Giant Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Treated with Multiorgan Resection: a Case Report and Review of the Literature.

Malignant peripheral nerve sheath tumors (MPNST) are rare. Although they originate from Schwann cells or pluripotent neural crest cells, they constitute less than 10% of all soft tissue sarcomas and more than 60% develop on the basis of neurofibromatosis. It is difficult to diagnose MPNST. Although it mostly occurs in the head and neck region or upper extremities, only 1% of cases are located in the retroperitoneal region. The main treatment is surgery, but survival results are not satisfactory even after surgery with R0 resection. They are not sensitive to chemotherapy and radiotherapy and tend to recur locally. The mass detected by imaging in a 57-year-old male patient who admitted to hospital with the complaint of abdominal pain was excised with clear surgical margins. The tumor was located in the left upper quadrant of the abdomen and seemed to invade the pancreas and left kidney in the computed tomography images. The patient had no history of neurofibromatosis or radiation. In this study, it was aimed to present our case diagnosed with retroperitoneal MPNST and treated with multiorgan resection, which is a rare entity, and to increase the awareness of clinicians about the diagnosis, treatment and prognosis of this rare tumor.

A rare form of persistent Mullerian duct syndrome: Transverse testicular ectopia with germ cell testis cancer and hernia uteri inguinalis.

Persistent Mullerian duct syndrome is a rare form disorder of sexual differentiation characterised by the persistence of Mullerian derivatives (fallopian tubes, uterus and the proximal vagina) in males with an XY karyotype and normal virilisation. We report a case of a 29-year-old man with right transverse testicular ectopia, mix germ cell cancer at ectopic right testis and left-sided obstructed inguinal hernia containing a uterus and fallopian tube. We performed orchiectomy and hysterectomy on the patient.

Comparison of staging systems in gastric carcinoma.

AIM: Gastric carcinoma is one of the most frequent cancers and leading causes of cancer-related death worldwide. Treatment strategies are planned according to the Tumour-Node-Metastasis (TNM) stage of the disease. However, the prognosis varies substantially even within the same stage. Prognostic nomograms were designed to overcome this diversity. In this study, staging systems and prognostic tools are compared in the context of their ability to predict patients' prognosis. METHODS: Records of 391 patients operated for gastric cancer from January 2006 to September 2013 were analysed retrospectively. TNM staging system, Metastatic lymph node ratio (LNR), Kattan Prognostic Tool and Prognostic Tool of Italian Research Group on Gastric Cancer (GIRCG) were compared with the patients' survival times by their concordance indices and correlation coefficients. RESULTS: A total of 343 patients were included in the study. Concordance indices of the compared staging systems were 0.678 for TNM, 0.601 for GIRCG scale, 0.646 for LNR stage and 0.680 for Kattan scale. Pearson correlation coefficients were 0.404 for TNM staging, 0.314 for GIRCG scale, 0.304 for LNR stage and -0.406 for Kattan scale. Spearman correlation coefficients were 0.383, 0.311, 0.310 and -0.400 respectively. CONCLUSION: Based on these results, Kattan prognostic scale was found to be the most accurate system for predicting mortality. This was followed by TNM staging system.