scholarly journals A case of Pseudomyxoma Peritonei of an unexpected origin

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Marie Csanyi-Bastien ◽  
France Blanchard ◽  
Aude Lamy ◽  
Jean-Christophe Sabourin

Abstract Background Pseudomyxoma peritonei (PMP) is a complex and partially understood disease defined by mucin deposits in the peritoneal cavity, mostly of appendiceal origin caused by the rupture of a mucocele often containing Low or High grade Appendiceal Mucinous Neoplasm (LAMN/HAMN). Other origins include primitive ovarian mucinous cystadenoma or cystadenocarcinoma almost always with an associated teratoma, but to our knowledge no case of ovarian teratomatous appendiceal-like mucocele with LAMN has been reported as a cause of PMP. Case presentation A 25-year old female with infertility was diagnosed with an isolated left ovarian tumor in a context of PMP. Histological examination revealed an ovarian teratoma containing an appendiceal-like structure with mucocele and LAMN, without any associated lesion of the appendix on full histological analysis. Molecular characterization of the ovarian lesion showed co-KRAS and GNAS mutations, as described in PMP of appendiceal origin, while only KRAS mutations are reported in primitive ovarian mucinous tumor. Conclusions Detection of co-KRAS and GNAS mutations in our case of ovarian teratomatous appendiceal-like mucocele with LAMN shows that when PMP derives from a mucinous ovarian lesion (with histological proof of none-appendiceal involvement), it is probably of a digestive teratomatous origin, emphasizing the need to actively search for tetatomatous signs in a context of ovarian PMP.

2021 ◽  
Author(s):  
Marie Csanyi-Bastien ◽  
France Blanchard ◽  
Aude Lamy ◽  
Jean-Christophe Sabourin

Abstract Background:Pseudomyxoma peritonei (PMP) is a complex and partially understood disease defined by mucin deposits in the peritoneal cavity, mostly of appendiceal origin caused by the rupture of a mucocele often containing Low or High grade Appendiceal Mucinous Neoplasm (LAMN/HAMN). Other origins include primary ovarian mucinous cystadenoma or cystadenocarcinoma almost always with an associated teratoma, but to our knowledge no case of ovarian teratomatous appendiceal-like mucocele with LAMN has been reported as a cause of PMP.Case presentation:A 25-year old female with infertility was diagnosed with an isolated left ovarian tumor in a context of PMP. Histological examination revealed an ovarian teratoma containing an appendiceal-like structure with mucocele and LAMN, without any associated lesion of the appendix on full histological analysis. Molecular characterization of the ovarian lesion showed co-KRAS and GNAS mutations, as described in PMP of appendiceal origin, while only KRAS mutations are reported in primary ovarian mucinous tumor.Conclusions:Detection of co-KRAS and GNAS mutations in our case of ovarian teratomatous appendiceal-like mucocele with LAMN shows that when PMP derives from a mucinous ovarian lesion (with histological proof of none-appendiceal involvement), it is probably of a digestive teratomatous origin, emphasizing the need to actively search for tetatomatous signs in a context of ovarian PMP.


Author(s):  
Bijal M. Patel ◽  
Ava D. Desai ◽  
Shilpa M. Patel ◽  
Meeta H. Mankad ◽  
Pariseema S. Dave ◽  
...  

Background: Appendectomy is performed in all mucinous ovarian tumors (MOT) identified intraoperatively to ensure microscopic metastases from appendix are not missed. Several recent studies suggested that appendectomy should only be performed in cases with a grossly abnormal appendix or with evidence of pseudomyxoma peritonei. Our study aimed to determine the frequency of malignancy in a grossly normal appendix in women undergoing surgery for borderline or malignant MOT.Methods: In a single institution retrospective study, women undergoing surgery for MOT from January 1, 2008 to June 30, 2016 were included. Women with benign MOT, those with a history of either prior appendicectomy or prior gastrointestinal (GI) malignancy were excluded.Results: Of 266 women identified with MOT, 153 with borderline and malignant MOT were included in the study after application of inclusion criteria. The study population comprised of 29 (18.95%) borderline and 124 (81.05%) malignant MOT. Among the borderline MOT, 13/29 had undergone appendectomy. Five (38.46%) had grossly abnormal appendices of whom 1 had mucinous cystadenoma, 3 had borderline mucinous tumor and 1 had mucinous cystadenocarcinoma of the appendix. Histology was normal in all 8 (61.54%) grossly normal appendices. Among the malignant MOT, 80/124 (64.52%) underwent appendicectomy. Nineteen (23.46%) had grossly abnormal appendices and histology was suggestive of adenocarcinoma of appendix. Histology was normal in all 62 (76.54%) macroscopically normal appendices.Conclusions: Present results suggest that appendectomy be performed only for those appendices that are grossly abnormal or associated with pseudomyxoma peritonei at surgery for MOT


2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Hugo Palma Rios ◽  
André Goulart ◽  
Pedro Leão

Pseudomyxoma peritonei (PMP) and extragastrointestinal stromal tumors (E-GISTs) are both rare entities. Most of the time, PMP is associated with an appendiceal tumor. An ovarian mucinous tumor can mimic appendiceal metastases. E-GIST is a mesenchymal tumor that can arise from the omentum, retroperitoneum, mesentery, or pleura. We present a case of an 87-year-old woman with mucinous carcinomatosis and acute intestinal occlusion submitted to an emergency laparotomy. She has found to have a borderline mucinous tumor of the ovary from the intestinal type with several lesions of pseudomyxoma peritonei and an E-GIST from the epiploons retrocavity (intermediated risk). In the literature, no relation was found between these two rare tumors. E-GIST was an incidental finding in the context of a mucinous carcinomatosis.


