Case 295: Pseudomyxoma from Mucinous Adenocarcinoma of the Appendix Extending from the Retroperitoneum into the Thigh.

History A 57-year-old man with no remarkable past medical history presented to an outside institution with painless swelling in his right thigh of 6 months duration. He denied any trauma to the site. At that time, physical examination demonstrated swelling in his right upper thigh. All other work-up, including complete blood count and chest radiography, yielded negative results. The initial diagnosis was lymphangioma of the thigh. He continued to experience worsening swelling in his right upper thigh with no other symptoms over the next year. He was referred to our facility, where he underwent US evaluation of the thigh lesion, an MRI scan encompassing the entire extent of his thigh lesion, and a CT scan of his abdomen and pelvis.

Endometrioid Adenocarcinoma With Solitary Metastasis to the Appendix, Mimicking Primary Appendiceal Adenocarcinoma: A Case Report and Literature Review.

Metastatic tumors of the appendix are rare. Endometrial cancer tends to metastasize by directly invading neighboring structures; the lung, liver, bones, and brain are common sites of distant metastasis. Herein, we present a case of a solitary endometrial metastatic tumor in the appendiceal mucosa without serosal involvement that mimicked a primary adenocarcinoma of the appendix. The patient who had undergone a radical hysterectomy for an endometrioid adenocarcinoma 3 years earlier presented to the hospital with a history of persistent right-lower abdominal pain. Physical examination showed tension of the abdominal muscles, tenderness, and rebounding pain on the McBurney's point. Open appendectomy for suspected appendicitis revealed a perforation of the distal appendiceal tip. Opening of the surgical specimen showed a mass that was located in the mucosa of the appendix near the appendicular root and resembled a primary tumor of the appendix. Microscopically, the adenocarcinoma of the appendiceal mucosa showed a transitional relationship with the normal mucosa, involving the submucosa and muscle but not invading the serosa. Based on the patient's medical history and the results of immunohistochemical staining, we made a diagnosis of metastatic endometrioid adenocarcinoma. The gross anatomy and histologic features of solitary metastatic tumors can mimic those of primary tumors. A correct diagnosis should be made by combining the patient's medical history with morphologic and immunohistochemical test results.

Rate of Appendiceal Metastasis with Non-Serous Epithelial Ovarian Cancer in Manitoba.

OBJECTIVE: This study sought to evaluate the rate of appendiceal involvement in non-serous mucinous and endometrioid-associated epithelial ovarian cancers. METHODS: The Manitoba Cancer Registry and CancerCare database were used to find all women with non-serous epithelial ovarian, fallopian tube, or primary peritoneal cancer between 1995 and 2011. All patients with an appendectomy were then identified, and their final pathology findings were reviewed. Women who did not receive treatment or lacked follow-up were excluded. RESULTS: We identified 338 patients from 1995-2011 with no prior appendectomy. Of these, 16.6% received an appendectomy, and 22.8% were clinically evaluated. Most cases within this cohort were mucinous (62%) and stage 1 (63%). Four appendiceal metastases were identified (7.2%), and one half appeared clinically normal at the time of surgery (3.6%). Within the mucinous histologic type, 32.7% of patients received an appendectomy, with a metastatic rate of 5.7%. Of the 127 endometrioid cases, only 10 patients received an appendectomy, and 2 were found to have metastases. No metastases were found in the 85 patients in the clear cell cohort, only 5 of whom received an appendectomy. CONCLUSION: Routine appendectomy or clinical assessment of the appendix is valuable for all non-serous ovarian cancers. The rate of involvement for endometriosis-associated ovarian cancers may be significantly higher than expected, and further studies need to be conducted.

Is it necessary to perform routine appendectomy for mucinous ovarian neoplasms? A retrospective study and meta-analysis.

