Abdominal splenosis mimicking peritoneal carcinomatosis of ovarian cancer.

We present the clinical case of a 53-year-old woman referred for suspicion of recurrence of a mesonephric-like adenocarcinoma of the ovary. Abdominal and pelvic CT revealed multiple round/oval solid nodules with similar density scattered throughout the abdomen and pelvis, the biggest ones appearing in the left hypochondrium; no normal-appearing spleen or ascites were observed. These radiological findings and the absence of significant elevation of CA 125 levels made the radiologists hypothesize that these aspects were related to abdominal splenosis. They asked the patient about previous medical history of splenic injury, which she confirmed, referring it was a consequence of a remote major trauma. A 99mTc-labeled heat-denatured erythrocytes (99mTc-DRBC) scintigraphy/ hybrid SPECT/CT was then performed for definitive diagnosis; it showed spleen remnants as foci of increased radiopharmaceutical uptake in the same locations as the nodules appearing in the CT. This diagnostic work-up was consistent with abdominal splenosis, mimicking peritoneal carcinomatosis of ovarian cancer.

End of treatment FDG-PET in primary mediastinal B-cell lymphoma treated with R-chemotherapy: Prognostic indicator and implications for consolidation radiotherapy.

The ideal therapeutic regimen in primary mediastinal B-cell lymphoma (PMBCL) is controversial and may include consolidation radiotherapy (RT). An adequate strategy is essential in a population where long-term effects of RT are significant. We evaluated the prognostic value of end-of-treatment (EOT) FDG-PET in 50 patients receiving rituximab and anthracycline-containing chemotherapy and its implications for consolidative RT. Thirty patients (60%) obtained complete metabolic response (CMR), five received consolidation RT. The remaining patients had partial response (14) and progression (6). Of these, 12 received mediastinal RT, six salvage chemotherapy, and two no further treatment. Five-year progression free survival was 100% and 48% (95% CI 30%-77%) in patients with negative and positive EOT FDG-PET, respectively (P < .001). Five-year overall survival for negative and positive EOT FDG-PET was 100% and 67% (95% CI 48%-93%) respectively (P = .001). Within positive EOT FDG-PET cases, an association was found between Deauville score and survival. The negative predictive value (NPV) of EOT FDG-PET for disease relapse/progression was 100% (95% CI 0.88-1.00); the positive predictive value was 47% (95% CI 0.24-0.71). This study demonstrates the importance of metabolic assessment in PMBCL and is relevant for its high NPV. Our data favor the use of EOT FDG-PET for decisions concerning RT.

Selumetinib for plexiform neurofibromas in neurofibromatosis type 1: a single-institution experience.

BACKGROUND: Plexiform neurofibromas (PN) are the most frequent tumors associated with Neurofibromatosis type 1 (NF-1). PN can cause significant complications, including pain, functional impairment, and disfigurement. There is no efficient medical treatment and, surgical resection of large PN is frequently infeasible. Selumetinib (AZD6244/ARRY-142886) is a mitogen-activated protein kinase enzyme (MEK1/2) inhibitor and works by targeting the MAPK pathway. It is an investigational treatment option for inoperable symptomatic PN associated with NF-1. Herein, we describe a single institutional experience with selumetinib for inoperable PN in NF-1. METHODS: Case series study of demographics, clinical, baseline characteristics, treatment effect, and follow-up of consecutive genetically confirmed NF1 patients with inoperable PN associated with significant or potential significant morbidity treated with selumetinib (April 2018 to April 2019). RESULTS: Nineteen patients were treated with selumetinib. Predominant target locations were head and neck (31.6%, 6/19), chest (26.3%, 5/19) and pelvis (21%, 4/19) and the most important comorbidities were disfigurement (47.4%, 9/19) and pain (26.3%, 5/19). The mean follow-up time was 223 days (range 35-420 days). All but one had sustained clinical improvement, mainly in the first 60-90 days of treatment. In one patient, the treatment was suspended after 168 days (lack of clear benefit and left ventricular ejection fraction drop). There were no adverse effects leading to treatment suspension. CONCLUSIONS: In the first observational study of selumetinib for NF-1 associated PN we showed that the drug was associated with clinical and radiological improvement. Our study also confirms the safety described in the clinical trials.

