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AJR 2001; 177:881-885
© American Roentgen Ray Society


Original Report

Idiopathic Multilocular Thymic Cyst

CT Features with Clinical and Histopathologic Correlation

Yo Won Choi1, H. Page McAdams2, Seok Chol Jeon1, Eun Kyung Hong3, Yun-Hyeon Kim4, Jung-Gi Im5 and Seung Ro Lee1

1 Department of Radiology, Hanyang University Hospital, 17 Haengdang-dong, Sungdong-ku, Seoul 133-792, South Korea.
2 Department of Radiology, Duke University Medical Center, P.O. Box 3808, Durham, NC 27710.
3 Department of Pathology, Hanyang University Hospital, Seoul 133-792, South Korea.
4 Department of Diagnostic Radiology, Cheonnam University Hospital, 8 Hakdong Dongku, Kwangju 501-757, South Korea.
5 Department of Radiology, Seoul National University Hospital, 28 Yongon-dong, Chongro-gu, Seoul 110-744, South Korea.

Received January 30, 2001; accepted after revision April 18, 2001.

 
Address correspondence to Y. W. Choi.


Abstract
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Abstract
Introduction
Materials and Methods
Results
Discussion
References
 
OBJECTIVE. We performed this study to describe the CT features of idiopathic multilocular thymic cysts with clinical and histopathologic correlation.

CONCLUSION. Multilocular thymic cysts typically manifest on CT as unilocular or multilocular cystic thymic masses, often with soft-tissue attenuation components. CT cannot be used to distinguish neoplastic from nonneoplastic soft-tissue components.


Introduction
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Abstract
Introduction
Materials and Methods
Results
Discussion
References
 
Benign thymic cysts are uncommon lesions that account for approximately 3% of all anterior mediastinal masses [1]. Such cysts can be either congenital [2, 3] or acquired in origin [2]. Congenital cysts are typically unilocular, contain clear fluid, have walls that are thin to the point of translucency, and show no evidence of inflammation on careful histopathologic examination [2, 3]. In contrast, acquired thymic cysts result from an inflammatory process [2]. They are usually multilocular, hence the commonly used term "multilocular thymic cyst." The cysts contain turbid fluid or gelatinous material, have walls that are thick and fibrous, and typically have evidence of significant inflammation and fibrosis on histopathologic examination [2]. Multilocular thymic cysts should be distinguished from congenital cysts for several reasons: they may recur after excision, they may be associated with thymic neoplasms such as thymoma or thymic carcinoma, and they may adhere to adjacent structures and simulate an invasive neoplasm at thoracotomy [2].

Relatively few reports exist concerning the imaging features of acquired multilocular thymic cyst [4,5,6,7]. Most cases are described in the setting of AIDS [6, 7]. In this article, we describe the CT features of eight patients who had idiopathic multilocular thymic cyst with clinical and histopathologic correlation.


Materials and Methods
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Abstract
Introduction
Materials and Methods
Results
Discussion
References
 
Eight patients without AIDS who had pathologically proven multilocular thymic cyst were identified by search of radiologic teaching files accessed over 6 years at three tertiary referral hospitals. The original hospital and related medical records for all patients were obtained for this review. Lesions were diagnosed in two males and six females whose ages ranged from 15 to 58 years (mean, 35 years). In all patients, a diagnosis of multilocular thymic cyst was made when the completely resected specimen showed a thymic lesion characterized by multiple cysts with a prominent inflammatory and fibrotic component, as defined by Suster and Rosai [2]. Clinical records of affected patients were reviewed, and presenting complaints, associated conditions, and follow-up results were noted.

Six patients had CT scans obtained before and after administration of IV contrast material; contrast-enhanced scans were obtained in two patients. Contiguous 8- or 10-mm sections (n = 2 or n = 6, respectively) were obtained after bolus injection of 100 mL of iopromide (Ultravist 300; Schering, Berlin, Germany) in two patients and during drip infusion of 50 mL of various contrast media after bolus injection of 50 mL in the other six patients. All images were printed at a window width of 350-450 H and a window level of 20-35 H.

All CT examinations were analyzed simultaneously by two chest radiologists; findings were recorded by consensus. CT scans were evaluated for lesion size, shape, laterality (unilateral or bilateral), calcification, and presence of mass effect on adjacent structures. Border characteristics were classified as well marginated or poorly defined. Lesions with well-marginated borders were further classified as smooth (spherical), lobulated, or normal thymus-shaped. The presence of cystic (defined as an area of water attenuation equivalent to that of the gallbladder) or solid (defined as an area of soft-tissue attenuation with thickness greater than that of the adjacent cyst wall) components was noted. When present, the maximum horizontal dimension of the solid component was measured.

