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Atypical Presentation of Lymphangioleiomyomatosis as Acute Abdomen: CT Diagnosis

Prince of Wales Hospital Shatin, New Territories, Hong Kong

Lymphangioleiomyomatosis is a rare disorder of unknown origin that almost exclusively affects women of childbearing age. It is characterized by proliferation of abnormal smooth-muscle cells (lymphangioleiomyomatosis cells) in the pulmonary interstitium and along the thoracic and abdominal lymphatics. Lymphangioleiomyomatosis may be associated with tuberous sclerosis complex. The most common manifestations of lymphangioleiomyomatosis are pulmonary symptoms including progressive dyspnea, recurrent pneumothoraces, and chylous effusions. Extrapulmonary lymphangioleiomyomatosis as the initial presentation of the disease is highly unusual. We describe a patient with extrapulmonary lymphangioleiomyomatosis presenting as an acute abdomen.

A previously healthy 32-year-old woman presented with right lower quadrant abdominal pain and fever. Abdominal sonography was performed, and a complex cystic lesion was found in the right adnexa. Clinical suspicion of a tuboovarian abscess was raised by the attending surgeon. Gynecologists were consulted, and an emergency laparoscopy was performed. At surgery, the uterus and ovaries were normal, but a pulsatile retroperitoneal mass of uncertain origin was found.

Contrast-enhanced CT was then performed. A diffuse encapsulated mass containing low-attenuation (8–24 H) material was present in the retroperitoneum (Fig. 1A). The lesion extended from the retroperitoneum down to the pelvis along the common iliac vessels (Fig. 1B). Neither hepatic nor renal angiomyolipoma could be found, and no as-cites was present. Multiple thin-walled pulmonary cysts were found in the lungs, but the intervening lung parenchyma was normal (Fig. 1C). Small pleural effusions were present bilaterally. The overall picture was compatible with lymphangioleiomyomatosis with retroperitoneal lymphangiomyoma.

View larger version (130K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A. —32-year-old woman with lymphangioleiomyomatosis. Contrast-enhanced CT scan shows diffuse, encapsulated mass (arrow) containing low-attenuation material in retroperitoneum, compatible with retroperitoneal lymphangiomyoma.

View larger version (115K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B. —32-year-old woman with lymphangioleiomyomatosis. Contrast-enhanced CT scan shows retroperitoneal lymphangiomyoma (arrows) extending into pelvis.

View larger version (77K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C. —32-year-old woman with lymphangioleiomyomatosis. CT scan shows multiple thin-walled pulmonary cysts with intervening normal lung parenchyma.

Manifestation of extrapulmonary lymphangioleiomyomatosis as an initial presenting symptom is rare. The most common forms of extrapulmonary lymphangioleiomyomatosis include renal angiomyolipoma (54% of cases), enlarged abdominal lymph nodes (39%), and lymphangiomyoma (16%). Less commonly, ascites (10%) and hepatic angiomyolipoma (4%) may be present [1, 2]. If the diagnosis of extrapulmonary lymphangioleiomyomatosis precedes that of pulmonary lymphangioleiomyomatosis, the patient usually develops chest symptoms within 1–2 years [3].

No effective treatment for lymphangioleiomyomatosis has been found. Hormonal therapy can control chylothoraces [4]. However, for patients with severe pulmonary involvement, lung transplantation is mandatory.

References

1. Avila NA, Kelly JA, Chu SC, Dwyer AJ, Moss J. Lymphangioleiomyomatosis: abdominopelvic CT and US findings. Radiology2000; 216:147 –153[Abstract/Free Full Text]
2. Woodring JH, Howard RS 2nd, Johnson MV. Massive low-attenuation mediastinal, retroperitoneal, and pelvic lymphadenopathy on CT from lymphangioleiomyomatosis: case report. Clin Imaging1994; 18:7 –11[Medline]
3. Matsui K, Tatsuguchi A, Valencia J, et al. Extra-pulmonary lymphangioleiomyomatousis (LAM): clinicopathologic features in 22 cases. Hum Pathol2000; 31:1242 –1248[Medline]
4. Korzeniewska-Kosela M, Maziarka D, Wesolowski S, et al. Pulmonary lymphangioleimyomatosis: presentation and results of treatment [in Polish]. Pneumonol Alergol Pol2001; 69:626 –634[Medline]

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This Article Figures Only Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Wong, Y.-Y. Articles by Chu, W. C.-W. Search for Related Content PubMed PubMed Citation Articles by Wong, Y.-Y. Articles by Chu, W. C.-W. Social Bookmarking                      What's this?