OBJECTIVES: To evaluate whether routine appendectomy is necessary in all patients with mucinous borderline ovarian tumor (mBOT) or mucinous ovarian cancer (MOC) who undergo gynecologic surgery. METHODS: The database of Qilu Hospital was searched for women who underwent appendectomy in a primary surgery for an ovarian tumor between June 2005 and June 2015 and whose final diagnosis was mBOT, MOC or primary appendiceal tumor. A retrospective review was performed, as well as a meta-analysis of the literature to further validate the findings. RESULTS: Seventy-one patients, 29 with mBOT and 42 with malignant mucinous tumors (including 40 with primary MOC and 2 with appendiceal mucinous adenocarcinoma), underwent appendectomy at the time of primary surgery. Among those with mBOT, two (6.9%) appendices were grossly abnormal and pathologically diagnosed with appendiceal implantation by mBOT. In the 42 patients with malignant disease, five (12%) appendices had a grossly abnormal appearance, one (2.4%) was diagnosed with an appendiceal metastasis from MOC and two (4.7%) were primary appendiceal adenocarcinoma. For grossly normal appendices, only one (2.4%) was confirmed to have microscopic metastasis from MOC. The meta-analysis included a total of 914 mBOT and MOC cases with appendectomies, including our current cases. The estimated rate of overall appendiceal pathology is 4.97%, and the pooled odds ratio (OR) showed statistical differences between MOC and mBOT (MOC vs. mBOT, OR=2.15, P<0.05). The estimated malignant pathology rate in macroscopically normal vs. abnormal appendices is 1.4% and 59%, respectively, with an estimated OR up to 97.5 (95% CI 28.1-338.5, P<0.05). CONCLUSION: There is not sufficient evidence to support a routine appendectomy for patients with a grossly normal appendix in mBOT and MOC. A careful intra-operative exploration of the appendix is crucial, but appendectomy is only warranted when the appendix is abnormal.

The Importance of Appendectomy in Surgery for Mucinous Adenocarcinoma of the Ovary.

OBJECTIVE: The aim of this study was to assess the importance of appendectomy during surgery for mucinous ovarian cancer. It can be difficult to distinguish between primary ovarian and primary appendiceal cancers clinically, histologically, and immunohistochemically. Removal of the appendix may facilitate differential diagnosis, improve staging, and possibly increase 5-year survival but may also be associated with increased postsurgical morbidity. In the largest population published to date, we analyze and discuss these matters. METHODS: Prospectively gathered data on 269 patients with confirmed mucinous ovarian adenocarcinoma from a national database were analyzed. The impact of appendectomy and metastases to the appendix on 5-year and overall survival was analyzed. RESULTS: Appendectomy was performed in 172 cases (64%), and in 10 cases (4%), pathologic evaluation of the removed appendix revealed metastases from ovarian cancer. Three of the cases were macroscopically normal, and metastases were discovered only during microscopic evaluation. Patients with metastatic disease to the appendix had significantly worse 5-year survival (22%) compared with patients without metastases (73%) (χ = 31.998, P < 0.0001). Equally, 5-year survival was significantly higher in patients who had been adequately staged with hysterectomy, omentectomy, bilateral salpingo-oophorectomy, and appendectomy (74% vs 52%, χ = 7.322, P = 0.007). In multivariate analysis, increase in revised 2013 International Federation of Gynecology and Obstetrics classification stage (IA reference) was significantly associated with worsened prognosis (hazard ratio, 1.13; P < 0.0001). Equally, each stepwise increase in performance status score was related to a poorer prognosis with hazard ratio of 1.63 (P < 0.0001). Metastases to the appendix and staging did not remain significant factors of survival in multivariate analysis. CONCLUSIONS: Univariate analysis suggests that metastatic disease to the appendix and failure to perform complete staging including appendectomy are related to a worsened prognosis. A normal-looking appendix does not exclude metastatic disease, and because appendectomy is easily performed and does not increase morbidity, it should be performed during surgery for suspected mucinous ovarian cancer.

Computed Tomography Features of Appendiceal Metastasis in Patients With Underlying Malignancy: Comparison With Pathological Findings.

