Citations

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 time he underwent a wide field laryngectomy, right thyroid lobectomy, and biopsy of the right digastric node. He was a clinical stage III, pT3N0M0. No adjuvant radiation therapy was given at that time. The patient remained asymptomatic until February 2014, when he presented with dysphagia and neck swelling. Positron emission tomography/computed tomography showed evidence of recurrence. The patient was treated with definitive intensity modulated radiation therapy (IMRT) with concurrent chemotherapy. Treatment for this disease is gathered by scattered case reports. If surgery is a possibility it is considered as first line therapy. Post-surgical radiation is then offered. However, in this case the recurrent tumor was located near the carotid artery, and thus surgery was not a possibility. Therefore, concurrent chemotherapy and radiation with IMRT and weekly cis-platinum was given. While the optimum combination of treatment has not yet been established because of the rarity of this cancer's location site, the current patient appeared to have an excellent response from the definitive IMRT and chemotherapy treatment.

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 origin of hormone secretion. A diagnostic and therapeutic bilateral salpingo-oophorectomy confirmed steroid cell tumor NOS of the left ovary. Post-operatively, the patient had complete resolution of her symptoms and normalization of testosterone level. Our case emphasizes the importance of a clinical suspicion for an occult testosterone secreting ovarian tumor in a symptomatic patient without obvious ovarian mass on imaging.

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, DFS, and OS were 87, 73, 63, and 83%, respectively. Angiosarcomas were found to be associated with worse LC, LRC, DFS, and OS. Patients over the age of 60 had lower rates of DFS. HNSTSs comprise a diverse group of tumors that can be managed with various treatment regimens involving RT. Angiosarcomas have higher recurrence and mortality rates.

Rare Tumors is an international peer-reviewed 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 encourage involvement from physicians across the globe and from all medical disciplines.

Introduction. High BMI is a risk factor for upper body breast cancer-related lymphedema (BCRL) onset. Black cancer survivors are more likely to have high BMI than White cancer survivors. While observational analyses suggest up to 2.2 times increased risk of BCRL onset for Black breast cancer survivors, no studies have explored race or other social factors that may affect BCRL severity, operationalized by interlimb volume difference (ILD).Materials and Methods. ILD was measured by perometry for 296 overweight (25 > BMI < 50) Black (n=102) or White (n=194) breast cancer survivors (>6 months from treatment) in the WISER Survivor trial. Multivariable linear regression examined associations between social and physical factors and ILD.Results. Neither Black race (−0.26,p=0.89) nor BMI (0.22,p=0.10) was associated with ILD. Attending college (−4.89,p=0.03) was the strongest factor associated with ILD, followed by having more lymph nodes removed (4.75,p=0.01), >25% BCRL care adherence (4.10,p=0.01), and years since treatment (0.55,p<0.001).Discussion. Neither race nor BMI was associated with ILD among overweight cancer survivors. Education, a proxy for resource level, was the strongest factor associated with greater ILD. Tailoring physical activity and weight loss interventions designed to address BCRL severity by resource rather than race should be considered.

OBJECTIVES: To describe the demographic and clinical characteristics of pediatric multiple sclerosis (MS) in the United States. METHODS: This prospective observational study included children and adolescents with MS. Cases were evaluated across 9 geographically diverse sites as part of the US Network of Pediatric MS Centers. RESULTS: A total of 490 children and adolescents (324 girls, 166 boys) were enrolled; 28% developed symptoms before 12 years of age. The proportion of girls increased with age from 58% (&lt;12 years) to 70% (≥12 years). Race and ethnicity as self-identified were: white, 67%; African American, 21%; and non-Hispanic, 70%. Most (94%) of the cases were born in the United States, and 39% had 1 or both foreign-born parents. Fifty-five percent of cases had a monofocal presentation; 31% had a prodrome (most frequently infectious), most often among those aged &lt;12 years (P &lt; .001). Children aged &lt;12 years presented more commonly with encephalopathy and coordination problems (P &lt; .001). Sensory symptoms were more frequently reported by older children (ie, those aged ≥12 years) (P &lt; .001); 78% of girls had MS onset postmenarche. The initial Expanded Disability Status Scale score for the group was &lt;3.0, and the annualized relapse rate was 0.647 for the first 2 years. Interval from symptom onset to diagnosis and from diagnosis to initiation of disease-modifying therapy was longer among those &lt;12 years of age. CONCLUSIONS: Pediatric MS in the United States is characterized by racial and ethnic diversity, a high proportion of children with foreign-born parents, and differences in clinical features and timing of treatment among those &lt;12 years of age compared with older children.