2021 ◽  
Vol 5 (1) ◽  
pp. 76-79
Author(s):  
Manju Pandey ◽  
Binuma Shrestha ◽  
Bijay Chandra Acharya ◽  
Suraj Upreti

Pseudomyxoma peritonei is an extremely rare clinical syndrome with incidence of approximately two per 10000 laparotomies. Approximately 44% of women with PMP have ovarian mucinous tumor. It is generally accepted that the ovarian involvement is secondary; PMP is nearly always gastrointestinal origin, usually from mucinous adenoma of the appendix1. Exception to this, we report a 35 years lady who presented with the complaint of abdominal pain and abdominal distention for two months, clinically and radiologically gross mucinous ascites with large abdominopelvic mass was evident. Final histopathology was reported as mucinous cystadenoma, with mature teratoma components, appendix was normal. 


Author(s):  
Hanny Aditanzil ◽  
Bayu Mahendra ◽  
Ketut Suwiyoga

Objectives: To improve skill in making a diagnosis and management of pseudomyxoma peritoneum originating borderline mucinous ovarian tumor during pregnancy. Methods: Case report. Conclusion: Diagnosis of pseudomyxoma peritoneum during pregnancy is difficult before surgery. Management is based on grade of malignancy and gestational age of pregnancy. [Indones J Obstet Gynecol 2013; 1-3: 161-5] Keywords: mucinous tumor, pregnancy, pseudomyxoma peritoneum


2021 ◽  
pp. 106689692199945
Author(s):  
Christine J. Salibay ◽  
Valentina Zanfagnin ◽  
Heather Miller ◽  
Saloni Walia ◽  
Laurie L. Brunette ◽  
...  

Ovarian Brenner tumors, accounting for ∼5% of overall ovarian epithelial neoplasm, are often reported in association with mucinous neoplasm. Histogenetically, the two tumors are thought to arise from similar precursors. To date, fewer than 60 borderline Brenner tumors alone have been reported, and the concomitant presence of atypical proliferative components in Brenner and mucinous tumors is even rarer. Therefore, the clinicopathological characteristics and prognosis of patients with the borderline Brenner tumors alone or coexisting with mucinous neoplasm are extremely limited. Herein, we report a unique case of a 53-year-old woman with a unilateral ovarian borderline Brenner tumor associated with focal atypical mucinous epithelial proliferation and her clinical presentations. The clinicopathological features of the tumor are documented and the literature review along with the clinical molecular advances are summarized in this study.


2015 ◽  
Vol 34 (3) ◽  
pp. 293-297 ◽  
Author(s):  
Enoe Quiñonez ◽  
Maolly Schuldt ◽  
Juan A. Retamero ◽  
Francisco F. Nogales

2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Kimberly Da Costa ◽  
Sivakumaran Sabanathan

Abstract A mucocele refers to an appendix that has dilated due to progressive accumulation of mucus within its lumen. Appendiceal mucocele is a rare cause of an acute abdomen. They represent 0.2-0.7% of all appendix specimens. LAMN account for less than 0.3% of appendicectomy specimens.  We present a 38 year old man with an acute RIF’s pain who went on to have CT scan which revealed a mucocele of appendix but did not show any features of perforation or pseudomyxoma peritonei. He had a laparoscopic converted to open appendicectomy. The proximal 2 cm of appendix was oedematous but normal calibre. Histology revealed a low grade appendiceal mucinous neoplasm (LAMN) that was completely excised.  The mucocele of the appendix was first described by Rokitansky in 1842. Appendix mucocele may come as a consequence of obstructive or inflammatory processes, cystadenomas or cystadenocarcinomas. Appendiceal mucinous neoplasms commonly presents in the sixth decade of life and our patient was much younger in comparison. Several literatures suggest the value of preoperative CT imaging in obtaining diagnosis and also in planning further treatment. Appendicectomy or a right hemicolectomy is treatment of choice based on presence or absence of following factors 1. Perforated mucocele 2. Involvement of the base of the appendix. 3. Positive lymph nodes of mesoappendix and ileocolic. Patients with malignancy or pseudomyxoma peritonei are likely to require cytoreductive surgery, heated intraoperative intraperitoneal chemotherapy, early postoperative intraperitoneal chemotherapy.


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