scholarly journals Neuroendocrine tumour arising inside a tailgut cyst

2017 ◽  
Vol 99 (2) ◽  
pp. e91-e93 ◽  
Author(s):  
I Mora-Guzmán ◽  
A Alonso-Casado ◽  
A Rodríguez Sánchez ◽  
E Bermejo Marcos
Keyword(s):  
Malignant Transformation ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Surgical Excision ◽  
Tailgut Cyst

Tailgut cysts are rare benign retrorectal cysts arising from persistent remnants of an embryonic hindgut. Malignant transformation inside this lesion is very uncommon and occurrence of neuroendocrine tumours in this context is extremely rare. We report the case of a 56-year-old woman who underwent surgical excision of a presacral tailgut cyst, which was found incidentally to include a neuroendocrine tumour.

scholarly journals Malignancy within a Tail Gut Cyst: A Case of Retrorectal Carcinoid Tumour

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
A. A. Abukar ◽  
B. J. Parcell ◽  
C. B. Lim ◽  
P. V. Patil ◽  
A. Ramsanahie ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Neuroendocrine Tumour ◽  
Carcinoid Tumour ◽  
Surgical Excision ◽  
Presacral Space ◽  
Tailgut Cyst ◽  
Diagnostic Strategy ◽  
Rectal Pain ◽  
Ct Guided ◽  
Complete Surgical Excision

Purpose.Tailgut cysts with malignant transformation are rare entities. We discuss the diagnostic strategy and treatment of a malignancy within a tailgut cyst.Methods.In this study we report on the case of a 61-year-old man with a malignant neuroendocrine tumour arising within a tailgut cyst and an overview of the literature emphasising the histopathological characteristics and differential diagnosis.Results.Our patient presented with lower back pain, rectal pain, and increased urgency of defecation. MRI scan and CT-guided biopsy on histological analysis revealed a diagnosis of carcinoid tumour of the presacral space. The patient subsequently underwent an abdominoperineal excision of the rectum.Conclusions.This case highlights the importance of tailgut cysts as a differential diagnosis of presacral masses. It is a rare congenital lesion developing from remnants of the embryonic postanal gut and is predominantly benign in nature. Approximately half of cases remain asymptomatic; therefore, diagnosis is often delayed. Magnetic resonance imaging is the investigation of choice and an awareness of the possibility of malignant potential is critical to avoiding missed diagnosis and subsequent morbidity. Complete surgical excision allows accurate diagnosis, confirmation of oncological clearance, and prevention of mortality.

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

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S55-S55
Author(s):  
Hatem Kaseb ◽  
Xuchen Zhang
Keyword(s):  
Malignant Transformation ◽  
Resection Margin ◽  
Surgical Excision ◽  
Pathology Report ◽  
Tailgut Cyst ◽  
First Case ◽  
Retrorectal Cystic Hamartoma ◽  
Surgical Data ◽  
Tail Gut Cyst ◽  
Cystic Hamartoma

Abstract 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.

scholarly journals Retrorectal Mucinous Adenocarcinoma Arising from a Tailgut Cyst: A Case Report

2021 ◽  
pp. 147-151
Author(s):  
Manon Baverez ◽  
Emilie Thibaudeau ◽  
Vincent Libois ◽  
Olivier Kerdraon ◽  
Hélène Senellart ◽  
...  
Keyword(s):  
Case Report ◽  
Surgical Treatment ◽  
Anal Canal ◽  
Malignant Transformation ◽  
Mucinous Adenocarcinoma ◽  
Tailgut Cyst ◽  
Local Invasion

We report the case of a 57-year-old woman who presented with local invasion of the anal canal by mucinous adenocarcinoma, the malignant transformation of a long-term preexisting retrorectal tailgut cyst. This progression is infrequent and justifies preemptive surgical treatment of retrorectal cysts.

A Comparison of ree Chromogranin A Assays in Patients with Neuroendocrine Tumours

2014 ◽  
Vol 23 (4) ◽  
pp. 419-424 ◽  
Author(s):  
Lise Brehm Hoej ◽  
Tina Parkner ◽  
Cindy Soendersoe Knudsen ◽  
Henning Grønbaek
Keyword(s):  
Liver Metastases ◽  
Chromogranin A ◽  
Neuroendocrine Tumours ◽  
Single Photon ◽  
Neuroendocrine Tumour ◽  
Photon Emission ◽  
Enzyme Linked Immunosorbent Assay ◽  
Emission Computed Tomography ◽  
Clinical Sensitivity ◽  
Disease Free