Perceptibility of the cyst wall on CT was recorded. We primarily evaluated cyst walls adjacent to the mediastinum because the thickness of cyst walls adjacent to the lung might be overestimated because of adjacent atelectatic lung or thickened pleura. The cystic portion of the lesions, when present, was characterized as either unilocular or multilocular. Lesion attenuation and pattern of contrast enhancement were also recorded. When only contrast-enhanced CT scans were available, enhancement of the lesion was considered present when its attenuation exceeded that of the skeletal muscle. Associated findings in the lung, pleura, and mediastinum were also noted.

Descriptions of the gross pathologic specimens were available in all eight patients; 35-mm slides of the gross specimens were available in five patients. H and E—stained microscopic sections were available in all eight patients and were reviewed by an experienced pathologist to confirm the diagnosis. CT findings, with particular attention to the cyst wall, internal septa, and solid components, were correlated with histopathologic features. The time interval between CT and the histopathologic study ranged from 3 to 14 days.


Results
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Abstract
Introduction
Materials and Methods
Results
Discussion
References
 
Clinical Findings
Five patients were asymptomatic at presentation; three complained of chest pain, dyspnea, fever, or hemoptysis. The five asymptomatic patients underwent chest CT for the evaluation of myasthenia gravis (n = 3) or anterior mediastinal masses detected on chest radiographs (n = 2). Associated diseases included myasthenia gravis (n = 4) and systemic lupus erythematosus (n = 2). A small focus of thymoma was incidentally diagnosed at histopathologic examination in one patient. No patient had HIV infection, a history of prior thoracotomy or median sternotomy, or a history of prior mediastinal malignancy. All patients were treated by surgical excision and had an uneventful recovery. On follow-up examination, six patients were alive without evidence of cyst recurrence 12-96 months (mean, 34 months) after surgery. Two patients were lost to follow-up.

CT Findings
On CT, all lesions manifested as anterior mediastinal masses inseparable from the thymus. Five lesions were unilateral (four, right; one, left) and three were bilateral. They ranged from 4 to 9 cm (mean, 6.5 cm) in maximum dimension. Seven lesions were well marginated; of these, five were smooth, one was lobulated, and one conformed to the normal shape of the thymus. One lesion had poorly defined margins and one exerted mass effect upon adjacent mediastinal structures.

On contrast-enhanced CT (n = 8), six lesions were heterogeneous and had solid components that ranged from 8 to 23 mm (mean, 14 mm) in maximum horizontal dimension (Figs. 1A,1B,1C,2A,2B,2C,3,4). The cystic components of these six lesions were multilocular in three patients (Figs. 1A,1B,1C and 2A,2B,2C) and unilocular in three (Figs. 3 and 4). Two of the eight lesions did not have solid components; one of these lesions was unilocular and the second was multilocular. In seven of the eight patients, thymic cyst(s) occupied more than half the total volume of the lesion. In the eighth patient, the cystic component was so small that the mass was initially misdiagnosed as purely solid. All lesions had clearly definable walls around the entire (n = 1) or partial (n = 7) circumference of the cyst (Figs. 1A,1B,1C,2A,2B,2C,3,4). Cyst walls, internal septa, and solid components enhanced uniformly and became more distinct after contrast administration. More septa were seen on enhanced CT than on unenhanced CT in three lesions.



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Fig. 1A. Multilocular thymic cyst in 39-year-old woman with 6-year history of systemic lupus erythematosus. Contrast-enhanced CT scan shows well-defined cyst walls (closed arrow), multiple internal septa, and enhancing soft-tissue attenuation components (open arrows) more easily distinguished from cysts than on unenhanced CT.

 


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Fig. 1B. Multilocular thymic cyst in 39-year-old woman with 6-year history of systemic lupus erythematosus. Photograph of gross specimen shows cystic mass involving both lobes of thymus. Cyst contents are variable. Note prominent soft-tissue component in cephalad aspect of right lobe of thymus (arrows), corresponding to enhancing portion of mass (open arrows, A) on enhanced CT.

 


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Fig. 1C. Multilocular thymic cyst in 39-year-old woman with 6-year history of systemic lupus erythematosus. Photomicrograph shows multiple cysts containing eosinophilic fluid (F) and intervening hyperplastic thymic tissue (T). (H and E, x1)

 


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Fig. 2A. Multilocular thymic cyst in 29-year-old woman with 8-month history of myasthenia gravis. Contrast-enhanced CT scan at level of aortic arch shows well-marginated multilocular cystic mass in anterior mediastinum. Note small nodular calcifications (arrowhead) in cyst wall (arrow) and soft-tissue attenuation.

