-
Indian Journal of Pathology &... Apr 2024Well-differentiated neuroendocrine tumors of the appendix (A-WDNET) are the most common neoplasms of the appendix but are rare and incidental. INSM1 is a novel...
Evaluation of immunohistochemical expression of novel neuroendocrine marker INSM1 and histological tumor growth pattern in well-differentiated neuroendocrine tumors of the appendix: 15-year single tertiary center experience.
INTRODUCTION
Well-differentiated neuroendocrine tumors of the appendix (A-WDNET) are the most common neoplasms of the appendix but are rare and incidental. INSM1 is a novel transcription factor marker with high sensitivity and specificity for neuroendocrine differentiation. It may display architecturally diverse tumor growth patterns including solid, nest, insular trabecular, and acinar.
AIMS
The aim of this study was to determine the staining expression of INSM1 in A-WDNETs and detail the morphological tumor growth patterns.
MATERIALS AND METHODS
INSM1 immunohistochemistry was performed on 35 A-WDNET patients. Tumors were histologically classified according to their growth patterns.
RESULTS
Thirty-five patients, 60% of whom were male, had a mean age of 30 years at diagnosis and a mean tumor size of 0.9 cm. Eight percent of the tumors invaded the mucosa/submucosa, 34.3% showed invasion into the muscularis propria, 57.1% showed invasion into the subserosa or mesoappendix, LN metastasis was observed in two patients, lymph-vascular invasion in two patients, and perineural invasion in five patients. Ninety-four percent of the tumors were grade 1, the mitotic rate was >2% in two cases, and Ki-67 PI was >3% in two cases. INSM1 was positive in all cases (100%), 1+ 8.6%, 2+ 5.7%, 3+ 17.1%, 4+ 68.6%, weak staining in 11.4%, moderate staining in 22.9%, and strong staining in 67.7%. Type A histological growth pattern was observed in 54.3%, type B in 31.4%, and type C in 14.3% architecturally.
CONCLUSIONS
INSM1 was positive in all A-WDNET morphological patterns and was 100% sensitive. INSM1 IHC can be used as an alternative to traditional neuroendocrine markers or in combination with the diagnosis of neuroendocrine tumors.
Topics: Humans; Male; Neuroendocrine Tumors; Female; Adult; Repressor Proteins; Immunohistochemistry; Biomarkers, Tumor; Middle Aged; Appendiceal Neoplasms; Young Adult; Adolescent; Tertiary Care Centers; Appendix; Aged
PubMed: 38427752
DOI: 10.4103/ijpm.ijpm_514_23 -
JAMA Network Open Feb 2024Serum tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA125) have been useful in the management of...
IMPORTANCE
Serum tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), and cancer antigen 125 (CA125) have been useful in the management of gastrointestinal and gynecological cancers; however, there is limited information regarding their utility in patients with appendiceal adenocarcinoma.
OBJECTIVE
To assess the association of serum tumor markers (CEA, CA19-9, and CA125) with clinical outcomes and pathologic and molecular features in patients with appendiceal adenocarcinoma.
DESIGN, SETTING, AND PARTICIPANTS
This is a retrospective cohort study at a single tertiary care comprehensive cancer center. The median (IQR) follow-up time was 52 (21-101) months. Software was used to query the MD Anderson internal patient database to identify patients with a diagnosis of appendiceal adenocarcinoma and at least 1 tumor marker measured at MD Anderson between March 2016 and May 2023. Data were analyzed from January to December 2023.
MAIN OUTCOMES AND MEASURES
Association of serum tumor markers with survival in patients with appendiceal adenocarcinoma. Cox proportional hazards regression analyses were also performed to assess associations between clinical factors (serum tumor marker levels, demographics, and patient and disease characteristics) and patient outcomes (overall survival).
