Case Report Urol Int 1992;49:171-174

Noriyoshi Yamakitaa Miyuki Sugimotoa Noriyuki Takeda3Shinobu Gotoa Keigo Yasudaa Hiromi Unob Kuniyasu Shimokawac Kiyoshi Miura3

Pseudo-Adrenal Incidentaloma: Magnetic Resonance Imaging in a Patient with Para-Adrenal Castleman's Disease

Third Department of Internal Medicine, Department of Urology, and Department of Clinical Laboratory Medicine, Gifu University School of Medicine, Gifu, Japan

Abstract

Introduction

Case Report

The development and recent increase in the use of imaging procedures in abdominal examinations have given rise in the incidental discovery of adrenal masses, the so-called adrenal incidentaloma [1, 2]. This disease entity, however, must be distinguished from incidentally discovered para-adrenal masses. Castleman’s disease is a lymphogenic proliferative dis­ ease of unknown etiology [3, 4], It is also known as giant lymph node hyperplasia [4-6], angiofollicular lymph node hyperplasia [7], and angiomatous lymphoid hamartoma [8,9]. It is a relatively rare disorder and usually develops in the mediastinum. Although there have been several re­ ports describing its appearance by computed axial tomog­ raphy (CT), there has been little information on its charac­ teristic image by magnetic resonance imaging (MRI) [ 10], In this article, we present a rare case of para-adrenal Cas­ tleman’s disease which was investigated using MRI.

Received: December 23. 1991 Accepted: March 10, 1992

A 37-year-old female consulted a physician with a complaint of heartburn. She was referred to Gifu University Hospital because of the ultrasonographic finding of a left suprarenal mass which was thought to be an adrenal mass. On physical examination, no abnor­ mal symptoms and signs suggestive of adrenal disorders and other diseases were noted. Laboratory' results showed normal complete blood counts, renal and hepatic functions and immunological tests. The endocrine function of the adrenal cortex and medulla was found to be completely normal. Chest roentgenography, barium examina­ tion of the upper gastrointestinal tract and colon, and excretory urog­ raphy showed no remarkable findings. l3lI-Iodocholesterol adrenal scintigraphy demonstrated symmetrical uptake of adrenals. Ultrasonography was performed with YGM RT-3600™ (Yokogawa Medical Co., Tokyo) with a 3.5-MHz transducer. A suprarenal round mass was delineated when scanned from the left flank. The mass image was solid, homogenous, and hypoechoic (fig. la). CT was performed with GE CT/T 8800™ (General Electric Co., Milwaukee, Wise., USA), with a matrix number of 250 X 250, a scanning dis­ tance of 5 mm and a width of 5 mm. The mass image was round and relatively homogeneous on plain CT, and was not enhanced by con-

Noriyoshi Yamakita, MD Institute of Clinical Medicine University ofTsukuba Tsukuba City 305 (Japan)

€> 1992 S. Karger AG. Basel 0042-1138/92/0493-0171 $2.75/0

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Keywords

Castleman's disease Giant lymph node hyperplasia Angiofollicular lymph node hyperplasia Adrenal incidentaloma

We report a case of para-adrenal angiofollicular lymph node hyperplasia (Castleman’s disease) of the hyaline-vascular type. The mass could not be differen­ tiated from an adrenal tumor by ultrasonography and computed axial tomog­ raphy (CT). However, magnetic resonance imaging (MRI) suggested the possi­ bility of an extra-adrenal origin of the mass. The intensity of the mass by MRI was homogeneous and of a higher intensity in the T2-weighted image than in the T1 -weighted image, a finding similar to lymphadenopathy, lymphatic tumorous mass or metastatic tumor of the lymph node. Ultrasonography, CT and MRI may not be useful in characterizing Castleman’s disease, but MRI was useful to distinguish asymptomatic para-adrenal masses from those of adrenal origin.

