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Successful management of perivascular epithelioid cell tumor of the rectum with recurrent liver metastases: A case report.

Abstract：

RATIONALE:The perivascular epithelioid cell tumor (PEComa) is rare in young man and rarely occurs in the large intestine. PATIENT CONCERNS:The clinical characteristics, diagnosis, and managements in a 28-year-old boy who presented with sudden onset of cramping and abdominal pain and intermittent melena with a blood pressure of 74/39 mm Hg was retrospectively reviewed. CT scan of the abdomen revealed a 8.9 × 7.2 cm mass in the pelvic floor. DIAGNOSES:Given the difficulty of obtaining a diagnostic specimen, surgical resection was performed. The pathology report of lower anterior resection was malignant PEComa of the rectum in 2006. INTERVENTIONS:Treatment consisted of surgical resection only without additional adjuvant therapy. Over the next 49 months (until 2010) after surgery, abdominal CT showed a 0.6-cm hypodense mass over the liver with suspected liver metastasis. He refused any further evaluation and treatment. After 4 years (2014), abdominal CT showed that the original mass had increased from 0.6 to 1.5 cm and the number of tumors had increased from 1 to 3. In August 2014, he underwent a metastatic hepatectomy without additional chemotherapy or radiotherapy. OUTCOMES:We noted that the metastatic progression was slow in the 4 years after the first operation. At 28 months after metastatic hepatectomy, the patient was doing well. There was also no recurrence of the PEComa of the rectum at the 120-month follow-up in 2016. LESSONS:To the best of our knowledge, this is the first report of a PEComa of the rectum with liver metastases treated with only surgical resection. At approximately 8.8 cm, this is the largest PEComa of the rectum reported in the recent literature.

journal_name

Medicine (Baltimore)

journal_title

Medicine

authors

Lin KH,Chang NJ,Liou LR,Su MS,Tsao MJ,Huang ML

doi

10.1097/MD.0000000000011679

subject

Has Abstract

pub_date

2018-08-01 00:00:00

pages

e11679

issue

31

eissn

0025-7974

issn

1536-5964

pii

00005792-201808030-00070

journal_volume

97

pub_type

杂志文章

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