Computed Tomography Follow-Up Assessment of Patients with Low-Grade Appendiceal Mucinous Neoplasms: Evaluation of Risk for Pseudomyxoma Peritonei

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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Low-Grade Appendiceal Mucinous Neoplasms: A Single Institution Experience of 64 Cases With Clinical Follow-up and Correlation With the Current (Eighth Edition) AJCC Staging

International Journal of Surgical Pathology ◽

10.1177/1066896919883679 ◽

2019 ◽

Vol 28 (3) ◽

pp. 252-258 ◽

Cited By ~ 2

Author(s):

Mary Wong ◽

Brad Barrows ◽

Alexandra Gangi ◽

Stacey Kim ◽

Richard B. Mertens ◽

...

Keyword(s):

Progressive Disease ◽

Low Grade ◽

Single Institution ◽

Mucinous Neoplasms ◽

Pathologic Staging ◽

Eighth Edition ◽

Ajcc Staging ◽

Disease Free ◽

Histopathologic Features

Background. In this single-institution study, we applied the current (eighth edition) American Joint Committee on Cancer pathologic staging criteria to 64 low-grade mucinous neoplasms of the appendix (LAMNs), examined their histopathologic features, and studied the patients’ clinical outcomes. Design. Sixty-four LAMNs, with a median follow-up of 52 months, were reviewed. Results. The distribution of pathologic stages was pTis (n = 39), pT3 (n = 1), pT4a (n = 5), pT4aM1a (n = 8), and pT4aM1b (n = 11). Recurrence was observed in only 2 patients (both with pT4aM1b disease), one of whom died of disease. All remaining patients were disease-free after a median clinical follow-up of 60 months. Conclusions. Our study confirms that pTis LAMNs have an excellent prognosis and suggests that pT4a and pT4aM1a LAMNs may also have a low risk of developing progressive disease.

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Necrotizing Fasciitis Secondary to a Primary Suture for Anoperineal Trauma by Motorcycle Accident in a Healthy Adult

Case Reports in Emergency Medicine ◽

10.1155/2015/956156 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4

Author(s):

Susumu Saigusa ◽

Masaki Ohi ◽

Hiroki Imaoka ◽

Ryo Uratani ◽

Minako Kobayashi ◽

...

Keyword(s):

Computed Tomography ◽

Necrotizing Fasciitis ◽

Healthy Adult ◽

Perineal Pain ◽

Suture Repair ◽

Low Grade ◽

Primary Suture ◽

Left Chest ◽

Motorcycle Accident

A 41-year-old man experienced a swollen scrotum three days after a motorcycle accident and presented to our hospital. He had had a primary suture repair for anoperineal trauma in an outside hospital at the time of the injury. He presented to us with general fatigue, low grade fevers, and perineal pain. Abdominal computed tomography showed subcutaneous emphysema from the scrotum to the left chest. The sutured wound had foul-smelling discharge and white exudate. We made the diagnosis of necrotizing fasciitis and immediately opened the sutured wound and performed initial debridement and lavage with copious irrigation. We continued antibiotics and lavage of the wound until the infection was controlled. Fortunately, the necrotizing fasciitis did not worsen and he was discharged after 15 days. Our experience indicates that anoperineal injuries should not be closed without careful and intensive follow-up due to the potential of developing necrotizing fasciitis.

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Zap it track it: the application of Lean Six Sigma methods to improve the screening system of low-grade mucinous neoplasms of the appendix in an acute hospital setting

International Journal for Quality in Health Care ◽

10.1093/intqhc/mzz075 ◽

2019 ◽

Author(s):

kathleen McGrath ◽

Mairéad Casserly ◽

Freda O’mara ◽

Jurgen Mulsow ◽

Conor Shields ◽

...

