Inferior vena cava leiomyosarcoma is a malignant neoplasm rarely diagnosed. The study presented two cases of inferior vena cava leiomyosarcomas subject to treatment at the Department of Oncology, Center of Oncology in Warsaw. The first patient underwent surgery after a two-year thorough gastroenterological diagnostic process, being suspected of pancreatic cancer. Proper diagnosis was established intraoperatively, the tumor was completely excised. After ten months the patient was subject to chemotherapy, due to the presence of distant metastatic lesions. Chemotherapy was stopped after 24 months, due to disease progression. The patient died six months, thereafter. The second patient underwent surgery after a short diagnostic process, being suspected of an adrenal gland tumor. Proper diagnosis was established intraoperatively, and the tumor was completely excised. The patient remained under close oncological control. Three years after the procedure disease recurrence was not observed. Retroperitoneal space tumors pose a significant clinical problem. Rapid diagnosis and proper therapeutic decisions often determine the future of such patients. The five-year survival rate of patients subject to radical surgical treatment, due to inferior vena cava leiomyosarcomasa ranges between 33 and 55%.
Tumors of the pancreas and the pancreatoduodenal area constitute a group characterized by highly variable histological structure and differing prognosis. Adenocarcinoma of the pancreas is the most common tumor of this area and prognosis of patients with this tumor still remains poor.The aim of the study was to present characteristics of tumors of the pancreatoduodenal are and the pancreas in our own material.Material and methods. Since May 2005, a systematic prospective assessment was started of patients with tumors of the pancreas that underwent surgery in Department of Surgery, Clinic of Oncology, Center of Oncology in Warsaw. Within 22 months, between 1.03.2005 and 31.12.2006, 85 patients (42 women, 43 men) with lesions of the pancreas or ampulla of Vater, potentially amenable to resection, were qualified for surgical treatment. An average age of patients that underwent surgery was 61.2 years.Results. Surgical resection was performed in 43 patients (50.6%), including: 17 pancreatoduodenectomies (39.5%), 7 total pancreatectomies (16.3%), 16 peripheral splenopancreatectomies (37.2%) and 3 local resections/lesion extirpation (7%).The most common reasons for refraining from resection included: involvement of mesenteric vessels (12/42 - 28.6%), infiltration of portal vein system (19%), liver metastases (19%).Adenocarcinoma of the pancreas was the predominant histological diagnosis (32/85 - 37.64%), followed by chronic pancreatitis (14.1%). Tumors of uncertain or unknown malignant potential (neuroendocrine tumors, mucinous cystic neoplasm, solid cystic papillary tumor) were diagnosed in 13 patients (15.3%).Conclusions. Despite more and more precise preoperative diagnosis, significant percentage of patients qualified for radical resection of tumors of the pancreas and pancreatoduodenal area, have lesions not amenable to surgical resection. Significant percentage of tumors of uncertain or unknown malignant potential in the presented material must be emphasized. This group of patients requires different management approach than patients with adenocarcinoma.
Multiple endocrine neoplasia type 1 (MEN1) syndrome is an autosomal dominant hereditary disorder characterised by coexistence of pancreatic neuroendocrine tumours (pNETs) with parathyroid and pituitary tumours. PNETs, including mostly non-functioning tumours, gastrinoma and insulinoma, occur in nearly 95% of MEN1 patients and account for over 50% of disorder-related mortality. Therefore, early initiation of screening for pNET using biochemical and imaging tests as well as appropriate surgical and systemic treatment are of particular importance for this group of patients. Currently, there are no clearly defined guidelines which determine the optimal methods for detection and treatment of pNET in MEN1. Caution should be exercised when applying the guidelines designed for patients with sporadic pNET to MEN1 patients as the clinical course of the disorder is slightly different, involving multifocality of lesions and younger age of patients at onset. This paper discusses the distinctive features and challenges in diagnosing and treating pNETs in MEN1 patients.
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