Clinical Case Summary

Introduction: Kaposi's Sarcoma is a neoplasia in blood and lymphatic vessels, often linked to HHV-8, affecting 40% of HIV-positive patients. Upper gastrointestinal endoscopy reveals thickened folds with rounded nodular violaceus lesions or hemorrhagic macules, hence it represents an unusual cause of digestive bleeding in HIV patients.
Case Report: A 67-year-old male, previously healthy, was admitted to the gastroenterology department for symptoms including progressive dysphagia, weight loss of 6 kg over 2 months, and intermittent episodes of melena. Laboratory tests revealed iron deficiency anemia (Hb 11.5g/dL). Gastroscopy showed a diffuse distribution of multiple prominent whitish plaques throughout the esophagus, indicative of candidiasis (Figure 1). In the stomach, several elevated erythematous-violaceous vascular-like lesions were observed in the greater curvature of the gastric body (7-8) (likely related to KS) (Figures 2 and 3). Histological analysis confirmed the presence of KS, showing vascular spindle-shaped cells, with immunohistochemical tests positive for CD34 and HHV-8 (Figure 4) leading to a HIV testing, turning positive with a high viral load and critically low CD4+ count. The patient is now receiving antiretroviral therapy and is monitored by infectious disease specialists. KS was also found in the skin and palate but not in the lungs.
Discussion: Improved access to antiretroviral therapy has led to a reduction in the incidence of AIDS-related illnesses among individuals with HIV. However, it is imperative not to disregard the presence of certain endoscopic lesions that may indicate this diagnostic probability, such as esophageal candidiasis or gastric/duodenal vascular lesions. Gastric KS, as a cause of digestive bleeding is rare; nevertheless, it merits consideration in the differential diagnosis of HIV-positive patients.

References

Figure 1: Endoscopic image showing a diffuse distribution of multiple prominent whitish plaques throughout the entire length of the esophagus, indicative of candidiasis.

Figures 2 and 3: Endoscopic images displaying elevated erythematous-violaceous vascular-like lesions in the greater curvature of the gastric body, likely related to Kaposi's sarcoma (KS).

Figure 4: Displays histopathological examination results, showing the proliferation of vascular spindle-shaped cells consistent with KS, with positive immunohistochemistry for CD34 and HHV8.

Clinical Case Summary

Introduction: Myeloid sarcoma (MS) is a rare neoplasm composed of immature myeloid cells, which can occur de novo or in association with hematologic malignancies such as acute myeloid leukemia (AML). Gastric involvement is rare.
Case Report: A 56-year-old woman with a medical history of acute myeloid leukemia (AML) with inv(16). Following two unsuccessful induction therapy cycles, she underwent two unrelated allogeneic hematopoietic stem cell transplantations (HSCT). During the second post-HSCT period, she developed grade I gastrointestinal graft-versus-host disease (GI GVHD), confirmed by biopsy and managed with budesonide.
Five months post-HSCT, the patient was admitted due to upper gastrointestinal bleeding (melena) and worsening anemia (Hb: 5,6 g/dL). Gastroscopy revealed multiple hemorrhagic suffusions, petechial areas, nodular formations with erythematous borders, and eroded centers that bled upon contact and were not amenable to endoscopic treatment (Figure 1). Biopsies were performed to exclude GI GVHD and viral superinfection. Histological examination revealed acute myeloid leukemia (myeloid sarcoma) infiltration in the gastric mucosa (Figure 2). Six months post-HSCT, the patient succumbed to AML progression.
Discussion:
- Myeloid Sarcoma (MS) typically affects the skin, lymph nodes, and brain, making its occurrence in the gastrointestinal tract uncommon.
- Endoscopic imaging may mimic other conditions therefore biopsies are required to confirm the diagnosis.
- In hematologic patients post-HSCT, endoscopic evaluations are essential for diagnosing gastrointestinal complications such as opportunistic infections, GI graft-versus-host disease (GI GVHD), and progression of their underlying disease. Our case highlights the rarity of gastric MS.

References

Figure 1: Gastroscopic images (A and B) depicting multiple hemorrhagic suffusions, petechial areas, nodular formations with erythematous borders, and eroded centers.

Figure 2: Histological examination of gastric and oxyntic mucosa biopsies revealing lamina propria occupation by small to medium immature blast cells, consistent with acute myeloid leukemia infiltration of the gastric mucosa (previously identified).

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.

Introduction

Adenomatous polyposis syndromes comprise familial adenomatous polyposis (FAP), linked to a mutation in the APC gene, and MUTYH-associated polyposis (MAP), when there is a biallelic mutation in the MUTYH gene. These conditions result in a high risk of colorectal and duodenal cancers, with well-established recommendations for screening and preventive treatment for these locations. Recent studies also suggest an increased incidence of gastric cancer in these patients, associated with different types of dysplastic lesions in contact or at distance from the classic fundic gland polyps (FGP).

