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小儿盲肠未分化多形性肉瘤

Pediatric Undifferentiated Pleomorphic Sarcoma of the Cecum.

作者信息

McCombs Desiree, Condon Kathleen, Roybal Jessica, Warrier Rajasekharan, Falcon Corey

机构信息

The University of Queensland Medical School, Ochsner Clinical School, New Orleans, LA.

Department of Pediatrics, Tulane University School of Medicine, New Orleans, LA.

出版信息

Ochsner J. 2023 Spring;23(1):77-81. doi: 10.31486/toj.22.0042.

Abstract

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm typically diagnosed in older adults and localized to the extremities or retroperitoneum. Because of poor response to therapy and high rates of recurrence, this neoplasm is associated with a poor prognosis. A 12-year-old female presented with weight loss, abdominal pain, fatigue, and diarrhea. She was profoundly anemic with occult blood-positive stools. On endoscopy, a fungating cecal mass was biopsied and diagnosed as malignant sarcomatoid neoplasm. The neoplasm was resected with clear margins during subsequent surgery, and on final pathology was diagnosed as UPS. A suspicious lung nodule was also removed via video-assisted thoracoscopic surgery and found to be a granuloma positive for for which the patient received antifungal therapy. The patient did not receive additional chemotherapy or radiotherapy and was doing well without signs of recurrence at 12 months postresection. This report of cecal UPS in a 12-year-old is rare because of the patient's age and tumor location. We have identified only 2 other case reports of pediatric gastrointestinal UPS. This case illustrates the need for a broad differential and prompt workup in pediatric patients presenting with weight loss and abdominal complaints. More information regarding the management and outcomes in cases of gastrointestinal UPS is needed to assist providers in determining the best treatment course and to allow for better prognostication.

摘要

未分化多形性肉瘤(UPS)是一种高级别肿瘤,通常在老年人中诊断出来,且多局限于四肢或腹膜后。由于对治疗反应不佳且复发率高,这种肿瘤的预后较差。一名12岁女性出现体重减轻、腹痛、疲劳和腹泻症状。她严重贫血,大便潜血阳性。在内镜检查中,对一个呈蕈状的盲肠肿物进行活检,诊断为恶性肉瘤样肿瘤。在随后的手术中,该肿瘤被切除,切缘清晰,最终病理诊断为UPS。一个可疑的肺结节也通过电视辅助胸腔镜手术切除,发现是一个肉芽肿,患者接受了抗真菌治疗。患者未接受额外的化疗或放疗,术后12个月情况良好,无复发迹象。这份关于一名12岁儿童盲肠UPS的报告很罕见,原因在于患者的年龄和肿瘤位置。我们仅发现另外2例儿童胃肠道UPS的病例报告。该病例表明,对于出现体重减轻和腹部不适的儿科患者,需要进行广泛的鉴别诊断和及时的检查。需要更多关于胃肠道UPS病例的管理和结果的信息,以帮助医疗人员确定最佳治疗方案并实现更好的预后评估。

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