{"paper_id":"9aff3615-b5c8-4466-89c6-a7c08a2c38ec","body_text":"Submit Manuscript | http://medcraveonline.com\nIntroduction\nEndometriosis is defined as the presence of endometrial glands and \nstroma outside the uterine cavity1,2 and is thought to affect up to 10% \nof women.3 Endometriotic lesions can be found anywhere in the body, \ncommon sites are the ovaries, pelvic peritoneum and fallopian tubes. \nIt is less commonly found at the cervix, bladder, lungs and bowel.4\nAn association between endometriosis and malignancy has been \nwell documented in literature despite reported controversy of the \nrelationship.5,6 Brinton et al. 7 evaluated a large cohort of Swedish \nwomen (20,868) with a diagnosis of endometriosis, were an \nincreased risk of cancer was found; in particular ovarian, breast and \nhaematopoietic malignancies. Reports of endometriosis associated \nwith cancers of the bowel are rare.\nAppendicular tumours are rare and account for only 0.4% of all \ngastrointestinal tumours. 8 They are usually found incidentally or \nduring investigations for other disease processes (as in our case), \naccounting for 1% of appendectomies.9\nReports of endometriosis associated with bowel cancer are rare. \nOne similar case has been reported by Azordgean et al., 10 which \ndocumented the coexistence of a carcinoid tumour of the appendix \nand ileal endometriosis, each found at separate locations. 10 This \ncase presented with right lower abdominal pain mimicking acute \nappendicitis and was treated surgically with excision of the ileum and \nappendix. We describe the unique, not yet reported case of a 36 year \nold woman presenting with primary infertility, who was found to have \na mucinous cyst-adenoma and carcinoid tumour of the appendix with \nco-existent endometriosis. \nCase presentation\nA 36 year old asymptomatic woman was referred with a 3 year \nhistory of primary infertility. Her periods occurred every 35-38 days \nwith an average bleed and she engaged in regular unprotected coitus. \nNo other personal or family medical history was noted and she led a \nhealthy lifestyle as a non-smoker with minimal alcohol intake.\nRoutine infertility tests for herself and her partner were mostly \nnormal. Except her ultrasound scan revealed 3-4 endometriotic \ncysts in the right ovary, the largest being 3.5cm in diameter with a \nsmall cyst also found in the left ovary. Endocervical swabs revealed \nChlamydia which was promptly treated. It was decided to proceed \nwith a diagnostic laparoscopy with tubal patency dye test and excision \nof endometriosis if appropriate.\nObstet Gynecol Int J. 2014;1(1):5‒7. 5\n©2014 Dunkerton. This is an open access article distributed under the terms of the Creative Commons Attribution License, which \npermits unrestricted use, distribution, and build upon your work non-commercially.\nCombined mucinous cystadenoma and carcinoid \ntumour of the appendix with coexistent features of \nan endometrioma: a case report\nVolume 1 Issue 1 - 2014\nSuzanna Dunkerton,1 Neena K Pankhania,1 \nChris Johnson,2 T erek Gelbaya1\n1Gynaecology, Leicester Royal Infirmary, UK \n2Pathology, Leicester Royal Infirmary, UK\nCorrespondence: Suzanna Dunkerton, Leicester General \nHospital, Leicester, Leics, UK, T el 07979591333, \nEmail \nReceived: August 15, 2014 | Published: August 20, 2014\nAbstract\nIntroduction:  Endometriosis is defined as ‘the presence of endometrial glands and \nstroma outside the uterine cavity’. Appendiceal cancers are rare tumours of the \ngastrointestinal tract. There are some reported cases of these two disease processes \noccurring simultaneously within separate lesions. However, there are no reported \ncases of the two diseases occurring within the same entity. We report the unique case \nof an appendiceal cancer (combined mucinous cyst adenoma and carcinoid tumour) \nwith coexistant histological features of an endometrioma. \nCase report:  A 36 year old nulliparous woman was referred to clinic with primary \ninfertility, with an unremarkable past medical history. After routine infertility \ninvestigations, diagnostic laparoscopy was carried out and endometriosis was \ndiagnosed. A suspicious lesion was also seen on the appendix. A right hemicolectomy \nand appendectomy was performed. Histological results showed mucinous cystadenoma \nwith well differentiated carcinoid tumour and coexistent features of an endometrioma. \nDiscussion:  An association between endometriosis and cancer has been well \ndocumented in literature. Existence with appendiceal cancers is rare. Carcinoids and \ncyst adenomas are both common types of appendiceal cancers, found incidentally or \nmimicking acute appendicitis. Occurrence in the same lesion is rare and unique to be \nfound with features of an endometrioma.