RESULTS
A total of 1338 patients with appendiceal adenocarcinoma were included, with a median (range) age at diagnosis of 56.5 (22.3-89.6) years. The majority of the patients had metastatic disease (1080 patients [80.7%]). CEA was elevated in 742 of the patients tested (56%), while CA19-9 and CA125 were elevated in 381 patients (34%) and 312 patients (27%), respectively. Individually, elevation of CEA, CA19-9, or CA125 were associated with worse 5-year survival; elevated vs normal was 81% vs 95% for CEA (hazard ratio [HR], 4.0; 95% CI, 2.9-5.6), 84% vs 92% for CA19-9 (HR, 2.2; 95% CI, 1.4-3.4), and 69% vs 93% for CA125 (HR, 4.6; 95% CI, 2.7-7.8) (P < .001 for all). Quantitative evaluation of tumor markers was associated with outcomes. Patients with highly elevated (top 10th percentile) CEA, CA19-9, or CA125 had markedly worse survival, with 5-year survival rates of 59% for CEA (HR, 9.8; 95% CI, 5.3-18.0), 64% for CA19-9 (HR, 6.0; 95% CI, 3.0-11.7), and 57% for CA125 (HR, 7.6; 95% CI, 3.5-16.5) (P < .001 for all). Although metastatic tumors had higher levels of all tumor markers, when restricting survival analysis to 1080 patients with metastatic disease, elevated CEA, CA19-9, or CA125 were all still associated worse survival (HR for CEA, 3.4; 95% CI, 2.5-4.8; P < .001; HR for CA19-9, 1.8; 95% CI, 1.2-2.7; P = .002; and HR for CA125, 3.9; 95% CI, 2.4-6.4; P < .001). Interestingly, tumor grade was not associated with CEA or CA19-9 level, while CA-125 was slightly higher in high-grade tumors relative to low-grade tumors (mean value, 18.3 vs 15.0; difference, 3.3; 95% CI, 0.9-3.7; P < .001). Multivariable analysis identified an incremental increase in the risk of death with an increase in the number of elevated tumor markers, with an 11-fold increased risk of death in patients with all 3 tumor markers elevated relative to those with none elevated. Somatic mutations in KRAS and GNAS were associated with significantly higher levels of CEA and CA19-9.
CONCLUSIONS AND RELEVANCE
In this retrospective study of serum tumor markers in patients with appendiceal adenocarcinoma, CEA, CA19-9, and CA125 were associated with overall survival in appendiceal adenocarcinoma. Given their value, all 3 biomarkers should be included in the initial workup of patients with a diagnosis of appendiceal adenocarcinoma.
Topics: Humans; Middle Aged; Aged; Aged, 80 and over; Biomarkers, Tumor; Retrospective Studies; CA-19-9 Antigen; Carcinoembryonic Antigen; Adenocarcinoma; Appendiceal Neoplasms; Neoplasms, Second Primary; CA-125 Antigen
PubMed: 38416491
DOI: 10.1001/jamanetworkopen.2024.0260 -
Cureus Jan 2024Appendicular mucinous neoplasms, constituting less than 1% of gastrointestinal tract neoplasms, are heterogeneous entities. They may be asymptomatic, discovered...
Appendicular mucinous neoplasms, constituting less than 1% of gastrointestinal tract neoplasms, are heterogeneous entities. They may be asymptomatic, discovered incidentally, or present as large tumors due to mucin accumulation. The lack of standardized treatment complicates management. Imaging studies, particularly CT scans, are crucial for diagnosis and follow-up. This case report presents two clinical cases of women in their sixth and seventh decades of life with a history of lower gastrointestinal bleeding, mild anemia in laboratory studies, and incomplete colonoscopies. The diagnosis, confirmed through CT scans, led to the decision for surgical intervention in both cases, involving laparoscopic right hemicolectomy with ileotransverse anastomosis. Subsequently, histopathological reports confirmed the diagnosis of high-grade appendicular mucinous neoplasms, and a follow-up plan was established with imaging studies every six months with no recurrence at two years. Over 50% of appendicular tumors are mucinous neoplasms originating from low-grade mucinous neoplasms. Given the low lymph node invasion (2%), appendectomy may suffice if the entire tumor is excised. Extensive resections or right hemicolectomy are reserved for larger tumors or high-grade neoplasms to minimize local recurrence risk. Mucinous neoplasms with acellular mucin and peritoneal invasion may require cytoreduction or right hemicolectomy, while those with mucinous epithelium may need hyperthermic intraperitoneal chemotherapy (HIPEC) due to the risk of local recurrence, worsened by the presence of extra appendiceal epithelial cells. Disease-free and overall survival depend on treatment and initial lesion characterization. A five-year survival rate of 86% is reported for low-grade mucinous neoplasms. Follow-up approaches lack an ideal standard, generally involving physical examinations and imaging studies every six months to one year during the first six years.
