scholarly journals First Report of a Low-Grade Pseudomyxoma peritonei Originating from Gall Bladder

2020 ◽  
pp. 1-4
Author(s):  
Aruna Prabhu ◽  
Andreas Brandl ◽  
Satoshi Wakama ◽  
Shouzou Sako ◽  
Haruki Ishibashi ◽  
...  

Pseudomyxoma peritonei (PMP) refers to accumulation of mucinous ascites with or without neoplastic cells in the peritoneal cavity. It most commonly originates from a low or a high grade primary appendiceal mucinous neoplasm. Though adenocarcinoma of gall bladder has been reported to give rise to PMP, to the best of our knowledge, this is the first report of a PMP arising from a low grade mucinous tumour of the gall bladder. A 72-year-old patient was diagnosed with PMP 1.5 years after a cholecystectomy. After initial oral TS1 (combination of tegafur, gimeracil and oteracil) and later intraperitoneal (IP) chemotherapy with docetaxel and cisplatin, the patient was operated with the goal of tumour debulking, including removal of 5.5 L of mucinous ascites, an appendectomy, and ovariectomy. The histopathologic report showed a normal appendix and metastasis of PMP to the right ovary. After the exclusion of the 2 most common sites of origin (appendix and ovary), the specimen of the cholecystectomy was reviewed. It showed low grade mucinous tumour in the gall bladder, with immuno-histochemical markers (IHCs) suggestive of CK7, CDX2, MUC 2 positive and CK20, MUC5AC negative. MIB-1 index was 12%. The pathologic report of cytoreductive surgery performed after 7 cycles of IP chemotherapy confirmed the diagnosis of PMP originating from low grade mucinous tumour of the gall bladder. Our case report illustrates a rare disease and highlights that, though peritoneal metastasis from gall bladder cancers are known to have a poor prognosis, the peritoneal dissemination from a low grade mucinous neoplasm of gall bladder (PMP) has a significantly better prognosis due to a better disease biology and improved treatment options currently available for the treatment of PMP.

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.


2018 ◽  
Vol 37 (4) ◽  
pp. 338-343
Author(s):  
Michiel Simons ◽  
Inge Ebisch ◽  
Joanne de Hullu ◽  
Maaike van Ham ◽  
Marc Snijders ◽  
...  

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Vera Vavinskaya ◽  
Joel M. Baumgartner ◽  
Albert Ko ◽  
Cheryl C. Saenz ◽  
Mark A. Valasek

Primary appendiceal mucinous lesions are uncommon and represent a spectrum from nonneoplastic mucous retention cysts to invasive adenocarcinoma. Low-grade appendiceal mucinous neoplasms (LAMNs) represent an intermediate category on this spectrum and can be classified according to whether or not they are confined to the appendix. Although LAMNs are frequently confined to the appendix, they can also spread to the peritoneum and clinically progress as pseudomyxoma peritonei (i.e., mucinous ascites). Thus, the appropriate classification of appendiceal primary neoplasia is essential for prognosis and influences clinical management. In addition, the precise classification, management, and clinical outcome of patients with disseminated peritoneal disease remain controversial. Here, we report an unusual case of LAMN with pseudomyxoma peritonei that initially presented with mucinous and bloody vaginal discharge. Pathological evaluation revealed low-grade appendiceal mucinous neoplasm with secondary involvement of the peritoneum, ovaries, and endometrial surface. Therefore, LAMN should be considered in the differential diagnosis of mucinous vaginal discharge.


2020 ◽  
Vol 42 (1) ◽  
pp. 65-67
Author(s):  
Nisha Sharma ◽  
Alina Karna ◽  
Abhimanyu Jha

Low-grade appendiceal mucinous neoplasm (LAMN) is an uncommon malignancy with varied clinical features. The most alarming complication of this neoplasm is seeding of mucin into the adjacent peritoneum leading to pseudomyxoma peritonei (PMP). We report three cases of LAMN. Two cases were seen post appendicectomy in 23-year old and 36-year old females. The third case was seen in 53-year-old male post Whipple procedure and hemicolectomy. LAMNs are often diagnosed incidentally, as clinical presentation is barely specific and resembles acute appendicitis. Hence it is imperative to examine all appendicectomy specimens not to miss cases of appendiceal mucinous neoplasm which can have implications on the further management of the patient.


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