304 First Reported Case of Metachronous Tumours With Delayed Recurrence from The Neuroendocrine Tumour Arising in A Tailgut Cyst

Background Malignant transformation of tailgut cysts (benign retrorectal cysts) into Neurodendocrine tumours (NETS) is extremely rare with less than 30 reported cases in the literature. A tiny proportion of these cases report early recurrence (within 2 years) and to our knowledge none have reported delayed recurrence 6 years after primary resection. Case history We report a 55-year-old male who underwent laparoscopic excision of a 5 cm presacral tumour found incidentally and confirmed histologically to be a G1 NET (WHO Classification) arising from a pre-existing tail gut cyst, with no evidence of metastasis.16 months later the patient presented with bowel obstruction secondary to adenocarcinoma of the colon at the splenic flexure. The patient underwent laparoscopic left hemicolectomy; histology confirming a moderately differentiated adenocarcinoma (TNM stage pT3pN0). The patient underwent adjuvant chemotherapy post-operatively based upon immunohistochemistry. The patient was followed up for both tumours with CEA, serum chromogranin A, colonoscopies, and CT scans. At 5th year post hemicolectomy, CT scan revealed a new asymptomatic 18 mm left internal iliac lymph node which was avid on octreotide scan consistent with metastasis secondary to NET resected previously. He, therefore, underwent 30Gy sabre radiotherapy. Conclusions There is no consensus regarding follow up duration post early NET resection. The recurrent NET was picked up incidentally as part of the 5-year colorectal adenocarcinoma follow up at our unit. The recurrent NET would not have been identified had this not been a metachronous case. Thus, consideration must be given to both radiological and biochemical follow up post NET resections.

The Controversy of Surgical Intervention for Anal Canal Duplication in Children

Objective: Since the first definition of anal canal little has been discovered about the etiology of this rare condition. We present four asymptomatic cases of anal canal duplication with diverse clinical and surgical findings. Methods: A retrospective chart review was performed on four infants presenting with asymptomatic anal canal duplication, born between 2014 and 2016. Clinical characteristics and pathologic findings of patients either by radiological imaging or pathology were evaluated. The primary outcome measure was the complications. Results: All patients were followed-up with physical examination and ultrasound for a mean of 3.5±1.0 years, lastly seen at the beginning of 2018. The female to male ratio was 3:1. Duplicate anal canal length varied between 12-20mm, and two of the four patients had a presacral cystic mass confirmed as a tail gut cyst following surgery. At follow-up, none of the patients had developed symptoms related to anal canal duplication, regardless of whether they had surgical intervention. Conclusion: Though surgical management is the preferred treatment for anal canal duplication, it seems that patients who do not undergo surgery might remain free of symptoms, suggesting that surgical intervention may be unnecessary. doi: https://doi.org/10.12669/pjms.36.6.1832 How to cite this:Akova F, Altinay S, Aydin E. The Controversy of Surgical Intervention for Anal Canal Duplication in Children. Pak J Med Sci. 2020;36(6):---------. doi: https://doi.org/10.12669/pjms.36.6.1832 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Retrorectal Cystic Hamartoma (Tail Gut Cyst): An Institutional Experience

Objectives Tailgut cyst (TGC) is a rare congenital benign cystic lesion arising from a remnant of the embryonic postnatal hindgut. TGCs are typically multinodular, uncapsulated, but usually well circumscribed. TGCs are generally rare in adults and the majority of the lesions are benign. Malignant transformation is rare and malignancies reported include adenocarcinomas and neuroendocrine tumors. Methods A retrospective database search was conducted and coded as “tailgut cyst or retrorectal cystic hamartoma” to identify cases of possible TGC between January 2005 and January 2019. The search utilized the pathology files of Yale-New Haven Hospital using CoPath laboratory information system. Clinical and surgical data were gathered from the surgical pathology report and surgical operative note when available. Results A total of 13 cases (10 women) were identified with a median age of 51 years. All the specimens were obtained by surgical excision. Most of the cases were excised from the presacreal area (11 cases), and two cases were excised from the retrorectal area. The two cases resected from the retrorectal area had a malignant component. The first case was a 33-year-old female who had a 1.5-cm tailgut cyst that showed a multifocal neuroendocrine tumor (carcinoid); the resection margin was also positive. The second case was a 59-year-old female who had a tailgut cyst that showed mucinous adenocarcinoma; the resection margin was also positive. Conclusion Diagnosis of tailgut is sometimes challenging. Possible differential diagnoses in this location include teratoma, dermoid cyst, anterior sacral meningocele, and presacral abscess. Imaging is an important component of the initial workup. Surgical resection is the treatment of choice when the cyst grows in size and if a solid component is present. Exclusion of other benign entities and malignant transformation/component on histological examination is crucial.