Khare Manika, Gupta Manish Kumar, Sharma Umesh Babu, Garg K M
Department of Pathology, JNUIMSRC, Jaipur, Rajasthan, India.
Department of Surgery, JNUIMSRC, Jaipur, Rajasthan, India.
Indian J Pathol Microbiol. 2018 Jul-Sep;61(3):404-406. doi: 10.4103/IJPM.IJPM_334_17.
Paragangliomas (PGLs) are rare tumors of neural crest origin, with a malignancy rate of approximately 10% and a 5-year survival rate of <50%. We present a case of malignant PGL arising from the porta hepatis with metastasis to the portal lymph node and bilateral ovaries. PGLs arising from the porta hepatis are very rare. As per our knowledge, only three cases of hepatic duct PGL have been reported. It is important to detect it earlier because the treatment modality and prognosis of benign and malignant PGL differs and defines the prognosis of the patient.
副神经节瘤（PGLs）是起源于神经嵴的罕见肿瘤，恶性率约为10%，5年生存率<50%。我们报告一例起源于肝门的恶性PGL，伴有门静脉淋巴结和双侧卵巢转移。起源于肝门的PGL非常罕见。据我们所知，仅报道过3例肝管PGL。早期检测很重要，因为良性和恶性PGL的治疗方式和预后不同，这决定了患者的预后。