 


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Fig. 2B. Multilocular thymic cyst in 29-year-old woman with 8-month history of myasthenia gravis. Contrast-enhanced CT scan at level of carina shows multilocular cystic mass (arrows) with soft-tissue attenuation.

 


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Fig. 2C. Multilocular thymic cyst in 29-year-old woman with 8-month history of myasthenia gravis. Photograph of gross specimen shows many small cysts separated by thick fibrous septa in right thymic lobe (R) and two large cysts in left thymic lobe (L). Note dark hemorrhagic fluid in left lobe cysts and yellow fluid in right lobe cysts. Number of right lobe cysts is greater than noted on CT (A and B). Also note nodular and linear calcifications in left lobe cyst wall (open arrows) corresponding to calcification (arrowhead, A) on CT. Aggregated small cysts (black arrowheads) in top of right thymic lobe and soft-tissue region (white arrowheads) in lower portion of left thymic lobe (arrows, in A and B) corresponded to soft-tissue attenuation components on CT.

 


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Fig. 3. Multilocular thymic cyst associated with incidental thymoma in asymptomatic 25-year-old man. Contrast-enhanced CT scan shows smooth unilocular cystic mass in right anterior mediastinum. Note focal thickening of medial cyst wall (arrows) corresponding to thymoma in cyst wall of pathologic specimen (not shown).

 


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Fig. 4. Multilocular thymic cyst in asymptomatic 58-year-old woman. Contrast-enhanced CT scan shows homogeneous, unilocular cystic mass (arrows) in anterior mediastinum. Only inferior portion of mass had perceptible cyst wall (not shown).

 

Small calcifications were seen in the cyst wall in three lesions (Fig. 2A,2B,2C). In three lesions, the attenuation of cystic components was measured and ranged from -20 to 17 H (mean, 3 H). Bilateral pleural effusions were noted in one patient with systemic lupus erythematosus, and focal pneumonic consolidation was noted in one patient.

CT—Histopathologic Correlation
All eight lesions were multilocular at histopathologic examination. However, four of the eight lesions appeared unilocular on CT (Figs. 3 and 4). And, although the other four lesions appeared multilocular on CT, the number of cysts revealed at histopathologic examination was always greater than the number seen on CT (Figs. 1A,1B,1C and 2A,2B,2C). Correlation of CT findings with available pathologic material suggested several reasons for these discrepancies. First, some of the lesions were composed of a single dominant cyst with multiple small associated cysts that were likely too small to be revealed as separate cysts on thick-section CT (Fig. 4). Second, the internal septa of one lesion were much too thin to be visualized on thick-section CT. Third, two lesions had cysts that were filled with proteinaceous debris or blood and thus may have appeared as soft-tissue components on CT, a phenomenon described in other thymic cysts [8].

Six lesions had solid components seen on CT. At histopathologic examination, these components were found to represent thymoma (n = 1) or various nonneoplastic tissues (n = 5) such as thymic hyperplasia (n = 3), normal thymic remnants (n = 2), hemorrhagic cysts (n = 2), or numerous small cysts septated by thick inflammatory thymic tissue (n = 1). In all patients, cyst walls were thick and fibrous and encompassed the entire perimeter of the cyst at histopathologic examination. Linear or nodular calcific foci were histopathologically identified in the cyst wall in four patients (Fig. 2C), including the three lesions that had calcification visible on CT.


Discussion
Top
Abstract
Introduction
Materials and Methods
Results
Discussion
References
 
Multilocular thymic cysts are acquired lesions of the thymus and are most likely caused by an inflammatory process [2]. In many cases—perhaps most—the specific cause of inflammation is unknown. Multilocular thymic cysts have been reported in patients with Sjögren's syndrome [2], aplastic anemia [2], and myasthenia gravis [8], suggesting the possibility of an immune-mediated inflammatory process. In our study, two of the eight patients had systemic lupus erythematosus and four had myasthenia gravis. Although a pathogenic role for HIV infection [6, 7], radiation [9], or surgical trauma [10] has also been suggested, no such cases were included in this study.

True multilocular thymic cysts can occur in association with thymic neoplasia, including thymoma [2, 4] and thymic carcinoma [2]; one of our patients had a small focus of thymoma. In addition, up to half the patients with nodular sclerosing Hodgkin's disease or seminoma of the anterior mediastinum have cystic thymic lesions that are histopathologically identical to idiopathic multilocular thymic cysts [2]. Cystic degeneration of a thymoma can also result in a gross pathologic appearance that simulates multilocular thymic cyst. Thus, in cases of suspected multilocular thymic cyst, it is of vital importance that the histopathologic specimen be carefully inspected to exclude coexisting neoplasia [2].

