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781
Case Report
Castleman Features Jorg
F. Debatin,1
Disease Charles
of the Adrenal
E. Spritzer,
and N. Reed
manifests in the thorax. involved [i].
The
adrenal
MR imaging can be used to distinguish nant causes of adrenal masses. However, be distinguished by using MR, even with ment [2, 3]. We present a case of a benign of the hyaline
vascular
has the MR imaging of a malignant
type
affecting
glands
are
benign from malignot all lesions can contrast enhanceCastleman tumor
an adrenal
signal and enhancement
MR Imaging
Dunnick
Castleman disease is a benign lymphoid neoplasm. Although it can arise wherever Iymphoid tissue is found, it most frequently only rarely
Gland:
gland
During laparotomy, a dark yellow tumor, measuring 5 x 6 cm, that had replaced the right adrenal gland was resected en bloc. Histologic examination revealed giant lymph node hyperplasia (Castleman disease) of the hyaline vascular type. Surrounding lymph nodes were normal, and the postoperative convalescence was uneventful.
Discussion Castleman disease is a benign proliferation of mature lymphocytes and/or plasma cells, with preservation of the lymph node architecture [i ]. Also referred to as giant lymph node
that
characteristics
lesion.
hyperplasia, this benign lymphoid neoplasm was first reported by Castleman and Towne [4] and was thought to represent
Case Report
hyperplasia
A 43-year-old underwent
woman
excretory
with
a long
urography
for
history
of arterial
hematuria.
enal mass was found, and subsequent
hypertension
A right-sided
CT examination
suprar-
confirmed
a
right adrenal mass measuring 4.7 x 3.0 x 3.2 cm (Fig. 1 A). Laboratory analysis showed the lesion was not hyperfunctioning. The mass was stable for 3 years until July 1 990, when it increased in size to 5.5 x 6.1 x 5.8 cm. An MR examination showed the mass was hypointense to liver on Ti -weighted images and uniformly hyperintense on T2-weighted images (Figs. 1 B and 1C). The calculated
T2 value obtained from a multislice, two-echo image (2500/40, 80 [TR/TE]) was 70 msec (Fig. 1 D). After IV administration of gadopentetate dimeglumine, the mass showed in its signal intensity and relatively
a more than threefold slow washout (Fig.
minutes
the
after
contrast
administration,
signal
intensity
increase 1 E). Ten
of the mass
was still twice as high as before contrast injection (Figs. 1 E-1G). The calculated T2 value and contrast enhancement characteristics were consistent with a malignant tumor of the adrenal gland. Received I
March 11 , 1991 ; accepted
All authors: Radiology Department,
AJA 157:781-783,
of mediastinal
lymph
nodes.
Two
distinct
histo-
logic patterns are recognized. The hyaline vascular variety, accounting for up to 90% of Castleman tumors, is usually asymptomatic [5]. Histologically, this type is characterized by small, flat, concentrically layered, follicle-centered cells, exten-
sive capillary proliferation, and defacement of lymphoid sinuses. Various amounts of hyaline tissues encase the proliferated capillaries [5]. The plasma cell subtype is a much less common form of Castleman tumor and is characterized by the presence of sheets of mature plasma cells [5]. The follicle centers are normal, without evidence of vascularization or hyalinization. About half these patients have systemic signs and symptoms; the most
common
are anemia,
fever,
fatigue,
hyperglobuli-
nemia, and hypoalbuminemia. Whereas the hyaline vascular subtype is usually a solitary lesion between i .5 and 16.0 cm in size, the plasma cell
after revision April 30, 1991. Box 3808, Duke University Medical Center, Erwin Rd. , Durham,
October 1991 0361-803x/91/1
574-0781
© American
Aoentgen
Ray Society
NC 2771 0. Address
reprint
requests
to N. A. Dunnick.
Downloaded from www.ajronline.org by 177.87.223.162 on 10/14/15 from IP address 177.87.223.162. Copyright ARRS. For personal use only; all rights reserved
782
DEBATIN
.
ETAL.
AJA:157,
October
1991
1:?