PubMed: 38406052
DOI: 10.7759/cureus.52908 -
Indian Journal of Pathology &... Feb 2024Mucinous carcinomas arising within an ovarian dermoid tumor are rare. Most of the cases reported in the literature show morphological features resembling an appendiceal...
Mucinous carcinomas arising within an ovarian dermoid tumor are rare. Most of the cases reported in the literature show morphological features resembling an appendiceal mucinous neoplasm. They exhibit CK7-/CK20+ immunophenotype similar to carcinomas of the lower gastrointestinal tract. In this report, we have described a case of a well-differentiated mucinous carcinoma arising within a mature cystic teratoma. The mucinous carcinoma showed a spectrum of morphological patterns, including cystadenoma-like areas, proliferative/borderline areas, villous adenoma-like areas, and areas of invasive carcinoma. In addition, our case showed some unusual findings, namely, diffuse CK7 positivity, associated pseudomyxoma peritonei, and metastasis to lungs. These features were not demonstrated in any of the previously reported cases. Our case shows that the teratomatous mucinous neoplasm of the ovary may show CK7+/CK20+/CDX2 + immunoprofile making it immunohistochemically indistinguishable from a primary ovarian mucinous neoplasm or a metastatic mucinous carcinoma of the lower gastrointestinal tract.
PubMed: 38391380
DOI: 10.4103/ijpm.ijpm_1255_21 -
Asian Journal of Surgery May 2024
Topics: Humans; Appendiceal Neoplasms; Adenocarcinoma, Mucinous; Male; Appendectomy; Female; Middle Aged
PubMed: 38378407
DOI: 10.1016/j.asjsur.2024.01.160 -
Internal Medicine (Tokyo, Japan) Feb 2024A 71-year-old woman was found to have submucosal tumor-like lesion on colonoscopy (CS) before gastric surgery, and computed tomography (CT) showed a 12-mm structure at...
A 71-year-old woman was found to have submucosal tumor-like lesion on colonoscopy (CS) before gastric surgery, and computed tomography (CT) showed a 12-mm structure at the base of the appendix. The lesion could not be clearly detected on CT nine months later, but it had enlarged again on CT one year later; therefore, CS and endoscopic ultrasound (EUS) were performed. The lesion was determined to be cystic with viscous contents, and laparoscopic appendicectomy was performed. This is the first report of low-grade appendiceal mucinous neoplasm (LAMN) diagnosed by a histopathologic examination of a resected specimen showing shrinkage and re-expansion of the appendix.
PubMed: 38369352
DOI: 10.2169/internalmedicine.3136-23 -
Annals of Surgical Oncology May 2024Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic...
BACKGROUND
Ovarian pseudomyxoma peritonei (OPMP) are rare, without well-defined therapeutic guidelines. We aimed to evaluate cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) to treat OPMP.
METHODS
Patients from the French National Network for Rare Peritoneal Tumors (RENAPE) database with proven OPMP treated by CRS/HIPEC and with histologically normal appendix and digestive endoscopy were retrospectively included. Clinical and follow-up data were collected. Histopathological and immunohistochemical features were reviewed.