OBJECTIVE: To evaluate the computed tomography (CT) findings compared with pathologic features and to determine whether CT findings are useful for diagnosis of appendiceal metastasis in patients with underlying malignancy. MATERIALS AND METHODS: Preoperative CT examinations of 59 patients who had underlying malignancy and underwent appendectomy were evaluated. Appendiceal metastasis (n = 21) and nonmetastasis (n = 38) were pathologically confirmed. Computed tomography features (appendiceal morphology and diameter, presence of ascites, rectal shelf, omental cake, lymphadenopathy or organ metastasis) were retrospectively reviewed, and compared with histopathological findings. Fisher exact test and Wilcoxon rank sum test were performed for statistical analysis. RESULTS: Mean diameter of appendiceal metastasis was significantly larger (9.1 mm) than that of nonmetastasis (5.2 mm), (P < 0.0001). The CT morphology of appendiceal metastasis showed broad spectrum, and 3 cases of nodular thickening was only detected in appendiceal metastasis. There was a significant difference between groups (P = 0.0102). Appendiceal metastasis was more frequently associated with peritoneal seeding (ascitis, rectal shelf, omental cake) than nonmetastasis (P < 0.0001). Histopathological invasion of appendiceal metastasis was more frequently seen in serosa-mesoappendix (n = 20, 100%) or muscularis propria (n = 17, 85%), than in submucosa (n = 10, 50%) or mucosa layer (n = 7, 35%). Acute appendicitis was more commonly detected in metastasis (n = 4) than in nonmetastasis (n = 1) (P = 0.0495). CONCLUSIONS: Evaluation of CT features of appendix and peritoneum may be useful for prediction of appendiceal metastasis in patients with underlying malignancy.

Pulmonary toxicity after intraperitoneal mitomycin C: a case report of a rare complication of HIPEC.

BACKGROUND: Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) has become a common treatment approach for disseminated appendiceal neoplasms. Systemic absorption of intraperitoneal chemotherapeutics may lead to drug-induced toxicity, most commonly neutropenia. Mitomycin C has been the most commonly used chemotherapeutic in HIPEC for the past several decades. CASE PRESENTATION: Here, we describe a rare pulmonary complication secondary to intraperitoneal administration of mitomycin C. CONCLUSIONS: While rare, intraperitoneal mitomycin C has the potential to cause serious pulmonary toxicity that should be considered with administration. To our knowledge, this report represents only the second case described in the literature.

Microscopic Omental Metastasis in Clinical Stage I Endometrial Cancer: A Meta-analysis.

BACKGROUND: A patient with early-stage endometrial cancer may possibly have microscopic metastasis in the omentum, which is associated with a poor prognosis. The purpose of this study was to identify risk factors for microscopic omental metastasis in patients with clinical stage I endometrial cancer to establish the indications for selective omentectomy. METHODS: We searched the PubMed, EMBASE, and Cochrane Library databases for published studies from inception to August 2014, using terms such as 'endometrial cancer' or 'uterine cancer' for disease, 'omentectomy' or 'omental biopsy' for intervention, and 'metastasis' for outcome. Two reviewers independently identified the studies that matched the selection criteria. We calculated the pooled risk ratios (RRs) with 95 % confidence intervals (CI) of each surgicopathologic finding for microscopic omental metastases in clinical stage I endometrial cancer. We also calculated the prevalence of microscopic omental metastases. RESULTS: Among 1163 patients from ten studies, 22 cases (1.9 %) of microscopic omental metastases were found, which accounted for 26.5 % of all omental metastases. Positive lymph nodes (RR 8.71, 95 % CI 1.38-54.95), adnexal metastases (RR 16.76, 95 % CI 2.60-107.97), and appendiceal implants (RR 161.67, 95 % CI 5.16-5061.03) were highly associated with microscopic omental metastases. CONCLUSIONS: Microscopic omental metastases were not negligible in patients with clinical stage I endometrial cancer. Those with a risk factor of microscopic omental metastases were recommended for selective omentectomy.

[A case of appendix cancer treated as cancer of unknown primary origin].

A 57-year-old man initially presented with chief complaints of abdominal distension and anorexia. Positron emission tomography- computed tomography (PET-CT) scan showed ascites and multiple peritoneal metastases with abnormal uptake of fluorodeoxyglucose (FDG). The patient underwent endoscopy, biopsy, and cytology and was diagnosed with adenocarcinoma of unknown primary origin. He was treated with systematic chemotherapy, including carboplatin/paclitaxel (CBDCA/ PTX) and gemcitabine regimens. However, progressive disease (PD) complicated by intestinal obstruction was indicated. He was referred to our department for management. We performed surgery to resolve the intestinal obstruction and confirm the diagnosis. Appendix cancer was diagnosed intraoperatively. He was administered a modified fluorouracil plus Leucovorin and oxaliplatin(mFOLFOX6) /panitumumab regimen following surgery. The tumor had a good response to treatment, and the primary lesion was resected. After resection , the tumor was controlled by systemic chemotherapy for six months. However, the patient unfortunately died owing to arrhythmia. Most patients with cancer of unknown primary origin have a very poor prognosis because it is difficult to select appropriate treatment. Laparotomy can be effective in making a definitive diagnosis, as in the case described here.

