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Guo W, Deng M, Chen Q. A neuroendocrine tumor arising in a tailgut cyst: Case report and literature review. Int J Surg Case Rep 2024; 120:109912. [PMID: 38889516 PMCID: PMC11231592 DOI: 10.1016/j.ijscr.2024.109912] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2024] [Revised: 06/09/2024] [Accepted: 06/13/2024] [Indexed: 06/20/2024] Open
Abstract
INTRODUCTION AND IMPORTANCE Cystic lesions in the retrorectal space include developmental abnormality, inflammatory process, and tumor-relevant cysts. Among them, the tailgut cyst is the most common lesion which is featured by the complex epithelium lining the wall. It is generally accepted that tailgut cysts are embryonic residues and are mostly benign, but there are also reports about malignant transformation and even metastasis. CASE PRESENTATION A 44-year-old female complained a sacrococcygeal discomfort more than one year. The imaging diagnosis was an infectious cyst. After surgery, a solid region was defined in a cyst. Morphologically, the region was composed of bland epithelia forming glandular or ribbon-like structure, with round nuclei and fine chromatin. Immunohistochemically, the cells were positive for CK7, CD56 and synaptophysin. The Ki-67-positive cells were about 1 %. The final diagnosis is a low-grade neuroendocrine tumor arising in a tailgut cyst. The patient was living without recurrence by the follow-up of 20 months after surgery. CLINICAL DISCUSSION By reviewing the previously reported NET arising from tailgut cysts, we summarized 29 cases of neuroendocrine neoplasms that reported detailed information, and the majority are women. We found that the higher-grade tumor presented a higher tendency of distant metastasis or recurrence after surgery. Complete resection and full evaluation by pathologists are necessary to get a correct diagnosis and avoid disease progression. CONCLUSION We reported the rare case of NET G1 arising from a tailgut cyst and reviewed relevant reports, in order to broaden differential diagnoses when an isolated mass is identified in the retrorectal space.
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Affiliation(s)
- Wei Guo
- Department of Pathology, Zhongnan Hospital of Wuhan University, Wuhan, China; Department of Pathology, Wuhan University Taikang Medical School (School of Basic Medical Sciences), Wuhan, China
| | - Ming Deng
- Department of Radiology, Zhongnan Hospital of Wuhan University, Wuhan, China
| | - Qiongrong Chen
- Department of Pathology, Zhongnan Hospital of Wuhan University, Wuhan, China.
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Matrood S, Apostolidis L, Schrader J, Krug S, Lahner H, Ramaswamy A, Librizzi D, Kender Z, Kröcher A, Kreutzfeldt S, Gress TM, Rinke A. Multicenter Analysis of Presacral Neuroendocrine Neoplasms-Clinicopathological Characterization and Treatment Outcomes of a Rare Disease. Front Endocrinol (Lausanne) 2021; 12:709256. [PMID: 34690926 PMCID: PMC8527170 DOI: 10.3389/fendo.2021.709256] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/13/2021] [Accepted: 09/10/2021] [Indexed: 11/17/2022] Open
Abstract
Background and Aims Neuroendocrine neoplasms (NENs) of the presacral space are an extremely rare disease entity with largely unknown outcome and no established standard of care treatment. Therefore, we wanted to analyze clinical presentation, histopathological findings, treatment outcomes, and prognosis in a multicentric patient cohort. Methods We searched local databases of six German NEN centers for patients with presacral NEN. Retrospective descriptive analyses of age, sex, stage at diagnosis, symptoms, grade, immunohistochemical investigations, biomarkers, treatment, and treatment outcome were performed. Kaplan-Meier analysis was used to determine median overall survival. Results We identified 17 patients (11 female, 6 male) with a median age of 50 years (range, 35-66) at diagnosis. Twelve cases presented initially with distant metastases including bone metastases in nine cases. On pathological review the majority of patients had well-differentiated G2 tumors. Immunohistochemical profile resembled rectal NENs. All but one patient had non-functioning tumors. Somatostatin receptor imaging was positive in 14 of 15 investigated cases. Eight patients were treated surgically including palliative resections; 14 patients received somatostatin analogs with limited efficacy. With 14 PRRTs completed, 79% showed clinical benefit, whereas only one patient with neuroendocrine carcinoma (NEC) responded to chemotherapy. Treatment with everolimus in three patients was not successful, whereas cabozantinib resulted in a disease stabilization in a heavily pretreated patient. During a median observation period of 44.5 months, 6 patients died. Median overall survival was not reached. Conclusion Presacral NEN are histopathologically similar to rectal NENs. Presacral NEN should be considered as possible primary in NEN of unknown primary. The majority of tumors is non-functioning and somatostatin receptor positive. PRRT demonstrated promising activity; tyrosine kinase inhibitors warrant further investigations. Further molecular characterization and prospective evaluation of this rare tumor entity are needed.
