Document Detail


Primary retroperitoneal lymphangioleiomyomatosis in a postmenopausal woman: a case report and review of the literature.
MedLine Citation:
PMID:  17362325     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Lymphangioleiomyomatosis (LAM) is a rare progressive disease of unknown etiology that typically affects women of childbearing age. It is characterized by an abnormal proliferation of smooth muscle cells causing gradual obstruction of small airways, frequently resulting in respiratory failure and death. While LAM is predominantly a lung disorder, we report a case of retroperitoneal LAM in a patient who had no evidence of pulmonary involvement. A 59-year-old female presented with postmenopausal bleeding and no other complaints. She was found to have a low attenuation retroperitoneal mass on abdominal and pelvic computed tomography (CT) scan suspicious for lymphoma. CT-guided biopsy was nondiagnostic. Laparoscopic resection of some of the enlarged retroperitoneal lymph nodes confirmed the diagnosis of LAM. This case is an atypical presentation of LAM. The disease typically presents in premenopausal women, with the initial site of involvement being the lungs and mediastinum. In rare cases, such as this of extrapulmonary LAM, patients typically present with a palpable abdominal mass, abdominal pain, or chylous ascites. As in our case, radiographic findings can mimic malignancies such as lymphoma. Laparoscopic lymph node biopsy is a valuable tool in these situations of diagnostic dilemma.
Authors:
M Kebria; D Black; C Borelli; I Modica; M Hensley; D S Chi
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  International journal of gynecological cancer : official journal of the International Gynecological Cancer Society     Volume:  17     ISSN:  1048-891X     ISO Abbreviation:  Int. J. Gynecol. Cancer     Publication Date:    2007 Mar-Apr
Date Detail:
Created Date:  2007-03-16     Completed Date:  2007-05-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9111626     Medline TA:  Int J Gynecol Cancer     Country:  United States    
Other Details:
Languages:  eng     Pagination:  528-32     Citation Subset:  IM    
Affiliation:
Gynecology Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
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MeSH Terms
Descriptor/Qualifier:
Female
Humans
Lymphangioleiomyomatosis / diagnosis*,  pathology
Middle Aged
Postmenopause
Retroperitoneal Neoplasms / diagnosis*,  pathology

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