Embryonal tumor with abundant neuropil and true rosettes: a report of three cases of a rare tumor, with an unusual case showing rhabdomyoblastic and melanocytic differentiation.

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is an increasingly recognized entity that belongs to the family of embryonal tumors of the CNS. The authors present three cases of this rare tumor that were encountered at King Hussein Cancer Center, Amman, Jordan. Discussion of the clinicopathological findings is presented along with a recent literature review. Sixteen-, 57- and 30-month-old children presented with tumors located in the pineal gland, the right fronto- parieto-temporal region and the cerebellum, respectively. The findings of hypocellular neuropil as well as the characteristic ependymoblastic rosettes were seen. In addition the third case showed an abnormal combination of patterns including melanocytic and rhabdomyoblastic differentiation. The tumors stained positively for synaptophysin in the neuropil and small cell component, while the ependymoblastic rosettes stained for vimentin only. Epithelial membrane antigen and CD99 were negative in all components. One of the cases showed tetraploidy of chromosome 2. All cases exhibited an aggressive course. This is a rare and recently recognized tumor with dismal outcome, and reporting of additional new cases should help in gaining more knowledge about it.

Popliteal lymph node dissection for metastatic squamous cell carcinoma: a case report of an uncommon procedure for an uncommon presentation.

Lymph node metastasis from cutaneous squamous cell carcinoma is uncommon. The popliteal fossa is rarely involved with metastasis. Popliteal lymph node dissection is uncommonly performed and not frequently discussed in the literature. We present a case of squamous cell carcinoma of the heel with popliteal and inguinal metastasis. This is followed by a description of the relevant anatomy of the popliteal fossa and the technique of popliteal lymphadenectomy.

Upper abdominal normal organ contouring guidelines and atlas: a Radiation Therapy Oncology Group consensus.

PURPOSE: To standardize upper abdominal normal organ contouring guidelines for Radiation Therapy Oncology Group (RTOG) trials. METHODS AND MATERIALS: Twelve expert radiation oncologists contoured the liver, esophagus, gastroesophageal junction (GEJ), stomach, duodenum, and common bile duct (CBD), and reviewed and edited 33 additional normal organ and blood vessel contours on an anonymized patient computed tomography (CT) dataset. Contours were overlaid and compared for agreement using MATLAB (MathWorks, Natick, MA). S95 contours, defined as the binomial distribution to generate 95% group consensus contours, and normal organ contouring definitions were generated and reviewed by the panel. RESULTS: There was excellent consistency and agreement of the liver, duodenal, and stomach contours, with substantial consistency for the esophagus contour, and moderate consistency for the GEJ and CBD contours using a Kappa statistic. Consensus definitions, detailed normal organ contouring recommendations and high-resolution images were developed. CONCLUSIONS: Consensus contouring guidelines and a CT image atlas should improve contouring uniformity in radiation oncology clinical planning and RTOG trials.

Brain stem gliomas: a clinicopathological study from a single cancer center.

Brain stem gliomas (BSG) are rare tumors occurring predominantly in childhood. They are mostly of astrocytic origin and are divided into infiltrative versus circumscribed types, with different prognoses. The diagnosis is mainly based on MRI findings, and biopsy is rarely performed. This is a retrospective study of BSG with available biopsies diagnosed at our center over 6-year period. Fifteen cases were identified, with a predominance of females. The median age was 7 years, and the mean duration of symptoms was <6 weeks in 58.3% (n = 7) of cases. MRI was typical of diffuse pontine gliomas in 64.3% (n = 9) of cases. Radiotherapy was the commonest modality of treatment, and the median overall survival was 21.7 months. Twelve cases were consistent with infiltrative astrocytoma of various grades (2 grade II, 7 grade III and 3 grade IV). Entrapped normal neurons and mitosis were the commonest findings indicating infiltrative growth and high grade, respectively, and those correlated significantly with immunostaining for neurofilament protein and Ki-67 of ≥3%. Overall survival correlated only with the duration of symptoms and tumor grade on biopsies. A limited panel of immunostains might be useful in undetermined cases to decide on the growth pattern and grade.

Stereotactic body radiation therapy for hepatocellular carcinoma.

Stereotactic body radiotherapy (SBRT), in which highly conformal potent radiation doses are delivered in fewer fractions than traditional radiation therapy (RT), is an increasingly popular treatment for hepatocellular carcinoma (HCC). The great majority of HCCs smaller than 6 cm and with Child-Pugh A liver function are controlled with SBRT with limited toxicity. Long-term local control is reduced in larger tumors, and toxicity is increased in patients with Child-Pugh B or C liver function. SBRT is an effective treatment for tumor vascular thrombi and can lead to sustained vascular recanalization. The first site of recurrence following SBRT is most often within the liver, away from the high dose volume, providing rationale for combining SBRT with regional or systemic therapies. Randomized trials of SBRT are warranted.

Management strategies for locoregional recurrence in early-stage gastric cancer: retrospective analysis and comprehensive literature review.