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Affiliation(s)
- Sami Matrood
- Department of Gastroenterology and Endocrinology, UKGM Marburg and Philipps University, Marburg, Germany
| | - Leonidas Apostolidis
- Department of Medical Oncology, National Center for Tumor Diseases (NCT) Heidelberg, Heidelberg University Hospital, Heidelberg, Germany
| | - Jörg Schrader
- I. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
| | - Sebastian Krug
- Clinic for Internal Medicine I, Martin-Luther University Halle/Wittenberg, Halle, Germany
| | - Harald Lahner
- Department of Endocrinology and Metabolism, University Hospital Essen, Essen, Germany
| | - Annette Ramaswamy
- Institute of Pathology, UKGM Marburg and Philipps University, Marburg, Germany
| | - Damiano Librizzi
- Department of Nuclear Medicine, UKGM Marburg and Philipps University, Marburg, Germany
| | - Zoltan Kender
- Department of Internal Medicine I and Clinical Chemistry, Heidelberg University Hospital, Heidelberg, Germany
| | - Anke Kröcher
- Clinic for Internal Medicine I, University Hospital Carl Gustav Carus Dresden, Dresden, Germany
| | - Simon Kreutzfeldt
- Department of Translational Medical Oncology, National Center for Tumor Diseases (NCT) Heidelberg and German Cancer Research Center (DKFZ), Heidelberg, Germany
| | - Thomas Matthias Gress
- Department of Gastroenterology and Endocrinology, UKGM Marburg and Philipps University, Marburg, Germany
| | - Anja Rinke
- Department of Gastroenterology and Endocrinology, UKGM Marburg and Philipps University, Marburg, Germany
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Liang F, Li J, Yu K, Zhang K, Liu T, Li J. Tailgut Cysts with Malignant Transformation: Features, Diagnosis, and Treatment. Med Sci Monit 2020; 26:e919803. [PMID: 31926113 PMCID: PMC6977638 DOI: 10.12659/msm.919803] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
A tailgut cyst is a type of benign congenital disease that mainly develops in the retro-rectal space. However, malignant transition can occur in some cases of tailgut cysts. Early and precise diagnosis, and proper treatment, are vital for patients with tailgut cysts with malignant transformation. In this review, we aim to summarize the similarities and differences in the diagnosis and treatment methods among the 3 most frequently reported types of tailgut cysts with malignant transformation. In our study, PubMed and Web of Science databases were used to search for the studies and the key words were “tailgut cysts” and “malignancy”. We found 176 articles and selected 75 articles in our survey, with 9 reviews, 35 case reports, and 31 case reports and reviews.
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Affiliation(s)
- Feng Liang
- Department of General Surgery, The Second Hospital of Jilin University, Changchun, Jilin, China (mainland)
| | - Jian Li
- Department of Pathology, The Second Hospital of Jilin University, Changchun, Jilin, China (mainland)
| | - Ke Yu
- Operating Theater and Department of Anesthesiology, The Second Hospital of Jilin University, Changchun, Jilin, China (mainland)
| | - Kai Zhang
- Department of General Surgery, The Second Hospital of Jilin University, Changchun, Jilin, China (mainland)
| | - Tongjun Liu
- Department of General Surgery, The Second Hospital of Jilin University, Changchun, Jilin, China (mainland)
| | - Jiannan Li
- Department of General Surgery, The Second Hospital of Jilin University, Changchun, Jilin, China (mainland)
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Nicoll K, Bartrop C, Walsh S, Foster R, Duncan G, Payne C, Carden C. Malignant transformation of tailgut cysts is significantly higher than previously reported: systematic review of cases in the literature. Colorectal Dis 2019; 21:869-878. [PMID: 30932326 DOI: 10.1111/codi.14628] [Citation(s) in RCA: 24] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/08/2018] [Accepted: 03/16/2019] [Indexed: 02/08/2023]
Abstract
AIM The best treatment for tailgut cysts has not been firmly established. We report a systematic review of the cases in the available literature in order to provide an evidence base for treatment. METHOD A systematic search of articles wholly or partly in English was made of PubMed, Embase and Google Scholar; additional studies were discovered by searching reference lists and contacting authors directly. Search terms 'tailgut cyst', 'tail gut cyst', 'retrorectal hamartoma' and 'retrorectal tumour' were used for case reports or case series; no publication date restrictions were imposed. Only studies with histological confirmation of diagnosis and reporting the age and gender of patients were included. Papers were excluded by consensus between the first two authors. RESULTS A total of 196 individual cases were analysed in detail including 51 cases of neoplasia. The overall rate of neoplastic transformation was 26.6%. Although the male:female cyst incidence ratio was 1:4, men over 18 had a significantly greater relative risk of neoplasm at 1.94 (P = 0.0055). Radiological evidence of nodular thickening of the cyst wall significantly increased the relative risk of the presence of cancer (P = 0.0023). CONCLUSIONS Current orthodoxy that these are not dangerous embryological remnants is unfounded and may be false. The available data suggest the risk of malignant transformation is high and will apply to any residual tissue after excision. The same rationale behind total mesorectal excision in rectal cancer applies to tailgut cysts. Consequently they should be resected with similar oncological margins.
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Neuroendocrine tumour developing within a long-standing tailgut cyst: case report and review of the literature. Clin J Gastroenterol 2019; 12:539-551. [PMID: 31147970 DOI: 10.1007/s12328-019-00998-4] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/22/2019] [Accepted: 05/22/2019] [Indexed: 12/13/2022]
Abstract
A tailgut cyst is a rare congenital lesion that can develop in the presacral space from the remnants of an embryonic hindgut. It is unusual for malignant change to occur in a tailgut cyst. We report a case of a large long-standing tailgut cyst, which was removed during a laparotomy. Histopathology showed a well-differentiated neuroendocrine tumour (primary carcinoid tumour) arising in a tailgut cyst. We reviewed the English literature for all adult cases with this condition. All original articles were reviewed, and data were compiled and tabulated. Including this report, 29 cases of NET developing in a tailgut cyst were found in the English literature. Tailgut cysts have been reported as more common in females, with a mean age of presentation in the fifth decade (Devine, in: Zbar A, Wexner S (eds) Coloproctology. Springer specialist surgery series, Springer, London, 2010; Hjermstad and Helwig in Am J Clin Pathol 89:139-147, 1988). Tailgut cysts may undergo malignant change including adenocarcinoma, sarcoma, and NET (Mathis et al. Br J Surg 97:575-579, 2010; Messick in Dis Colon Rectum 61:151-153, 2018; Patsouras et al. in Colorectal Dis 17:724-729, 2015; Chereau et al in Colorectal Dis 15:e476-e482, 2013). It is difficult to estimate the true incidence of malignant change in a tailgut cyst, with the literature reports only limited to case reports and small-case series. Although rare, our case confirms need to consider the possibility of a malignant component, even in a benign process such as a tailgut cyst. This prompts consideration for upfront definitive management.
