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Lymphangioleiomyomatosis: CT of Diurnal Variation of Lymphangioleiomyomas¹

¹ From the Department of Diagnostic Radiology, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bldg 10, Rm 1C-660, 10 Center Dr MSC 1182, Bethesda, MD 20892-1182 (N.A.A., A.J.D.); and Pulmonary-Critical Care Medicine Branch (J.B., J.M.) and Pathology Section (V.J.F.), National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Md. Received August 30, 2000; revision requested October 5; revision received March 9, 2001; accepted April 3. Address correspondence to N.A.A. (e-mail: navila@nih.gov).

	ABSTRACT

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PURPOSE: To evaluate the imaging and clinical features of lymphangioleiomyomas and to describe the phenomenon of diurnal variation in the size of lymphangioleiomyomas in patients with lymphangioleiomyomatosis.

MATERIALS AND METHODS: One hundred twenty-eight patients with lymphangioleiomyomatosis underwent chest and abdominopelvic computed tomography (CT). Thirteen patients underwent CT in the morning and afternoon of the same day to assess diurnal variation in lymphangioleiomyoma size.

RESULTS: Twenty-seven of 128 patients (21%) had 54 lymphangioleiomyomas. The vast majority (96%) of these masses contained material of low attenuation at CT. Associated CT findings included enlarged abdominal lymph nodes, pleural effusions, ascites, and dilatation of the thoracic duct. The prevalence of lymphangioleiomyomas was 15% in patients who had mild pulmonary disease, 19% in patients who had moderate disease, and 26% in patients who had severe disease. Diurnal variation in size of masses was demonstrated in 12 of 13 patients. Seven of the 27 patients who had masses underwent biopsy; all seven were confirmed to have lymphangioleiomyomas. The most common symptoms associated with lymphangioleiomyomas were bloating, abdominal pain, and edema of the lower extremities. The majority of the patients reported worsening of symptoms as the day progressed.

CONCLUSION: Lymphangioleiomyomas are common in patients with lymphangioleiomyomatosis. Diurnal variation in size may explain worsening of symptoms during the day.

Index terms: Lung, diseases, 60.799 • Lymphangiomyomatosis, 87.829, 60.799 • Lymphatic system, diseases, 99.829 • Lymphatic system, flow dynamics • Retroperitoneal space, diseases, 87.829

	INTRODUCTION

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Lymphangioleiomyomatosis (LAM) is a rare multisystem disorder seen almost exclusively in premenopausal women that is characterized by proliferation of abnormal smooth muscle cells in the lungs and the lymph vessels of the thorax and retroperitoneum (1–5) and by the formation of pulmonary parenchymal cysts. The chest radiographic findings include the classic triad of reticular interstitial pattern, chylous pleural effusions, and recurrent pneumothoraces (6). The linear abnormalities seen on chest radiographs represent the superimposition of the walls of thin-walled cysts, which are a hallmark of LAM at computed tomography (CT) (7). An associated finding in LAM, previously thought to be rare, is the dilation of lymph vessels due to blockage by the proliferation of smooth muscle cells in their walls. This process results in complex lymphatic masses termed lymphangioleiomyomas. We have previously briefly reported the imaging characteristics of lymphangioleiomyomas in 13 patients with LAM and abdominopelvic lymphangioleiomyomas (8). We have subsequently examined additional patients with lymphangioleiomyomatosis and have found information that we believe may be useful in better characterizing lymphangioleiomyomas. Early in our investigations, we discovered that lymphangioleiomyomas can change in size during the course of the day; we termed this effect diurnal variation. To the best of our knowledge, this finding has not been previously reported but is important, particularly to radiologists, because it means that patients must be imaged at similar times of the day to avoid misdiagnosis of the diurnal variation in size as an enlarging neoplasm. The purpose of our study, therefore, was to evaluate the imaging and clinical features of lymphangioleiomyomas and to describe the previously unreported phenomenon of diurnal variation in the size of lymphangioleiomyomas in patients with LAM.