Appendiceal carcinoids in children--management and outcomes.

BACKGROUND: Appendiceal carcinoid tumors are very rare in children, and management has been guided by adult presentations and outcomes. Here, we present our experience with pediatric appendiceal carcinoid tumors. METHODS: We undertook a retrospective review of all cases of appendiceal carcinoids in children over a 20-y period. Data regarding clinical presentation, diagnosis, pathology, follow-up, and outcomes were collected and analyzed. RESULTS: We identified 13 cases of appendiceal carcinoids. All cases were diagnosed after appendectomy for presumed appendicitis (nine acute and four interval;), with no patient having carcinoid syndrome. Mean age at diagnosis was 13.7 y, and all but one case was female. Tumor size ranged from microscopic foci of tumor cells to 2.1 cm (mean, 0.93 cm). Seven cases had invasion of the mesoappendix, three of which underwent a subsequent right hemicolectomy. The patient with the largest tumor (2.1 cm) had evidence of lymphatic invasion with three nodes positive for tumor after right colectomy. No patient had elevation of 5-hydroxyindoleacetic acid or serum chromogranin A, and surveillance computed tomographic scans did not reveal any liver metastases. CONCLUSIONS: This is a relatively large series of carcinoids of the appendix in children. We found no evidence of carcinoid syndrome or metastatic disease in these cases other than lymphatic. The need for a secondary colectomy is perhaps questionable.

Appendectomy with cytoreductive surgery for ovarian and type 2 endometrial carcinoma.

UNLABELLED: There is considerable variation within and between cancer centers in the practice of appendectomy as part of cytoreductive surgery for ovarian carcinoma and in the surgical staging of endometrial carcinoma. The purpose of this study was to determine the prevalence and the type of appendiceal pathology, the morbidity associated with appendectomy in gynaecologic cancer surgery. MATERIALS AND METHODS: This is a retrospective review of all cytoreductive surgery for ovarian carcinoma and surgical staging for endometrial carcinoma with appendectomy over a four year period. RESULTS: Two hundred and fifty-one patients (38 patients for endometrial carcinoma surgery and 213 patients for ovarian cytoreduction) had an appendectomy performed. Metastases to the appendix was present in 46 (23.2%) of primary ovarian carcinoma and one (2.6%) primary endometrial carcinosarcoma. The appendix was more likely to be involved in advanced stage ovarian cancer with positive peritoneal washings, omental deposits, grade 3 differentiation, and papillary serous histology. Sixteen (6.4%) co-incidental primary appendiceal tumours were detected. No postoperative morbidity specific to appendectomy was identified. One case of ovarian carcinoma was upstaged from IC to IIIA by the appendiceal metastases. There was no upstaging of disease in the endometrial carcinoma group. DISCUSSION: Appendectomy is an integral part of ovarian cytoreductive surgery but the authors found it did not upstage the disease in a clinically significant manner. The incidence of co-incidental appendiceal primary tumours was high in this series and may add value to the procedure in preventing further surgeries. The absence of procedure related morbidity is reassuring. The authors recommend appendectomy for all ovarian staging surgery and its consideration in type 2 endometrial cancer.

Intraoperative predictors of appendiceal abnormalities in patients with mucinous ovarian neoplasms.