Seven (88%) of the eight multilocular thymic cysts in our series manifested on CT as well-defined, heterogeneous, unilocular, or multilocular cystic masses of the thymus. In all patients, walls were clearly evident and surrounded either part of, or the entire perimeter of, the cyst. The radiologic differential diagnosis for such lesions of the anterior mediastinum includes cystic teratoma, lymphangioma, hemangioma, and, as noted earlier, cystic degeneration of seminoma, Hodgkin's disease, and thymoma. Cystic teratoma cannot be confidently differentiated from multilocular thymic cyst because cystic teratona commonly manifests as a unilocular or multilocular cystic mass and frequently calcifies. Identification of fat, cartilaginous, or tooth-like calcification in the lesion suggests teratoma.

As opposed to multilocular thymic cysts, lymphangiomas are usually contiguous with a cervical or axillary component, may infiltrate across tissue planes, and enhance only minimally after IV contrast administration [11]. However, hemangiomas enhance intensely after IV contrast administration [12]. In our experience, congenital thymic cysts typically do not have a definable wall on CT, probably because cyst walls are typically very thin at histopathologic examination [2, 3]. Thus, the presence of a clearly definable cyst wall on CT, or the presence of calcification within the wall, argues for the diagnosis of multilocular thymic cyst. However, only the cyst walls adjacent to the mediastinum should be evaluated because the presence and thickness of the wall adjacent to the lung might be overestimated as a result of adjacent compressed lung or thickened pleura.

Solid components that were identified on CT in six (75%) of the eight patients did not usually represent thymic neoplasm but corresponded to various benign tissues. In only one of the six patients was a small thymoma discovered. Nevertheless, because neoplasia cannot be confidently differentiated from multilocular thymic cyst on CT, these lesions must undergo resection and careful histopathologic examination. Clues to alternative diagnoses include associated lymphadenopathy in cases of lymphoma and pleural metastases in cases of thymoma. Invasive features on CT suggest malignancy. Elevated serum levels of ß-human chorionic gonadotropin or {alpha}-fetoprotein suggest malignant germ cell tumor.

In summary, multilocular thymic cysts are rare acquired lesions of the thymus that most likely result from inflammation but may also be associated with neoplasia. They typically manifest on CT as unilocular or multilocular cystic masses. The cysts have well-defined walls, and the lesions frequently have prominent soft-tissue attenuation components. Because CT cannot be used to reliably distinguish neoplastic from non-neoplastic soft-tissue components, complete surgical resection and careful histopathologic examination is recommended in all patients with suspected multilocular thymic cyst.


References
Top
Abstract
Introduction
Materials and Methods
Results
Discussion
References
 

  1. Davis RD Jr, Oldham HN Jr, Sabiston DC Jr. Primary cysts and neoplasms of the mediastinum: recent changes in clinical presentation, methods of diagnosis, management and results. Ann Thorac Surg 1987;44:229 -237[Abstract]
  2. Suster S, Rosai J. Multilocular thymic cyst: an acquired reactive process—study of 18 cases. Am J Surg Pathol 1991;15:388 -398[Medline]
  3. Indeglia RA, Shea MA, Grage TB. Congenital cysts of the thymus gland. Arch Surg 1967;94:149 -152[Abstract/Free Full Text]
  4. Liang SB, Ohtsuki Y, Sonobe H, et al. Multilocular thymic cysts associated with thymoma: a case report. Path Res Pract 1996;192:1283 -1287
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  6. Avila NA, Mueller BU, Carrasquillo JA, Kontny HU, Jaffe ES, Pizzo PA. Multilocular thymic cysts: imaging features in children with human immunodeficiency virus infection. Radiology 1996;201:130 -134[Abstract/Free Full Text]
  7. Leonidas JC, Berdon WE, Valderrama E, et al. Human immunodeficiency virus infection and multilocular thymic cysts. Radiology 1996;198:377 -379[Abstract/Free Full Text]
  8. Brown LR, Muhm JR, Sheedy PF, et al. The value of computed tomography in myasthenia gravis. AJR 1983;140:31 -35[Free Full Text]
  9. Shapiro J, Gallant A, Wechsler RJ, Steiner RM. Thymic cyst secondary to cervical irradiation: a complication of treated laryngeal cancer. Comput Med Imaging Graph 1991;15:319 -322[Medline]
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  11. Shaffer K, Rosado-de-Christenson ML, Patz EF Jr, Young S, Farver CF. Thoracic lymphangioma in adults: CT and MR imaging features. AJR 1994;162:283 -289[Abstract/Free Full Text]
  12. Seline TH, Gross BH, Francis IR. CT and MR imaging of mediastinal hemangiomas. J Comput Assist Tomogr 1990;14:766 -768[Medline]

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