	MATERIALS AND METHODS

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Study Population
One hundred twenty-eight women with LAM, who ranged in age from 27 to 70 years (mean, 43 years), were enrolled consecutively from December 1995 to December 1999 in a protocol approved by our institutional review board. (Our institution is a referral center for patients with LAM, in whom we study the natural history of the disease among other factors.) As part of the protocol, the patients underwent CT studies of the chest, abdomen, and pelvis. Two patients also underwent cervical CT examinations to evaluate neck masses that were seen on the chest CT images. All patients provided written informed consent.

Imaging Studies
Every patient underwent CT examination of the chest, abdomen, and pelvis with either a HiSpeed Advantage (GE Medical Systems; Milwaukee, Wis) or a CTi (GE Medical Systems) scanner. The images of the abdomen were acquired helically with a pitch of 1 and were reconstructed at 10-mm intervals; the kidneys were studied with 5-mm-thick sections. The examinations were performed following the administration of 130 mL of an oral contrast agent and an intravenous nonionic contrast agent (ioxilan, Oxilan; Cook Imaging, Bloomington, Ind) at a rate of 2.0 mL/sec. There was a 40-second delay between the administration of the contrast agent and the scanning. Thin-section CT of the chest was also performed; the images were obtained with 1.0-mm collimation at 3-cm intervals during end inspiration with the patient in a supine position. The images were reconstructed with a high-resolution algorithm. In addition, 13 patients with lymphangioleiomyomas underwent a CT scan of the masses late in the afternoon of that same day (between 3 and 5 PM; the time differential between the two studies was 4–8 hours). The afternoon scans were performed helically and reconstructed at 10-mm intervals without the administration of additional oral or intravenous contrast material.

Medical History and Review of Previous Pathology Reports
All patients were interviewed before the imaging studies were performed. They were asked about the presence of abdominal and pelvic manifestations of lymphangioleiomyomas, including bloating, abdominal pain, abdominal swelling, lower extremity swelling or paresthesia, chyluria, and chylous vaginal discharge. Because of the known association between LAM and tuberous sclerosis complex (TSC), all patients were examined for skin manifestations of TSC and were questioned about family history of lung disease, pneumothoraces, skin lesions, and seizures (9). The pathology reports from any prior abdominal surgeries or biopsies performed at other institutions were reviewed, and the findings were recorded.

Image Analysis
Imaging studies were reviewed by a board-certified radiologist (N.A.A.) who was aware of the diagnoses of LAM but not of other radiologic, clinical, or pulmonary function findings. The thoracic, abdominal, and pelvic CT images were obtained on the same day and were reviewed simultaneously.

Lymphangioleiomyomas were considered to be present if the CT scans showed large, contiguous, lobulated masses sometimes infiltrating the retroperitoneum (8). Size and attenuation measurements were obtained for all masses. Attenuation measurements were obtained by one of the authors (N.A.A.) by placing the largest region of interest possible within the mass and calculating the mean attenuation in Hounsfield units by means of software available with the CT scanners. The location, border definition, and thickness of the walls of the masses were also documented. The volume of each lymphangioleiomyoma was estimated from CT images by measuring its maximum anterior-posterior (ap), transverse (tr), and craniocaudal (cc) diameters and applying the following formula: volume = ap x tr x cc. The contents of the lymphangioleiomyomas were characterized as fluid or solid on the basis of their attenuation at CT; specifically, homogeneous areas of attenuation less than or equal to 25 HU were interpreted as fluid, and the remainder were interpreted as solid. In contrast to lymphangioleiomyomas, lymph nodes appeared on the CT scans as round or ovoid and well-circumscribed masses. They were judged enlarged if they met previously published region-specific size criteria (ie, if they had a short-axis diameter 6 mm, 10 mm, and 15 mm when in the retrocrural space, upper abdomen, and pelvis, respectively) (10). The presence of enlarged (>1.5 cm in maximum diameter) axillary and inguinal lymph nodes was also noted. For the 13 patients who underwent two CT examinations during the same day, the percentage difference in volume of the lymphangioleiomyomas was calculated using the following formula: percentage difference in volume = (larger volume - smaller volume)/smaller volume x 100. Other CT findings noted were pleural effusion, abdominal or pelvic ascites, and thoracic duct dilatation.