\nConclusion:  This case illustrates the broad spectrum of appendiceal and endometrial \ndisease. We hope to highlight the interesting asymptomatic presentation of this patient \nand therefore the importance of requesting routine histopathological analysis after \nappendicectomy. \nKeywords: endometrioma, appendix, mucinouscystadenoma\nObstetrics & Gynecology International Journal\nCase Report\n Open Access\n\n\nCombined mucinous cystadenoma and carcinoid tumour of the appendix with coexistent features of an \nendometrioma: a case report\n6\nCopyright:\n©2014 Dunkerton et al.\nCitation: Dunkerton S, Pankhania NK, Johnson C, et al. Combined mucinous cystadenoma and carcinoid tumour of the appendix with coexistent features of \nan endometrioma: a case report. Obstet Gynecol Int J . 2014;1(1):5‒7. DOI: 10.15406/ogij.2014.01.00001\nDiagnostic laparoscopy found extensive uterovesical, peritoneal \nand ovarian endometriosis. \nFigure 1: Right lateral bowel adhesions were dissected and \nendometriomas were excised from both ovaries and were mobilised as \nper RCOG guidelines.2 The dye test revealed the left tube was patent \nand the right blocked. Overall, grade 4 endometriosis was noted. On \nfurther visualisation of the abdomen, a suspicious looking mucoid \nlesion on the tip of the appendix was seen and biopsied.\nFigure 1 Endometriosis seen at initial laparoscopy.\nFigure 2: Histology of the appendix tip showed mucinous \nmaterial within the bowel wall and stroma suggesting a mucocele or \nmucinous tumour of the appendix. The patient was referred to lower \ngastrointestinal surgeons. A CT scan found it difficult to visualise the \nappendix but adjacent to the caecum a 10mm low attenuation lesion \nwith mural calcification was noted suggestive of an appendicular \nmucocele. No pelvic or retroperitoneal lymphadenopathy was noted \nand no lesions were noted within the chest.\nFigure 2 Histology of the appendix tip showed mucinous material within \nthe bowel wall and stroma suggesting a mucocele or mucinous tumour of the \nappendix.\nFigure 3: The patient underwent a right hemicolectomy during \nwhich further endometriotic deposits were seen. The appendix \nappeared abnormal as it was thickened and adherent to caecum with \nmucinous material extruding from the tip. Three proximal puckered \nlesions were noted in last 20cm of the terminal ileum but no other \nperitoneal disease was noted.\nHistological results found the same appendix showed.\ni) Mucinous cystadenoma of the appendix\nii) Well differentiated carcinoid tumour measuring 10mm in diameter\niii) Coexistent features of endometriosis \nFigure 4 & Figure 5: Currently, the patient is well and conceived \nnaturally whilst waiting to start IVF treatment.\nFigure 3 CT scan. Adjacent to the caecum a 10mm low attenuation lesion \nwith mural calcification was noted suggestive of an appendicular mucocele.\nFigure 4 Appendis take from hemicolectomy specimen, showing mucin on \nthe serosal surface.\nFigure 5 Nests of carcinoid tumour cells (red arrow) infiltrating appendiceal wall. \n\n\nCombined mucinous cystadenoma and carcinoid tumour of the appendix with coexistent features of an \nendometrioma: a case report\n7\nCopyright:\n©2014 Dunkerton et al.\nCitation: Dunkerton S, Pankhania NK, Johnson C, et al. Combined mucinous cystadenoma and carcinoid tumour of the appendix with coexistent features of \nan endometrioma: a case report. Obstet Gynecol Int J . 2014;1(1):5‒7. DOI: 10.15406/ogij.2014.01.00001\nDiscussion\nEndometriosis is one of the most common diseases seen by \ngynaecologists. The prevalence is difficult to estimate as women can \nbe asymptomatic or present with varying degrees of pelvic pain. 11 \nEndometriosis is associated with infertility and has been identified \nin 38.5% of infertile women as opposed to 5.2% of fertile women.12\nAppendicular tumours have diverse histology. Carcinoid neoplasms \nare the most common, accounting for 66% of all tumours of the \nappendix. They are comprised of enterochromaffin cells and contain \nmany neurosecretory granules that release serotonin, histamine and \nprostaglandins.13 As with endometriosis, the incidence and prevalence \nis unknown, as the disease is mainly asymptomatic. The average time \nfor a carcinoid tumour to become symptomatic is 9 years. Carcinoid \ntumours may present late and be associated with metastasis if found \nwithin the tip of the appendix.\nThe most common benign appendix tumours are mucinous \ncystadenomas. These tumours are composed of intestinal epithelium \nare dysplastic and secrete mucin, giving rise to a mucocele. This often \ncauses the appendix to dilate and can present as acute appendicitis. The \npresence of a mucinous cystadenoma with associated mucin within \nthe appendix wall increases the risk of psuedomyxoma peritonei.