RESULTS
Fifteen patients with a median age of 56 years were included. The median Peritoneal Cancer Index was 16. Following CRS, the completeness of cytoreduction (CC) score was CC-0 for 9/15 (60%) patients, CC-1 for 5/15 (33.3%) patients, and CC-2 for 1/15 (6.7%) patients. The median tumor size was 22.5 cm. After pathological review and immunohistochemical studies, tumors were classified as Group 1 (mucinous ovarian epithelial neoplasms) in 3/15 (20%) patients; Group 2 (mucinous neoplasm in ovarian teratoma) in 4/15 (26.7%) patients; Group 3 (mucinous neoplasm probably arising in ovarian teratoma) in 5/15 (33.3%) patients; and Group 4 (non-specific group) in 3/15 (20%) patients. Peritoneal lesions were OPMP pM1a/acellular, pM1b/grade 1 (hypocellular) and pM1b/grade 3 (signet-ring cells) in 13/15 (86.7%), 1/15 (6.7%) and 1/15 (6.7%) patients, respectively. Disease-free survival analysis showed a difference (p = 0.0463) between OPMP with teratoma/likely-teratoma origin (groups 2 and 3; 100% at 1, 5, and 10 years), and other groups (groups 1 and 4; 100%, 66.6%, and 50% at 1, 5, and 10 years, respectively).
CONCLUSION
These results suggested that a primary therapeutic strategy using complete CRS/HIPEC for patients with OPMP led to favorable long-term outcomes.
Topics: Female; Humans; Middle Aged; Pseudomyxoma Peritonei; Hyperthermic Intraperitoneal Chemotherapy; Cytoreduction Surgical Procedures; Retrospective Studies; Hyperthermia, Induced; Neoplasms, Cystic, Mucinous, and Serous; Teratoma; Appendiceal Neoplasms; Combined Modality Therapy; Survival Rate
PubMed: 38341381
DOI: 10.1245/s10434-023-14850-0 -
European Journal of Surgical Oncology :... Mar 2024Treatment of peritoneal metastasis from appendicular adenocarcinoma consists of cyto-reductive surgery (CRS) and Hyperthermic IntraPEritoneal Chemotherapy (HIPEC). In...
INTRODUCTION
Treatment of peritoneal metastasis from appendicular adenocarcinoma consists of cyto-reductive surgery (CRS) and Hyperthermic IntraPEritoneal Chemotherapy (HIPEC). In case of acute appendicular syndrome (AAS) the tumor is likely to be perforated. In that case, there is no treatment recommendation. We propose CRS and HIPEC.
MATERIALS AND METHOD
We listed 21 consecutive patients who were addressed for discovery of appendiceal adenocarcinoma. The emergency surgery was performed in a primary-care hospital. We evaluated the therapeutic algorithms, per operative decision, survival and recurrent rate.
RESULTS
Among the 21 patients, 4 patients were diagnosed as synchronous appendicular peritoneal metastasis, and underwent CRS and HIPEC. The other 17 patients with diagnosis of adenocarcinoma on anatomopathological samples, without peritoneal metastasis during appendectomy, were addressed. Between them 2 patients were denied CRS. Among the 15 operated patients, 8 patients had no peritoneal metastasis discovery during surgery, and therefore underwent prophylactic CRS and HIPEC. Peritoneal metastasis were discovered for the other 7 patients, who also underwent CRS and HIPEC. For the prophylactic group, the recurrence rate is 12,5 %, overall survival (OS) is 100 %. The rate of grade III-IV surgical complications after CRS and HIPEC was 36 % among the 19 patients who underwent surgery.
CONCLUSION
In case of appendectomy in emergency situations for perforated adenocarcinoma, half of the patients may have peritoneal metastasis. In case of non-identified peritoneal metastasis during CRS, performing a prophylactic HIPEC seems to be associated with an encouraging rate of peritoneal disease free situation at 5 years.
Topics: Humans; Hyperthermic Intraperitoneal Chemotherapy; Peritoneal Neoplasms; Combined Modality Therapy; Appendicitis; Antineoplastic Combined Chemotherapy Protocols; Hyperthermia, Induced; Appendiceal Neoplasms; Adenocarcinoma; Acute Disease; Cytoreduction Surgical Procedures; Survival Rate; Retrospective Studies
PubMed: 38340494
DOI: 10.1016/j.ejso.2024.107959 -
Annals of Medicine and Surgery (2012) Feb 2024Actinomycosis is an uncommon bacterial infection caused by bacteria that typically progresses slowly and leads to the formation of masses. Although it commonly affects...
