Primary esophageal diffuse large B-cell lymphoma

Autor: Paloma, Álvarez Martínez, Pedro José, Ramos Martínez, Patricia, García González, Alejandro, Nieto-Jara, Celia, Del Caño Cerdán, Emilia, García Riesco
Rok vydání: 2022
Předmět:
Zdroj: Revista Española de Enfermedades Digestivas.
ISSN: 1130-0108
Popis: A 76-year-old man presented with dysphagia, epigastric pain and weight loss for the last two months. Heavy sweating was also presented. Past medical conditions included type 2 diabetes. He had no evidence of any immunosupressive disease including HIV infection. Physical examination only revealed low-grade fever. Laboratory data showed leukocytosis. Gastroscopy evidenced a complete esophageal stenosis starting at 30 cm, with a severely friable mucosa of malignant appearance. The results of biopsies were insufficient for diagnosis of malignancy. Computed tomography demonstrated a 10-cm irregular tumor located in the distal and middle thirds of the esophagus, which resulted in narrowing of the lumen. Involving tracheal carina, bronchus, and descending aorta were observed. Perforation signs were also seen. Distant metastases were not found. Empirical treatment with piperacillin/tazobactan was started. A surgical gastrostomy to allows nutritional support was performed. Two other gastroscopies were performed resulting in an inconclusive diagnosis. Finally, flow cytometry performed in samples obtained by endobronchial ultrasound-guided biopsy evidenced prominent clonal B-cell populations consistent with extranodal diffuse large B-cell lymphoma exhibing CD10 expression. A treatment with Rituximab in combination with cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) was started. DISCUSSION Primary esophageal diffuse large B-cell lymphoma (DLBCL), a variant of non-Hodgkin's lymphoma, accounts for less than 1% of all cases of gastrointestinal lymphomas (1). Primary esophageal DLBCL may pose a diagnostic dilema due to the marked heterogeneity and nonspecificity observed in diagnostic investigations (2). Thus, radiological and endoscopic findings are often unconclusive. Therefore, repeated endoscopic biopsies are frequently required (3), as in the present case. In our patient, we firstly performed endopscopic biopsy, but, endoscopic and bite-on-bite biopsy in submucosal infiltrating tumors have a very low diagnostic yield. With the clinical application of ultrasound-guided biopsy, great progress has been made in the diagnostic accuracy and in the evaluation of the depth of invasion in gastrointestinal tumors (2, 4). Physicians should be vigilant for this tumour in individuals presenting with consistent clinical features, as the response to prompt chemotherapy with or whitout radiotherapy is considerably good (5).
Databáze: OpenAIRE