Keyword(s):

Six Sigma ◽

Lean Six Sigma ◽

Acute Hospital ◽

Tumour Markers ◽

Low Grade ◽

Screening System ◽

Post Intervention ◽

Number Of Patients ◽

Mucinous Neoplasms

Abstract Objective To improve the number of patients receiving annual computed tomography (CT) scan and tumour markers, who are diagnosed with low-grade mucinous neoplasms (LAMN). Design A pre-/post-intervention design was employed using Lean Six Sigma methods to identify gaps in the screening system and to develop and implement solutions for a more robust, auditable screening programme. Setting The patients diagnosed with LAMN of the appendix referred to the acute hospital and are enrolled in the screening service. Participants Consultant colorectal surgeons, cancer nurse specialist, colorectal medical team and quality improvement staff. Interventions Diagnostic tools identified gaps in the current process. A set of improvements were implemented to standardize the pathway for referral and surveillance of patients, provide information on the condition and treatment and standardize and track information received by patients and their referring hospital. Main Outcome Measure(s) Pre and post-intervention outcome measures were taken for the number of patients who receive an annual CT of thoracic, abdomen and peritoneum and tumour markers and number of patients who receive information and contact details. Results At baseline, of the 28 patients that met the inclusion criteria only 61% had a correct follow-up. Following the implementation of improvements, 78% of patients had correct follow-up and 90% had received information. Conclusions Gaps in the current cancer screening system were identified and improvements implemented a reduced number of patients having an incorrect follow-up. Findings are applicable across all precancerous screening systems irrespective of the type of malignancy. The methods used empowered patients and fostered an interdisciplinary team approach to care.

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Pancreatic sphincterotomy improves pain symptoms due to branch-duct intrapapillary mucinous neoplasia without worrisome features: a multicenter study

Endoscopy International Open ◽

10.1055/a-0841-3385 ◽

2019 ◽

Vol 07 (09) ◽

pp. E1130-E1134

Author(s):

Jean-Michel Gonzalez ◽

Diane Lorenzo ◽

Jean-Philippe Ratone ◽

Adrian Culetto ◽

Frédérique Maire ◽

...

Keyword(s):

Multicenter Study ◽

Median Number ◽

Low Grade ◽

Branch Duct ◽

Biliary Sphincterotomy ◽

Mucinous Neoplasms ◽

Pain Symptoms ◽

Pancreatic Sphincterotomy ◽

Retrospective Multicenter Study

Abstract Introduction Branch duct intraductal papillary mucinous neoplasms (BD-IPMNs) require follow-up to detect worrisome features (WF). Data are missing about endoscopic pancreatic sphincterotomy (PS) for symptomatic IPMN. Patients and methods This was a retrospective multicenter study in four expert centers. Patients treated with endoscopic PS for symptomatic (painful) BD-IPMN without WF were included. Age, sex, follow-up time, characteristics of IPMNs and endoscopic retrograde cholangiopancreatographies (ERCPs), and indications for surgery were recorded. Results In total, 21 patients were included (median age 68 years, range 45 – 87 years). The median number of cysts was 2 (range 1 – 10), located in the head (59 %), body/tail (17 %), or multifocal (24 %). ERCP including PS was completed in all of the cases, with biliary sphincterotomy in 33 %. Clinical efficacy after one session was 81 % (17/21). Among the failures, one had a second successful PS and three were operated. The final efficacy was 86 % (18/21). Seven patients were operated after a mean of 19 months: four for WF, three for pain. The histopathology showed four low grade dysplasia, one high grade dysplasia, and two no dysplasia. No adenocarcinoma occurred during a follow-up of 99 months (range 14 – 276 months). Conclusions Endoscopic PS for symptomatic IPMN without WF is effective in more than 80 % of cases, without increasing the risk for adenocarcinoma.

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Low-Grade Appendiceal Mucinous Neoplasm Involving the Endometrium and Presenting with Mucinous Vaginal Discharge

Case Reports in Obstetrics and Gynecology ◽

10.1155/2016/6841989 ◽

2016 ◽

Vol 2016 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Vera Vavinskaya ◽

Joel M. Baumgartner ◽

Albert Ko ◽

Cheryl C. Saenz ◽

Mark A. Valasek

Keyword(s):

Pseudomyxoma Peritonei ◽

Vaginal Discharge ◽

Low Grade ◽

Invasive Adenocarcinoma ◽

Peritoneal Disease ◽

Mucinous Neoplasm ◽

Mucinous Neoplasms ◽

Pathological Evaluation ◽

Classification Management

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.