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Iwata E, Orosz Z, Teh J, Reynolds J, Whitwell D, Tanaka Y, Athanasou NA. Neuroendocrine Tumor Arising in a Tailgut Cyst: A Rare Presacral Tumor. Int J Surg Pathol 2018; 27:336-342. [PMID: 30176741 DOI: 10.1177/1066896918796291] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/11/2023]
Abstract
A tailgut cyst (retrorectal cystic hamartoma) is an uncommon lesion that develops in the presacral (retrorectal) space. Malignant change in a tailgut cyst is extremely rare and presents as a soft tissue (presacral) or bone (sacral) neoplasm. We report a case of tailgut cyst in which a neuroendocrine tumor developed in a 25-year-old female. Computed tomography and magnetic resonance imaging scans revealed a sacrococcygeal malformation with absent left S4 and S5 and a partly cystic lesion within the right presacral space. Histologically, the lesion contained cystic and solid elements. The cysts were lined by columnar and stratified squamous epithelial cells with underlying patchy smooth muscle. The solid element was a partly necrotic neuroendocrine tumor composed mainly of ribbons of tumor cells, which showed mitotic activity and expressed cytokeratin, chromogranin, and synaptophysin. Histologically, tailgut cysts are lined by epithelium and contain scattered smooth muscle bundles in the cyst wall. Although rare, the possibility of tailgut cyst with neuroendocrine tumor should be included in the differential diagnosis of an enlarging presacral tumor.
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Affiliation(s)
- Eiichiro Iwata
- 1 University of Oxford, Oxford, UK.,2 Nara Medical University, Kashihara-shi, Nara, Japan
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Al Khaldi M, Mesbah A, Dubé P, Isler M, Mitchell A, Doyon J, Sideris L. Neuroendocrine carcinoma arising in a tailgut cyst. Int J Surg Case Rep 2018; 49:91-95. [PMID: 29966957 PMCID: PMC6039892 DOI: 10.1016/j.ijscr.2018.05.032] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/04/2018] [Accepted: 05/21/2018] [Indexed: 02/07/2023] Open
Abstract
INTRODUCTION A tailgut cyst, also called retrorectal cystic hamartoma, is a rare congenital lesion that forms most commonly in the retrorectal space. It is presumed to arise from remnants of early embryogenesis. PRESENTATION OF CASE The following report describes a unique case of a retrorectal cystic hamartoma in a 53 year-old French Canadian man with a history of low back pain. The tumour underwent malignant transformation into a well-differentiated neuroendocrine carcinoma three years after the beginning of symptoms. DISCUSSION This condition can be found at any age, but occurs especially among middle-aged women. Not only is it frequently misdiagnosed, but also several complications associated to the cyst have been reported such as infection and malignant transformation. This is why complete surgical excision of the tailgut cyst is currently recommended.
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Affiliation(s)
- Maher Al Khaldi
- Department of Surgery, Division of General Surgical Oncology, Hôpital Maisonneuve-Rosement, University of Montreal, Canada.
| | - Amanda Mesbah
- Department of Surgery, Division of General Surgical Oncology, Hôpital Maisonneuve-Rosement, University of Montreal, Canada
| | - Pierre Dubé
- Department of Surgery, Division of General Surgical Oncology, Hôpital Maisonneuve-Rosement, University of Montreal, Canada
| | - Marc Isler
- Department of Surgery, Division of Orthopedic Surgery, Hôpital Maisonneuve-Rosement, University of Montreal, Canada
| | - Andrew Mitchell
- Department of Pathology, Hôpital Maisonneuve-Rosement, University of Montreal, Canada
| | - Josée Doyon
- Department of Pathology, Hôpital Maisonneuve-Rosement, University of Montreal, Canada
| | - Lucas Sideris
- Department of Surgery, Division of General Surgical Oncology, Hôpital Maisonneuve-Rosement, University of Montreal, Canada
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Diagnosis of Tailgut Cyst in Gynecologic Patients: Systematic Review of the Literature. JOURNAL OF ENDOMETRIOSIS AND PELVIC PAIN DISORDERS 2017. [DOI: 10.5301/jeppd.5000293] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Tailgut cysts, also called retrorectal cystic hamartomas, are rare findings that usually occur in the retrorectal space. The incidence is estimated to be about 1 in 40,000.The aim of our review is to evaluate the potential pitfalls in the diagnosis of this rare pathologic condition, according to diagnostic procedures for this rare but misleading and subtle entity.We conducted a Medline literature review of the English literature discussing tailgut cysts in female patients. We concentrated on imaging technology used for diagnosis in gynecologic patients. For our search, we used the key words “tailgut cyst”, “retrorectal cystic hamartoma”, “gynecology”, focusing on clinical presentation, imaging technology used for diagnosis, presence of malignancy, and misdiagnosis with more common gynecologic pathology.Our search revealed 143 articles during the years 1975–2016 and, of these, 30 articles describing 91 female patients were considered.We concluded that tailgut cyst should be included in the differential diagnosis of pelvic lesions in gynecologic patients.
