ABSTRACT
Background: Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by mucinous ascites, typically related to appendiceal or ovarian tumours. Current standard treatment involves cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), but recurrences occur in 20-30 per cent of patients. The aim of this study was to define the timing and patterns of recurrence to provide a basis for modifying follow-up of these patients. Methods: This observational study examined a prospectively developed multicentre national database (RENAPE working group) to identify patients with recurrence after optimal CRS and HIPEC for PMP. Postoperative complications, long-term outcomes and potential prognostic factors were evaluated. Results: Of 1411 patients with proven PMP, 948 were identified who had undergone curative CRS and HIPEC. Among these patients, 229 first recurrences (24·2 per cent) were identified: 196 (20·7 per cent) occurred within the first 5 years (early recurrence) and 30 (3·2 per cent) occurred between 5 and 10 years. Three patients developed a first recurrence more than 10 years after the original treatment. The mean(s.d.) time to first recurrence was 2·36(2·21) years. Preoperative chemotherapy and high-grade pathology were significant factors for early recurrence. Overall survival for the entire group was 77·9 and 63·1 per cent at 5 and 10 years respectively. The principal site of recurrence was the peritoneum. Conclusion: Recurrence of PMP was rare after 5 years and exceptional after 10 years.
Subject(s)
Cytoreduction Surgical Procedures/methods , Hyperthermia, Induced/methods , Neoplasm Recurrence, Local/diagnosis , Peritoneal Neoplasms/therapy , Pseudomyxoma Peritonei/therapy , Adult , Aged , Combined Modality Therapy/methods , Disease-Free Survival , Female , Follow-Up Studies , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Neoplasm Recurrence, Local/epidemiology , Neoplasm Recurrence, Local/prevention & control , Peritoneal Neoplasms/mortality , Peritoneal Neoplasms/pathology , Peritoneum/pathology , Peritoneum/surgery , Prognosis , Prospective Studies , Pseudomyxoma Peritonei/mortality , Pseudomyxoma Peritonei/pathology , Retrospective Studies , Time FactorsSubject(s)
Carcinoma/secondary , Carcinoma/surgery , Colectomy , Colorectal Neoplasms/pathology , Cytoreduction Surgical Procedures/methods , Peritoneal Neoplasms/secondary , Peritoneal Neoplasms/surgery , Antineoplastic Agents/therapeutic use , Carcinoma/drug therapy , Chemotherapy, Adjuvant , Chemotherapy, Cancer, Regional Perfusion/methods , Colorectal Neoplasms/surgery , Humans , Hyperthermia, Induced , Male , Middle Aged , Peritoneal Neoplasms/drug therapyABSTRACT
INTRODUCTION: Diffuse esophageal leiomyomatosis is a rare disease. Misdiagnosis is frequent and previous surgeries can complicate surgical management. The only treatment described for severe symptomatic cases is esophagectomy. PRESENTATION OF CASE: We describe a case of diffuse esophageal leiomyomatosis associated with Alport syndrome in a 21 year-old female where endoscopic ultrasonography (EUS) with concomitant fluoroscopy and 3D-gastric computed tomography (3D-GCT) modified surgical management. DISCUSSION: The diagnosis of diffuse esophageal leiomyomatosis is difficult but can be greatly facilitated by extensive endoscopic and radiologic workup. Esophagectomy should only be entertained after complete anatomic mapping of the lesions, especially after previous surgeries. CONCLUSION: EUS and 3D-GCT should strongly be considered as part of routine preoperative workup in these patients.