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599 Acute Abdomen Secondary to Appendiceal volvulus: A Case Report

British Journal of Surgery ◽

10.1093/bjs/znab134.304 ◽

2021 ◽

Vol 108 (Supplement_2) ◽

Author(s):

A Alamassi

Keyword(s):

Acute Appendicitis ◽

Fluid Collection ◽

Treatment Modalities ◽

Low Grade ◽

Case Presentation ◽

Acute Peritonitis ◽

Mucinous Neoplasms ◽

Neoplastic Process ◽

Pathological Subtypes

Abstract Introduction Appendiceal torsion is an uncommon precipitating factor for acute appendicitis. It is found to be in association with appendiceal mucocele. Case presentation A 38-year-old lady presented with acute peritonitis. CT Scan showed tubular fluid collection. This was suggested to be a torsion/volvulus of the appendix. The intraoperative finding were a distended appendix filled with mucus and twisted 720° at the base. Appendectomy was done. The histopathology showed low grade mucinus neoplasia. Discussion: Approximately, 50% of mucoceles are asymptomatic and incidentally discovered, whilst 40% present with acute appendicitis. There are two suggested pathogenesis: one being an obstructive mechanism causing high pressure in the appendix, resulting into gross mucinous accumulation and second being a neoplastic process. Appendiceal mucinous neoplasms are classified into LAMN, HAMN and mucinous appendiceal adenocarcinoma. It is important to differentiate between the pathological subtypes as the risk of occurrence of PMP may vary according to that. This would affect the treatment modalities; follow up planning, prognosis and risk of recurrence. It is important that extra precautions should be taken whilst handling the specimens intraoperatively to avoid spillage of the content as that might lead to implantation of neoplastic cells in the peritoneum and that might affect the overall survival rate.

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The many faces of pseudomyxoma peritonei: a radiological review based on 30 cases

Radiologia Brasileira ◽

10.1590/0100-3984.2019.0044 ◽

2019 ◽

Vol 52 (6) ◽

pp. 372-377

Author(s):

Cássia Fonseca ◽

Saulo Carvalho ◽

Teresa Margarida Cunha ◽

Rui Tiago Gil ◽

Nuno Abecasis

Keyword(s):

Computed Tomography ◽

Pseudomyxoma Peritonei ◽

Primary Tumor ◽

Ovarian Tumors ◽

Preoperative Imaging ◽

Low Grade ◽

Imaging Features ◽

Imaging Findings ◽

Primary Tumors ◽

Histologic Subtype

Abstract Objective: To determine the most common imaging features of pseudomyxoma peritonei (PMP), as well as the histologic subtypes of the primary tumors. Materials and Methods: We reviewed 30 cases of women with pathologically confirmed PMP. Only computed tomography scans were available. All cases were retrospectively studied by four radiologists, working independently. We identified the most common imaging findings, the predominant primary site of the disease, and the growth pattern. The most common sites of recurrence were also analyzed. Results: The most common computed tomography finding was peritoneal/omental nodules (including “omental caking”), followed by visceral scalloping and non-mucinous ascites. The most common site of the primary tumor was the appendix (in 63.3%), followed by the ovaries (in 16.6%), and 16.6% of the tumors were of undetermined origin. There was one case of synchronous appendiceal and ovarian tumors. Low-grade mucinous neoplasm was the most common histologic subtype, accounting for 84.2% of the appendiceal tumors and 40% of the ovarian tumors. Conclusion: Although PMP is a relatively rare entity, radiologists must be aware of its possible imaging findings, common locations, and possible patterns of recurrence. The origin of the primary tumor should also be investigated. Future studies are needed in order to determine which preoperative imaging findings predict surgical outcomes and to characterize the main findings of radiological recurrence.