The extent and severity of lung disease was determined by visual inspection of high-resolution CT images of the chest and was assigned one of the following three grades: I (mild), in which pulmonary cysts involved less than one-third of the lung parenchyma; II (moderate), in which pulmonary cysts involved one-third to two-thirds of the lung parenchyma; and III (severe), in which pulmonary cysts involved two-thirds or more of the lung parenchyma or the patient had already undergone lung transplantation. This grading method has been used by previous authors (11–13).

The sign test was used to assess the statistical significance of the difference between the morning and afternoon volumes of the masses. The Cochran-Armitage test was used to assess the association between the extent and severity of lung disease and the presence of lymphangioleiomyomas (14).

	RESULTS

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Of the 128 patients, 27 (21%) had 54 lymphangioleiomyomas (Table 1). The imaging features of 13 of the 27 patients with lymphangioleiomyomas have previously been described (8). The lymphangioleiomyomas were located in various areas of the body, as shown in Table 1 and Figure 1. The range of volumes of the lymphangioleiomyomas was 10–1,500 cm³. Two masses had infiltrated the retroperitoneum and had ill-defined borders; the rest had well-defined borders. Two masses appeared solid at CT (at 59 and 71 HU—similar in attenuation to the liver); the rest (52 of 54) contained low-attenuating material (3–25 HU) consistent with fluid. Of the 52 apparently fluid-filled masses, 37 had thin walls, 10 had thick walls, and five had a combination of thin and thick walls (Fig 2). Associated CT findings in patients with lymphangioleiomyomas were enlarged abdominal lymph nodes (see size criteria in Materials and Methods) (16 of 27 patients [59%]), pleural effusions (eight of 27 patients [30%]), ascites (four of 27 patients [15%]), and dilatation of the thoracic duct (five of 27 patients [19%]). No patient had enlarged axillary or inguinal lymph nodes.

View larger version (120K): [in this window] [in a new window] [Download PPT slide]   Figure 1a. Examples of different locations of lymphangioleiomyomas. (a) Transverse contrast-enhanced 5-mm CT section through the neck of a 41-year-old patient shows a low-attenuating left cervical mass (white arrows) that displaces the carotid artery and jugular vein (black arrow) anteriorly. (b) Transverse contrast-enhanced 10-mm CT section through the chest of a 32-year-old patient demonstrates a low-attenuating mass (arrows) posterior to the heart. (c) Transverse contrast-enhanced 10-mm CT section through the abdomen of a 49-year-old patient demonstrates a low-attenuating mass (arrow) that infiltrates the retroperitoneum, has ill-defined borders, and elevates the aorta (A) and inferior vena cava (I) anteriorly from the spine. (d) Transverse contrast-enhanced 10-mm CT section through the pelvis of a 30-year-old patient demonstrates a multilobular pelvic mass (arrows) displacing the bladder (B) medially.

View larger version (140K): [in this window] [in a new window] [Download PPT slide]   Figure 1b. Examples of different locations of lymphangioleiomyomas. (a) Transverse contrast-enhanced 5-mm CT section through the neck of a 41-year-old patient shows a low-attenuating left cervical mass (white arrows) that displaces the carotid artery and jugular vein (black arrow) anteriorly. (b) Transverse contrast-enhanced 10-mm CT section through the chest of a 32-year-old patient demonstrates a low-attenuating mass (arrows) posterior to the heart. (c) Transverse contrast-enhanced 10-mm CT section through the abdomen of a 49-year-old patient demonstrates a low-attenuating mass (arrow) that infiltrates the retroperitoneum, has ill-defined borders, and elevates the aorta (A) and inferior vena cava (I) anteriorly from the spine. (d) Transverse contrast-enhanced 10-mm CT section through the pelvis of a 30-year-old patient demonstrates a multilobular pelvic mass (arrows) displacing the bladder (B) medially.

