Perivascular Epithelioid Cell Tumor of the Retroperitoneum in a Young Woman Resulting in an Abdominal Chyloma

Abstract

Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm which appears to have predominancy for young, frequently Asian, women. The neoplasm is composed chiefly of HMB-45-positive epithelioid cells with clear to granular cytoplasm and usually showing a perivascular distribution. These tumors have been reported in various organs under a variety of designations. Malignant PEComas exist but are very rare. The difficulty in determining optimal therapy, owing to the sparse literature available, led us to present this case. We report a retroperitoneal PEComa discovered during emergency surgery for abdominal pain in a 28-year-old Asian woman. The postoperative period was complicated by chylous ascites that was initially controlled by a wait-and-see policy with total parenteral nutrition. However, the chyle production gradually increased to more than 4 l per day. The development of a bacterial peritonitis resulted in cessation of production of abdominal fluid permitting normal nutrition without chylous leakage. Effective treatment for this rare complication of PEComa is not yet known; therefore, we have chosen to engage in long-term clinical follow-up.

A review of the postoperative lymphatic leakage

“…Increasing rapidly from fasting to oral intake and/or turning clear to milky drainage fluid are the evidences of lymphatic leakage, especially the leakage of gastrointestinal tract [ 14 ]. High fat diet, an extensive amount of double cream [ 25 , 85 ], is used to detect the leakage of lymphatic channels [ 38 , 86 ]. That can be called “chylous lymphangiographic effect” [ 86 ].…”

“…CT, ultrasonography (US) or magnetic resonance(MR) [ 53 , 87 ] used in the diagnosis of lymphatic leakage, especially lymphocele and chylous ascites [ 57 ] can show us the hydrops [ 88 ], the cyst of body cavity [ 31 ] and location the leakage [ 89 ] particularly when there is suspicion of thoracic duct injury [ 85 ]. US- or CT- or MR- guided paracentesis and following by laboratory examination are widely used in diagnosis.…”

“…Several complications have been described of lymphangiography, including the tissue necrosis, fat embolism and hypersensitivity relating to the volume and type of contrast agents [ 85 ]. However, since it is difficult to treat at the meantime even on direct vision of lipiodol leakage, lymphangiography is not appropriate in dealing with lymphocele [ 36 ].…”

Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature

“…Since retroperitoneal PEComas cannot easily be differentiated from sarcomas and because half of them may develop a potentially malignant biological behaviour, radical surgical resection (usually organ sparing) constitutes the treatment of choice for localized disease[3,15,18,23,38,56]. Chemo-, immune- and/or radiotherapy have little efficacy[3,12,18,56,57].…”

Causes and Treatment of Inguinal Lymphadenopathy: Literature Review

“…The cause may be fibrosis and lymphatic occlusion caused by the inflammatory response caused by the contrast agent. Lymphangiography has reported several complications, including tissue necrosis, fat embolism, and allergic reactions [37], with some limitations. Radiotherapy has been shown to promote the recovery of inguinal lymphadenopathy and may be related to tissue sclerosis [38].…”