Cerebellopontine angle paraganglioma – report of a case and review of literature

Summary Majority of the cerebellopontine angle (CPA) tumors are acoustic neuromas, while bulk of the non-acoustic tumors are formed by meningiomas and epidermoid cysts. Primary paraganglioma is a rare tumor in this location, with only two such cases having been reported in the literature, till date. Recently, a case has been described wherein a paraganglioma was apparently arising as a primary lesion in the cerebellar hemisphere. We report another case of an intracranial paraganglioma of the CPA in a 40-year-old female, which did not have any vascular attachment but had focal cerebellar extension.     

Primary Leiomyosarcoma of the Pancreas—a Case Report and a Comprehensive Review

Purpose

Primary mesenchymal tumors of the pancreas are rare, with leiomyosarcomas the most encountered entities among the pancreatic sarcomas. With few exceptions, single case reports published over the last six decades constitute the entire scientific literature on this topic. Thus, evidence regarding clinical decision-making is scant.

Methods

Based on a case report and an extensive literature search in PubMed, we discuss the clinical aspects and current management of this rare malignancy.

Results

We identified only two papers with more than a single case presentation; these institutional patient series were limited to five and nine patients. Additionally, a few papers sought to summarize the individual case reports published in the English and/or Chinese language. The clinical presentation is rather non-specific. Moreover, modern imaging modalities are insufficiently accurate to diagnose leiomyosarcoma of the pancreas. Treatment goals include a complete resection with free margins. Proper morphologic examination using immunohistochemistry and the application of a grading system are clinically important for prognostication. The efficacy of adjuvant treatments has not been established.

Conclusion

Primary pancreatic leiomyosarcoma is extremely rare, and the scientific literature is primarily based on single case reports. Conclusions on management and prognosis should be drawn with caution. A multidisciplinary team consultation is warranted to discuss a thorough individual treatment plan based on the available scientific literature, despite its low evidence level.

     

Rhabdoid Meningioma in a Child： Report of a Case and Literatures Review

Meningiomas occur in 1%-4% of primary intracranial tumors in the pediatric group, and is increasing in incidence with age. Some authors have reported that meningioma is more prevalent among adult males, but there is no gender prevalence. The accepted origin of meningiomas is from the arachnoid ceils lining the meninges, or the choroid plexuses. Since Beckwith and Palmer introduced the term ＇rhabdoid tumor＇ in 1978 in reference to a subgroup of childhood malignant renal tumors, many tumors with a rhabdoid morphology have been reported in various sites, including the central nervous system. In 1998 Kepes et al.     

Intake of fruit and vegetables and risk of bladder cancer: a dose–response meta-analysis of observational studies

Background

Observational studies suggest an association between fruit and vegetables intake and risk of bladder cancer, but the results are controversial.

Methods

We therefore summarized the evidence from observational studies in categorical, linear, and nonlinear, dose–response meta-analysis. Pertinent studies were identified by searching EMBASE and PubMed from their inception to August 2013.

Results

Thirty-one observational studies involving 12,610 cases and 1,121,649 participants were included. The combined rate ratio (RR, 95 % CI) of bladder cancer for the highest versus lowest intake was 0.83 (0.69–0.99) for total fruit and vegetables, 0.81 (0.70–0.93) for total vegetables, 0.77 (0.69–0.87) for total fruit, 0.84 (0.77–0.91) for cruciferous vegetables, 0.79 (0.68–0.91) for citrus fruits, and 0.74 (0.66–0.84) for yellow–orange vegetables. Subgroup analysis showed study design and gender as possible sources of heterogeneity. A nonlinear relationship was found of citrus fruits intake with risk of bladder cancer (P _{for nonlinearity} = 0.018), and the RRs (95 % CI) of bladder cancer were 0.87 (0.78–0.96), 0.80 (0.67–0.94), 0.79 (0.66–0.94), 0.79 (0.65–0.96), and 0.79 (0.64–0.99) for 30, 60, 90, 120, and 150 g/day. A nonlinear relationship was also found of yellow–orange vegetable intake with risk of bladder cancer risk (P _{for nonlinearity} = 0.033). Some evidence of publication bias was observed for fruit, citrus fruits, and yellow–orange vegetables.

Conclusion

This meta-analysis supports the hypothesis that intakes of fruit and vegetables may reduce the risk of bladder cancer. Future well-designed studies are required to confirm this finding.     