ILl
LD
Fig. 1.-A, Contrast-enhanced CT scan shows a 4.7 x 3.2 cm right adrenal mass (arrow). Mass is of homogeneous attenuation without evidence of calcification, necrosis, or hemorrhage. B, Coronal Ti-weighted (500/20) MR image shows a large right adrenal mass (arrow) characterized by homogeneous signal mildly hypointense to that of hepatic parenchyma. C, Axial T2-weighted (2500/80) MR image shows a uniformly hyperintense right adrenal mass. D, Absolute T2 value of adrenal lesion was 70.8 msec. E-G, Coronal spoiled gradient-recalled echo images (28/5, flip angle 45#{176}) obtained before (E), 3 mm after (F), and 10 mm after (G) IV administration of gadopentetate dimeglumine (0.1 mmol/kg) show rapid contrast enhancement and relatively slow washout of paramagnetic contrast agent from right
adrenal mass.
subtype surgical
may be either focal or multifocal [i , 5]. Complete resection is the treatment of choice and is generally
curative.
As in this case,
Castleman
tumors
may enlarge
over
time [1]. Castleman disease occurs most often in young, otherwise healthy patients. Males and females are equally affected [i] and range in age from 8 to 66 years. Castleman tumors may occur in almost any area in which lymphoid tissue is normally found [1]. Of the 400 reported cases [6], 70% involved the thorax, 40% the neck, 12% the abdomen, and 4% the axilla. Most intraabdominal lesions are located in the pelvic, enteric, and perinephric regions [7], but they have described
throughout
the
The only other report adrenal gland described in an asymptomatic tion of the surgical
mesbeen
abdomen.
of Castleman a voluminous
47-year-old
woman.
specimen showed associated with an adrenal myelolipoma man tumor [8]. It is conceivable that in present case, the Castleman tumor may
disease involving the retroperitoneal tumor Histologic
examina-
adrenal hyperplasia adherent to a Castlethat case, and in the have originated from
lymphoid
tissue
in the adrenal
periphery
and
extended into the adrenal gland itself. The MR appearance of Castleman disease
subsequently
in the abdomen
has not been described. In our patient, the signal characteristics were similar to those reported in adrenal lymphoma:
hypointense compared with hepatic parenchyma weighted images and hypenntense on T2-weighted
on Ti images
-
[3]. Several investigators reported that most lesions (80%) with a T2 value greater than 60 msec are malignant [2]. According to this criterion, the calculated T2 value of 70 msec indicated
a malignant cause. Similarly, the prompt increase in signal intensity after the IV injection of gadopentetate dimeglumine and the delayed washout of the paramagnetic contrast agent from the lesion strongly suggest a malignant cause according to criteria reported by Krestin et al. [3]. This may be a reflection of the extensive capillary proliferation typical of
Castleman tumors of the hyaline geneity of the signal characteristics administration
was
unusual
vascular form. The homobefore and after contrast
for a malignant
tumor.
In a me-
AJA:157,
October
MR
1991
OF
CASTLEMAN
tastasis or a primary adrenal carcinoma of this size, areas of cystic degeneration and tumor necrosis are expected. This case emphasizes that signal intensities and enhancement characteristics of MR cannot be applied to all adrenal
Downloaded from www.ajronline.org by 177.87.223.162 on 10/14/15 from IP address 177.87.223.162. Copyright ARRS. For personal use only; all rights reserved
lesions.
Castleman
tumor
of the adrenal
gland
has to be
added to the list of exceptions to the rule that benign adrenal masses have a relatively short T2 and only mild enhancement with quick washout after administration of gadopentetate
DISEASE
783
2. Baker ME, Blinder A, Spritzer C, Leight GS, Herfkens AJ, Dunnick NA. MR evaluation of adrenal masses at 1.5 T. AJR 1989;153:307-312 3. Krestin GP, Steinbrich W, Friedmann G. Adrenal masses: evaluation with fast gradient-echo MR imaging and Gd-DTPA enhanced dynamic studies. Radiology 1989;171 :675-680 4. Castleman B, Towne VW. Case records of the Massachusetts General Hospital: case 4001 1 . N EngI J Med 1954;250:26-30 5. Goldberg MA, Deluca SA. Castleman’s disease. Am Fam Physician 1989;49: 151 -1 53 6. Fizzera G. Castleman’s disease: more questions than answers. Hum Pathol
1985:3:202-205
dimeglumine. REFERENCES 1 . Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer i972;29:670-683
7. Ebisana S, Yamauchi T, Fukani T, Ohkawa T. Aetroperitoneal Castleman’s disease: a case report and brief review of tumors of the pararenal area. Urol Int 1989;44: 169-172 8. Seniuta P, Cazenave-Mahe JP, Le Treut A, Trojani M. Adrenal myelolipoma and Castleman’s pseudotumor: a case of association in a retroperitoneal tumor (review). J Urol (Paris) 1989:95:511-514
American Come
Roentgen to the
Ray Society Scientific
Program
Instructional
(200
Course Neuroradiologv
Categorical
The Caldwell Award Scientific Social,
Golf,
hours) Ofl
Lecture
ANNUAL MEETING
Papers
Orlando,
Exhibits
and Tennis
Guest
92’
tapers)
( 60
Courses
Programs
Programs
Marriott’s
FL
OrlandoWorid May 10-15, 1992
Center
781
Case Report
Castleman Features Jorg
F. Debatin,1
Disease Charles
of the Adrenal
E. Spritzer,
and N. Reed
manifests in the thorax. involved [i].
