Geramizadeh Bita, Nabavizadeh Sara, Rezvani Alireza, Shamsolvaezin Nadereh, Zahedinassab Alireza, Khodadadi Neda, Iranpour Pouya
Department of Pathology, Medical School of Shiraz University, Shiraz University of Medical Sciences, Shiraz, Iran.
Transplant Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Gastrointest Tumors. 2023 Jan 5;10(1):1-5. doi: 10.1159/000528887. eCollection 2023 Jan-Dec.
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.
胃肠道肥大细胞肉瘤是肥大细胞增多症的一种罕见变体。它是一种具有高破坏能力和转移潜能的单灶性肿瘤。肥大细胞肉瘤的诊断可能具有挑战性，且可能延迟到预期预后不佳的程度。在本病例报告中，我们将描述我们对一名老年女性小肠肥大细胞肉瘤病例的经验，该病例在疾病全过程中早期得到诊断并成功治疗。患者为一名59岁女性，出现腹痛、潮红、体重减轻和呕吐症状。影像学检查支持空肠存在浸润性肿瘤。随后，进行了肿瘤切除手术。通过免疫组织化学、组织病理学和吉姆萨染色证实切除的肿瘤中存在肥大细胞。经过近一年的随访，患者总体状况良好，未发现复发迹象。这是首例成功治疗的胃肠道肥大细胞肉瘤报告病例。所有先前报告的病例均在复发后诊断，且对治疗无反应。我们的病例显示了本病早期诊断和治疗的重要性及其对结局和预后的影响。只有当病理学家对这种罕见疾病高度怀疑并牢记于心时，才能实现这一点。
