Zegarra Buitron Elizabeth, Vidal Panduro Daniel A, Morales Luna Domingo
Internal Medicine, School of Medicine, Universidad Peruana de Ciencias Aplicadas, Lima, PER.
Pathology, Hospital Nacional Hipólito Unanue, Lima, PER.
Cureus. 2022 Dec 26;14(12):e32962. doi: 10.7759/cureus.32962. eCollection 2022 Dec.
Epithelioid sarcoma is a rare entity that shows a predilection for the young and middle-aged population. There are two subtypes, i.e., the distal or conventional type, which is located in distal extremities, and the proximal type, located in proximal areas of the extremities, pelvis, perineal, and genital region. The latter is characterized by more aggressive behavior, a higher recurrence rate, and poor prognosis. Histopathological and immunohistochemical diagnoses are key to correct and timely treatment and a higher survival rate. We report a case of a 41-year-old man who presented a palpable progressive growth mass in the hypogastrium. The disease time was of nine months, and the tumor was resected, but it recurred a few months later in the same location. Computed tomography (CT) scans showed images suggestive of lung metastasis and the patient had to undergo a second surgery. He received eight cycles of chemotherapy and a subsequent CT scan control showed the progression of the disease, so a new chemotherapy regimen was established. The patient received three cycles of chemotherapy without improvement, so he decided to discontinue treatment. His last outpatient medical consultation was in January 2022. A systematic review of the studies published in PubMed and Google Scholar was performed. We identified 291 articles, but only 41 reports and case series were included, with a total of 55 patients. It is important to include this type of tumor in the differential diagnosis of epithelial tumors due to its aggressive behavior. Correct and timely diagnosis is crucial to obtain lower recurrence rates, lower mortality, and higher survival rates in these patients.
上皮样肉瘤是一种罕见的疾病，好发于中青年人群。它有两种亚型，即远端型或传统型，位于远端肢体；以及近端型，位于肢体近端、骨盆、会阴和生殖区域。后者的特点是行为更具侵袭性、复发率更高且预后较差。组织病理学和免疫组化诊断是正确及时治疗以及提高生存率的关键。我们报告一例41岁男性病例，其下腹部出现可触及的进行性生长肿块。病程为9个月，肿瘤被切除，但几个月后在同一部位复发。计算机断层扫描（CT）显示有肺转移迹象，患者不得不接受二次手术。他接受了8个周期的化疗，随后的CT扫描复查显示疾病进展，因此制定了新的化疗方案。患者接受了3个周期的化疗但无改善，于是决定停止治疗。他最后一次门诊就诊是在2022年1月。我们对发表在PubMed和谷歌学术上的研究进行了系统综述。我们识别出291篇文章，但仅纳入了41篇报告和病例系列，共55例患者。鉴于其侵袭性行为，在鉴别诊断上皮性肿瘤时纳入这类肿瘤很重要。正确及时的诊断对于降低这些患者的复发率、死亡率并提高生存率至关重要。