Background & Aims: Chromogranin A (CgA) is the most important general tumour marker used in the diagnosis and follow-up of patients with neuroendocrine tumours (NET). Chromogranin A assays may have different sensitivities, which is of importance for the clinical diagnosis and handling of NET patients. The aim of this study was to compare the clinical sensitivities of three different CgA assays in NET patients.Methods: We measured CgA level in 42 NET patients (male/female: 23/19, median age: 63 years, range 29- 85 years). Twenty-five patients had liver metastases, eight had local disease, and nine were disease free after surgery. We studied an in-house RIA: RH RIA assay (Rigshospitalet, Copenhagen, Denmark); NEOLISATM (Euro Diagnostica, Malmö, Sweden) and EURIA CgA RIA (Euro Diagnostica, Malmö, Sweden).Results: The RH RIA assay showed a clinical sensitivity of 97%, while the NEOLISA and EURIA assays both showed similar clinical sensitivities of 79%. Patients with liver metastases had significantly higher CgA levels compared to disease free patients by all three assays (P<0.001), but only the RH RIA assay was able to discriminate between patients with liver metastases and with regional disease (P<0.01).Conclusion: Chromogranin A measurements are significantly assay-dependent and caution should be applied in the interpretation of CgA measurement for assessment of NET status. The in-house RH RIA assay was better at predicting NET status than the NEOLISA and EURIA assays.List of abbreviations: CgA: chromogranin A; EURIA: Eurodiagnostica, radioimmunoassay; GFR: glomerular filtration rate; NEOLISA: Eurodiagnostica, enzyme-linked immunosorbent assay; NET: neuroendocrine tumour; PET-CT: positron emission tomography; PPI: proton pump inhibitor; RH RIA: Rigshospitalet, radioimmunoassay ; SPECT-CT: single photon emission computed tomography.

scholarly journals “The Innate Tail”: An Unusual Cause of Rectal Bleeding in an Adult Male

2020 ◽  
pp. 1-5
Author(s):  
Sujit Padmanabhan Nair ◽  
Sanjay Chandnani ◽  
Prasanta Debnath ◽  
Pravin M. Rathi ◽  
Parmeshwar Junare ◽  
...  
Keyword(s):  
Malignant Transformation ◽  
Adult Male ◽  
Rectal Bleeding ◽  
Treatment Modality ◽  
Surgical Excision ◽  
Women Physicians ◽  
Imaging Modalities ◽  
Rare Tumor ◽  
Congenital Lesions ◽  
High Index Of Suspicion

Retrorectal cystic hamartomas or tailgut cysts are infrequent congenital lesions presenting as presacral masses originating from the embryonic hindgut. They are commonly diagnosed in middle-aged women. Physicians must have a high index of suspicion to diagnose this rare tumor. We report a case of retrorectal hamartoma in a 70-year-old male presenting as rectal bleeding instead of the usual compressive symptoms. The utility of imaging modalities and the paramount importance of histopathology has been described. The treatment modality is surgical excision to prevent the potential malignant transformation.

Positivity for SATB2 distinguishes Islet1 positive rectal neuroendocrine tumours from pancreaticoduodenal neuroendocrine tumours

2020 ◽  
pp. jclinpath-2020-206645
Author(s):  
Sambit Kumar Mohanty ◽  
Ankit Tiwari ◽  
Nitin Bhardwaj ◽  
Fai Chuang ◽  
Evelyn Kim ◽  
...  
Keyword(s):  
Predictive Value ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Tissue Microarrays ◽  
Characteristic Analysis ◽  
Therapeutic Implications ◽  
Weak Staining ◽  
Metastatic Setting ◽  
Strong Staining ◽  
Paraffin Tissue