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Mitsuyama T, Kubota M, Nakamura Y, Yuzurihara M, Hoshi K, Okada Y. Neuroendocrine tumor arising from tailgut cyst with spinal cord tethering: case report and literature review. Spine J 2015; 15:e1-8. [PMID: 25305642 DOI: 10.1016/j.spinee.2014.09.027] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/20/2013] [Revised: 08/21/2014] [Accepted: 09/30/2014] [Indexed: 02/09/2023]
Abstract
BACKGROUND CONTEXT Neuroendocrine tumors (NETs) from tailgut cysts are rare; only 15 cases have been reported until now. A tailgut cyst with spinal cord tethering has not been previously reported, although both diseases are congenital anomalies in the early stage of gestation. PURPOSE To report a rare case of NET from tailgut cyst associated with spinal cord tethering and review the literature. STUDY DESIGN Case report and literature review. METHODS We describe the clinical course of a 53-year-old man, who presented with gluteal pain and bladder dysfunction. Magnetic resonance images showed that a tumor of the sacral spinal canal extended into the retrorectal space and connected to a thickened fatty filum terminale, which was tethering the spinal cord. RESULTS Because of tumor malignancy on a computed tomography-guided biopsy and the imaging data of involvement of presacral lymph nodes, we performed total removal of the tumor. Pathologic examination revealed NET (Grade 2) arising from a tailgut cyst. The patient received somatostatin analog therapy after surgery, followed by local radiation because of the further enlargement of the lymph nodes. Later, we started everolimus therapy for the metastases to the retroperitoneal lymph nodes. He presented with no local recurrence or further disease progression at 28 months after surgery. The review indicated that tumors in Grade 2 or 3 showed progressive clinical course after surgery and three of seven patients with biopsy were misdiagnosed. CONCLUSIONS The correct preoperative diagnosis of NETs from tailgut cysts is difficult, but extremely important because Grade 2 or 3 tumors show disease progression even after surgery. Presacral congenital tumors, such as tailgut cysts, have the potential of malignant transformation into neuroendcrine tumors or adenocarcinomas. Comorbidity of spinal cord tethering and tailgut cyst suggests some relationship to common developmental errors in embryogenesis.
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Affiliation(s)
- Tetsuryu Mitsuyama
- Department of Neurosurgery, Tokyo Women's Medical University, 8-1 Kawada-chou, Shinjuku, Tokyo, 162-8666, Japan; Department of Spinal Surgery, Kameda Medical Center, 929 Higashi-chou, Kamogawa, Chiba, 296-8602, Japan.
| | - Motoo Kubota
- Department of Spinal Surgery, Kameda Medical Center, 929 Higashi-chou, Kamogawa, Chiba, 296-8602, Japan
| | - Yoshiaki Nakamura
- Department of Oncology, Kameda Medical Center, 929 Higashi-chou, Kamogawa, Chiba, 296-8602, Japan
| | - Masahito Yuzurihara
- Department of Spinal Surgery, Kameda Medical Center, 929 Higashi-chou, Kamogawa, Chiba, 296-8602, Japan
| | - Kazuei Hoshi
- Department of Pathology, Kameda Medical Center, 929 Higashi-chou, Kamogawa, Chiba, 296-8602, Japan
| | - Yoshikazu Okada
- Department of Neurosurgery, Tokyo Women's Medical University, 8-1 Kawada-chou, Shinjuku, Tokyo, 162-8666, Japan
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Malignancy within a Tail Gut Cyst: A Case of Retrorectal Carcinoid Tumour. Case Rep Surg 2014; 2014:454502. [PMID: 25478281 PMCID: PMC4247923 DOI: 10.1155/2014/454502] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2014] [Accepted: 10/28/2014] [Indexed: 02/06/2023] Open
Abstract
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.
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Misawa SI, Horie H, Yamaguchi T, Kobayashi S, Kumano H, Lefor AT, Yasuda Y. A Unique Retrorectal Tumor With Neuroendocrine Differentiation. Int J Surg Pathol 2013; 21:271-7. [DOI: 10.1177/1066896913476738] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
Retrorectal or presacral tumors are very rare. We report a unique case of a retrorectal tumor with neuroendocrine differentiation, consisting of high- and low-grade components. A 53-year-old woman treated for a perianal abscess at another clinic was referred to our hospital for continued anal pain. Digital rectal examination identified a soft tumor with a smooth surface in the lower rectum. Pelvic computed tomography and magnetic resonance imaging detected a large cystic tumor measuring 8 cm in diameter in the retrorectal space of the pelvis. The border between the tumor and rectal wall, levator ani and vaginal wall was obscure. Fine-needle aspiration cytology was highly suggestive of carcinoma. Abdominoperineal resection was performed, and the tumor was histologically diagnosed as a neuroendocrine carcinoma based on immunohistochemical staining. No previous case has been reported with cystic growth of a neuroendocrine carcinoma in the retrorectal space.
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Affiliation(s)
| | | | | | | | | | - Alan T. Lefor
- Jichi Medical University, Shimotsuke, Tochigi, Japan
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A large presacral tailgut cyst with a carcinoid tumor in a male: report of a case. Surg Today 2013; 44:961-6. [DOI: 10.1007/s00595-012-0482-4] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/05/2012] [Accepted: 10/26/2012] [Indexed: 12/31/2022]
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Herráiz Hidalgo L, Cano Alonso R, Carrascoso Arranz J, Álvarez Moreno E, Martínez de Vega Fernández V. [3.0 T MRI with a high resolution protocol for the study of benign disease of the anus and rectum. Part one: High resolution protocol for 3.0 T MRI, anatomic review, benign tumors, and congenital or acquired alterations of the sphincter complex]. RADIOLOGIA 2012; 56:154-66. [PMID: 22998847 DOI: 10.1016/j.rx.2012.05.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2011] [Revised: 04/29/2012] [Accepted: 05/03/2012] [Indexed: 11/18/2022]
Abstract
Benign anorectal disease comprises a broad group of processes with very diverse origins; these processes may be congenital or acquired as well as inflammatory or tumor related. However, benign anorectal disease has received less attention in the scientific literature than malignant disease. We present an image-based review of the most common benign diseases of the anus and rectum. In this first part, we review the anatomy of the region and provide a brief description of the peculiarities of the high resolution protocol that we use with 3.0 T MRI. We go on to describe the most common benign anorectal tumors and developmental cystic lesions, together with their differential diagnoses, as well as congenital and acquired anomalies of the anorectal sphincter complex.