OBJECTIVE: To evaluate intraoperative factors predicting appendiceal pathology during gynecologic oncology surgery for suspected mucinous ovarian neoplasms. METHODS: We conducted a retrospective study on 225 patients with mucinous ovarian neoplasms who underwent surgery for an adnexal mass with concurrent appendectomy between 2000 and 2018. Regression analyses were used to evaluate intraoperative factors, such as frozen section of the ovarian mass and surgeon's impression of the appendix in predicting appendiceal pathology. RESULTS: Most patients (77.8%) had a normal appendix on final pathology. Abnormal appendix cases (n = 26) included: metastasis from high-grade adenocarcinoma of the ovary (n = 1), neuroendocrine tumor of the appendix (n = 4), and low-grade appendiceal mucinous neoplasms (n = 26; 23 associated with a mucinous ovarian adenocarcinoma, 2 with a benign mucinous ovarian cystadenoma, and 1 with a borderline mucinous ovarian tumor). Combining normal intraoperative appearance of the appendix with benign or borderline frozen section yielded a negative predictive value of 85.1%, with 14.9% of patients being misclassified, and 6.0% having a neuroendocrine tumor or low-grade appendiceal neoplasm. CONCLUSION: Benign or borderline frozen section of an ovarian mucinous neoplasm and normal appearing appendix have limited predictive value for appendiceal pathology. Appendectomy with removal of the mesoappendix should be considered in all cases of mucinous ovarian neoplasm, regardless of intraoperative findings.

Recurrence of hepatocellular carcinoma presenting as an asymptomatic appendiceal tumor: report of a case.

This report presents the case of a 66-year-old male with appendicular metastasis from hepatocellular carcinoma. He had a clinical history of right lobectomy of the liver after the diagnosis of hepatocellular carcinoma 3 years prior, and was admitted because of an asymptomatic appendiceal tumor detected by computed tomography. The appendiceal tumor was preoperatively suspected to be a recurrence of hepatocellular carcinoma, because of the patient's elevated level of serum α-fetoprotein and protein induced by vitamin K absence or antagonist II, and based on the magnetic resonance imaging findings. An appendectomy was thus performed, and the histopathological findings confirmed the diagnosis of a metastatic tumor from hepatocellular carcinoma. Appendicular metastasis is extremely rare. This is only the second case of a metastatic appendiceal tumor from hepatocellular carcinoma reported in the English literature.

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) for colorectal and appendiceal peritoneal metastases - The Hong Kong experience and literature review.

INTRODUCTION: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) is increasingly used to treat peritoneal metastases from appendiceal or colorectal origin. We evaluate our institution's experience and survival outcomes with this procedure, and its efficacy in symptom relief. METHODS: This is a single-centre retrospective observational study on patients with peritoneal metastases (PM) from appendiceal neoplasm or colorectal cancer who underwent CRS/HIPEC in Queen Mary Hospital. Our primary endpoints were overall survival (OS) and morbidity and mortality of this procedure; secondary endpoints included disease-free survival (DFS) and symptom-free survival. RESULTS: Between 2006 and 2018, thirty CRS/HIPEC procedures were performed for 28 patients - 17 (60.7%) had appendiceal PM while 11 (39.9%) had colorectal PM. The median peritoneal cancer index was 20; complete cytoreduction was achieved in 83.3% patients. High-grade morbidity occurred in 13.3% cases. There was no 30-day mortality. Two-year OS were 71.6% and 50% for low-grade appendiceal PM and colorectal PM patients (p = 0.20). Complete cytoreduction improved OS (2-year OS 75.4% vs 20%, p = 0.04). Median DFS was 11.8 months. Median symptom-free duration was 36.8 months; patients with complete cytoreduction were more likely to remain asymptomatic (82.9% at 1 year, vs 60% in incomplete cytoreduction group, p < 0.01). 91.7% low-grade appendiceal PM patients and 58.4% colorectal PM patients remained asymptomatic at post-operative one year (p = 0.31). CONCLUSION: CRS/HIPEC is beneficial to appendiceal PM and selected colorectal PM patients - improving survival and offering prolonged symptom relief, with reasonable morbidity and mortality. Complete cytoreduction is key to realising this benefit.

Acute appendicitis as primary symptom of prostatic adenocarcinoma: report of a case.