AimsDetermining the site of origin of a metastatic neuroendocrine tumour (NET) can be challenging and has important prognostic and therapeutic implications. An immunohistochemical (IHC) panel consisting of TTF1, CDX2, PAX8/PAX6 and Islet1 is often employed. However, there can be a significant IHC overlap among different primary sites. Herein, we sought to determine the utility of including Special AT-rich sequence binding protein-2 (SATB2) in the IHC panel that is used for determining the site of origin of a metastatic NET.MethodsParaffin tissue microarrays consisting of 137 primary NETs (26 lung, 22 jejunoileal, 8 appendix, 5 stomach, 4 duodenum, 17 rectum and 55 pancreas) were stained for SATB2, in addition to the well-described lineage-associated markers, such as TTF1, CDX2, PAX6 and Islet1. Additionally, a tissue microarray consisting of 21 metastatic NETs (1 lung, 1 stomach, 8 jejunoileal and 11 pancreas) was stained for TTF1, CDX2, SATB2 and Islet1. The results were recorded as no staining, weak staining and moderate to strong staining.ResultsAll appendiceal NETs and majority (88%) of the rectal NETs were positive for SATB2. All primary foregut NETs (stomach, pancreas, duodenum and lung) were negative for SATB2, except for one pulmonary NET with weak staining. However, among the metastatic tumours, 5 of 11 pancreatic NETs, 1 stomach NET, 1 lung NET and 2 of 8 jejunoileal NETs showed weak staining. Receiver operating characteristic analysis incorporating sensitivity and specificity data of IHC panel, considering moderate to strong staining as truly positive cases, showed that inclusion of SATB2 to the previously described NET IHC panel outperformed the panel without SATB2, raising the specificity for pancreaticoduodenal NETs from 81.2% to 100%, with a positive predictive value (PPV) of 100% and negative predictive value (NPV) of 82.22% (p<0.0001); for appendiceal NETs the specificity changed from 99.1% to 98.5% and sensitivity increased from 11.8% to 80%, with a PPV and NPV of 66.67% and 99.26%, respectively (p<0.0001); and for rectal NETs the specificity increased from 97.6% to 99.3% and sensitivity raised from 7.1% to 66.7%, with a PPV and NPV of 80% and 98.53%, respectively (p<0.0001).ConclusionsSATB2 stain is useful in differentiatingIslet1/PAX6 positive pancreatic and rectal NETs, as rectal NETs are typically moderately to strongly positive for SATB2 and pancreatic NETs are usually negative or weakly positive for SATB2. Moderate to strong staining for SATB2 is suggestive of an appendiceal or a rectal primary. SATB2 may complement the panel of CDX2, TTF1 and Islet1 in determining the site of origin of an NET in a metastatic setting.

Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms

2011 ◽  
Vol 18 (S1) ◽  
pp. S1-S16 ◽  
Author(s):  
Günter Klöppel
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Tnm Stage ◽  
Neuroendocrine Neoplasms ◽  
Gastroenteropancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Neuroendocrine Carcinomas

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are composed of cells with a neuroendocrine phenotype. The old and the new WHO classifications distinguish between well-differentiated and poorly differentiated neoplasms. All well-differentiated neoplasms, regardless of whether they behave benignly or develop metastases, will be called neuroendocrine tumours (NETs), and graded G1 (Ki67 <2%) or G2 (Ki67 2–20%). All poorly differentiated neoplasms will be termed neuroendocrine carcinomas (NECs) and graded G3 (Ki67 >20%). To stratify the GEP-NETs and GEP-NECs regarding their prognosis, they are now further classified according to TNM-stage systems that were recently proposed by the European Neuroendocrine Tumour Society (ENETS) and the AJCC/UICC. In the light of these criteria the pathology and biology of the various NETs and NECs of the gastrointestinal tract (including the oesophagus) and the pancreas are reviewed.

Nurse developments in neuroendocrine tumour management.

2003 ◽  
pp. 487-488 ◽  
Author(s):  
C Bouvier
Keyword(s):  
Multidisciplinary Team ◽  
Significant Contribution ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Nurse Specialist ◽  
Royal Free Hospital ◽  
The Past ◽  
Standards Of Practice ◽  
High Standards ◽  
Set Up

Nurse developments in the management of neuroendocrine tumours have changed significantly over the past three years. At the Royal Free Hospital we set up the nurse specialist role due to the expansion of patients being referred to the specialist unit, and the obvious need for a nursing input into their care. The nurse specialist can make a significant contribution within the context of a multidisciplinary team especially in the production of guidelines and policies to ensure and maintain high standards of practice, education for the patient, and the provision of expertise and security that the patient requires when diagnosed with a rare disease.

scholarly journals Neuroendocrine Tumour of the Appendix in Pregnancy

2013 ◽  
Vol 13 (2) ◽  
pp. 81-83
Author(s):  
Andrejs Vanags ◽  
Ilze Strumfa ◽  
Zane Simtniece ◽  
Jānis Gardovskis
Keyword(s):  
Acute Appendicitis ◽  
Digestive System ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Pathological Examination ◽  
Solitary Lesion ◽  
Documented Case ◽  
Pubmed Database ◽  
Anatomic Localization ◽  
In Pregnancy

Abstract Despite the fact that the appendix is one of the rarest anatomic localization of neuroendocrine tumours (NETs) in the digestive system, the appendicular neuroendocrine tumour (A-NET) remains among the most common tumours of the appendix. The A-NET usually presents as a small solitary lesion revealed only after pathological examination of appendix that has been surgically removed for acute appendicitis. Only 18 papers in the Pubmed database (1965-2012) are dedicated to A-NET in pregnancy. Here we present a well-documented case of A-NET in a pregnant lady.

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