Second Primary Cancer in Patients with Differentiated Thyroid Cancer: Does Radioiodine Play a Role?

BACKGROUND: Well-differentiated thyroid cancer (WDTC) is the most common endocrine neoplasia, and its incidence is rising. Studies have reported an increased risk of second primary cancer (SPC) in WDTC survivors, but its relationship with radioiodine treatment (RAIT) and other risk factors remains controversial. This study evaluated whether RAIT is an independent risk factor for SPC in WDTC patients. METHODS: This was a retrospective single-center study. A total of 2031 patients with WDTC diagnosed between 1998 and 2009, treated and followed at the authors' tertiary cancer center, were included. RESULTS: The median age of patients was 48 years (range 5-90 years); 83% were women and 77% underwent RAIT. The median follow-up was 8.8 years (range 5.0-17.0 years). A total of 130 SPC were diagnosed: 108/1570 (6.9%) received RAIT (RAIT+) and 22/461 (4.8%) did not (RAIT-). The most common SPC was breast cancer (31%), followed by genitourinary and gastrointestinal cancer (18% each). The 10-year cumulative incidence of SPC was 8.2% in RAIT+ and 4.5% in RAIT-. The absolute risk increase in the RAIT+ group versus the RAIT- group at 10 years of follow-up was 0.039 [confidence interval (CI) 0.011-0.067] per patient-year. The number needed to harm (NNH) was 25.6 [CI 15.0-87.2], indicating that on average during a 10-year follow-up period, there is one additional case of SPC for every 26 patients receiving RAIT. When controlling for age, sex, and familial and personal histories of cancer, there was an 84% increase in the risk of SPC in the RAIT+ group compared to the RAIT- group (p = 0.026; relative risk = 1.84 [CI 1.02-3.31]). There was an association between SPC incidence and total cumulative activity administered, which was statistically significant >200 mCi. The incidence of SPC was higher in both the WDCT and the RAIT+ cohorts compared to the general population (standardized incidence ratios = 1.32 and 1.40, respectively). CONCLUSION: These results indicate that in spite of the low incidence of SPC in WDTC patients, the risk is increased after RAIT, particularly for activities >200 mCi. Thus, considering the excellent survival of patients with WDTC, clinicians need to weigh the risks and benefits of RAIT, especially in patients with low-risk thyroid cancer.

Does Hypoxic Response Mediate Primary Resistance to Sunitinib in Untreated Locally Advanced Breast Cancer?

BACKGROUND: The antiangiogenic drug sunitinib has never been evaluated as single agent in untreated breast cancer patients. OBJECTIVE: We aimed to characterize the activity of sunitinib, alone and with docetaxel, in untreated locally advanced or operable breast cancer and to uncover the mechanisms of response. METHOD: Patients were treated with an upfront window of sunitinib followed by four cycles of sunitinib plus docetaxel. Response, resistance and toxicity were evaluated according to standard clinical parameters, magnetic resonance imaging, positron emission tomography, standard pathology characterization, molecular pathology and gene expression profiling. RESULTS: Twelve patients were included. We detected primary resistance to sunitinib in the upfront window in untreated breast cancer, as evidenced by four non-responding patients. At surgery, five patients had viable tumor in the breast and axilla, four had viable tumor cells in the breast alone and three were taken off study and thus not evaluated, due to unacceptable toxicity. Early functional imaging was useful in predicting response. There were no clinical complete responses. Comparison of tumor gene expression profiling data between early responders and non-responders allowed us to identify the up-regulation of VEGF and angiogenic pathways in non-responders. Specifically, in tumors resistant to single-agent sunitinib we detected a transcriptional response to hypoxia characterized by over-expression of several HIF1α target genes. CONCLUSION: In this report of single-agent sunitinib treatment in untreated localized breast cancer patients, we found evidence of primary resistance to sunitinib, likely mediated by up-regulation of hypoxia responsive genes.

