“软糖豆”：诊断难题与当前概念
"The Jelly Belly": Diagnostic Dilemmas and Current Concepts.
作者信息
Kedia Priyanka Punit, Ravikumar Gayatri, Mohanty Suravi, Crasta Julian, Vallikad Elizabeth
机构信息
Department of Pathology, St. John's Medical College, Sarjapur Road, Bangalore, Karnataka 560034 India.
Department of Gynecologic Oncology, St. John's Medical College, Sarjapur Road, Bangalore, Karnataka 560034 India.
出版信息
J Obstet Gynaecol India. 2016 Oct;66(Suppl 1):573-7. doi: 10.1007/s13224-016-0921-z. Epub 2016 Jul 1.
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare and poorly understood clinicopathological entity characterized by gelatinous ascites with neoplastic or non-neoplastic mucinous implants in the peritoneum. Although its origin was debated, current evidence in literature favours the appendix as the origin of the disease, over the ovaries. The changing terminologies in the classification of this entity pose diagnostic and management challenges.
CASE REPORTS
Herein, we report three cases of PMP in postmenopausal women, their clinical presentation, pathological staging based on the peritoneal tumor deposits and the treatment administered. Two patients recovered uneventfully, while one had recurrence of adenocarcinoma.
CONCLUSION
The rarity of this disease and the diagnostic challenges associated with it are discussed with an emphasis on the current concepts in its origin and management. Appropriate classification and complete removal of the tumor is mandated to prevent disease-related mortality.
背景
腹膜假黏液瘤（PMP）是一种罕见且了解甚少的临床病理实体，其特征为胶冻样腹水，伴有腹膜内肿瘤性或非肿瘤性黏液种植。尽管其起源存在争议，但目前文献证据表明，相较于卵巢，阑尾是该疾病的起源部位。该实体分类中术语的不断变化给诊断和治疗带来了挑战。
病例报告
在此，我们报告3例绝经后女性PMP病例，包括其临床表现、基于腹膜肿瘤种植的病理分期及所给予的治疗。2例患者顺利康复，1例出现腺癌复发。
结论
讨论了该疾病的罕见性及其相关诊断挑战，重点阐述了其起源和治疗的当前概念。必须进行适当分类并完整切除肿瘤以预防疾病相关死亡。
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