Giant idiopathic benign retroperitoneal cyst: a case report.

BACKGROUND: Primary retroperitoneal cyst formation without connection to adjacent anatomical structures is a rare and benign entity with the majority of these cysts being discovered incidentally. If symptoms develop, they are generally non-specific and related to compression of the adjacent retro-/or intraperitoneal structures. Complete resection of the cyst is curative and therefore the preferred treatment option. We report a case of giant retroperitoneal cyst for which a total surgical removal was performed. CASE REPORT: A 79-year-old female consulted our department with complaints of long lasting abdominal pain and progressive abdominal distension. CT abdomen revealed a giant retroperitoneal cyst (35 cm × 25 cm × 21 cm) without radiological features of malignancy but with severe mass effect on the surrounding intra-abdominal structures. A median laparotomy was performed with a complete excision of cyst. No signs of malignancy or atypia were observed on histological examination. CONCLUSION: Idiopathic retroperitoneal cysts can slowly grow to giant proportions and subsequently lead to chronic vague abdominal symptoms. Complete surgical excision is curative and should be pursued.

A goblet cell carcinoma of the appendix presenting as an acute appendicitis: a case report.

Background: A goblet cell carcinoma of the appendix is a rare neoplasm with histological features of both adenocarcinomas and carcinoid tumours. It has a more aggressive behaviour in comparison to the more common classic neuroendocrine appendiceal tumours. Clinical presentation is variable and a significant proportion of patients present with an acute appendicitis. The aggressive evolution of these tumours makes the management more challenging.Case report: We report the case of a 43-year-old man with acute right iliac fossa pain who was initially diagnosed with an acute appendicitis. Histological analysis of the resected appendix demonstrated the presence of a goblet cell carcinoma with perineural and lymphovascular invasion. No distant metastasis was present. A right hemicolectomy was performed and adjuvant chemotherapy was administered.Conclusion: Goblet cell carcinomas is a distinct entity which should be delineated from the classical appendiceal carcinoids. Its clinical evolution obliges a more aggressive therapeutic strategy.

Deep brain stimulation in Bassen-Kornzweig syndrome: Still effective after 22 years.

Introduction: Bassen-Kornzweig syndrome or abetalipoproteinemia is a rare autosomal recessive disorder characterized by a malabsorption of dietary fat and fat-soluble vitamins. This deficiency can lead to a variety of symptoms, including hematological (acanthocytosis, bleeding tendency), neurological (tremor, spinocerebellar ataxia), neuromuscular (myopathy), ophthalmological symptoms (retinitis pigmentosa). The thalamic ventral intermediate nucleus (VIM) is a well-established target for deep brain stimulation (DBS) in the treatment of refractory tremor. Research question: We evaluated the clinical long-term follow-up (22 years) after VIM-DBS for refractory tremor in abetalipoproteinemia. We also evaluated the adjustments of stimulation settings and medication balance after DBS procedure. Material and methods: We report a 53-year-old male who suffers from abetalipoproteinemia since the age of 17. He underwent bilateral VIM-DBS to treat his disabling refractory intentional tremor at the age of 31. He still has a very good response to his tremor with limited stimulation adaptations over 22 years. For more than two decades follow-up, the treatment significantly improved his ADL functions and therefore also the QoL. Discussion and conclusion: The VIM target for DBS in the treatment of refractory tremor has been extensively reported in the literature. Thalamic VIM-DBS is a safe and effective treatment for a severe, refractory tremor as a neurological symptom caused by abetalipoproteinemia. It also highlights the importance of a multidisciplinary follow-up, to adjust and optimize the stimulation/medication balance after VIM-DBS surgery.