View larger version (122K): [in this window] [in a new window] [Download PPT slide]   Figure 1c. Examples of different locations of lymphangioleiomyomas. (a) Transverse contrast-enhanced 5-mm CT section through the neck of a 41-year-old patient shows a low-attenuating left cervical mass (white arrows) that displaces the carotid artery and jugular vein (black arrow) anteriorly. (b) Transverse contrast-enhanced 10-mm CT section through the chest of a 32-year-old patient demonstrates a low-attenuating mass (arrows) posterior to the heart. (c) Transverse contrast-enhanced 10-mm CT section through the abdomen of a 49-year-old patient demonstrates a low-attenuating mass (arrow) that infiltrates the retroperitoneum, has ill-defined borders, and elevates the aorta (A) and inferior vena cava (I) anteriorly from the spine. (d) Transverse contrast-enhanced 10-mm CT section through the pelvis of a 30-year-old patient demonstrates a multilobular pelvic mass (arrows) displacing the bladder (B) medially.

View larger version (111K): [in this window] [in a new window] [Download PPT slide]   Figure 1d. Examples of different locations of lymphangioleiomyomas. (a) Transverse contrast-enhanced 5-mm CT section through the neck of a 41-year-old patient shows a low-attenuating left cervical mass (white arrows) that displaces the carotid artery and jugular vein (black arrow) anteriorly. (b) Transverse contrast-enhanced 10-mm CT section through the chest of a 32-year-old patient demonstrates a low-attenuating mass (arrows) posterior to the heart. (c) Transverse contrast-enhanced 10-mm CT section through the abdomen of a 49-year-old patient demonstrates a low-attenuating mass (arrow) that infiltrates the retroperitoneum, has ill-defined borders, and elevates the aorta (A) and inferior vena cava (I) anteriorly from the spine. (d) Transverse contrast-enhanced 10-mm CT section through the pelvis of a 30-year-old patient demonstrates a multilobular pelvic mass (arrows) displacing the bladder (B) medially.

View larger version (137K): [in this window] [in a new window] [Download PPT slide]   Figure 2a. Examples of lymphangioleiomyomas demonstrating variations in wall thickness. (a) Transverse contrast-enhanced 10-mm CT section through the pelvis of a 30-year-old patient shows a lymphangioleiomyoma with thin walls (arrow) displacing the bladder anteriorly and to the right. (b) Transverse contrast-enhanced 10-mm CT section through the pelvis of a 49-year-old patient demonstrates a mass with thick walls (arrow).

View larger version (139K): [in this window] [in a new window] [Download PPT slide]   Figure 2b. Examples of lymphangioleiomyomas demonstrating variations in wall thickness. (a) Transverse contrast-enhanced 10-mm CT section through the pelvis of a 30-year-old patient shows a lymphangioleiomyoma with thin walls (arrow) displacing the bladder anteriorly and to the right. (b) Transverse contrast-enhanced 10-mm CT section through the pelvis of a 49-year-old patient demonstrates a mass with thick walls (arrow).

View larger version (136K): [in this window] [in a new window] [Download PPT slide]   Figure 3a. Transverse contrast-enhanced 10-mm CT scans through the pelvis of a 44-year-old patient (Patient 13, Table 2) obtained in the morning and afternoon of the same day demonstrate an increase in the size of the mass (arrow). (a) Scan obtained in the morning (10:34 AM). Volume of the mass: 45 cm3. (b) Scan obtained in the afternoon (2:35 PM). Volume of the mass: 120 cm3.

View larger version (132K): [in this window] [in a new window] [Download PPT slide]   Figure 3b. Transverse contrast-enhanced 10-mm CT scans through the pelvis of a 44-year-old patient (Patient 13, Table 2) obtained in the morning and afternoon of the same day demonstrate an increase in the size of the mass (arrow). (a) Scan obtained in the morning (10:34 AM). Volume of the mass: 45 cm3. (b) Scan obtained in the afternoon (2:35 PM). Volume of the mass: 120 cm3.

