Pseudomyxoma peritonei with endometrial mucinous carcinoma and appendicular mucinous tumor: An unusual association

Only 28 cases of pseudomyxoma peritonei (PMP) arising from urachal neoplasms have been reported. We report one example of this extremely rare disease with KRAS mutational status in its spectrum of pathology. A 45-year-old woman presented with urachal frankly invasive mucinous cystadenocarcinoma confined to the dome of the bladder, which clinically manifested as PMP and was not detected at the first surgery. The primary tumour was revealed 6 months later because of its recurrence as PMP. Microscopic investigation revealed tubular adenoma and cystadenocarcinoma communicating with the bladder lumen and transitioning from the urachal urothelium to the mucinous epithelium. A urachal remnant was identified near the neoplasm. On immunohistochemistry, the tumour proved positive for CK7, CK20, CEA, and CDX2. Staining for β-catenin revealed expression in both the cytoplasm and cell membrane. Mismatch repair protein expression was normal. Somatic KRAS-mutation (G12V) was revealed in tubular adenoma, cystadenocarcinoma, and mucinous carcinoma peritonei and may play an oncogenic role in the malignant transformation of urachal mucosa and the development of PMP.

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Pseudomyxoma Peritonei Caused by Acute Pancreatitis in Intraductal Papillary Mucinous Carcinoma of the Pancreas

Pancreas ◽

10.1097/01.mpa.0000194611.62723.51 ◽

2006 ◽

Vol 32 (2) ◽

pp. 223-224 ◽

Cited By ~ 20

Author(s):

Hiroshi Imaoka ◽

Kenji Yamao ◽

Ahmed A. S. Salem ◽

Nobumasa Mizuno ◽

Kuniyuki Takahashi ◽

...

Keyword(s):

Acute Pancreatitis ◽

Pseudomyxoma Peritonei ◽

Mucinous Carcinoma ◽

Carcinoma Of The Pancreas

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Pre-operative diagnosis of invasive mucinous carcinoma derived from intraductal papillary mucinous tumor

Suizo ◽

10.2958/suizo.20.31 ◽

2005 ◽

Vol 20 (1) ◽

pp. 31-38 ◽

Cited By ~ 4

Author(s):

Yuzo SASADA ◽

Masataka KIKUYAMA ◽

Takeshi KANEKO ◽

Yoshihiro IDE ◽

Takachika OZAWA ◽

...

Keyword(s):

Mucinous Carcinoma ◽

Intraductal Papillary Mucinous Tumor ◽

Mucinous Tumor

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Pseudomyxoma peritonei presenting with port site hernia

Journal of Surgical Arts ◽

10.14717/jsurgarts-210208 ◽

2021 ◽

pp. 89-92

Keyword(s):

Pseudomyxoma Peritonei ◽

Case Reports ◽

Abdominal Distension ◽

Right Hemicolectomy ◽

Port Site ◽

Mucinous Tumor ◽

Ovarian Mass ◽

First Case ◽

Port Site Hernia ◽

History Of

Pseudomyxoma peritonei (PMP) is a rare disease with an incidence of two per million. Acute appendicitis, ovarian mass, and abdominal distension are the most common presentations. A 72-year-old male patient with a history of laparoscopic cholecystectomy was admitted to the hospital with abdominal pain and increased supraumbilical port site swelling. Radiological examination revealed a mass in the terminal ileum and severe intraabdominal mucinous fluid. Intraabdominal gelatinous fluid protruding from the port site defect and a mass in the distal ap-pendix were observed during operation. He underwent a right hemicolectomy, ileocolic anasto-mosis and peritoneal debridement; cytoreductive surgery was administered two months later due to mucinous tumor of the appendix. There are only a few case reports describing PMP presen-ting with an incisional hernia after open surgical procedures. To the best of our knowledge, this report describes the first case of PMP presenting with a port site hernia after a laparoscopic in-tervention.

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Should the appendix always be removed during surgery for mucinous ovarian tumors?

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20180193 ◽

2018 ◽

Vol 7 (2) ◽

pp. 677

Author(s):

Bijal M. Patel ◽

Ava D. Desai ◽

Shilpa M. Patel ◽

Meeta H. Mankad ◽

Pariseema S. Dave ◽

...

Keyword(s):

Pseudomyxoma Peritonei ◽

Mucinous Cystadenoma ◽

Ovarian Tumors ◽

Mucinous Tumor ◽

Inclusion Criteria ◽

Single Institution ◽

Normal Appendix ◽

History Of ◽

Study Population ◽

Mucinous Ovarian Tumors

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

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Mucinous Carcinomatosis: A Rare Association between an Ovarian Tumor and an E-GIST

Case Reports in Surgery ◽

10.1155/2018/6897372 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Hugo Palma Rios ◽

André Goulart ◽

Pedro Leão

Keyword(s):

Pseudomyxoma Peritonei ◽

Ovarian Tumor ◽

Incidental Finding ◽

Intestinal Type ◽

Mesenchymal Tumor ◽

Emergency Laparotomy ◽

Mucinous Tumor ◽

Appendiceal Tumor ◽

Stromal Tumors ◽

Rare Association

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.

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A Primary Retroperitoneal Mucinous Tumor

Case Reports in Surgery ◽

10.1155/2015/157613 ◽

2015 ◽

Vol 2015 ◽

pp. 1-3

Author(s):

Alicia A. Heelan Gladden ◽

Max Wohlauer ◽

Martine C. McManus ◽

Csaba Gajdos

Keyword(s):

Case Reports ◽

Mucinous Carcinoma ◽

Malignant Potential ◽

Mucinous Tumor ◽

Retroperitoneal Mass ◽

Surveillance Imaging ◽

Mucinous Neoplasms ◽

Low Malignant Potential ◽

Mucinous Cystadenomas ◽

Primary Retroperitoneal

A twenty-five-year-old female presented with a large retroperitoneal mass. Workup included history and physical exam, imaging, biopsy, colonoscopy, and gynecologic exam. After surgical resection, the mass was determined to be a primary retroperitoneal mucinous tumor (PRMT). Clinically and histologically, these tumors are similar pancreatic and ovarian mucinous neoplasms. PRMTs are rare and few case reports have been published. PRMTs are divided into mucinous cystadenomas, mucinous borderline tumors of low malignant potential, and mucinous carcinoma. These tumors have malignant potential so resection is indicated and in some cases adjuvant chemotherapy and/or surveillance imaging.

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