Shoaib Daania, Khan Saqib Raza, Rashid Yasmin Abdul, Zahir Muhammad Nauman
Aga Khan University Hospital, Karachi, Pakistan.
Dr. Ziauddin Hospital, Karachi, Pakistan.
Int J Surg Case Rep. 2022 Oct;99:107618. doi: 10.1016/j.ijscr.2022.107618. Epub 2022 Sep 9.
Myoepithelial carcinomas are a diverse group of tumors exhibiting myoepithelial differentiation. There have been increasing reports of extra-salivary sites of origin for myoepithelial carcinomas such as soft tissues, bone and visceral areas. Due to this entity's rarity, definite diagnostic and treatment parameters are somewhat limited. We present the case of a myoepithelial carcinoma arising from the abdominal wall, a rare site of origin of an uncommon tumor.
A 37-year-old gentleman presented to our institution in Oct 2018 with a recurrent abdominal mass for which he underwent wide local excision after completing the workup, which included systemic scans and relevant blood investigations. The histopathology report was consistent with malignant abdominal myoepithelial carcinoma. However, subsequent follow-up scans in May 2019 showed disease progression with the appearance of multiple lung metastases. After a detailed discussion, he was started on Pazopanib 800 mg orally once a day, on which he remained stable till May 2022. It was then when he experienced clinical disease progression confirmed on systemic scans, so he was offered palliative systemic chemotherapy.
Abdominal malignant myoepithelial carcinomas are an infrequent entity. However, this case highlights its critical diagnostic markers and primary and recurrent abdominal myoepithelial carcinoma management.
Abdominal myoepithelial carcinomas, although rare, are also under-recognized. Thus, keeping an index of high suspicion for these tumors and being armed with knowledge regarding the heterogeneity of its features would lead to better diagnostic awareness and documentation, paving the way for better evidence-based treatments.
肌上皮癌是一组表现出肌上皮分化的多样化肿瘤。关于肌上皮癌起源于涎腺外部位（如软组织、骨骼和内脏区域）的报道越来越多。由于该实体罕见，明确的诊断和治疗参数在一定程度上受到限制。我们报告一例起源于腹壁的肌上皮癌，这是一种罕见肿瘤的罕见起源部位。
一名37岁男性于2018年10月因复发性腹部肿块就诊于我院，在完成包括全身扫描和相关血液检查在内的检查后，他接受了广泛局部切除。组织病理学报告与恶性腹部肌上皮癌一致。然而，2019年5月的后续扫描显示疾病进展，出现了多个肺转移灶。经过详细讨论，他开始口服帕唑帕尼800mg，每日一次，直至2022年5月病情一直稳定。就在那时，全身扫描证实他出现了临床疾病进展，因此他接受了姑息性全身化疗。
腹部恶性肌上皮癌是一种罕见的实体。然而，该病例突出了其关键诊断标志物以及原发性和复发性腹部肌上皮癌的管理。
腹部肌上皮癌虽然罕见，但也未得到充分认识。因此，对这些肿瘤保持高度怀疑指数，并掌握其特征异质性的相关知识，将提高诊断意识和记录水平，为更好的循证治疗铺平道路。
