagnosed cases of CRC seen in the two laboratories (University teaching and a private facility) in Uyo, Akwa-Ibom state, Nigeria during the study period were retrieved noting their bio-data, pathological and clinical variables. A total of 45 patients of age range 26-80 years with a mean of 55.9 years (SD 3.9) and a male to female ratio of 1.4:1 were seen. The two most co...
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
Head and neck soft tissue sarcomas treated with radiation therapy
Head and neck soft tissue sarcomas (HNSTSs) are rare and heterogeneous cancers in which radiation therapy (RT) has an important role in local tumor control (LC). The purpose of this study was to evaluate outcomes and patterns of treatment failure in patients with HNSTS treated with RT. A retrospective review was performed of adult patients with HNSTS treated with RT from January 1, 1998, to December 31, 2012. LC, locoregional control (LRC), disease-free survival (DFS), overall survival (OS), and predictors thereof were assessed. Forty-eight patients with HNSTS were evaluated. Five-year Kaplan-Meier estimates of LC, LRC, DF...
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
Rare malignancies in Eastern India, socio-economic impact
The etiology of cancer is multifactorial. Various factors, including physical carcinogens, chemicals and viral carcinogens affect patients with known predisposing factors who subsequently develop malignancies. Here is a retrospective study of 18 patients who developed rare malignancies in clinical situations like xeroderma pigmentosum, tuberous sclerosis, neurofibromatosis, hereditary multiple exostosis, second malignancies due to radiotherapy and chronic irritation. The predisposing factors like chronic infection in leprosy, filariasis, poverty and ignorance leading to the chronicity of the lesion, lack of available healt...
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
Three years sustained complete remission achieved in a primary refractory ALK-positive anaplastic T large cell lymphoma treated with crizotinib
We report a case of a primary refractory ALK+ anaplastic largecell lymphoma that sustains complete response after 3 years of crizotinib monotherapy. (Source: Rare Tumors)
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
Appendiceal hemangioma, mimicking acute appendicitis in a 17-year-old girl
Epithelial tumors of appendix are not so common, and mesenchymal tumors of the appendix are even less common. Capillary hemangioma of the appendix is an extremely rare event and to the best of our knowledge only 4 cases have been reported in the English literature so far. In this case report we want to explain our experience with an extremely rare occurrence of capillary hemangioma of appendix in a 17-year-old girl presented with right lower quadrant pain that was operated with the clinical impression of acute appendicitis. The patient has been operated as a routine appendectomy with a completely uneventful postoperative p...
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
A rare case of exclusively oncocytic mucoepidermoid carcinoma with MAML2 translocation
Mucoepidermoid carcinoma is the most common malignant tumor of the salivary gland. The oncocytic variant of mucoepidermoid carcinoma (OMEC) is rare and a small subset shows exclusive oncocytic morphology. Here we report an OMEC case of the parotid gland in a 74-year-old woman with exclusive oncocytes and rare mucocytes. The oncocytes showed diffuse nuclear positivity with p63 immunostaining. The MAML2 translocation was present, supporting the diagnosis of OMEC. Distinguishing OMEC with exclusive oncocytes from oncocytoma and oncocytic carcinoma can be very challenging for pathologists and is critical for proper clinical ma...
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
Nuclear protein of the testis midline carcinoma masquerading as a primary mediastinal seminoma
This article reviews the clinicopathologic features and the differential diagnosis of these malignancies. (Source: Rare Tumors)
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
Characteristics and patterns of rare malignancies published in Rare Tumors
Rare Tumors is an international peerreviewed medical journal established in 2009. The journal is focused on rare cancers and aims to expand upon current knowledge on their presentation, diagnosis, management, and outcomes. We reviewed the 335 case reports published from 2009 to 2015. We found great diversity in both the country of origin as well as specialty of first authors. Outside of the United States (US) and European Union (EU), there were 20 countries with contributions to the journal. Similarly, there was representation from twelve medical specialties with first authorship of reports. Rare Tumors continues to encour...
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
Paratesticular fibrous pseudotumors
We reported a young patient’s findings about this rare pathology. (Source: Rare Tumors)
Source: Rare Tumors - June 28, 2016 Category: Cancer & Oncology Source Type: research