The most common disease ofappendix is acute appendicitis. There have been at least 20 case reports of metastasis-induced appendicitis in English literature. The authors reported a 62-year-old Thai man who underwent appendectomy due to classical symptoms and signs of acute appendicitis and was subsequently referred to Siriraj Hospital for proper management after a pathological report of metastatic adenocarcinoma to the appendix. By morphology, the primary site was thought to be the prostate gland. Confirmation was done based on serum PSA study, transrectal ultrasound-guided core needle biopsy, and review of the material from Hua Hin Hospital. Orchidectomy was performed with satisfactory outcome. The patient was doing well for two years before upper gastrointestinal hemorrhage and obstructive jaundice caused by another aggressive tumor developed. He died of the second tumor associated with subsequent renal failure in spite of good response to orchidectomy.

Missed Gastric Cancer Metastasis to the Appendix: Case Report and Literature Review.

BACKGROUND Gastric cancer metastasis to the appendix is a rare condition that might present with symptoms of acute appendicitis or remain asymptomatic and be diagnosed incidentally. This report summaries 6 previously reported cases in addition to the presented case. CASE REPORT We report a 54-years-old female patient who presented with gastric cancer metastasis to the appendix that was found incidentally in the second surgery when she underwent bowel resection due to bowel entrapment in internal hernia, a complication of her primary gastric cancer surgical intervention. Six case-reports on gastric cancer metastasis to the appendix were reviewed. The metastasis was symptomatic in 4 cases, and solitary in 3 cases. The diagnosis was delayed in 4 cases as there was no evidence of metastasis at the diagnosis of the primary tumor; appendectomy was performed in all cases. The prognosis of the cases varied considerably. CONCLUSIONS We question the real incidence of appendiceal metastasis in gastric cancer, and the benefit-risk ratio of appendectomy in every gastrectomy. Guidelines on management of similar cases is also needed.

Unusual Pattern of Invasive Lobular Carcinoma Metastasis: A Case Report.

BACKGROUND Metastasis of breast cancer to the gastrointestinal (GI) tract is not common, however, invasive lobular carcinoma is more likely to metastasize to the GI tract than ductal carcinoma. The simultaneous metastasis of invasive lobular carcinoma to the GI tract and ovaries is an extremely uncommon presentation, which may mimic another rare entity that is peritoneal carcinomatosis secondary to breast cancer. Diagnosis of this entity is difficult as it can masquerade as a primary disease process instead of a secondary one. Treatment is even more difficult due to the sparsity of guidelines regarding this presentation. CASE REPORT A 58-year-old female with a history of invasive lobular carcinoma of the left breast treated 5 years prior to presentation with GI symptoms. Workup revealed a stenosis of the sigmoid colon; however, colonoscopy and biopsy did not show signs of malignancy. The patient was initially diagnosed with diverticulitis and given appropriate treatment which mildly improved her symptoms but did not eradicate them. Continued symptoms and failed attempts at diagnosis prompted the decision to perform an exploratory laparotomy which revealed metastasis of invasive lobular carcinoma to the sigmoid colon, appendix, and ovaries. CONCLUSIONS GI metastasis of breast cancer is a difficult entity to diagnose and treat. Concomitant metastasis to the GI tract and genitourinary system is even more challenging to diagnose and treat. These variable metastasis presentations of breast cancer indicate a need for more specific modalities for follow-up of breast cancer patients especially those with the invasive lobular subtype which tends to metastasize to unusual distant sites and present years after diagnosis and treatment of the primary disease.

Anti-angiogenesis treatment in a patient with appendix metastasis of small cell lung cancer: A case report.

RATIONALE: Small-cell lung cancer (SCLC) is a common pathological type of lung cancer, but appendiceal metastasis of SCLC was rare. At present, clinical studies on the maintenance therapy of SCLC have not reached a significant conclusion. PATIENT CONCERNS: We reported on a 52-year-old man who diagnosed as extensive stage SCLC with abdominal pain for 2 months, aggravated for 2 days. DIAGNOSES: The patient was diagnosed with extensive-stage SCLC, relapsed with appendix metastasis after treatment by emergency abdominal surgery. INTERVENTIONS: The patient received systemic treatments, including surgery, bevacizumab in combination with chemotherapy and bevacizumab alone was continued as maintenance therapy. OUTCOMES: The patient had an overall survival would more than 23 months, and he gained another 8 months of progression-free survival after first-line radiochemotherapy. LESSONS: Although SCLC appendix metastasis is rare, continuous anti-angiogenic therapy combined with bevacizumab maintenance therapy after surgical treatment can prolong survival.