Correlation of Abdominopelvic Imaging Findings with the Severity of Lung Disease
The severity of lung disease in the 128 patients was as follows: 40 (31%) had grade I lung disease, 27 (21%) had grade II disease, and 61 (48%) had grade III disease (Table 4, Fig 4). A positive association was observed between the severity of lung disease and the probability of lymphangioleiomyomas being present; the higher the grade, the higher the probability. Lymphangioleiomyomas were observed in six of 40 patients (15%) with grade I disease, in five of 27 patients (19%) with grade II disease, and in 16 of 61 patients (26%) with grade III disease. Four of the patients with grade III disease had undergone lung transplantation at the time of this study. Thus, an approximately linear trend was observed between the grade of lung disease and the probability of lymphangioleiomyomas; this was not a statistically significant trend (P = .12, Cochran-Armitage test).

View larger version (141K): [in this window] [in a new window] [Download PPT slide]   Figure 4a. High-resolution CT images of the chest (1-mm-thick transverse sections obtained in patients in the prone position) demonstrate the extent of pulmonary disease in patients with LAM. (a) Image in a 43-year-old patient with mild (grade I) pulmonary LAM. Note scattered pulmonary cysts (arrows) involving less than one-third of the parenchyma. (b) Image in a 51-year-old patient with severe (grade III) pulmonary LAM. Note diffuse pulmonary cysts involving more than two-thirds of the parenchyma. Very little normal lung can be seen.

View larger version (127K): [in this window] [in a new window] [Download PPT slide]   Figure 4b. High-resolution CT images of the chest (1-mm-thick transverse sections obtained in patients in the prone position) demonstrate the extent of pulmonary disease in patients with LAM. (a) Image in a 43-year-old patient with mild (grade I) pulmonary LAM. Note scattered pulmonary cysts (arrows) involving less than one-third of the parenchyma. (b) Image in a 51-year-old patient with severe (grade III) pulmonary LAM. Note diffuse pulmonary cysts involving more than two-thirds of the parenchyma. Very little normal lung can be seen.

	DISCUSSION

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View larger version (9K): [in this window] [in a new window] [Download PPT slide]   Figure 5a. Diagrams of normal lymph vessels and the effect of LAM on them. (a) Unidirectional valves and lymph flow in normal lymph vessels. (b) Proliferation of smooth muscle cells in lymph vessels causes mural thickening. (c) Obstruction of lymph flow by LAM cells results in dilatation of the vessels and the formation of collections of chylous material. The resulting masses are termed lymphangioleiomyomas.

View larger version (13K): [in this window] [in a new window] [Download PPT slide]   Figure 5b. Diagrams of normal lymph vessels and the effect of LAM on them. (a) Unidirectional valves and lymph flow in normal lymph vessels. (b) Proliferation of smooth muscle cells in lymph vessels causes mural thickening. (c) Obstruction of lymph flow by LAM cells results in dilatation of the vessels and the formation of collections of chylous material. The resulting masses are termed lymphangioleiomyomas.

View larger version (11K): [in this window] [in a new window] [Download PPT slide]   Figure 5c. Diagrams of normal lymph vessels and the effect of LAM on them. (a) Unidirectional valves and lymph flow in normal lymph vessels. (b) Proliferation of smooth muscle cells in lymph vessels causes mural thickening. (c) Obstruction of lymph flow by LAM cells results in dilatation of the vessels and the formation of collections of chylous material. The resulting masses are termed lymphangioleiomyomas.

CT scans in 12 of the 13 patients examined in the morning and afternoon showed an increase in the size of the lymphangioleiomyomas between morning and afternoon CT examinations. This diurnal pattern may explain the patients’ report that symptoms of lymphangioleiomyomas worsen as the day progresses. It also provides a potentially useful radiologic feature in distinguishing lymphangioleiomyomas from lymphomas and other solid lymphatic masses, because the latter commonly demonstrate no or slow changes over time rather than daily undulations in volume. However, this pattern may mean that follow-up scans must be obtained at the same time of day the first scan was obtained to accurately assess the progression and stability of the mass in question. We speculate that the increase in size of these lymph collections during the course of the day is the result of several factors: first, greater lymph flow through the pelvis and abdomen during the day than at night because of the increase in chyle production after meals; second, an increase in lymphatic return from the lower extremities during the day due to increased muscular activity; third, the gravitational effect of increased intraluminal pressure within the lymph collections and draining lymph vessels when the patient is upright rather than recumbent (Fig 6).

