Clinical Case Summary

Background: Mucinous cystic neoplasms are rare benign tumours of the liver, which occur in less than 5% of all liver cysts [1]. However, their etiology is not quite clear, female dominance and estrogen receptor positivity suggest their embryological origin. Without any specific laboratory, biomarker, imaging and cytological characteristics, the diagnosis left for histology, most of the time after variously radical operations.
Aim: The aim of our presentation is to show the severity of evaluating this rare condition through a case report.
Methods: A 27-year-old woman was admitted to our department with jaundice and abdominal pain. Past medical history was irrelevant, she had obesity and smoking as risk factors. Laboratory results showed total bilirubin level of 92.6 μmol/l with elevated obstructive liver enzymes. Abdominal ultrasound revealed a dilatated common bile duct and a 60 mm large septated cystic lesion in the liver. To evaluate the indeterminate etiology, abdominal CT was performed, which described an unspecific hypodensity in the head of the pancreas, and suggested Echinococcus cyst in the liver. Serology was negative twice. Due to progressing jaundice an ERCP was performed. An unremovable mass was found in the hepatic bifurcation, plastic stents were placed. CA19-9 was moderately elevated (156,3 U/ml). For further identification cholangioscopy was performed, where a soft, regular surfaced tissue was found at the level of hepatic bifurcation. Targeted biopsies were undiagnostic. Ultrasound follow up showed slight enlargement of the cystic lesion. Fine needle sampling was postponed several times due to the suspicion of echinococcoid cyst, eventually it was done. No specific cytological diagnosis was made, microbiology identified Acinetobacter baumannii. Lack of clinical and laboratory signs of systematic inflammation, no antibiotic was added. Follow up CT described further enlargement. After almost a half year of diagnostic steps, surgical resection was left to step forward. During the laparotomy huge pathological lymph nodes were seen, therefore left hepatolobectomy, radical lymphadenectomy, and hepato-jejunostomy were performed. Macroscopically two bigger parts were recognizable, one of them grown inside the bile duct. No early complications were noted.
Results: Histological process at first sight seen ovarial-like strome. Estrogen dye was added, which shown high density for oestrogen receptors. Final diagnosis was concluded as mucinous cystic neoplasm with low grade dysplasia.
Conclusion: Mucinous cystic neoplasms are rare benign tumors of the liver. As the case above has shown, diverse and sophisticated diagnostic modalities aren’t sensitive enough to determinate and differentiate cystic liver deformations. Fortunately, no malignant focus was found this time.

References

1.Soni S, Pareek P, Narayan S, Varshney V. Mucinous cystic neoplasm of the liver (MCN-L): a rare presentation and review of the literature. Med Pharm Rep. 2021 Jul;94(3):366-371. doi: 10.15386/mpr-1543. Epub 2021 Jul 29. PMID: 34430860; PMCID: PMC8357353.

Clinical Case Summary

Background: Pancreatic Adenocarcinoma (PA) represents an aggressive disease affected by very poor prognosis worldwide. In the early phases, PA remains asymptomatic favoring delayed diagnosis and affecting the disease prognosis. Recently, Acute Pancreatitis (AP) has been described by some anecdotical cases as a potential early manifestation of PA, in particular if case of pancreatic head development.
Case presentation: An 80-year-old female patient affected by diabetes mellitus type 2 complaining of acute epigastric pain was admitted to the emergency room receiving an AP diagnosis. After 2 days she was hospitalized at Gastroenterology Department to receive medical treatment. On admission, the patient presented hyper-neutrophilia, elevated levels of pancreatic enzymes, cholestasis indexes, and phlogosis markers. Abdominal ultrasound revealed a hypoechoic nodular lesion of 40 mm located at the pancreas body, associated with ectasia of the upstream Wirsung duct. The contrast enhancement US (CEUS), evidenced a hypoenhancement pattern in the arterial phase suggesting the need for a second-level imaging examination. The abdominal CT scan revealed the lesion ventral to the splenic-portal confluence without signs of vascular infiltration, presenting a late contrast enhancement suggestive of a hetero-primitive lesion of the pancreatic body. Echo-endoscopy-guided sampling confirmed the adenocarcinoma diagnosis. After this, the patient received a distal pancreatectomy to achieve surgical radicality. No progression of disease was observed after surgery, and she is currently on stable oncological follow-up.
Conclusion: Even when the neoplasm is not located in the head of the organ, AP has herein been reported as a rare exordial manifestation of PA whose onset can help clinicians to achieve early diagnosis of this aggressive tumor.