Aims & Methods

The aim of this study was to determine the prevalence, treatment, and follow-up of gastric dysplastic lesions in patients with an adenomatous polyposis syndrome.
We retrospectively analysed clinical and endoscopic data from all patients with adenomatous polyposis syndrome (FAP or MAP) who underwent follow-up esophagogastroduodenoscopy (EGD) in our center, between January 2015 and May 2022. Data was collected from a national prospective database of patients with digestive polyposis diseases created in 2011. We distinguished two types of dysplastic lesions: on the one hand, well-defined umbilicated sessile nodules of the antrum, and on the other hand, whitish flat lesions showing a "blue crest" sign in NBI, often fundic and within FGPs.

Results

A total of 227 patients were included. Thirteen (5.7%) presented a MUTYH mutation. Twenty-seven patients (11.9%) had gastric dysplasia (with 17 (63%) women and a mean age of 52 [23-68] years), located proximally in 70.4% of cases and mostly corresponding to low-grade dysplasia (18 cases, 66.7%), followed by high-grade dysplasia (8 cases, 29.6%) and 1 case of cancer (3.7%). One patient died of metastatic gastric cancer not detected by endoscopy, within a very dense fundic polyposis. None of these patients had an Helicobacter pylori (HP) infection (vs 13 (6.5%) in the control group). Whitish flat areas of the proximal stomach were observed in 27 (11.9%) patients, with confirmed (low-grade) dysplasia in 17 cases (63%). Sessile antral lesions were reported in 21 cases (9.2%), with confirmed dysplasia in 6 (29%). One of the flat fundic lesions was already invasive adenocarcinoma (pT2). Three other cases of dysplasia were revealed by systematic biopsies. The presence of fundic whitish flat lesions was strongly correlated with the presence of dysplasia (relative risk of 31 (95% CI [12.7-74.9], p <0.001)). The mean age and smoking were significantly higher in the dysplasia group (p <0.001). In multivariate analysis, smokers or patients with high-density FGPs (>50) had a significantly higher risk of gastric dysplasia (absolute risk of 13.08, 95% CI [2.67-64.10] and 22.06, 95% CI [4.28-113.58], respectively). Among the cases of dysplasia, 21 (77.8%) were treated by endoscopic resection, mostly ESD (10 cases (47.6%), for lesions with a median diameter of 62.5 [35-160] mm). One of these patients had a deeply invasive pT2 adenocarcinoma and therefore received systemic treatment. A patient with multiple and extensive adenomatous lesions was referred for gastrectomy, which he refused. The remaining patients are still under endoscopic surveillance. The median follow-up duration after treatment was 21 [0-72] months, with 2 proximal recurrences (7%, low grade dysplasia) treated endoscopically.

Conclusion

We present one of the largest gastric follow-up cohorts of patients with familial polyposis, with a non-negligible frequency of gastric dysplasia and risk of cancer. The latter is possibly preventable by endoscopic resection if the dysplasia is recognized early. This accentuates the importance of educating gastroenterologists on recognizing these sometimes very difficult to see lesions (dyschromic flat lesions).

Clinical Case Summary

Introduction: Among gastric malignancies, lymphomas rank as the second most common type, following gastric adenocarcinoma.
Primary gastric Burkitt lymphoma, is a subtype of non-Hodgkin's lymphoma, represents an exceptionally rare and aggressive malignancy, with only a limited number of reported cases globally.
A case: An 81-year-old man, smoker and chronic alcoholic, who presented to the emergency unit for epigastralgia evolving for a month with intermittent vomiting complicated on the same day of his hospitalization by the occurrence of hematemesis and melena evolving in a context of altered general condition.
Physical examination revealed pallor and epigastric tenderness.
The upper gastrointestinal endoscopy revealed an antral pre-pyloric ulcerative process, bleeding easily on contact, as well as two fundic ulcerations with raised edges.
Histological examination of the endoscopic gastric biopsy revealed diffuse infiltration of the gastric mucosa by a lymphomatous tumoral process, the immune histochemical evaluation revealed that lymphoid cells were positive for CD20, CD10, and Bcl6, negative for CD23, CD5 and Bcl2. All of the neoplastic cells were positive for Ki-67. These findings pointed toward a diagnosis of gastric Burkitt's lymphoma.
A CT scan of the abdomen showed diffuse and irregular endoluminal thickening of the antropyloric wall. Lymph nodes measuring 1 to 2 cm were present around the stomach, with no secondary location.
Considering all the clinical, radiological, and pathological findings, a final diagnosis of Burkitt's lymphoma arising from the stomach was made. The case was discussed in the multidisciplinary clinic, it was decided to go ahead with intensive systemic chemotherapy with cyclophosphamide and rituximab.

Disclosure

no conflict of interest