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Pediatric and Developmental Pathology :... 2023Pediatric neuroendocrine tumors (NET) of the GI tract are rare and appendiceal NET are typically incidental. Few studies have been done in the pediatric population and...
BACKGROUND AND AIMS
Pediatric neuroendocrine tumors (NET) of the GI tract are rare and appendiceal NET are typically incidental. Few studies have been done in the pediatric population and practice guidelines are mainly based on adult data. There are currently no diagnostic studies specific for NET. Our study aimed to identify clinical, radiological, and pathological findings in pediatric appendiceal NET, test criteria for follow up surgical treatment, review potential prognostic pathological findings, and possible pre-operative diagnostic radiological studies.
MATERIALS AND METHODS
A retrospective data search was conducted for well-differentiated NET of the appendix in patients ≤21 years between 1/1/2003 and 7/1/2022. Available clinical, radiologic, pathological, and follow-up information was recorded.
RESULTS
Thirty-seven patients with appendiceal NET were identified. No masses were reported in the patients who underwent presurgical imaging. Appendectomy samples showed NET (0.2->4 cm), most located in the tip. Most cases were WHO G1 (34/37), with negative margins (n = 25). Sixteen cases extended to the subserosa/mesoappendix (pT3). Lymphovascular (6), perineural (2), and both lymphovascular and perineural invasion were also noted (2). The specified tumor stages were pT1 (10/37), pT3 (16/37), and pT4 (4/37). Patients who underwent laboratory testing for chromogranin A (20) and urine 5HIAA (11) had normal limits. Subsequent surgical resection was recommended in 13 cases and performed in 11. To date, all patients have no recurrent or additional metastatic disease.
CONCLUSIONS
Our study showed that all pediatric well-differentiated appendiceal NET were incidentally found as part of acute appendicitis management. Most NET were localized with low-grade histology. Our small cohort support the previously suggested management guidelines with follow up resection in certain cases. Our radiologic review didn't identify a best modality for NET. Comparing cases with and without metastatic disease, no tumors under 1 cm had metastasis, but serosal and perineural invasion along with G2 status were associated with metastasis in our limited study.
Topics: Adult; Humans; Child; Adolescent; Appendix; Neuroendocrine Tumors; Appendiceal Neoplasms; Retrospective Studies
PubMed: 37334832
DOI: 10.1177/10935266221146001 -
Cureus Apr 2023The origin of endometriosis has multiple theories, with controversy over which may demonstrate the prominent pathophysiology. The most common extra-pelvic organ system...
The origin of endometriosis has multiple theories, with controversy over which may demonstrate the prominent pathophysiology. The most common extra-pelvic organ system affected by endometriosis is the gastrointestinal tract. Gastrointestinal endometriosis (GE) accounts for 3 to 37% of all endometriosis cases, and appendiceal endometriosis is present in around 3% of GE cases, therefore constituting less than 1% of all endometriosis cases. In this report, we present a 24-year-old female with a past medical history significant for endometriosis status post two excisional laparoscopies who presented with eight months' duration of right lower quadrant pain, constant and stabbing, with rebound tenderness. Appendectomy and histopathology demonstrated focal endometriosis, diffuse serosal fibrovascular adhesions involving the appendiceal serosa/subserosa, as well as a dilated lumen filled with hemorrhagic content. When the appendix is not considered in endometriosis pathology, patients are at increased risk for unresolved pain and further laparoscopic procedures. Prophylactic appendectomy appears to be a worthwhile consideration in patients with chronic pelvic pain, given the high frequency of appendiceal pathology.
PubMed: 37214070
DOI: 10.7759/cureus.37825 -
International Journal of Surgery Case... Jun 2023Jejunal diverticulum is a rare condition that affects less than 0.5 % of population. Pneumatosis is also a rare disorder marked by gas in the intestinal wall's...
INTRODUCTION AND IMPORTANCE
Jejunal diverticulum is a rare condition that affects less than 0.5 % of population. Pneumatosis is also a rare disorder marked by gas in the intestinal wall's submucosa and subserosa. Both the conditions are rare cause of pneumoperitoneum.
PRESENTATION OF CASE
A case of 64 years female presented with acute abdomen and upon investigation found to have pneumoperitoneum. Exploratory laparotomy was done and intraoperatively there was multiple jejunal diverticula and pneumatosis intestinalis in separate segments of bowel and closure was done without any resection of bowel segments.
