Leiomyosarcoma of the inferior vena cava. Report of two cases and review of the literature.

INTRODUCTION: Leiomyosarcoma (LMS) occurs most frequently in the inferior vena cav (IVC). Since Perl's first description in 1871, about 300 cases of IVX have been reported in English literature. The Authors present their personal experience of two case of IVC leiomyosarcoma treated in their Institution. PATIENTS AND METHODS: Clinical and pathologic data, surgical management, ourcomes and follow-up of two patients admitted nb the Authors' Division respectively on February and November 2004 were collected. RESULTS: There were a 49-years-old female with rare epigastric pain and a 42-years-old female with one-month history of abdominal discomfort and weight loss. After preoperative evaluation, both of the patients had localised and resectable tumors and underwent radical surgical excision, with prosthetic replacement of the IVC in the first patient and ligation of the IVC in the other one. The first patient is still alive but in an advanced disease. The second patient died of recurrence at seven months. DISCUSSION: LMS of IVC is a rare tumor. The diagnosis is often delayed because of non-specificic symptoms. Radical resection with surgical margins free of tumor is the treatment of choice. The extent of venous resection does not seem to affect the survival. The type of IVC reconstruction is based on the extent of venal wall involvement. The prognosis is often poor because of tumor recurrence and/or metastatic disease. The role of chemo and radio therapy is not clear yet. CONCLUSION: Long term survival is related to an extensive surgery, being surgical margins free of tumor the only prognostic factor with significant influence on patients survival.

"Small is beautiful" A series of ileo-anal anastomoses performed with the 25 - mm circular stapler.

AIM: With the idea that a small diameter stapler should cause less sphincter trauma, we began to use the 25mm circular stapler to perform ileo-pouch-anal anastomosis (IPAA) and we report our experience. MATERIAL OF STUDY: A retrospective study using a bowel function questionnaire and a quality of life questionnaire has been conducted on a group of patients who underwent IPAA using a 25mm stapler RESULTS: We performed IPAA using a 25mm circular stapler in 37 patients. Postoperative mortality was nil and morbidity was 27%. One anastomotic stenosis occurred. Long term follow-up information was available on 28 patients. Mean follow-up was 70 months (range 8-177). Mean number of bowel movements was 4.5 (range 2-10, median 4.5) during the day and 0.9 (range 0-10, median 0) at night. Out of 28 patients, 19 (68%) were fully continent and 32% had occasional soiling, no one reported incontinence. All patients except one were able to withold their stool for more than 15 minutes. Daytime pad use was: never 86%, occasionally 3%, frequently 11%; nightime pas use was never 86%, occasionally 7% and frequently 7%. Bowel regulating drugs use was never 82%, occasionally 14%, regularly 4%. Evacuation difficulties were: never 75%, occasionally 21%, frequently 4%. DISCUSSION: Our results compare favourably with the literature, which reports median bowel frequency 6-7.6/24h, 9.4- 33% urgency, 17-44% daytime soiling and 32-61% nighttime soiling. CONCLUSIONS: Our results must be considered preliminary but we found the 25-mm stapler safe and adequate to perform IPAA. KEY WORDS: IPAA, Ulcerative Colitis, Stapler, Function.

Unusual Presentation of Recurrent Early Stage Endometrial Carcinoma 28 Years after Primary Surgery.

Endometrial carcinoma is the most common neoplasia of female genital tract. The prognosis of early stage disease (FIGO I and FIGO II) is excellent: recurrence after surgery is less than 15%, most of which are reported within 3 years after primary treatment. Herein we report a case of late rectal recurrence from FIGO Ib endometrial adenocarcinoma. Patient had also familiar and personal history of colonic adenocarcinoma and previous findings of microsatellite instability (MSI); molecular analysis evidenced heterozygotic somatic mutation in MLH1 gene. Twenty-eight years after hysterectomy and bilateral salpingoovariectomy, a rectal wall mass was detected during routine colonoscopy. Patients underwent CT scan, pelvic MRI, and rectal EUS with FNA: histopathological and immunohistochemical analysis revealed differentiated carcinoma cells of endometrial origin. No neoadjuvant treatment was planned and low rectal anterior resection with protective colostomy was performed; histology confirmed rectal lesion as metastasis from endometrial carcinoma. Recurrence of early stage endometrial carcinoma after a long period from primary surgery is possible. It is important to keep in mind this possibility in order to set a correct diagnostic and therapeutic algorithm, including preoperative immunohistochemical staining, and to plan a prolonged follow-up program.

A rare case of IgG4-related systemic disease manifesting with pancreatic head mass mimicking borderline resectable cancer.

INTRODUCTION: Autoimmune pancreatitis (AIP) is a rare pancreatic disorder among chronic pancreatitis that can mimick pancreatic cancer (PC). Patients with type 1 AIP usually present obstructive jaundice associated with high level of IgG4 in serum and a pancreatic mass at radiological imaging; these disorders may be associated with other organs lesions presenting the same histopathological features, and in these cases AIP should be considered a pancreatic localization of an IgG4-related systemic disease. PRESENTATION OF CASE: We report the case of a young man with initial suspect of PC to be treated with surgery, and final diagnosis of AIP in the context of an IgG4-related systemic disease. DISCUSSION: Because of its similar features, several algorithms have been proposed for AIP diagnosis, based on combination of clinical/serological and radiological criteria. However, histology represents the only way to obtain definitive diagnosis, even if sometimes it is difficult to obtain biological samples. CONCLUSION: IgG4-related systemic disease must be taken into account among differential diagnosis during the workup for PC, in order to avoid unnecessary surgery.