OBJECTIVE: To present a comprehensive account and literature review addressing the anatomical distribution, natural history, and management strategies for locoregional recurrence in early-stage gastric cancer (EGC). PATIENTS AND METHODS: Retrospective chart review of patients presenting with EGC recurrence at King Hussein Cancer Center (Amman, Jordan) between July 2006 and May 2009. A literature review of publications addressing recurrence following surgery for EGC was undertaken via a systematic search of PUBMED database and National Comprehensive Cancer Network (NCCN) guideline updates. RESULTS: Seventeen patients presented with EGC, three of whom (17.6%) were pathologically staged as T2N1 [1/33 lymph nodes (LNs)], T1N0, and T1N0 were afflicted by recurrence following R0 partial gastrectomy. Literature review yielded 18 studies specifically addressing recurrence in EGC. Several management strategies have been proposed for isolated recurrence following gastrectomy in EGC. NCCN clinical practice guideline updates do not take into consideration whether the recurrence is isolated or widespread and whether the initial stage is early or advanced. CONCLUSIONS: While acknowledging the limitations of this study, including the small sample size and the short follow-up period, it appears clear that oncologic treatment is possible for EGC recurrence, particularly, in patients with isolated relapse. Guideline updates should differentiate between management strategies suitable for recurrence occurring in early versus advanced initial cancer stage.

Radiotherapy with concurrent or sequential temozolomide in elderly patients with glioblastoma multiforme.

OBJECTIVE: The objective of this article was to evaluate therapeutic outcomes of elderly patients with glioblastoma multiforme (GBM) treated by surgery followed by combined modality therapy and compare achievable outcomes to those of a younger age population. METHODS AND MATERIALS: Seventy-eight adult patients with histologically confirmed grade IV astrocytoma were treated at King Hussein Cancer Center (Amman, Jordan) between September 2004 and December 2008. Records were retrospectively reviewed and included 55 males and 23 females between 19 and 78 years of age (median age 50 years). This case series included 20 patients aged 60 years or older. All patients underwent craniotomy followed radiotherapy and concurrent or sequential temozolomide. The follow-up ranged from 1 to 56 months (median 9.4 months). RESULTS: The median survival for the whole cohort was 13.8 months. The median survival for patients less than 60 years was 14.3 months and for patients 60 years or older was 12.3 months (P = 0.19). Among elderly patients, radical surgical resection (P = 0.002), concurrent delivery of chemoradiation (0.041) and radiotherapy dose ≥ 5400 cGy (P = 0.0001) conferred statistically significant improvements in overall survival. CONCLUSION: Management of GBM in elderly patients should include maximal surgical resection followed by radiotherapy and temozolomide whenever medically feasible. Outcomes comparable to those obtained in younger age groups can be expected. Our results indicate that concurrent chemoradiation is superior to sequential chemoradiation in these patients.

The challenges of managing glioblastoma multiforme in developing countries: a trade-off between cost and quality of care.

BACKGROUND AND OBJECTIVES: The management of glioblastoma multiforme (GBM) in developing countries is hindered by the paucity of clear protocols due in part to growing economic constraints and the lack of availability of expensive chemotherapeutic agents. We evaluated the deliverable treatment protocols and achievable outcomes for patients with GBM in a low-income country prior and subsequent to the worldwide adoption of temozolomide. DESIGN AND SETTING: Retrospective case series. PATIENTS AND METHODS: Charts of consecutive patients with a pathologic diagnosis of high-grade glioma diagnosed between January 2003 and December 2008 were retrospectively reviewed. RESULTS: We identified 146 adult patients, including 105 males and 41 females between 19 and 81 years of age (median age, 51 years), with histologically confirmed high-grade glioma. All patients underwent craniotomy. Eighty-two patients were treated with radiotherapy and temozolomide, of whom 42 patients received temozolomide concurrent with radiation followed by adjuvant temozolomide; 40 patients received irradiation followed sequentially by 6 cycles of temozolomide. In 40 patients irradiation was utilized as a single modality treatment adjuvant to surgery. The follow-up ranged from 1 to 56 months (median, 9.4 months). The median survival for the whole cohort was 10.2 months. The median survival for the radiotherapy-alone group was 5.3 months and for combined radiotherapy/temozolomide was 14.8 months. Survival was similar in both concurrent and sequential groups. Temozolomide conferred a statistically significant survival benefit of 9 months compared with standard therapeutic modalities. CONCLUSIONS: The results compare favorably to those reported in developed nations. Current management of GBM in developing countries should include maximal surgical resection followed by radiotherapy/temozolomide whenever medically and/or financially feasible. Outcomes comparable to those obtained within the context of randomized trials can be expected in low-income settings if healthcare delivery is carefully planned. Our results indicate that concurrent and sequential regimens are equally effective in these patients.

Ileocaecal intussusception secondary to metastatic phyllodes tumour of the breast.

A patient with phyllodes tumour of the breast is discussed. During follow-up, she presented with intestinal obstruction caused by ileocaecal intussusception. The cause of the intussusception was metastatic phyllodes tumour, which is a unique presentation.

Local control of the primary tumour in metastatic neuroblastoma.

The previous studies have stressed on the importance of loco-regional control in the management of high-risk neuroblastoma. We searched the Surveillance, Epidemiology and End-Results (SEER) database for patients older than 2years with metastatic neuroblastoma who were diagnosed from 1998 to 2005. We identified 291 patients (mean age, 4.35years). The 5-year survival estimate was 53.2%+/-6.4% for patients who had complete resection of their primary tumours (n=116) and 35.7%+/-4.7% for patients who did not have complete resection (p=0.003). External-beam radiotherapy did not affect survival (p=0.79); this finding has to be taken with caution due to the study limitations.