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Pulmonary Metastasis from Pseudomyxoma Peritonei

Gastroenterology Research and Practice ◽

10.1155/2012/690256 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 10

Author(s):

Toshiyuki Kitai

Keyword(s):

Pulmonary Metastasis ◽

Pseudomyxoma Peritonei ◽

Clinical Findings ◽

Therapeutic Modality ◽

Locoregional Therapy ◽

Rapid Progression ◽

Low Grade ◽

Abdominal Lesions ◽

Disease Free

Pseudomyxoma peritonei (PMP) is a rare clinical condition, where copious mucinous ascites accumulate in the peritoneal cavity due to dissemination of mucin-producing tumor. Because of this disseminating, yet nonmetastasizing, behavior, PMP attracts much interest from surgical oncologists in that aggressive locoregional therapy can give the opportunity of long survival and even cure. Although extra-abdominal metastasis is exceptionally rare, the lung is the most likely site in such a case. In this paper, the clinical findings and treatment of eleven cases with pulmonary metastasis from PMP were reviewed, including ten cases in the literature and one case which we experienced. The clinical features of PMP cases with pulmonary metastasis were similar to cases without pulmonary metastasis. The histological type was low-grade mucinous neoplasm in most cases. Pulmonary lesions were resected in seven cases in which abdominal lesions were controlled by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy or another therapeutic modality. Disease-free state was maintained in five cases at the end of the follow-up period. However, it should be noted that rapid progression after resection was seen in two cases, suggesting that biological features may have changed by surgical intervention.

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Altered linkage pattern of N-glycan sialic acids in pseudomyxoma peritonei

Glycobiology ◽

10.1093/glycob/cwaa079 ◽

2020 ◽

Author(s):

Pirjo Nummela ◽

Annamari Heiskanen ◽

Soili Kytölä ◽

Caj Haglund ◽

Anna Lepistö ◽

...

Keyword(s):

Pseudomyxoma Peritonei ◽

Peritoneal Dissemination ◽

Sialic Acids ◽

Low Grade ◽

High Grade ◽

Malignant Diseases ◽

Linkage Pattern ◽

Mucinous Neoplasms ◽

Sialic Acid Binding ◽

Tissue Specimens

Abstract Pseudomyxoma peritonei (PMP) is a highly mucinous adenocarcinoma growing in the peritoneal cavity and most commonly originating from the appendix. Glycans play an important role in carcinogenesis, and glycosylation is altered in malignant diseases, including PMP. We have previously demonstrated that fucosylation of N-glycans is increased in PMP, but we did not observe modulation of overall sialylation. As sialic acids can be attached to the rest of the glycan via α2,3- or α2,6-linkage, we have now analyzed the linkage patterns of sialic acids in tissue specimens of normal appendices, low-grade appendiceal mucinous neoplasms (LAMN), low-grade (LG) PMP and high-grade (HG) PMP. For the linkage analysis, the enzymatically released acidic N-glycans were first treated with ethyl esterification or α2,3-sialidase digestion followed by MALDI-TOF mass spectrometry. Significant increase in the relative abundance of α2,6-sialylated and decrease in α2,3-sialylated N-glycans was observed in PMP tumors as compared to the normal appendices (P < 0.025). More specifically, increased α2,6-sialylation (P < 0.05) and decreased α2,3-sialylation (P < 0.01) were detected in afucosylated and monofucosylated N-glycans of PMPs, whereas the less abundant multifucosylated glycans, containing terminal fucose, demonstrated increased α2,3-sialylation (P < 0.01). Importantly, the increase in α2,6-sialylation was also detected between PMP and the appendiceal precursor lesion LAMN (P < 0.01). The identified glycosylation alterations produce ligands for sialic acid-binding immunoglobulin-like lectins (Siglecs) and sialofucosylated glycans binding selectins, which play a role in the peritoneal dissemination and progression of the disease.

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