Heterogeneity and renal mass biopsy：a review of its role and reliability

Increased abdominal imaging has led to an increase in the detection of the incidental small renal mass（SRM）. With increasing recognition that the malignant potential of SRMs is heterogeneous, ranging from benign（15%-20%） to aggressive（20%）, enthusiasm for more conservative management strategies in the elderly and infirmed, such as active surveillance（AS）, have grown considerably. As the management of the SRM evolves to incorporate ablative techniques and AS for low risk disease, the role of renal mass biopsy（RMB） to help guide individualized therapy is evolving. Historically, the role of RMB was limited to the evaluation of suspected metastatic disease, renal abscess, or lymphoma. However, in the contemporary era, the role of biopsy has grown, most notably to identify patients who harbor benign lesions and for whom treatment, particularly the elderly or frail, may be avoided. When performing a RMB to guide initial clinical decision making for small, localized tumors, the most relevant questions are often relegated to proof of malignancy and documentation（if possible） of grade. However, significant intratumoral heterogeneity has been identified in clear cell renal cell carcinoma（ccRCC） that may lead to an underestimation of the genetic complexity of a tumor when single-biopsy procedures are used. Heterogeneous genomic landscapes and branched parallel evolution of ccRCCs with spatially separated subclones creates an illusion of clonal dominance when assessed by single biopsies and raises important questions regarding how tumors can be optimally sampled and whether future evolutionary tumor branches might be predictable and ultimately targetable. This work raises profound questions concerning the genetic landscape of cancer and how tumor heterogeneity may affect, and possibly confound, targeted diagnostic and therapeutic interventions. In this review, we discuss the current role of RMB, the implications of tumor heterogeneity on diagnostic accuracy, and highlight promising future directio     

Management of unresectable head and neck cancers — a retrospective analysis at a rural medical college of India

Objective

To study the natural course of disease in patients of advanced squamous cell carcinoma of head and neck (SCCHN) admitted in a rural tertiary hospital and to formulate an acceptable protocol of management and their prognosis.

Study design

A 5-year retrospective analysis of patients of advanced SCCHN attended in the department of Radiotherapy and admitted at department of ENT at B. S. Medical College, Bankura, West Bengal were analyzed for the present study.

Setting

B. S. Medical College, Bankura at West Bengal, which is a rural medical college and act as tertiary referral, center for three district of West Bengal namely Midnapur, Purulia, Bankura, part of Burdwan and adjoining area of Jharkhand state.

Patients

We investigated 100 patients in our hospital who presented with advanced and in-operable stage of SCCHN.

Eligibility criteria

Biopsy proved cases of SCCHN in stage III, stage IVA and stage IVB which is surgically unresectable and without any past history of radiotherapy and chemotherapy were included in this study.

Key demographic features

Eighty-nine men and 11 female with advanced SCCHN were included in this study. Their median age was 48 years (36–78 years) with a median weight of 44 kg and median Karnofsky score 70.

Interventions

Different types of palliative surgical intervention like Ryles’ tube insertion (16%), feeding jejunostomy (11%), tracheostomy (60%) and arterial ligation (2%) was done to relieve the symptoms. Forty-three patients received chemoradiation and 22 patients received only radiotherapy. Best supportive care was provided to 35 patients.

Outcome

Twenty-nine percent of patients had partial response in terms of disease control and none had complete response.

Conclusion

Symptomatic relief of respiratory obstruction, dysphagia and pain constitute the mainstay of treatment in the present study. A short course of radiotherapy is found to be effective in control of symptoms and helps to improve the quality-of-life. Complete response to therapy in advanced head and neck squamous cell carcinoma with a poor nutritional status is very difficult to achieve.     

The clinicopathological characteristics and surgical treatment of retroperitoneal angiomyolipoma： a case report and literature review of both English and Chinese

Angiomyolipoma usually present as incidental findings on routine imaging or laparotomy, but rarely they may give rise to massive hemorrhage. If bleeding occurs, the treatment of choice is to save life either by angiography with selective embolisation or surgery. Herein, we reported a case of a 32-year-old man who came to our hospital complaining of sudden colicky pain in the right hypochondria region, associated with nausea and vomiting. On physical examination, the patient was in a state of shock; blood pressure of 60/36 mmHg, heart rate of 160 beats/min, high abdominal distention, and non-coagulated blood was extracted from abdominal puncture. The abdominal computed tomography (CT) and arteriography showed a 20 × 15 × 15 cm mass suggestive of spontaneous rupture of liver neoplasm and intraperitoneal hemorrhage. On laparotomy, 1500 mL of non-coagulated blood was found, and the tumor located at right upper abdomen, which originated from retroperitoneum. The tumor was resected totally with hematoma and sutured the bleeding vessels. The histological study of the resected mass revealed the presence of angiomyolipoma.     