The
adrenal
MR imaging can be used to distinguish nant causes of adrenal masses. However, be distinguished by using MR, even with ment [2, 3]. We present a case of a benign of the hyaline
vascular
has the MR imaging of a malignant
type
affecting
glands
are
benign from malignot all lesions can contrast enhanceCastleman tumor
an adrenal
signal and enhancement
MR Imaging
Dunnick
Castleman disease is a benign lymphoid neoplasm. Although it can arise wherever Iymphoid tissue is found, it most frequently only rarely
Gland:
gland
During laparotomy, a dark yellow tumor, measuring 5 x 6 cm, that had replaced the right adrenal gland was resected en bloc. Histologic examination revealed giant lymph node hyperplasia (Castleman disease) of the hyaline vascular type. Surrounding lymph nodes were normal, and the postoperative convalescence was uneventful.
Discussion Castleman disease is a benign proliferation of mature lymphocytes and/or plasma cells, with preservation of the lymph node architecture [i ]. Also referred to as giant lymph node
that
characteristics
lesion.
hyperplasia, this benign lymphoid neoplasm was first reported by Castleman and Towne [4] and was thought to represent
Case Report
hyperplasia
A 43-year-old underwent
woman
excretory
with
a long
urography
for
history
of arterial
hematuria.
enal mass was found, and subsequent
hypertension
A right-sided
CT examination
suprar-
confirmed
a
right adrenal mass measuring 4.7 x 3.0 x 3.2 cm (Fig. 1 A). Laboratory analysis showed the lesion was not hyperfunctioning. The mass was stable for 3 years until July 1 990, when it increased in size to 5.5 x 6.1 x 5.8 cm. An MR examination showed the mass was hypointense to liver on Ti -weighted images and uniformly hyperintense on T2-weighted images (Figs. 1 B and 1C). The calculated
T2 value obtained from a multislice, two-echo image (2500/40, 80 [TR/TE]) was 70 msec (Fig. 1 D). After IV administration of gadopentetate dimeglumine, the mass showed in its signal intensity and relatively
a more than threefold slow washout (Fig.
minutes
the
after
contrast
administration,
signal
intensity
increase 1 E). Ten
of the mass
was still twice as high as before contrast injection (Figs. 1 E-1G). The calculated T2 value and contrast enhancement characteristics were consistent with a malignant tumor of the adrenal gland. Received I
March 11 , 1991 ; accepted
All authors: Radiology Department,
AJA 157:781-783,
of mediastinal
lymph
nodes.
Two
distinct
histo-
logic patterns are recognized. The hyaline vascular variety, accounting for up to 90% of Castleman tumors, is usually asymptomatic [5]. Histologically, this type is characterized by small, flat, concentrically layered, follicle-centered cells, exten-
sive capillary proliferation, and defacement of lymphoid sinuses. Various amounts of hyaline tissues encase the proliferated capillaries [5]. The plasma cell subtype is a much less common form of Castleman tumor and is characterized by the presence of sheets of mature plasma cells [5]. The follicle centers are normal, without evidence of vascularization or hyalinization. About half these patients have systemic signs and symptoms; the most
common
are anemia,
fever,
fatigue,
hyperglobuli-
nemia, and hypoalbuminemia. Whereas the hyaline vascular subtype is usually a solitary lesion between i .5 and 16.0 cm in size, the plasma cell
after revision April 30, 1991. Box 3808, Duke University Medical Center, Erwin Rd. , Durham,
October 1991 0361-803x/91/1
574-0781
© American
Aoentgen
Ray Society
NC 2771 0. Address
reprint
requests
to N. A. Dunnick.
Downloaded from www.ajronline.org by 177.87.223.162 on 10/14/15 from IP address 177.87.223.162. Copyright ARRS. For personal use only; all rights reserved
782
DEBATIN
.
ETAL.
AJA:157,
October
1991
1:?
