Leyrat Brice, Bernadach Maureen, Ginzac Angeline, Lusho Sejdi, Durando Xavier
Department of Medical Oncology, Centre Jean Perrin, Clermont-Ferrand, France.
UFR Medicine, University Clermont Auvergne, Clermont-Ferrand, France.
Case Rep Oncol. 2021 Apr 8;14(1):664-670. doi: 10.1159/000515298. eCollection 2021 Jan-Apr.
Umbilical skin metastases (or Sister Mary Joseph nodules) are rare. Their presence typically indicates the late manifestation of deep-seated abdominopelvic malignancy. They occur mainly in gynecological cancers, and gastrointestinal cancers in men. The most common histology is adenocarcinoma (∼75% of cases), but it can also rarely be squamous cell or undifferentiated carcinoma. These metastases can be present at diagnosis or appear at disease recurrence, and are associated with a very poor prognosis with an average survival of 11 months. We report the clinical case of a 58-year-old man with metastatic pancreatic adenocarcinoma and umbilical cutaneous metastasis after receiving first-line chemotherapy. The diagnosis was established upon liver biopsy in July 2019, after the patient presented with a complaint of transfixing abdominal pain. The first-line treatment consisted of six cycles of modified FOLFIRINOX chemotherapy. However, in November 2019, computed tomography (CT) scan showed disease progression. Second-line treatment with gemcitabine (Gemzar®) led to a 16% decrease in target lesions. During the fourth cycle, three periumbilical indurated nodules appeared. After six cycles, skin infiltration had increased, and the patient reported his abdominal pain had intensified. Reassessment by CT scan showed an increase in both hepatic and peritoneal disease progression. Third-line treatment with FOLFIRI, started on April 15, 2020, could not control the disease, leading to greater induration and subcutaneous infiltration, which were responsible for the increased pain and ultimate death. Umbilical skin metastases are rare, and they are associated with advanced metastatic disease and a very poor prognosis. Cases reporting Sister Mary Joseph nodules are needed to better understand the conditions and mechanisms of their appearance and dissemination.
脐部皮肤转移瘤（或玛丽·约瑟夫修女结节）较为罕见。它们的出现通常提示深部腹盆腔恶性肿瘤的晚期表现。主要发生于妇科癌症以及男性的胃肠道癌症。最常见的组织学类型是腺癌（约占病例的75%），但也很少见鳞状细胞癌或未分化癌。这些转移瘤可在诊断时即存在，或在疾病复发时出现，且预后极差，平均生存期为11个月。我们报告一例58岁男性患者的临床病例，该患者在接受一线化疗后出现转移性胰腺腺癌及脐部皮肤转移。2019年7月，患者因持续性腹痛就诊，经肝脏活检确诊。一线治疗为六个周期的改良FOLFIRINOX化疗。然而，2019年11月的计算机断层扫描（CT）显示疾病进展。吉西他滨（健择®）二线治疗使靶病灶缩小了16%。在第四个周期时，脐周出现三个硬结性结节。六个周期后，皮肤浸润增加，患者自述腹痛加剧。CT扫描重新评估显示肝脏和腹膜疾病进展均加重。2020年4月15日开始的FOLFIRI三线治疗未能控制疾病，导致硬结和皮下浸润加重，这导致疼痛加剧并最终死亡。脐部皮肤转移瘤罕见，与晚期转移性疾病及极差的预后相关。需要更多报告玛丽·约瑟夫修女结节的病例，以更好地了解其出现和播散的情况及机制。
