Mast Cell Sarcoma of Small Intestine, Early Diagnosis, and Good Prognosis: An Extremely Rare Case Report and Review of the Literature.

Gastrointestinal mast cell sarcoma is a rare variant of mastocytosis. It is a unifocal tumor with high destructive capacity and metastatic potential. Diagnosis of mast cell sarcoma can be challenging and might be so delayed that unfavorable prognosis may be expected. In this case report, we will describe our experience with a case of mast cell sarcoma in the small intestine of an elderly woman, which was diagnosed early on throughout the course of her disease and successfully treated. The patient was a 59-year-old woman who presented with abdominal pain, flushing, weight loss, and vomiting. Imaging studies supported the existence of an infiltrative neoplasm in the jejunum. Then, surgical removal of the tumor was performed. The presence of mast cells in the resected tumor was confirmed by immunohistochemistry, histopathology, and Giemsa staining. After almost a year of follow-up, the patient's overall condition was fine, and no signs of recurrence were found. This is the first reported case of successfully treated gastrointestinal mast cell sarcoma. All of the previously reported cases had been diagnosed after recurrence with no response to treatment. Our case shows the significance of early diagnosis and treatment in this condition and its impact on outcome and prognosis. That could be achieved only if the pathologist has a high suspicion for this rare disease and keeps it in the back of one's mind.

Diagnostic Value of Epithelial Cell Adhesion Molecule Flow Cytometry for Diagnosis of Metastatic Carcinoma in Serous Effusions.

INTRODUCTION: Malignant serous effusions are common in metastatic carcinomas. Although cytomorphology is recognized as the gold standard diagnostic method, it exhibits moderate sensitivity. This study aimed to assess the diagnostic value of immunophenotyping with a single epithelial marker, known as the epithelial cell adhesion molecule (EpCAM, CD326), in discriminating malignant metastatic carcinomas of serous fluids. METHODS: This prospective study was conducted on suspicious or confirmed cases of malignant tumors from September 16, 2019, to June 21, 2020. Serous fluid samples were assessed via cytomorphology using the Wright-Papanicolaou method and the anti-EpCAM mouse monoclonal antibody (clone VU-1D9) flow cytometry. The EpCAM(+)/CD45(-) immunophenotype was defined as the metastatic involvement of carcinoma in the serous cavity. RESULTS: A total of 118 samples (90 females and 28 males; mean age, 54.04 ± 16.14 years), collected from peritoneal and pleural fluids, were examined in this study. Five samples (4.24%) were positive in both EpCAM flow cytometry and cytology, while 102 samples (86.44%) were negative for both EpCAM flow cytometry and cytology, yielding an overall agreement of 92%, 84%, and 90.7% for the peritoneal, pleural, and total samples, respectively. Based on the Bayesian latent class model, the EpCAM flow cytometry showed sensitivity and specificity of 58.5% (95% CI: 0.3, 99.7) and 96.2% (95% CI: 47.8, 100), respectively. The corresponding values were 68.7% (95% CI: 0.3, 99.9) and 96.1% (95% CI: 47, 100) for cytology, respectively. CONCLUSION: The EpCAM flow cytometry and cytology showed comparable performance in detecting metastatic effusions. The EpCAM flow cytometry might have a diagnostic value in decreasing the false-negative rate of cytomorphology, while maintaining excellent specificity.

Comparative study of pulmonary circulation and myocardial function in patients with ß-thalassemia intermedia with and without hydroxyurea, a case-control study.

OBJECTIVES: To compare myocardial systolic and diastolic functions and pulmonary circulation by two-dimensional, M-mode, Doppler, and pulsed tissue Doppler imaging in patients with ß-thalassemia intermedia who received hydroxyurea at least for 1 yr, patients who did not receive hydroxyurea, and a healthy control group. METHODS: We assigned 84 patients with thalassemia intermedia into two groups: one of two was treated with hydroxyurea for at least 1 yr and no hydroxyurea. M-mode echocardiographic, Doppler, and pulsed Doppler tissue images were compared in these two groups and in 20 control participants who did not have thalassemia. For the estimation of pulmonary hypertension, pulmonary acceleration time below 100 ms was considered as an index of pulmonary hypertension. RESULTS: There were no significant differences in M-mode echocardiographic findings between three groups. Doppler echocardiography detected no significant differences between the treated and untreated groups. In treated hydroxyurea group, 11.7% patients had pulmonary acceleration time below 100 ms and in untreated group 21% (LR = 1.45, P = 0.22). Pulsed Doppler tissue imaging parameters did not differ significantly between treated and untreated patients (P > 0.05). Pulsed Doppler tissue images showed significant changes between patients and controls in peak systolic velocity of the septum, peak atrial velocity of the septum (Aas), the peak systolic velocity of the tricuspid valve (St), the peak early diastolic velocity of the tricuspid valve (Eat), and the peak late diastolic velocity of the tricuspid valve (Aat). CONCLUSION: This study revealed that in spite of decrease in estimated pulmonary hypertension in treated group, HU has no statistically significant effect on pulmonary acceleration time and M-mode, Doppler, and tissue Doppler changes of patients with TI.