CLINICAL DISCUSSION
Small bowel diverticulosis was considered to be an incidental anomaly; however, it is now thought to be acquired. Pneumoperitoneum is a common complication of diverticula perforation. The occurrence of pneumatosis cystoides intestinalis or subserosal dissection of air around the colon or adjacent structures has been linked to pneumoperitoneum. Complications should be managed accordingly however, occurrence of short bowel syndrome should be considered before doing resection anastomosis of involved segment.
CONCLUSION
Jejunal diverticula and pneumatosis intestinalis both are rare cause of pneumoperitoneum. Combination of both the condition giving rise to pneumoperitoneum is extremely rare. These conditions can give rise to diagnostic dilemma in clinical practice. One should always think these as differentials when patient with pneumoperitoneum are encountered.
PubMed: 37196478
DOI: 10.1016/j.ijscr.2023.108320 -
The American Journal of Surgical... Jul 2023For patients with locally advanced rectal cancer (LARC) with pathological complete response (pCR), the clinical significance of the distribution extent of acellular...
The Deepest Extent of Acellular Mucin Pools in Resected Locally Advanced Rectal Cancer With Pathological Complete Response After Preoperative Chemoradiotherapy: A Hidden Killer?
For patients with locally advanced rectal cancer (LARC) with pathological complete response (pCR), the clinical significance of the distribution extent of acellular mucin pools (AMP) distribution remains unclear, so this study was conducted to address key unanswered questions. We performed a retrospective analysis of 317 patients with LARC with pCR after preoperative chemoradiotherapy and total mesorectal resection from January 2011 to June 2020. Based on AMP existence and the deepest tissue layer of distribution, patients were assigned new stages. The patient information was recorded, and the main outcome measures included 5-year disease-free survival (DFS) and 5-year overall survival (OS). A total of 83/317 (26.2%) patients exhibited AMP, and disease recurrence occurred in 46/317 (14.5%) patients. Over the 5-year median follow-up period, the patients with AMP showed 5-year DFS rates (75.9% vs. 88.9%, P =0.004) and 5-year OS rates (85.5% vs. 95.7%, P =0.002) statistically lower than those of patients without AMP. Disease recurrence was seen in 15/54 (27.8%) patients with AMP within the subserosa and/or the serosa, or adipose tissue. Univariate and multivariate analysis showed that the existence of AMP within the subserosa and/or the serosa, or adipose tissue was an independent risk factor for DFS [hazard ratio (HR): 2.344; 95% confidence interval (CI): 1.256-4.376; P =0.007] and OS [HR: 3.374; 95% CI: 1.438-7.917; P =0.005]. The new stages based on the deepest extent of AMP were related to worse DFS ( P =0.004) and OS ( P =0.003) rates among patients with pCR. In conclusion, the presence of AMP might reduce the prognosis of LARC patients with pCR after chemoradiotherapy, especially in patients with AMP in deeper tissue layers. Therefore, the influence of the deepest AMP extent might be worth considering in staging. Moreover, the revised staging of patients with pCR according to the deepest extent of AMP, which is unrelated to the clinical T stage, might facilitate postoperative management.
Topics: Humans; Retrospective Studies; Neoadjuvant Therapy; Neoplasm Recurrence, Local; Chemoradiotherapy; Rectal Neoplasms; Prognosis; Disease-Free Survival; Mucins; Neoplasm Staging; Treatment Outcome
PubMed: 37194966
DOI: 10.1097/PAS.0000000000002055 -
Diagnostics (Basel, Switzerland) Apr 2023Pneumatosis cystoid intestinalis (PCI) is a rare condition, with a worldwide incidence of 0.3-1.2%. PCI is classified into primary (idiopathic) and secondary forms, with...
Pneumatosis cystoid intestinalis (PCI) is a rare condition, with a worldwide incidence of 0.3-1.2%. PCI is classified into primary (idiopathic) and secondary forms, with 15% and 85% of presentations, respectively. This pathology was associated with a wide variety of underlining etiologies to explain the abnormal accumulation of gas within the submucosa (69.9%), subserosa (25.5%), or both layers (4.6%). Many patients endure misdiagnosis, mistreatment, or even inadequate surgical exploration. In this case, a patient presented acute diverticulitis, after treatment, a control colonoscopy was performed that found multiple rounds and elevated lesions. To further study the subepithelial lesion (SEL), a colorectal endoscopic ultrasound (EUS) was performed with an overtube in the same procedure. For safe insertion of the curvilinear array EUS, an overtube with colonoscopy was positioned through the sigmoid as described by Cheng et al. The EUS evaluation evidenced air reverberation in the submucosal layer. The pathological analysis was consistent with PCI's diagnosis. The diagnosis of PCI is usually made by colonoscopy (51.9%), surgery (40.6%), and radiological findings (10.9%). Although the diagnosis can be made by radiological studies, a colorectal EUS and colonoscopy can be made in the same section without radiation and with high precision. As it is a rare disease, there are not enough studies to define the best approach, although colorectal EUS should be preferred for a reliable diagnosis.