Fig. 2. Axial images by MRI. a-d Tl-weighted images, e, f T2-weightcd images. Arrow­ heads show larger mass images, (a, b, e). The small arrow and asterisk show the left adrenal gland (b, c). The broad arrow and asterisk show smaller mass images (d, f).

trast medium (fig. lb). However, neither ultrasonography nor CT could distinguish whether the mass was adrenal or extra-adrenal. MRI was performed with a GE Signa™ superconducting magnet operating at 1.5 T. Images were obtained by spin echo pulse sequences. Tl - and T2-weighted images were obtained in the situa­ tions with repetition times of 600 and 2,000 ms, and echo times of 20 and 60 ms, respectively. MRI demonstrated two masses (fig. 2, 3): a larger one located in the left suprarenal region (fig. 2a, b, e, 3a-d) and the other smaller one located in the hilar region of the left kidney in the axial (fig. 2d, 0 and coronal images (fig. 3e). With regard to the axial images, the wing of the left adrenal gland was depicted together with the larger mass in one slice (fig. 2b). In another slice, however, the left adrenal gland but not the larger mass was demonstrated (fig. 2c). These findings suggested the possibility that the origin of the larger mass was extra-adrenal. Both masses were iso-intense to the liver on Tl-weighted image (fig. 2a-d) and were of higher intensity than the liver on T2-weighted images (fig. 2e, f). From these data, a retroperitoneal lymphogenic or neurogenic tumor was suspected, although the possibility of an adrenal mass could not be totally excluded.

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Intraoperatively, a dark yellowish mass, which was found to be unattached to the left adrenal gland, was resected. Moreover, five smaller masses (diameter 0.5-1.0 cm) in the retroperitoneum, one of which was detected by CT and MRI. were found and resected. The largest mass was 4.4 X 3.0 X 1.7 cm in size and 15.4 g in weight. It was capsulatcd but not calcified. Microscopic study of the mass revealed angiofollicular lymph node hyperplasia (Castleman’s dis­ ease) with small hyalinized follicular centers with radially penetrat­ ing vascular vessels and prominent interfollicular vascular prolifera­ tion (hyaline-vascular, HV type; fig. 4). However, only inflammatory findings of lymph nodes were found in all the other smaller masses resected. This might have been attributable to the inflammatory reaction to the previous cesarean section of this patient. The patient has remained free of disease for 30 months until the present.

Yamakita/Sugimoto/Takeda/Goto/Yasuda/ Uno/Shimokawa/Miura

Para-Adrenal Castleman’s Disease

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Fig. 1. a Ultrasonography of the larger mass, b CT image after administration of contrast material. Arrowheads show larger mass images.

Fig. 3. Sagittal and coronal images by MRI. All are Tl-weighted images, a-c Sagittal images, d, e Coronal images. Arrowheads show larger mass images (a-d). The small arrow and asterisk show the left adrenal gland (a, b). The large arrow and asterisk show a smaller mass image (e).

Castleman et al. [3] first reported angiofollicular lymph node hyperplasia in 1956 as a distinct pathologic entity. Thereafter, Keller et al. [4] further studied this rare disorder and divided it into two groups. The first group is characterized microscopically by small HV follicles and interfollicular capillary proliferation (HV type), and the second by large follicles with intervening sheets of plasma cells (plasma-cell, PC type). The former group usually has no abnormal symptoms and laboratory data, but the latter frequently shows systemic manifestations such as fever, anemia, hepatosplenomegaly and polyclonal hyperglobulinemia suggestive of systemic inflammation. Both groups, however, often show only solitary lesions in the mediastinum and the prognosis after resection of the tumor is good. However, multicentric lesions [11], which encompass both pathologic classifications, often show severe systemic symptoms and usually have a poor prog­