View larger version (13K): [in this window] [in a new window] [Download PPT slide]   Figure 6a. Diagrams show diurnal variation in size of lymphangioleiomyomas. (a) Lymphangioleiomyoma caused by partial obstruction of the lymphatic lumen by LAM cells. Normal lymphatic flow in the morning (AM) is shown by the arrow. (b) By late afternoon (PM), the lymphangioleiomyoma has increased in size. This phenomenon probably results from increased lymph flow (arrows) during the day caused by an elevation in chyle production after meals and the increased return of lymph from the lower extremities through normal daily exercise.

View larger version (15K): [in this window] [in a new window] [Download PPT slide]   Figure 6b. Diagrams show diurnal variation in size of lymphangioleiomyomas. (a) Lymphangioleiomyoma caused by partial obstruction of the lymphatic lumen by LAM cells. Normal lymphatic flow in the morning (AM) is shown by the arrow. (b) By late afternoon (PM), the lymphangioleiomyoma has increased in size. This phenomenon probably results from increased lymph flow (arrows) during the day caused by an elevation in chyle production after meals and the increased return of lymph from the lower extremities through normal daily exercise.

Pelvic lymphangioleiomyomas may be misdiagnosed as cystic pelvic masses such as tubo-ovarian masses (hydrosalpinx or pyosalpinx) or as ovarian cancer. Fourteen of our patients with lymphangioleiomyomas had pelvic masses (alone or in combination with other masses in other locations). A carefully obtained medical history may help to differentiate the cause of the pelvic masses. Of our patients with pelvic masses, none had a history of pelvic inflammatory disease, two had undergone hysterectomy, and one had undergone bilateral salpingo-oophorectomy. Furthermore, demonstration of diurnal variation in mass size should help to differentiate lymphangioleiomyomas from other causes.

The most common complications of lymphangioleiomyomas relate to symptoms described by our patients, including severe abdominopelvic pain, incontinence, and lymphedema and paresthesias of the lower extremities. In a majority of the patients the symptoms worsen as the day progresses; this phenomenon can be explained by the diurnal variation in mass size that we report in this article. Authors of case reports have also described the spontaneous rupture of lymphangioleiomyomas resulting in chylous ascites and ureteral involvement; ureteral involvement can in turn result in chyluria (20,21). To date, there is no effective treatment for lymphangioleiomyomas. Surgery may be considered for palliation in cases in which discrete masses cause severe symptoms, but it is not feasible for masses that diffusely involve the retroperitoneum and/or surround vital structures.

The principal limitation of this study was the paucity of pathologic correlation of the imaging findings. Nevertheless, all seven patients who underwent biopsy to exclude tumor were found to have lymphangioleiomyomas.

Lymphangioleiomyomas are common in patients with LAM; their imaging and clinical features, especially their diurnal variation in size, should assist in the diagnosis and prevent unnecessary biopsies.

	FOOTNOTES

 
Abbreviations: LAM = lymphangioleiomyomatosis, TSC = tuberous sclerosis complex

Author contributions: Guarantor of integrity of entire study, N.A.A.; study concepts and design, N.A.A.; literature research, N.A.A.; clinical studies, J.M., J.B.; data acquisition, N.A.A., J.B., V.J.F.; data analysis/interpretation, A.J.D.; statistical analysis, A.J.D.; manuscript preparation, N.A.A., A.J.D.; manuscript definition of intellectual content, N.A.A.; manuscript editing, A.J.D., J.M.; manuscript revision/review, A.J.D., J.M., V.J.F.; manuscript final version approval, N.A.A.

	REFERENCES

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This Article Abstract Figures Only Full Text (PDF) All Versions of this Article: 2212001448v1 221/2/415    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map 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 Avila, N. A. Articles by Moss, J. Search for Related Content PubMed PubMed Citation Articles by Avila, N. A. Articles by Moss, J.