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Affiliation(s)
- L Herráiz Hidalgo
- Departamento de Diagnóstico por la Imagen, Hospital Universitario Quirón Madrid, Pozuelo de Alarcón, Madrid, España.
| | - R Cano Alonso
- Departamento de Diagnóstico por la Imagen, Hospital Universitario Quirón Madrid, Pozuelo de Alarcón, Madrid, España
| | - J Carrascoso Arranz
- Departamento de Diagnóstico por la Imagen, Hospital Universitario Quirón Madrid, Pozuelo de Alarcón, Madrid, España
| | - E Álvarez Moreno
- Departamento de Diagnóstico por la Imagen, Hospital Universitario Quirón Madrid, Pozuelo de Alarcón, Madrid, España
| | - V Martínez de Vega Fernández
- Departamento de Diagnóstico por la Imagen, Hospital Universitario Quirón Madrid, Pozuelo de Alarcón, Madrid, España
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Delemer M, Detchev R, Dugue T, Gosset P, Houzé de l’Aulnoit D. L’hamartome kystique rétrorectal (HKRR) : à propos d’un cas réséqué par abord vaginal et revue de la littérature. ACTA ACUST UNITED AC 2011; 40:569-71. [DOI: 10.1016/j.jgyn.2010.12.009] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2010] [Revised: 12/09/2010] [Accepted: 12/23/2010] [Indexed: 12/28/2022]
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Abstract
Tailgut cysts, or retrorectal cystic hamartomas, are rare congenital developmental lesions, most commonly located in the retrorectal space, and are more common in women. We present a case of retrorectal tailgut cyst managed using a laparoscopic approach. A 36-year-old woman presented with incidentally detected retrorectal tumors during evaluation for a gallbladder polyp. Her past medical history revealed that she had undergone cesarean section twice. The tumor marker CA 19-9 level was 42.52 U/ml. CT of the pelvis with contrast and pelvic MRI revealed a 3.9 × 3.3 cm well-defined, homogeneous cystic mass in the right presacral area, and a 2.5 × 1.5 cm cystic mass in the precoccygeal space. The patient underwent laparoscopic exploration with a preoperative diagnosis of tailgut cysts based on radiological findings. The operative time was 90 min including 30 min of subsequent laparoscopic cholecystectomy without placement of additional trocars. The surgical specimens consisted of two fragments of fibrofatty tissues, unilocular cystic masses. The final pathologic diagnosis was tailgut cysts with no evidence of malignancy. Postoperative recovery was uneventful, and the patient was discharged after 3 days. In conclusion, surgical resection is recommended in the management of retrorectal tailgut cyst to establish a definite diagnosis and to rule out malignancy. The laparoscopic approach is a feasible and safe option.
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Affiliation(s)
- S W Lim
- Department of Surgery, Chonnam National University Hwasun Hospital, Gwangju, South Korea
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La Rosa S, Boni L, Finzi G, Vigetti D, Papanikolaou N, Tenconi SM, Dionigi G, Clerici M, Garancini S, Capella C. Ghrelin-producing well-differentiated neuroendocrine tumor (carcinoid) of tailgut cyst. Morphological, immunohistochemical, ultrastructural, and RT-PCR study of a case and review of the literature. Endocr Pathol 2010; 21:190-8. [PMID: 20532674 DOI: 10.1007/s12022-010-9127-6] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
Well-differentiated neuroendocrine tumors (carcinoids) arising in the presacral space are rare neoplasms that can arise in association with either sacrococcygeal teratomas or tailgut cysts. Although tumors arising in tailgut cysts are more frequent than those associated with teratomas, they are still very rare, and only 13 cases have been reported in the literature. We describe the first case of a carcinoid composed of ghrelin-producing cells arising in a tailgut cyst. Ghrelin production was demonstrated using immunohistochemistry, electron microscopy, and reverse transcription-polymerase chain reaction methods. A 73-year-old woman with back and pelvic pain was found to have a presacral mass histologically diagnosed, on needle biopsy, as a well-differentiated neuroendocrine tumor. Workup did not show another primary tumor or metastatic disease. The patient underwent laparoscopic resection of the mass, and the pathological diagnosis of the surgical specimen was of a tailgut cyst-associated carcinoid composed of ghrelin-producing cells. In addition, we have accurately reviewed the literature on presacral carcinoids, associated or unassociated with tailgut cysts, to give the reader a comprehensive overview of these very rare tumor types.
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Affiliation(s)
- Stefano La Rosa
- Department of Pathology, Ospedale di Circolo, viale Borri 57, 21100 Varese, Italy.
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Chong Y, Cho MY. First Report of a Gangliocytic Paraganglioma Arising in a Tailgut Cyst. KOREAN JOURNAL OF PATHOLOGY 2010. [DOI: 10.4132/koreanjpathol.2010.44.4.435] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Affiliation(s)
- Yosep Chong
- Department of Pathology, Yonsei University Wonju College of Medicine, Wonju, Korea
| | - Mee-Yon Cho
- Department of Pathology, Yonsei University Wonju College of Medicine, Wonju, Korea
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19
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Dujardin F, Beaussart P, de Muret A, Rosset P, Waynberger E, Mulleman D, de Pinieux G. Primary neuroendocrine tumor of the sacrum: case report and review of the literature. Skeletal Radiol 2009; 38:819-23. [PMID: 19360403 DOI: 10.1007/s00256-009-0693-y] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/02/2009] [Revised: 03/20/2009] [Accepted: 03/23/2009] [Indexed: 02/02/2023]
Abstract
Primary carcinoid tumor (well-differentiated neuroendocrine tumor) of the bone involving the sacrum is extremely rare. We report the case of a 72-year-old man who presented with a 20-year history of intermittent low back pain and was found to have an intraosseous sacral mass on imaging. A needle biopsy revealed that this lesion was a well-differentiated neuroendocrine tumor. Workup did not show any primary tumor or other metastatic disease. There was no associated tailgut cyst or sacrococcygeal teratoma. The lesion was treated with radiation therapy because a surgical approach was rejected. The patient is free of metastatic disease after 28 years evolution of the lesion, retrospectively seen to be present on a conventional radiography performed in 1980. A review of the literature revealed 20 case reports of neuroendocrine tumors arising from the presacral region (with or without associated tailgut cyst or sacrococcygeal teratoma) and sometimes extending to the sacrum. One additional case was located within the neural canal and involved the sacrum, the presacral region, and the rectal wall. Our case is the only tumor arising primarily from the sacrum. The long evolution of this lesion without any other location makes metastatic disease very improbable and this case appears to be a unique example of primary intraosseous sacral carcinoid tumor.