nosis, but they are rarely found. Although several etiologi­ cal hypotheses have been proposed [4-6, 8, 12], this disor­ der is likely to be caused by some immunological reac­ tions to inflammation [4-6], Since the most common type of the disease is HV, which is usually asymptomatic and found in the mediasti­ num [4, 13, 14], it is incidentally discovered by chest roentgenogram in most cases [15-17], Retroperitoneal development of these masses is considerably rare. Ac­ cording to Honda et al. [ 14], who analyzed 439 cases, only 28 lesions (6.4%) were found in the retroperitoneum. Moreover, the occurrence of the lesions in the pararenal area is much lower, 1.9 % (6 of 315 cases) in the review by Testas et al. [ 13], The absence of any systemic manifesta­ tion, the normal laboratory results and the ultrasonogra­ phic and CT findings in the present case posed difficulty in distinguishing whether the mass was adrenal or extra­ adrenal in origin. When HV-type Castleman’s disease develops around the adrenal glands, it is not easy to differ­

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Discussion

Fig. 4. Macroscopic finding of the re­ sected para-adrenal Castleman’s tumor and its microscopic finding (HV type).

entiate it from an adrenal mass, even if it is distinguish­ able from a renal mass. The ultrasonographic findings of Castleman’s disease were reported to be soft and hypoechoic [ 10, 18-21 ]. On CT, it is being depicted as a homo­ geneous soft mass [ 10, 15- 21], However, there is some controversy in the findings after the administration of contrast medium; either there is an enhancement [17, 18, 21 ], especially an early and homogeneous enhancement on the postcontrast dynamic CT study [ 16, 21], or no enhancement [ 19,21 ] as in the present case. Such a differ­ ence in enhancement has no correlation with the histo­ logic type (HV or PC type). Several cases of Castlcman’s disease show calcified shadows which are said to be char­ acteristic of the disease [10, 17, 18], but no calcification was found in our patient. The MRI findings of Castleman’s disease in this pa­ tient were not different from those of lymphadenopathy which usually is homogeneous and of low intensity in T lweighted images and of higher intensity in T2-weighted images [22, 23]. It is unlikely that Castleman’s disease can be distinguished by MRI from inflammatory lymphade­

nopathy and tumorous lymphatic lesions such as Hodg­ kin’s lymphoma or metastasis [22]. The MRI findings of neurogenic tumors also show almost the same features. Castleman’s disease could not, therefore, be character­ ized by imaging procedures such as ultrasonography, CT and MRI. However, since MRI can display the lymph nodes and adrenal gland more clearly in T1-weighted images than CT, because of its excellent soft-tissue con­ trast resolution, the left adrenal gland could be delineated separately from the mass in a few slices, suggesting the possibility of extra-adrenal origin. MRI is a suitable method to investigate an asymptomatic retroperitoneal mass, especially in the para-adrenal area.

Acknowledgment This study was partially supported by grants from the Commit­ tees on ‘Disorders of Adrenal Hormones, 1990-1991’ and ‘Dysfunc­ tion of Hypothalamo-Pituitary Gland, 1990-1991’ of the Japanese Ministry' of Health and Welfare.

1 Copeland PM: The incidentally discovered ad­ renal mass. Ann Intern Med 1983:98:940— 945. 2 Yamakita N, Saitoh M. Mercado-Asis LB, Kitada M. Morita H. Yasuda K. Miura K: Asymptomatic adrenal tumor: 386 cases in Ja­ pan including our 7 cases. Endocrinol Jpn 1990;37:671-684. 3 Castleman B, Iverson L, Meneudex V: Local­ ized mediastinal lymph-node hyperplasia re­ sembling thymoma. Cancer 1956:9:822-830. 4 Keller AR. Hochholzer L. Castleman B: Hyal­ ine-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1972:29:670-683. 5 Diamond LW, Braylan RC: Immunologic markers and DNA content in a case of giant lymph node hyperplasia (Castleman’s disease). Cancer 1980:46:730-735. 6 Dura WT, Miduszewska O. Porwit-Ksiazek A: Cytoplasmic immunoglobulins in giant lymph node hyperplasia (Castleman’s tumor). Vir­ chows Arch [B] 1981:38:239-246. 7 Harrison EG, Bcrnatz PF.: Angiofollicular me­ diastinal lymph node hyperplasia resembling thymoma. Arch Pathol 1963:75:284-292. 8 Abell MR: Lymphnodal hamartoma versus thymic choristoma of pulmonary hilum. Arch Pathol 1957;64:584-588. 9 Tung KSK, McCormack LJ: Angiomatous lym­ phoid hamartoma. Cancer 1967;20:525-536.