Exposure to acrylamide and human cancer—a review and meta-analysis of epidemiologic studies

BackgroundAcrylamide has been associated to cancer risk in rodents, but data on humans are inconclusive. We thus carried out a critical review and meta-analysis of studies of exposure to acrylamide and cancer.MethodsWe identified 586 publications, 25 presented relevant results. We conducted meta-analyses of studies of dietary intake based on random-effects models by calculating pooled relative risks (RR) and the corresponding 95% confidence intervals (CI). We combined results of occupational studies according to a fixed-effect model.ResultsThe summary RRs for an increase of 10 μg/day of acrylamide intake were close to unity for all the cancers considered, ranging from 0.98 for esophageal cancer to 1.01 for colon, endometrial, ovarian and kidney cancer. None of the estimates was significant. Exclusion of one case–control study from Sweden resulted in a summary RR of kidney cancer of 1.04 (95% CI 1.00–1.08). The combined standardized mortality ratios for high occupational exposure were 1.67 (95% CI 0.83–2.99) for pancreatic cancer and 2.22 (95% CI 0.81–4.84) for kidney cancer.ConclusionsAvailable studies consistently suggest a lack of an increased risk of most types of cancer from exposure to acrylamide. The main association that requires further monitoring involves kidney cancer.     

Inflammatory myofibroblastic tumor of the pancreatic head – a case report of a 6 months old child and review of the literature

Background

Inflammatory myofibroblastic tumors are rare in the pediatric population. Most common localizations were reported in the lungs. A localization in the pancreas needs differentiation from other tumors and chronic pancreatitis. Treatment is surgical resection, although there are reports of treatment with oral steroids and radiation therapy.

Case report.

A 6-month-old child was treated due to a tumor in the head of the pancreas. On admission he was jaundiced with pruritus. US and MRI confirmed pancreatic tumor. Preoperative biopsy wasn’t conclusive regarding the nature of the tumor. Duodenopancreatectomy was performed. Postoperative course was uneventful. Histologic examination confirmed the diagnosis of inflammatory myofibroblastic tumor. On follow up, he remained with no evidence of recurrence.

Conclusions

A literature review revealed 10 cases of pancreatic inflammatory myofibroblastic tumors in the pediatric age group. Our patient is the youngest reported. Despite major resection, there were no complications. However, management of this child might be possible with steroids, but conservative treatment might be insufficient, especially in aggressive forms of tumors.     

Mixed Medullary-follicular Thyroid Carcinoma： Report of a Case and Review of the Literarture

Mixed medullary-follicular carcinomas （MMFCs） are tumors of the thyroid that display morphological and immunohistochemical features of both medullary and follicular neoplasms. These tumors are rare and less than 40 cases have been described in the literature since the early 1980s The term medullary-follicular thyroid carcinoma denotes a tumor which exhibits the features of a medullary carcinoma and shows positive expression of calcitonin on immunohistochemistry. It is a follicular-derived thyroid carcinoma and is positive for thyroglobulin expression within the same tumor. Most reported cases have lymph node involvement at the time of diagnosis. In cases having disease progression, distant metastases develop in the lung, liver, mediastinum and bone. We report a case of a male patient with mixed medullary-follicular thyroid carcinoma and a review of the literature.     

Diagnosis and Surgical Treatment of 105 Cases with a Tumor of the Chest Wall

OBJECTIVE To summarize the experience in diagnosis and surgical treatment of 105 cases with a tumor of the chest wall,and to investigate re-construction of a large chest-wall defect after resection of a chest wall tumor.METHODS Clinical data from 105 patients with a tumor of the chest wall were retrospectively analyzed.There were 78 males and 27 females with ages ranging from 6 to 70 years.Of the 105 cases,94 had a primary tumor,among which 75 were benign,19 malignant and the other 11 metastatic.After a resection of a chest-wall tumor in 19 patients,reconstruction of the large chest-wall defect was conducted.RESULTS All surgical operations were smoothly performed,without an intraoperative death.The results of postoperative follow-up were as follows:48 patients with a benign tumor were still living and well,16 patients with a benign tumor died of other diseases,13 with a malignant tumor survived for a period from 21 months to 8 years,and the others with a malignancy died of local recurrence or distant metastasis.All of the 11 patients with a metastatic tumor died of carcinomatous deseases during a period from 10 to 76 months.CONCLUSION With regard to a primary costal tumor without a patho-logical diagnosis,a restricted radical excision should be conducted first.Use of suitable repairing materials is very important for reconstruction of a mas-sive chest-wall defect.     