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Affiliation(s)
- Fanny Dujardin
- Department of Pathology, Hôpital Trousseau, CHRU de Tours, 37042 Tours Cedex 09, France
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20
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Gunkova P, Martinek L, Dostalik J, Gunka I, Vavra P, Mazur M. Laparoscopic approach to retrorectal cyst. World J Gastroenterol 2008; 14:6581-3. [PMID: 19030218 PMCID: PMC2773352 DOI: 10.3748/wjg.14.6581] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Retrorectal cysts are rare benign lesions in the presacral space which are frequently diagnosed in middle-aged females. We report here our experience with two symptomatic female patients who were diagnosed as having a retrorectal cyst and managed using a laparoscopic approach. The two patients were misdiagnosed as having an ovarian cystic lesion after abdominal ultrasonography. Computer tomography (CT) scan was mandatory to establish the diagnosis. The trocar port site was the same in both patients. An additional left oophorectomy was done for a coexisting ovarian cystic lesion in one patient in the same setting. There was no postoperative morbidity or mortality and the two patients were discharged on the 5th and 6th post operative days, respectively. Our cases show that laparoscopic management of retrorectal cysts is a safe approach. It reduces surgical trauma and offers an excellent tool for perfect visualization of the deep structures in the presacral space.
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21
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Vestigial retrorectal cystic tumors in adults: A review of 30 cases. ACTA ACUST UNITED AC 2008; 32:769-78. [DOI: 10.1016/j.gcb.2008.03.011] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2007] [Revised: 02/28/2008] [Accepted: 03/01/2008] [Indexed: 12/24/2022]
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22
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Tailgut cysts: MRI findings. Eur Radiol 2008; 18:2586-93. [PMID: 18566821 DOI: 10.1007/s00330-008-1028-4] [Citation(s) in RCA: 36] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2007] [Revised: 03/18/2008] [Accepted: 04/09/2008] [Indexed: 01/16/2023]
Abstract
Magnetic resonance imaging (MRI) features of 11 surgically resected pelvic tailgut cysts were analyzed with reference to histopathologic and clinical data. Homogeneity, size, location, signal intensity, appearance and presence of septa and/or nodules and/or peripheral rim and involvement of surrounding structures were studied. Histological examination demonstrated 11 tailgut cysts (TGC), including one infected TGC and one TGC with a component of adenocarcinoma. Lesions (3-8 cm in diameter) were exclusively or partly retrorectal in all cases but one, with an extension down the anal canal in five cases. Lesions were multicystic in all patients but one. On T1-weighted MR images, all cystic lesions contained at least one hyperintense cyst. The peripheral rim of the cystic lesion was regular and non or moderately enhancing in all cases but the two complicated TGC. Nodular peripheral rim and irregular septa were seen in the degenerated TGC. Marked enhancement of the peripheral structures was noted in the two complicated TGC. Pelvic MRI is a valuable tool in the preoperative evaluation of TGC.
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23
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Vega Menéndez D, Quintáns Rodríguez A, Hernández Granados P, Nevado Santos M, García Sabrido JL, Rueda Orgaz JA, Ochando F, Ojeda Pérez F, Loinaz C. Hamartomas quísticos retrorrectales. Cir Esp 2008; 83:53-60. [DOI: 10.1016/s0009-739x(08)70506-2] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
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Kim T, Grobmyer SR, Liu C, Hochwald SN. Primary presacral neuroendocrine tumor associated with imperforate anus. World J Surg Oncol 2007; 5:115. [PMID: 17931412 PMCID: PMC2092435 DOI: 10.1186/1477-7819-5-115] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2007] [Accepted: 10/11/2007] [Indexed: 01/07/2023] Open
Abstract
Background Presacral masses are unusual growths that have a limited differential diagnosis, typically not including neuroendocrine tumors (NETs). Classically, NETs are well-differentiated gastroenteropancreatic tumors of probable benign behavior. These tumors are associated with a typical morphologic pattern and involve the distal colon, rectum, and genitourinary tract; they are considered less aggressive, frequently asymptomatic, and rarely cause carcinoid syndrome, even when metastatic. Neuroendocrine tumors of the presacral region are extremely rare and few have been described in the literature. They have not been previously reported as being associated with imperforate anus. Case presentation We present an interesting case of a woman with a history of imperforate anus that was found to have a primary neuroendocrine tumor of the presacral region with no rectal wall involvement. Conclusion We argue that this is a primary gastroenteropancreatic neuroendocrine tumor which likely originated from cells of hindgut origin that underwent an abnormal migration during embryonic development.
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Affiliation(s)
- Tad Kim
- Division of Surgical Oncology, University of Florida, College of Medicine, Gainesville, FL 32610, USA.
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25
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Abstract
Presacral tumours represent a heterogeneous group of predominantly benign and occasionally malignant neoplasms. Due to the rarity of these tumours, their management is often performed in an ad hoc fashion and an algorithm for optimal treatment remains undefined. This review aims to present an overview of presacral tumours, focusing on their presentation, pathology, investigation and management.