10 Bartkowski DP, Fcrrigni RG: Castleman’s dis­ ease: An unusual retroperitoneal mass. J Urol 1988:139:118-120. 11 Weisenburger DD. Nathwani BN, Winberg CD. Rappaport H: Multiccntric angiofollicular lymph node hyperplasia: A clinicopathologic study of 16 cases. Hum Pathol 1985:16:162— 172. 12 Zettergren L: Probably neoplastic proliferation of lymphoid tissue (follicular lympho-rcticuloma). Acta Pathol Microbiol Scand 1961:51: 113-126. 13 Testas P, Pigne A. Voinnesson A. Vieillefond A. Paillas J: Les hyperplasies lymphoïdes angiofolliculaires (maladie de Castleman). Prem­ ière localization mésosigmoïdicnne. Chirurgie 1980:106:156-160. 14 Honda K. Yoshioka M. Nakamura M, Kobayashi K. Ishii H. Tsuchiya M, Tsuzuki T, Tashiro M. Hosoda Y, Kano S. Hagiwara T: A case of Castleman lymphoma (plasma cell type) in retroperitoncum with high titer of anti EB virus antibodies. Jpn J Gastroenterol 1984:81:10831088. 15 Olscamp G. Weisbrod G, Sanders D, Delarue N, Mustard R: Castleman’s disease: Unusual manifestations of an unusual disorder. Radiol­ ogy 1980;135:43-48. 16 Onik G. Goodman PC: CT of Castleman dis­ ease. Am J Roentgenol 1983;140:691-692. 17 Charing MJ: Case report. Mediastinal Castleman’s disease: A missed pre-operative diagno­ sis? Clin Radiol 1990:42:440-442.

18 Joseph N, Vogelzang RL, Hidvegi D. Neiman HL: Computed tomography of retroperitoneal Castleman disease (plasma cell type) with sono­ graphic and angiographic correlation. J Cornput Assist Tomogr 1985;9:570-572. 19 Libson E. Fields S, Strauss S. Bloom RA. Okon E. Galun E, Polliack A: Widespread Castleman disease: CT and US findings. Radiology 1988; 166:753-755. 20 Ebisuno S, Yamauchi T. Fukatani T, Ohkawa T: Retroperitoneal Castleman’s disease: A case report and brief review of tumors of the para­ renal area. Urol Int 1989:44:169—172. 21 Ferreiros J. Gomez Leon N. Mata ML Casa­ nova R, Pedrosa CS. Cuevas A: Computed tomography in abdominal Castleman’s disease. J Comput Assist Tomogr 1989:13:433—436. 22 Lee JK, Heiken JP. Ling D, Glazer HS. Balfe DM. Levitt RG, Dixon WT. Murphy WA: Magnetic resonance imaging of abdominal and pelvic lymphadenopathy. Radiology 1984.153: 181-188. 23 Dooms GC, Hricak H, Crooks LE. Higgins CB: Magnetic resonance imaging of lymph nodes: Comparison with CT. Radiology 1984:153: 719-728.

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Para-Adrenal Castlcman’s Disease

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References

Pseudo-adrenal incidentaloma: magnetic resonance imaging in a patient with para-adrenal Castleman's disease.

We report a case of para-adrenal angiofollicular lymph node hyperplasia (Castleman's disease) of the hyaline-vascular type. The mass could not be diff...
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