INTRODUCTION
Actinomycosis is an uncommon bacterial infection caused by bacteria that typically progresses slowly and leads to the formation of masses. Although it commonly affects the cervicofacial area, about 20% of cases occur in the abdominopelvic region. Because the disease can be mistaken for a tumour due to its infiltrative mass-like nature on imaging, over 90% of cases are only diagnosed following surgery and histological confirmation. This report describes a case of an appendicular mass, initially suspected to be a malignant tumour, but eventually diagnosed as appendiceal actinomycosis.
PRESENTATION OF CASE
Upon initial presentation, a 53-year-old woman with type II diabetes mellitus and no prior surgical history, displayed abnormal appendiceal uptake during a PET-computed tomography (CT) scan conducted for a suspected spinal tumour. Colonoscopy did not indicate any notable observations, and the patient chose to defer immediate action. Several months later, a CT scan revealed an increased mass-like appearance of the appendix compared to the previous PET-CT scan. After multidisciplinary discussions, a right laparoscopic hemicolectomy was recommended due to suspected malignancy. However, histological staining on microscopy confirmed actinomycosis originating from the appendix.
DISCUSSION
Chronic appendicitis with radiologic features similar to appendiceal carcinoma, or abdominal masses located in the ileocecal area, in patients with or without a previous surgical history should raise suspicion of actinomycosis.
CONCLUSION
Appendiceal actinomycosis should be considered in the differential diagnosis in the aetiology of chronic appendicitis mimicking appendiceal carcinoma. Awareness and accurate diagnosis of appendiceal actinomycosis can prevent unnecessary extended surgery as was performed in this case.
PubMed: 38333266
DOI: 10.1097/MS9.0000000000001564 -
World Journal of Clinical Cases Jan 2024Low-grade appendiceal neoplasms (LAMN) are characterized by low incidence and atypical clinical presentations, often leading to misdiagnosis as acute or chronic...
BACKGROUND
Low-grade appendiceal neoplasms (LAMN) are characterized by low incidence and atypical clinical presentations, often leading to misdiagnosis as acute or chronic appendicitis before surgery. The primary diagnostic tool for LAMN is abdominal computed tomography (CT) imaging. Surgical resection remains the cornerstone of LAMN management, necessitating en bloc tumor excision to minimize the risk of iatrogenic rupture. Laparoscopy, known for its minimal invasiveness, reduced postoperative discomfort, and expedited recovery, is a safe and reliable approach for LAMN treatment. Despite the possibility of pseudomyxoma peritonei development, appendectomy and partial appendectomy generally result in negative tumor margins and favorable outcomes, which can be attributed to the disease's slow growth and lower malignancy.
CASE SUMMARY
A 71-year-old male patient was admitted to our hospital with a pelvic space-occupying lesion detected 1 mo prior. Physical examination showed a soft abdomen without tenderness or rebound and no palpable masses. No shifting dullness was noted, and digital rectal examination revealed no palpable mass. Enteroscopy revealed a raised, smooth-surfaced mass measuring 3.0 cm in the cecum. Abdominal contrast-enhanced CT showed a markedly thickened and dilated appendix with visible cystic shadows. Laparoscopic surgery was performed and revealed a significantly dilated appendix, leading to laparoscopic resection of the appendix and part of the cecum. Post-surgical pathologic analysis confirmed LAMN. The patient received symptomatic and supportive post-operative care and was discharged on postoperative day 4 without complications such as abdominal bleeding, intestinal obstruction, or incision infection. No tumor recurrence was observed during a 7-mo follow-up period.
CONCLUSION
LAMN is a rare disease that lacks specific clinical manifestations. Abdominal CT plays a crucial role in diagnosing LAMN, and laparoscopic surgery is a safe and effective diagnostic and therapeutic approach.
PubMed: 38313642
DOI: 10.12998/wjcc.v12.i2.361