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Affiliation(s)
- J Ghosh
- Department of General Surgery, Manchester Royal Infirmary, Oxford Road, Manchester, M13 9WL, England, UK
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26
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Lee CM, Lee SH, Jeon CW, Ahn BK, Baek SU. Carcinoid Tumor Arising within a Tailgut Cyst -A Case Report-. JOURNAL OF THE KOREAN SOCIETY OF COLOPROCTOLOGY 2007. [DOI: 10.3393/jksc.2007.23.1.65] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Affiliation(s)
- Chul Min Lee
- Department of Surgery, Kosin University College of Medicine, Busan, Korea
| | - Seung Hyun Lee
- Department of Surgery, Kosin University College of Medicine, Busan, Korea
| | - Chang Wan Jeon
- Department of Surgery, Kosin University College of Medicine, Busan, Korea
| | - Byung Kwon Ahn
- Department of Surgery, Kosin University College of Medicine, Busan, Korea
| | - Sung Uhn Baek
- Department of Surgery, Kosin University College of Medicine, Busan, Korea
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Abstract
Primary neoplasms of the retrorectal (presacral) space are very rare. These lesions may be congenital or acquired, benign or malignant. They often arise with subtle clinical symptoms, or they may be found incidentally during evaluation for other conditions. Preoperative imaging can provide useful information for operative planning; however, it does not eliminate the need for surgery. Approaches for resection include posterior only and combined abdominoperineal, depending on the characteristics of the lesion. Successful treatment of these enigmatic lesions requires extensive knowledge of pelvic anatomy and expertise in pelvic surgery.
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Affiliation(s)
- Sean C. Glasgow
- Section of Colon and Rectal Surgery, Department of Surgery, Washington University School of Medicine and Barnes-Jewish Hospital, St. Louis, Missouri
| | - David W. Dietz
- Section of Colon and Rectal Surgery, Department of Surgery, Washington University School of Medicine and Barnes-Jewish Hospital, St. Louis, Missouri
- Siteman Cancer Center, St. Louis, Missouri
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28
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Krivokapic Z, Dimitrijevic I, Barisic G, Markovic V, Krstic M. Adenosquamous carcinoma arising within a retrorectal tailgut cyst: Report of a case. World J Gastroenterol 2005; 11:6225-7. [PMID: 16273657 PMCID: PMC4436647 DOI: 10.3748/wjg.v11.i39.6225] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Retrorectal, developmental tail gut cysts, include dermoid cysts, rectal duplication cysts and retrorectal cyst-hama-rtomas. Retrorectal cyst-hamartomas (RCH) are derived from remnants of the tail gut, the most caudal part of the embryonic hind gut, which normally involutes by the 8th wk of embryonic development (3-8 mm stage). They have specific radiological and histopathological features that distinguish them from other similar formations (dermoid cysts, enteric duplication cysts and teratomas). We report a patient with adenosquamous carcinoma arising within RCH, who underwent complete resection of the cyst through anterior laparotomy, and reached complete (recurrence-free for 14 mo, so far) functional recovery. The cyst was incidentally discovered during hysterectomy 12 years ago. Diagnostic, therapeutic and histopathological aspects of this rare case are discussed. The mentioned period between diagnosis and surgical treatment suggests that RCH, given enough time, can develop malignant degeneration, and should be resected at the time of diagnosis.
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Affiliation(s)
- Zoran Krivokapic
- FRCS, First Surgical Clinic, Koste Todorovica 6, Belgrade 11000, Serbia and Montenegro.
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29
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Cho BC, Kim NK, Lim BJ, Kang SO, Sohn JH, Roh JK, Choi ST, Kim SA, Park SE. A carcinoembryonic antigen-secreting adenocarcinoma arising in tailgut cyst: clinical implications of carcinoembryonic antigen. Yonsei Med J 2005; 46:555-61. [PMID: 16127782 PMCID: PMC2815842 DOI: 10.3349/ymj.2005.46.4.555] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/26/2023] Open
Abstract
Tailgut cysts (TGCs) are rare congenital cysts that occur in the retrorectal or presacral spaces. Although most tailgut cysts have been reported as benign, there have been at least 9 cases associated with malignant change. We report herein on an unusual case of a 40-year-old woman with a carcinoembryonic antigen (CEA)-producing adenocarcinoma arising within a TGC who underwent surgical resection and local radiation therapy. Despite the complete resection, metastatic adenocarcinoma developed five months after surgery. CEA-producing adenocarcinoma from a TGC is extremely rare and only two cases, including this case, have been reported in the English medical literature. Besides CEA, the serum levels of CA 19-9 became markedly elevated in this patient. Given that the serum CEA level decreased to the normal range after complete resection of tumor and that the tumor recurrence was associated with a rebound of the CEA serum level, our case shows that serial measurements of serum CEA can be used for treatment planning and for assessing the patient's treatment response for this rare disease.
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Affiliation(s)
- Byoung Chul Cho
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Nam Kyu Kim
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
| | - Beom Jin Lim
- Department of Pathology, Yonsei University College of Medicine, Seoul, Korea
| | - Sang Ook Kang
- Department of Surgery, Yonsei University College of Medicine, Seoul, Korea
| | - Ju Hyuk Sohn
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Jae Kyung Roh
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Sang Tae Choi
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Sung Ai Kim
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
| | - Se Eun Park
- Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
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30
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Killingsworth C, Gadacz TR. Tailgut Cyst (Retrorectal Cystic Hamartoma): Report of a Case and Review of the Literature. Am Surg 2005. [DOI: 10.1177/000313480507100810] [Citation(s) in RCA: 44] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/16/2023]
Abstract
Tailgut cysts are rare congenital lesions arising from remnants of normally regressing postanal primitive gut. They often present in middle-aged women with perirectal symptoms and a retrorectal multicystic mass. These cysts have occasionally shown malignant transformation. We report a case of a tailgut cyst occurring in a 25-year-old African-American female. The differential diagnosis of a retrorectal mass is briefly explored, and the etiology, diagnostic strategy, and surgical approach for tailgut cysts is examined. We also report an extensive literature review to examine clinical characteristics and surgical data for 43 cases of tailgut cysts spanning 16 years.