\nThere have been reports in literature of the co-existence of two \ntypes of appendicular tumours. Alsaad et al. 14 reported a case of \ncombined goblet cell carcinoid and mucinous cyst-adenoma of the \nappendix in a patient presenting with right iliac fossa pain. The \noccurrence of appendicular tumours co-existing with endometriosis \nis rarely reported, 15 and there have been no reported cases of two \ndifferent appendicular tumours being found with co-existent features \nof an endometrioma within the same appendix. Appendicectomy \nspecimens should be routinely sent for histopathological analysis. \nDuzgan et al.15 reported an intra-operative detection rate of less than \n50% for all types of appendicular tumours. Jones et al (2007) evaluated \nthe histopathological reports of 1225 appendicectomy specimens. Of \nthe 1225, 46 (3.75%) revealed abnormal diagnoses and 24 (1.96%) \nwere clinically significant, altering patient management. 16 Routine \nhistopathological analysis avoids the potential to miss significant \npathologies which may need different treatments.\nCurrently in the literature there are no clear guidelines regarding \nthe treatment of appendicular tumours.17 There is considerable debate \nas to the use of appendectomy versus right hemicolectomy and the \neffects on long term results. A right hemicolectomy is indicated \nwith adenocarcinomas, tumours invading the mesoappendix, serosa, \nlymphatics or vasculature and benign tumours with a diameter \nof more than 2cm. 18 As preferred by some surgical units 19 a right \nhemicolectomy is considered the treatment of choice. There was \nsuspicion of a malignant adenocarcinoma after initial biopsy which \nrendered a right hemicolectomy a safer option. \nConclusion\nMost surgeons will only encounter a few carcinoid tumours of \nthe appendix in their career. We feel this unique case will add to the \nsmall database of similar reports and help with management of this \nrare disease.\nThis case illustrates the broad spectrum of appendicular and \nendometriotic disease. It also demonstrates the role of biopsy of \nabnormal areas atypical of endometriosis at diagnostic laparoscopy. \nWe hope to highlight the interesting asymptomatic presentation \nof this patient and therefore the importance of requesting routine \nhistopathological analysis. After prompt treatment with right \nhemicolectomy, this young woman has fortunately conceived \nnaturally without IVF.\nAcknowledgments\nNone. \nConflicts of interest\nAuthor has no any conflict of interest to declare.\nReferences\n1. Benagiano G, Brosens I. History of Adenomyosis. Best Pract Res Clin \nObstet Gynaecol. 2006;20(4):449–463.\n2. http://www.rcog.org.uk/files/rcog-corp/GTG2410022011.pdf\n3. Mounsey AL, Wilgus A, Slawson DC. Diagnosis and management of \nendometriosis. Am Fam Physician. 2006;74(4):594–600.\n4. Remorgida V , Ferrero S, Fulcheri E, et al. Bowel endometriosis: presentation, \ndiagnosis, and treatment. Obstet Gynecol Surv. 2007;62(7):461–470.\n5. Heaps JM, Nieberg RK, Berek JS. Malignant neoplasm arising in \nendometriosis. Obstet Gyncol. 1990;75(6):1023–1028.\n6. Slavin RE, Krun R, Van Dinh T. Endometriosis associated intestinal \ntumours: a clinical and pathological study of 6 cases with a review of the \nliterature. Hum Pathol. 2000;31(4):456–463.\n7. Brinton L, Gridley G, Persson I, et al. Cancer risk after a hospital \ndischarge diagnosis of endometriosis. American Journal of Obstetrics and \nGyanecology. Am J Obstet Gynecol. 1997;176(3):572–579.\n8. Connor SJ, Hanna GB, Frizelle FA. Retrospective clinicopathologic \nanalysis of appendiceal tumours from 7970 appendicectomies. Dis Colon \nRectum. 1998;41(1):75–80.\n9. Topkan E, Polat Y , Karaoglu A. Primary mucinous adenocarcinoma of \nappendix treated with chemotherapy and radiotherapy: a case report. \nTumori. 2008;94(4):596–599.\n10. Azordegan N, Yazdankhah A, Moghadasian MH. A rare case of coexistence \nof carcinoid tumour of appendix vermicularis and ileal endometriosis. Arch \nGynecol Obstet. 2009;279(2):183–187.\n11. Slaughter K, Gala RB. Endometriosis for the colorectal surgeon. Clin \nColon Rectal Surg. 2010;23(2):72–79.\n12. Verkauf BS. Incidence, symptoms and signs of endometriosis in fertile and \ninfertile women. J Fla Med Assoc. 1987;74(9):671–675.\n13. Robertson RG, Geiger WJ, Davis NB. Carcinoid tumours. Am Fam \nPhysician. 2006;74(3):429–434.\n14. Alsaad KO, Serra S, Chetty R. Combined goblet cell carcinoid and \nmucnious cystadenoma of the vermiform appendix. World J Gastroenterol. \n2009;15(27):3431–3433.\n15. Duzgan AP, Moran M, Uzun S, et al. Unusual findings in appendicectomy \nspecimens: Evaluation of 2458 cases and review of the literature. Indian J \nSurg. 2004;66(4):221–226.\n16. Jones AE, Phillips AW, Jarvis JR, et al. The value of routine histopathological \nexamination of appendicectomy specimens. BMC Surg. 2007;7:17.\n17. Carr NJ, Sobin LH. Unusual tumours of the appendix and pseudomyxoma \nperitonei. Semin Diagn Pathol. 1996;13(4):314–325.\n18. Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumours. \nCancer.1997;79(4):813–829.\n19. Cortina R, McCormick J, Kolm P, et al. Management and prognosis of \nadenocarcinoma of the appendix. Dis Colon Rectum. 1995;38(8):848–852.","source_license":"CC0","license_restricted":false}