The recent advent of laparoscopic splenectomy for the treatment of refractory idiopathic thrombocytopenic purpura (ITP) has been embraced by surgeons and hematologists in many institutions. However, the occurrence of accessory splenic tissue in a proportion of such splenectomies, either concurrently or later, raises the question of how to deal with this problem when it arises. We report that the laparoscopic approach, facilitated by lateral positioning, can be successfully used for the treatment of an accessory spleen causing recurrent ITP. The use of intraoperative nuclear imaging can greatly aid the localization and provide confirmation of complete excision of the nuclear focus, especially for a very small accessory spleen.
The recommended Phase II dose of this combination is level III: A 60 mg/m(2), I 6.0 g/m(2), T 60 mg/m(2), with mesna and granulocyte-colony stimulating factor. The RR is similar to that of AI in other trials, but the survival is better than anticipated.
BackgroundGastric cancer is the fifth most prevalent and the third most lethal cancer worldwide, causing approximately 720,000 deaths annually. Although most cases of gastric cancers are sporadic, one of its inherited forms, hereditary diffuse gastric cancer (HDGC), constitutes about 1–3% of cases. Interestingly, females in families with HDGC are also predisposed to developing lobular breast cancer (LBC). Recent analyses have identified loss-of-function germline mutations in cadherein-1 (CDH1) as a culprit in HDGC and LBC. This discovery fueled several sequencing analyses and case series reports analyzing the pattern of inheritance of CDH1 and its propensity to induce HDGC. In 2015, a multinational and multidisciplinary task force updated the guidelines and criteria for screening, diagnosing, and managing HDGC.Case presentationHere, we present a case series of three siblings with family history of HDGC who tested positive for the CDH1 mutation and describe their surgical treatment course, post-operative management, and follow-up as they pertain to the updated guidelines.ConclusionsDespite recent updates in guidelines in the diagnosis and management of HDGC, the disease remains challenging to address with patients given the high level of uncertainty and the comorbidities associated with prophylactic intervention. We strongly recommend that an interdisciplinary team inclusive of clinical and surgical oncologists, along with geneticists, social work, and psychological support, should follow the patients in a longitudinal and comprehensive manner in order to achieve full recovery and return to normalcy, as with our patients.
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