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Affiliation(s)
| | - Thomas R. Gadacz
- Department of Surgery, Medical College of Georgia, Augusta, Georgia
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31
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Glasgow SC, Birnbaum EH, Lowney JK, Fleshman JW, Kodner IJ, Mutch DG, Lewin S, Mutch MG, Dietz DW. Retrorectal tumors: a diagnostic and therapeutic challenge. Dis Colon Rectum 2005; 48:1581-7. [PMID: 15937630 DOI: 10.1007/s10350-005-0048-2] [Citation(s) in RCA: 126] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
PURPOSE Tumors occurring in the retrorectal space are heterogeneous and uncommon. The utility of newer imaging techniques has not been extensively described, and operative approach is variable. This study examined the diagnosis, treatment, and outcome of retrorectal tumors at a tertiary referral center. METHODS Patients with primary, extramucosal neoplasms occurring in the retrorectal space were identified using a prospectively maintained, procedural database of all adult colorectal surgical patients (1981-2003). Patients also were incorporated from the gynecologic oncology service. Exclusion criteria included inflammatory processes, locally advanced colorectal cancer, and metastatic malignancy. Medical records, radiology, and pathology reports were reviewed retrospectively. RESULTS Thirty-four patients with retrorectal tumors were treated. Malignant tumors comprised 21 percent. Older age, male gender, and pain were predictive of malignancy (P < 0.05). Sensitivity of proctoscopy was 53 percent; this increased to 100 percent with the use of transrectal ultrasound. Accuracy of magnetic resonance vs. computed tomographic imaging for specific histologic tumor type was 28 vs. 18 percent, respectively. Surgical approach was anterior (n = 14), posterior (n = 11), and combined abdominoperineal (n = 9). Eleven patients required en bloc proctectomy. Patients undergoing posterior resection had lower blood loss and required fewer transfusions (P < 0.05). All benign tumors were resected with normal histologic margins and none recurred (median follow-up, 22 months). All patients with malignancy had recurrence/recrudescence of their disease. For these patients, median disease-free and overall survivals were 38 and 61 months, respectively. CONCLUSIONS Retrorectal tumors remain a diagnostic and therapeutic challenge. Pain, male gender, and advanced age increase the likelihood of malignancy. Various imaging modalities are useful for planning resection but cannot establish a definitive diagnosis. Whereas benign retrorectal tumors can be completely resected, curative resection of malignant retrorectal tumors remains difficult.
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Affiliation(s)
- Sean C Glasgow
- Department of Surgery, Section of Colon and Rectal Surgery, Siteman Cancer Center, Washington University, St. Louis, Missouri 63110, USA
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32
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Andea AA, Klimstra DS. Adenocarcinoma arising in a tailgut cyst with prominent meningothelial proliferation and thyroid tissue: case report and review of the literature. Virchows Arch 2005; 446:316-21. [PMID: 15731926 DOI: 10.1007/s00428-004-1178-y] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2004] [Accepted: 11/10/2004] [Indexed: 01/26/2023]
Abstract
Tailgut cysts (retrorectal cystic hamartomas) are rare lesions occurring in the retrorectal/presacral space of young women, considered to be of developmental origin. In this study, we report the clinicopathological characteristics of an adenocarcinoma developing in an unusual tailgut cyst. A 47-year-old female presented with a 3-month history of an enlarging presacral mass, extending into the gluteal area. The patient underwent surgical resection. Microscopic examination revealed a mucinous adenocarcinoma with intestinal features arising in a tailgut cyst. In addition to the usual components previously described in this lesion (cuboidal, columnar, ciliated or squamous epithelial lining with scattered smooth muscle fibers in the wall), our case was unique in that it was associated with a meningothelial proliferation and benign thyroid tissue with oncocytic changes. These additional features bring into question the proposed hindgut origin of tailgut cysts.
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Affiliation(s)
- Aleodor A Andea
- Memorial Sloan-Kettering Cancer Center, Department of Pathology, 1275 York Avenue, New York, NY 10021, USA
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33
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Song DE, Park JK, Hur B, Ro JY. Carcinoid tumor arising in a tailgut cyst of the anorectal junction with distant metastasis: a case report and review of the literature. Arch Pathol Lab Med 2004; 128:578-80. [PMID: 15086297 DOI: 10.5858/2004-128-578-ctaiat] [Citation(s) in RCA: 44] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Tailgut cyst is a rare congenital presacral lesion and is believed to arise from the persistent remnants of the postanal gut. Malignancy occurring in a tailgut cyst is extremely rare, and to our knowledge only 5 cases of carcinoid tumor arising in a tailgut cyst have been reported in the literature to date. We report a sixth case of carcinoid tumor arising in a tailgut cyst. The patient was a 41-year-old woman who presented with perianal pain. Sigmoidoscopy showed a 2-cm submucosal mass located 4 cm above the anal verge. The mass was a multilocular cyst with gray-tan solid portions. The cyst was lined by ciliated columnar, squamous, and transitional epithelia with small foci of carcinoid tumor in the cystic wall. The carcinoid tumor showed a trabecular growth pattern with uniform oval or round cells containing fine chromatin and positive immunoreactivity for chromogranin, synaptophysin, and cytokeratin. This case was unique because the tumor occurred at the anorectal junction, not in the retrorectal space, and unlike previously reported cases showed aggressive behavior and distant metastases.
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Affiliation(s)
- Dong Eun Song
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Manuel Francos J, Parés D, Biondo S, Antonio Palacín J, de Oca J, Osorio A, del Río C, Martí-Ragué J. Abordaje perineal en el tratamiento quirúrgico del hamartoma quístico retrorrectal. Cir Esp 2004. [DOI: 10.1016/s0009-739x(04)72371-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
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