References

1. Saluja A, Maitra A. Pancreatitis and Pancreatic Cancer. Gastroenterology. 2019 May;156(7):1937-1940. doi: 10.1053/j.gastro.2019.03.050. Epub 2019 Mar 30. PMID: 30940522.
2. Bezuidenhout AF, Wei PK, Burstein D, Brook A, Brook OR, Freedman SD, Tsai LL. Unexplained Acute Distal Pancreatitis: Association With Subsequent Diagnosis of Pancreatic Cancer. AJR Am J Roentgenol. 2023 Aug;221(2):196-205. doi: 10.2214/AJR.23.28999. Epub 2023 Mar 22. PMID: 36946899.
3. Walling A, Freelove R. Pancreatitis and Pancreatic Cancer. Prim Care. 2017 Dec;44(4):609-620. doi: 10.1016/j.pop.2017.07.004. Epub 2017 Oct 5. PMID: 29132523.

Introduction

The severity of bleeding and the time-to-endoscopy are generally recognised as risk factors for mortality in patients with GI bleeding. However, how to grade the bleeding severity (BS) is not clearly defined and it is not clear its relationship with the time-to-endoscopy. We found that the bleeding severity correlates with 5 clinical items: altered mental status, systolic blood pressure ≤100 mmHg, blood ureic nitrogen ≥130 mg/dl, haemoglobin ≤8 gr/dl and clinical presentation with hematemesis.

Aims & Methods

The aim of the study is to evaluate if the interaction between bleeding severity and timing-to-endoscopy could impact on the death risk. A prospective multicentre cohort study, including all consecutive patients with upper gastrointestinal bleeding admitted to 50 Italian hospitals was conducted. Charlson's comorbidity index and ASA scores were used to define the patients’ performance status. According to the previously cited clinical items, we defined bleeding severity (BS) and graded it in low, intermediate, and high-risk classes depending on the presence and the number of items at the time of admission. Timing to endoscopy was defined according to ESGE* guidelines and was divided into 4 time-frames (<6h; 6/12h; 12/24h; >24h).

Results

2,525 patients were included (mean age 68 [±15.8], male 67.3%); the bleeding was non-variceal in 82.3%. Overall, 176 patients died (6.9%); 28 (1.9%) in the low BS group (1,463 patients), 28 (8.6%) in the intermediate BS group (606 pts) and 96 (21%) in the high BS group (p<0.000). In our population, the 4-time frames do not impact on the mortality in the low and intermediate classes of risk. In the high BS class, there is an interaction between the time to endoscopy and the mortality, with the lower mortality observed in the 6/12 hrs frame.

Conclusion

In our population, the death risk gradually rises as the bleeding severity increases. Bleeding severity identifies 3 different classes of death risk and the time to endoscopy seems unrelated to mortality in low and intermediate BS classes. In the high BS patients, the lower mortality was observed in the timing to endoscopy 6-12 hrs.

References

* Ian M. Gralnek et al. Endoscopy 2021; 53: 300–332

Disclosure

nothing to disclose

Introduction

According to guidelines, in order to strictly diagnose esophageal motility disorders, besides achalasia, high resolution manometry (HRM) findings should be documented in both the upright and the supine position. A common finding among patients with Gastro-Esophageal Reflux Disease GERD submitted to manometry is ineffective esophageal motility (IEM). The clinical impact of such a diagnosis in this setting is ambiguous though it may denote future disturbance of peristaltic reserve even if until now only inadequate response to multiple rapid swallows (MRS) has been correlated to post anti-reflux surgery dysphagia.

Aims & Methods

We aimed to evaluate differences in esophageal contractility in different positions in patients with definite GERD.We included consecutive patients with definite GERD according to Acid Exposure Time (>6%) submitted simultaneously to 24 hours pH impedance off PPI study and HRM in both the upright and supine position. Patients with previous esophago-gastric surgeries as also patients diagnosed with motility disorders besides IEM/absent contractility according to Chicago 4 guidelines were excluded.