Bone scan usefulness in patients with painful hip or knee prosthesis: 10 situations that can cause pain, other than loosening and infection.

In recent years, with the higher median life expectancy, the number of hip and knee replacements has increased. Clinical examination and morphological studies are essential to evaluate patients with a painful arthroplasty. Nuclear medicine examinations also play an important role, their main usefulness being the exclusion of prosthesis complications. Nevertheless, conventional examinations, namely bone scan and white blood cell scintigraphy, can also identify complications, such as loosening and infection. This study describes the normal and pathologic patterns of a bone scan and exemplifies ten common situations that can cause pain in patients with hip or knee arthroplasty, other than loosening and infection, which can be disclosed on a bone scintigraphy. The ten situations that should be considered and looked for when analysing a bone scan are: referred pain, patellofemoral pain syndrome, fractures, fissures, abscess/haematoma, bone insert behaviour, heterotopic ossification, greater trochanter pseudarthrosis, osteoarthritis extension in a knee with an unicompartmental prosthesis, and systemic disease with bone involvement.

Thyroid Carcinoma Detected by 18F-Fluorodeoxyglucose Positron Emission Tomography Among Individuals Without Prior Evidence of Thyroid Disease: Relevance and Clinicopathologic Features.

OBJECTIVE: The expanding use of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) has contributed to an increasing number of thyroid incidentalomas. The present study aimed to estimate the prevalence of 18F-FDG-PET thyroid incidentalomas and evaluate the clinicopathologic features of thyroid malignancies detected by 18F-FDG-PET. METHODS: We reviewed all 18F-FDG-PET exams performed at the Portuguese Institute of Oncology, Lisbon, between 2007 and 2012 (n = 9,374). The inclusion criteria were focal thyroid uptake and absence of known thyroid disease. RESULTS: Focal thyroid uptake was observed in 60 out of 9,374 18F-FDG-PET exams (prevalence of 0.64%). Fine-needle aspiration cytology (FNAC) was performed in 23 patients and reported as malignant in 14 cases (56.5% primary thyroid carcinoma; 4.3% secondary malignancy), as benign in 7 cases (30.5%) and as follicular lesion of undetermined significance in 2 cases (8.7%). Fourteen patients had surgery. A final histologic diagnosis of papillary thyroid carcinoma was established in 12 cases (52.2%). Three were multifocal (25.0%); 8 had extrathyroidal extension (66.7%); 5 had angioinvasion (41.7%); 3 had lymph nodes metastases (25.0%) and 2 showed lung metastases (16.7%). Overall, 91.7% were classified as intermediate or high risk. All patients had radioiodine therapy. At the last observation (mean follow-up was 29.9 months), persistent or recurrent disease was identified in 4 patients (33.3%) and none died from thyroid malignancy. CONCLUSIONS: Thyroid carcinomas disclosed by 18F-FDG-PET are associated with aggressive histological criteria likely to carry a worse prognosis.

[Treatment and follow up protocol in differentiated thyroid carcinomas of follicular origin]. / Protocolo de tratamento e seguimento dos carcinomas diferenciados da tiróide de origem folicular.

Differentiated thyroid carcinoma of follicular origin (DTCFO), although not very frequent, has registered a raising incidence in the last decades. In the majority of the cases, DTCFO is a curable disease when treated and monitored by experienced, multidisciplinary teams. These factors contribute to an increasing number of DTCFO survivors requiring life-long monitoring, due to the possibility of occurrence of recurrences many years after the initial treatment. Several aspects of the treatment and management of these patients are still controversial. The present protocol represents the consensus of the members of the Grupo de Estudo da Tiróide of the Sociedade Portuguesa de Endocrinologia, Diabetes e Metabolismo. It aims to define guidelines, in agreement with the current state of the art and contemplating the necessary adaptations to local constrains, that ensure decreased mortality and protection of patients' quality of life, avoiding unnecessarily aggressive or ineffective treatments, optimizing the use of the available resources.