Ugwuegbulem Emeka, Hlaing Swe Swe, deFreitas Gerson, McIntosh William, Patel Dhruvanshur
Internal Medicine, St. Luke's University Hospital, Bethlehem, USA.
Internal Medicine, Crozer-Chester Medical Center, Upland, USA.
Cureus. 2022 Jun 9;14(6):e25785. doi: 10.7759/cureus.25785. eCollection 2022 Jun.
Myoepithelial carcinoma is a rare malignant tumor arising from myoepithelial cells. The usual sites of occurrence are the oral cavity and pharynx with the majority of tumors arising from the salivary gland. However, there have been reported cases of myoepithelial carcinoma seen in the superficial soft tissue, upper respiratory tract, breast, skin, and GI tract. Deep soft tissue myoepithelial carcinoma is relatively uncommon. Due to the rarity of this malignancy, consensus on appropriate therapy remains sparse. However, complete resection and/or adjuvant chemotherapy and radiotherapy have been advocated for non-metastatic localized diseases. Sadly, the reported outcome in patients with metastatic disease remains dismal. In this case, we report a 79-year-old male incidentally found to have a deep soft tissue mass in the sacral area with a coexistent left axillary lymphadenopathy with biopsy and immunohistochemistry confirmation of metastatic myoepithelial carcinoma. He had a rapid clinical deterioration with subsequent fatality.
肌上皮癌是一种起源于肌上皮细胞的罕见恶性肿瘤。其常见发生部位是口腔和咽部,大多数肿瘤起源于唾液腺。然而,也有报道称在浅表软组织、上呼吸道、乳腺、皮肤和胃肠道发现了肌上皮癌病例。深部软组织肌上皮癌相对少见。由于这种恶性肿瘤罕见,关于适当治疗的共识仍然很少。然而,对于非转移性局限性疾病,主张进行完全切除和/或辅助化疗及放疗。遗憾的是,转移性疾病患者的报告结果仍然很差。在此病例中,我们报告一名79岁男性,偶然发现骶骨区域有一个深部软组织肿块,同时伴有左腋窝淋巴结肿大,经活检和免疫组化证实为转移性肌上皮癌。他临床病情迅速恶化,随后死亡。