Results

In total 49 patients (M/F:26 (53.1%)/23 (46.9%), mean age:51±14) were included. The main referral reason was typical reflux symptoms in 37/49 (75.5%) patients while 8/49 (16.3%) reported dysphagia. Mean Distal Contractile Integral (DCI) was significantly reduced in the upright position (1629.2±1175.6 mm²/s/mmHg) vs supine position (2762.4±2710.4 mm²/s/mmHg), p=0.010. The rate of total ineffective swallows in the upright position was significantly increased in the upright position (29.0%) vs the supine (12.3%), p=0.010 as was also the rate of weak contraction (10.3% vs 3.1%, p=0.033 ) and marginally not of absent contractions (17.0% vs 8.3%, p=0.078). The rate of diagnosis of IEM/absent contractility was doubled in the upright position (8/49, 16.3%) vs in the supine position (4/49, 8.2%) (k-value:0.473, p=0.001). Inadequate post MRS contraction was observed in 9/49 (18.4%) and more specifically among 3/4 (75%) patients with IEM/absent contractility diagnosis in the supine position (k:-0.436, p=0.002) vs 4/8 (50%) in the upright position (k:-0.361, p=0.011). Dysphagia was not correlated to diagnosis of IEM/absent contractility neither in the supine (p=0.675) nor the upright position (p=0.586).

Conclusion

Upright position in HRM is correlated to a decreased mean DCI and an increased rate of ineffective esophageal contractions leading to an increased rate of diagnosis of IEM/absent contractility. Such a diagnosis in the upright position is correlated in a lesser extent to inadequate post-MRS swallow and not correlated to symptom of dysphagia. Until new data arise such an overdiagnosis among patients with GERD may be misleading and therefore the procedure in such patient may be better to be undertaken only in the supine position.

Clinical Case Summary

Introduction: Kaposi sarcoma is the most common digestive malignancy in Human Immunodeficiency Virus (HIV)-positive patients, but it can also occur in HIV-negative individuals. It is a low-grade vascular tumor associated with Human Herpes Virus-8 (HHV-8) and can appear in immunosuppressed patients. Lesions can develop on the skin, but they could also be found in internal organs and lymph nodes.
Case-report: A 61-year-old Caucasian female with alcoholic liver cirrhosis and diabetes mellitus presented in the Dermatology Department with multiple painless red-to-purple papular nodules across the thorax, cervical area, limbs, and face. Skin biopsies from the posterior cervical area and internal part of the right foot revealed a well-defined nodular dermal tumor with submucosal vascular spindle cells forming slits containing red blood cells and low mitotic activity. Immunohistochemistry confirmed CD34, CD31, HHV-8, and Ki67>30% expression, indicative of cutaneous Kaposi sarcoma. HIV serological tests were negative. Further assessments performed in the Gastroenterology Department established decompensated liver cirrhosis. Moreover, upper digestive endoscopy revealed esophageal varices, portal hypertension gastropathy, along with multiple umbilicated nodules with central ulcerations in the gastric fundus and body. Biopsy samples from these lesions described histopathological findings suggestive of Kaposi sarcoma, while immunostaining for HHV-8 and CD31 was positive. Proposed systemic chemotherapy was declined by the patient. Tumors of the gut with spindle-shaped cells such as leiomyomas, rhabdomyosarcomas, or gastrointestinal stromal tumors must be considered in the differential diagnosis of Kaposi sarcoma.
Conclusion: Kaposi sarcoma can develop in HIV-negative immunosuppressed patients, with conditions such as liver cirrhosis and HHV-8 infections, potentially affecting the digestive tract.

References

1. Xu H, Edwards JR, Espinosa O, Banerji S, Jackson DG, Athanasou NA. Expression of a lymphatic endothelial cell marker in benign and malignant vascular tumors. Hum Pathol 2004;35:857–861.
2. Rossi S, Orvieto E, Furlanetto A, Laurino L, Ninfo V, Dei Tos AP. Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. Mod Pathol 2004;17:547–552.
3. Antman K, Chang Y. Kaposi's sarcoma. N Engl J Med. 2000;342:1027–1038.
4. Huang WY, Pantanowitz L, Dezube BJ. Unusual sites of malignancies: CASE 3. AIDS-related Kaposi's sarcoma of the gastrointestinal tract. J Clin Oncol. 2005;23:2098–2099.
5. Arora M, Goldberg EM. Kaposi sarcoma involving the gastrointestinal tract. Gastroenterol Hepatol (N Y). 2010 Jul;6(7):459-62. PMID: 20827371; PMCID: PMC2933764.

Disclosure

No conflict of interest.