Published online Oct 24, 2021. doi: 10.5306/wjco.v12.i10.960
Peer-review started: April 21, 2021
First decision: July 16, 2021
Revised: July 26, 2021
Accepted: September 19, 2021
Article in press: September 19, 2021
Published online: October 24, 2021
Processing time: 183 Days and 14.1 Hours
Myeloid sarcoma (MS) is a rare hematologic malignancy defined as an extramedullary tumor of immature granulocytic cells. It can occur as primary or de novo and be associated with myelodysplasia or myeloproliferative neoplasms. The most frequent locations are the skin, lymph nodes and bones. The case of a patient with a diagnosis of primary granulocytic de novo gastric MS is reported.
A 19-year-old female patient with MS, whose abdominal computed tomography showed a bulky tumor of 16.5 cm in the gastric chamber with infiltration in the retroperitoneal, pancreatic and bile duct region; the histological study showed gastric mucosa diffusely infiltrated by mononucleated cells and the immunohistochemistry expressed myeloperoxidase. After receiving induction chemotherapy based on the 3 + 7 regimen (daunorubicin/cytarabine), the patient developed severe hematological toxicity and neutropenic typhlitis which required a prolonged medical treatment. She presented a rapid disease progression. Although she received supportive treatment, the patient died.
Gastric primary de novo MS is a rare and aggressive course neoplasm, fostering knowledge is very important to decide its management and to promote more approaches focused on understanding this pathology and its particularities in our population.
Core Tip: This case report describes a gastric primary de novo myeloid sarcoma (MS) which is a very rare hematological neoplasm with poor prognosis in a young and symptomatic patient. After receiving chemotherapy, she presented severe toxicity (neutropenic typhlitis) and rapid disease progression. This case highlights the importance of detecting gastric primary MS as a rare form of extramedullary myeloid leukemia presentation. Moreover, management of gastric primary MS could lead to interventions to avoid deterioration of gastrointestinal system during treatment. There is limited information of management and outcomes regarding gastric primary MS. Furthermore, there is very limited data about de novo MS in Peruvian patients.