PubMed: 37189527
DOI: 10.3390/diagnostics13081424 -
Human Pathology Jul 2023Intracholecystic papillary neoplasm (ICPN) is a non-invasive epithelial tumor that presents as a grossly identifiable mass arising in the mucosa and protruding into the...
Intracholecystic papillary neoplasm (ICPN) is a non-invasive epithelial tumor that presents as a grossly identifiable mass arising in the mucosa and protruding into the lumen. ICPN is associated with invasive carcinoma. There are few studies on the clinicopathological features of ICPN, including that with invasive carcinoma. We evaluated the clinicopathological characteristics of 42 ICPNs and 41 conventional gallbladder adenocarcinomas (cGBAs). Subserosa or deeper (≥ss) invasion was significantly lower in ICPN (61.9%) than that in cGBA (90.2%) (P = 0.004). Cox regression analysis revealed that lymph node metastasis (hazard ratio [HR] [95% confidence interval (CI)]: 2.610 [1.131, 6.024], P = 0.025) and positive margin (HR [95% CI]: 5.143 [2.113, 12.516], P < 0.001), but not ≥ss invasion (HR [95% CI]: 1.541 [0.479, 4.959], P = 0.469), were independent prognostic factors. In addition, there was a significant interaction between histological type and lymph node metastasis (HR [95% CI]: 0.191 [0.042, 0.983], P = 0.033). In cGBA, the presence or absence of lymph node metastasis did not affect prognosis; however, ICPN without lymph node metastasis had better prognosis. Therefore, the histological classification of ICPN and cGBA and the pathological evaluation of lymph node metastasis in ICPN are crucial for determining prognosis.
Topics: Humans; Gallbladder; Lymphatic Metastasis; Gallbladder Neoplasms; Carcinoma; Prognosis; Lymph Nodes; Neoplasm Staging; Retrospective Studies
PubMed: 37127081
DOI: 10.1016/j.humpath.2023.04.013 -
International Journal of Surgery Case... May 2023Appendiceal goblet cell adenocarcinoma is in 0.3-0.9 % of appendectomy specimens. There is still controversy regarding whether surgery with dissection or additional...
INTRODUCTION AND IMPORTANCE
Appendiceal goblet cell adenocarcinoma is in 0.3-0.9 % of appendectomy specimens. There is still controversy regarding whether surgery with dissection or additional resection is necessary for goblet cell adenocarcinoma and whether adjuvant chemotherapy is practical. We present three cases of goblet cell adenocarcinomas.
CASE PRESENTATION
Case 1: A 30-year-old woman was diagnosed with appendicitis and underwent appendicectomy. Histopathological evaluation revealed a malignant neoplasm with goblet-like cells and tumour infiltration into the subserosa. The patient underwent laparoscopic ileocecal resection, and the main lymph nodes at the root of the feeding vessels were removed. Case 2: A 50-year-old man was diagnosed with appendicitis and underwent appendicectomy. Histopathological evaluation revealed a malignant neoplasm with goblet-like cells; malignant cells were found at the surgical resection margins. The patient underwent laparoscopic ileocolic resection. Case 3: A 60-year-old man undergoing treatment for malignant melanoma. He was diagnosed with appendicitis associated with an appendiceal tumour, and emergency laparoscopic caecal resection was performed and diagnosed as goblet cell adenocarcinoma. We decided to prioritize treatment for malignant melanoma, and the patient is under follow-up for goblet cell adenocarcinoma and no metastasis was detected.
CLINICAL DISCUSSION
We performed additional resection in two case of goblet cell adenocarcinoma. Diagnosing appendiceal goblet cell adenocarcinoma is difficult, and the prognosis of patients with positive lymph nodes is poor. Surgical treatment should be considered for the advanced stages of this disease.
CONCLUSION
Goblet cell adenocarcinoma, diagnosed after appendectomy, additional resection including lymph node dissection may provide a long-term prognosis.
PubMed: 37084554
DOI: 10.1016/j.ijscr.2023.108229 -
Case Reports in Medicine 2023Bowel perforation in a pregnant patient with COVID-19 infection is a rarely reported complication. With the uncovering of the different treatment modalities and...