Denosumab and giant cell tumour of bone—a review and future management considerations

Giant cell tumour of bone (gctb) is one type of giant-cell-rich bone lesion characterized by the presence of numerous multinucleated osteoclast-type giant cells. Giant cells are known to express rankl (receptor activator of nuclear factor κB ligand) and are responsible for the aggressive osteolytic nature of the tumour. No available treatment option is definitively effective in curing this disease, especially in surgically unsalvageable cases. In recent years, several studies of denosumab in patients with advanced or unresectable gctb have shown objective changes in tumour composition, reduced bony destruction, and clinical benefit.Denosumab is a fully human monoclonal antibody that targets and binds with high affinity and specificity to rankl. Several large phase iii studies have shown that denosumab is more effective than bisphosphonates in reducing skeletal morbidity arising from a wide range of tumours and that it can delay bone metastasis. The relevant articles are reviewed here. The controversies related to the future use of denosumab in the treatment of gctb are discussed.     

‘Drawing’ a Molecular Portrait of CIN and Cervical Cancer: a Review of Genome-Wide Molecular Profiling Data

In this review we summarize the results of studies employing high-throughput methods of profiling ofHPV-associated cervical intraepithelial neoplasia (CIN) and squamous cell cervical cancers at key intracellularregulatory levels to demonstrate the unique identity of the landscape of molecular changes underlying thisoncopathology, and to show how these changes are related to the ‘natural history’ of cervical cancer progressionand the formation of clinically significant properties of tumors. A step-wise character of cervical cancer progressionis a morphologically well-described fact and, as evidenced by genome-wide screenings, it is indeed the consistentchange of the molecular profiles of HPV-infected epithelial cells through which they progressively acquire thephenotypic hallmarks of cancerous cells. In this sense, CIN/cervical cancer is a unique model for studying thedriving forces and mechanisms of carcinogenesis. Recent research has allowed definition of the whole-genomespectrum of both random and regular molecular alterations, as well as changes either common to processes ofcarcinogenesis or specific for cervical cancer. Despite the existence of questions that are still to be investigated,these findings are of great value for the future development of approaches for the diagnostics and treatment ofcervical neoplasms.     

Safety and outcome of treatment of metastatic melanoma using 3-bromopyruvate：a concise literature review and case study

3-Bromopyruvate （3BP） is a new, promising anticancer alkylating agent with several notable functions. In addition to inhibiting key glycolysis enzymes including hexokinase II and lactate dehydrogenase （LDH）, 3BP also selectively inhibits mitochondrial oxidative phosphorylation, angiogenesis, and energy production in cancer cells. Moreover, 3BP induces hydrogen peroxide generation in cancer cells （oxidative stress effect） and competes with the LDH substrates pyruvate and lactate. There is only one published human clinical study showing that 3BP was effective in treating fibrolamellar hepatocellular carcinoma. LDH is a good measure for tumor evaluation and predicts the outcome of treatment better than the presence of a residual tumor mass. According to the Warburg effect, LDH is responsible for lactate synthesis, which facilitates cancer cell survival, progression, aggressiveness, metastasis, and angiogenesis. Lactate produced through LDH activity fuels aerobic cell populations inside tumors via metabolic symbiosis. In melanoma, the most deadly skin cancer, 3BP induced necrotic cell death in sensitive cells, whereas high glutathione （GSH） content made other melanoma cells resistant to 3BP. Concurrent use of a GSH depletor with 3BP killed resistant melanoma cells. Survival of melanoma patients was inversely associated with high serum LDH levels, which was reported to be highly predictive of melanoma treatment in randomized clinical trials. Here, we report a 28-year-old man presented with stage IV metastatic melanoma affecting the back, left pleura, and lung. The disease caused total destruction of the left lung and a high serum LDH level （4,283 U/L）. After ethics committee approval and written patient consent, the patient received 3BP intravenous infusions （1-2.2 mg/kg）, but the anticancer effect was minimal as indicated by a high serum LDH level. This may have been due to high tumor GSH content. On combining oral paracetamol, which depletes tumor GSH, with 3BP treatment, serum LDH level drop