BACKGROUND
Bowel perforation in a pregnant patient with COVID-19 infection is a rarely reported complication. With the uncovering of the different treatment modalities and complications of this disease, this case highlights diagnostic and therapeutic dilemmas and limitations in a special population. . A 35-year-old female, Gravida 2 Para 1 at 31 weeks age of gestation (AOG) who underwent cesarean section for nonreassuring fetal status in 2019, was admitted for severe COVID-19 infection presenting with dyspnea. During her hospitalization, she developed progressive dyspnea from worsening of COVID-19 infection. Patient was eventually intubated and was given a total of two doses of tocilizumab, adequate antibiotic treatment, remdesivir, and dexamethasone. An emergency repeat cesarean section was performed due to maternal deterioration and poor variability of the fetus. She delivered to a live preterm baby girl, with an Apgar score of 1 and 7 on the 1st and 5th minute of life respectively. During the postoperative days, patient remained intubated and sedated. Increasing residuals were noted per nasogastric tube (NGT). Serial scout film of the abdomen (SFA) and medical management were performed. Upon weaning from sedation, patient complained of right lower quadrant pain. A whole abdominal computed tomography (CT) scan with contrast was requested which revealed large bowel obstruction. Patient was referred to surgery service. Upon assessment, abdomen was noted to be rigid with guarding during palpation. A scout film of the abdomen was requested and revealed extensive amount of pneumoperitoneum with scanty to absent colonic gas suggestive of bowel perforation. Patient underwent emergency exploratory laparotomy. Upon opening the peritoneum, free air was evacuated. Approximately 1.4 liters of fecaloid peritoneal fluid was suctioned and adhesive band was noted at the pelvic area. A 2 cm full thickness perforation at the cecum with 17 cm serosal tear from the cecum to ascending colon was noted. Bowel loops proximal to the perforation were dilated. Nasogastric tube was inserted for bowel decompression. Right hemicolectomy was performed. Specimen was sent for histopathology. Acute inflammatory cells infiltrating the blood vessels and possible microthrombi and beginning thrombus formation were noted in the subserosa. Patient eventually expired on the 14 hospital day from sepsis.
CONCLUSION
This case highlights a rarely reported complication of COVID-19 infection. Confluence of factors that predisposed the patient include pregnancy, COVID-19 infection, use of tocilizumab, and recent surgery. High index of suspicion is vital in the management and improvement of outcomes.
PubMed: 36937039
DOI: 10.1155/2023/7737433 -
BMC Pulmonary Medicine Mar 2023Recently, deaths due to mucormycosis in immunocompromised hosts have increased; however, the clinical and pathological features of mucormycosis are not fully understood,...
BACKGROUND
Recently, deaths due to mucormycosis in immunocompromised hosts have increased; however, the clinical and pathological features of mucormycosis are not fully understood, especially in view of the associated high mortality and rare incidence in immunocompetent patients.
CASE PRESENTATION
We have described a rare autopsy case of a 67-year-old Japanese man with chronic obstructive pulmonary disease who contracted mucormycosis. He had not been on any immunosuppressants, and his immune functions were intact. Since 3 days prior to admission to our hospital, he had experienced progressive dyspnea, productive cough, and fever. Chest computed tomography revealed pleural effusion in the left lower hemithorax and consolidation in the right lung field. Although he was administered with tazobactam-piperacillin hydrate (13.5 g/day), renal dysfunction occurred on the ninth disease day. Therefore, it was switched to cefepime (2 g/day). However, his general condition and lung-field abnormality worsened gradually. Cytological analysis of the sputum sample at admission mainly revealed sporangiophores and unicellular sporangioles, while repeated sputum culture yielded Cunninghamella species. Therefore, he was diagnosed with pulmonary mucormycosis. Liposomal amphotericin B (5 mg/kg/day) was initiated on the 28 disease day. However, chest radiography and electrocardiography detected cardiomegaly and atrial fibrillation, respectively, and he died on the 37 disease day. A postmortem examination revealed clusters of fungal hyphae within the arteries of the right pulmonary cavity wall, the subpericardial artery, intramyocardial capillary blood vessels, and the esophageal subserosa vein. Direct sequencing revealed that all fungal culture samples were positive for Cunninghamella bertholletiae.
CONCLUSIONS
Cunninghamella bertholletiae could rapidly progress from colonizing the bronchi to infecting the surrounding organs via vascular invasion even in immunocompetent patients.
Topics: Male; Humans; Aged; Mucormycosis; Autopsy; Lung Diseases, Fungal
PubMed: 36932380
DOI: 10.1186/s12890-023-02382-y