ILl
LD
Fig. 1.-A, Contrast-enhanced CT scan shows a 4.7 x 3.2 cm right adrenal mass (arrow). Mass is of homogeneous attenuation without evidence of calcification, necrosis, or hemorrhage. B, Coronal Ti-weighted (500/20) MR image shows a large right adrenal mass (arrow) characterized by homogeneous signal mildly hypointense to that of hepatic parenchyma. C, Axial T2-weighted (2500/80) MR image shows a uniformly hyperintense right adrenal mass. D, Absolute T2 value of adrenal lesion was 70.8 msec. E-G, Coronal spoiled gradient-recalled echo images (28/5, flip angle 45#{176}) obtained before (E), 3 mm after (F), and 10 mm after (G) IV administration of gadopentetate dimeglumine (0.1 mmol/kg) show rapid contrast enhancement and relatively slow washout of paramagnetic contrast agent from right
adrenal mass.
subtype surgical
may be either focal or multifocal [i , 5]. Complete resection is the treatment of choice and is generally
curative.
As in this case,
Castleman
tumors
may enlarge
over
time [1]. Castleman disease occurs most often in young, otherwise healthy patients. Males and females are equally affected [i] and range in age from 8 to 66 years. Castleman tumors may occur in almost any area in which lymphoid tissue is normally found [1]. Of the 400 reported cases [6], 70% involved the thorax, 40% the neck, 12% the abdomen, and 4% the axilla. Most intraabdominal lesions are located in the pelvic, enteric, and perinephric regions [7], but they have described
throughout
the
The only other report adrenal gland described in an asymptomatic tion of the surgical
mesbeen
abdomen.
of Castleman a voluminous
47-year-old
woman.
specimen showed associated with an adrenal myelolipoma man tumor [8]. It is conceivable that in present case, the Castleman tumor may
disease involving the retroperitoneal tumor Histologic
examina-
adrenal hyperplasia adherent to a Castlethat case, and in the have originated from
lymphoid
tissue
in the adrenal
periphery
and
extended into the adrenal gland itself. The MR appearance of Castleman disease
subsequently
in the abdomen
has not been described. In our patient, the signal characteristics were similar to those reported in adrenal lymphoma:
hypointense compared with hepatic parenchyma weighted images and hypenntense on T2-weighted
on Ti images
-
[3]. Several investigators reported that most lesions (80%) with a T2 value greater than 60 msec are malignant [2]. According to this criterion, the calculated T2 value of 70 msec indicated
a malignant cause. Similarly, the prompt increase in signal intensity after the IV injection of gadopentetate dimeglumine and the delayed washout of the paramagnetic contrast agent from the lesion strongly suggest a malignant cause according to criteria reported by Krestin et al. [3]. This may be a reflection of the extensive capillary proliferation typical of
Castleman tumors of the hyaline geneity of the signal characteristics administration
was
unusual
vascular form. The homobefore and after contrast
for a malignant
tumor.
In a me-
AJA:157,
October
MR
1991
OF
CASTLEMAN
tastasis or a primary adrenal carcinoma of this size, areas of cystic degeneration and tumor necrosis are expected. This case emphasizes that signal intensities and enhancement characteristics of MR cannot be applied to all adrenal
Downloaded from www.ajronline.org by 177.87.223.162 on 10/14/15 from IP address 177.87.223.162. Copyright ARRS. For personal use only; all rights reserved
lesions.
Castleman
tumor
of the adrenal
gland
has to be
added to the list of exceptions to the rule that benign adrenal masses have a relatively short T2 and only mild enhancement with quick washout after administration of gadopentetate
DISEASE
783
2. Baker ME, Blinder A, Spritzer C, Leight GS, Herfkens AJ, Dunnick NA. MR evaluation of adrenal masses at 1.5 T. AJR 1989;153:307-312 3. Krestin GP, Steinbrich W, Friedmann G. Adrenal masses: evaluation with fast gradient-echo MR imaging and Gd-DTPA enhanced dynamic studies. Radiology 1989;171 :675-680 4. Castleman B, Towne VW. Case records of the Massachusetts General Hospital: case 4001 1 . N EngI J Med 1954;250:26-30 5. Goldberg MA, Deluca SA. Castleman’s disease. Am Fam Physician 1989;49: 151 -1 53 6. Fizzera G. Castleman’s disease: more questions than answers. Hum Pathol
1985:3:202-205
dimeglumine. REFERENCES 1 . Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer i972;29:670-683
7. Ebisana S, Yamauchi T, Fukani T, Ohkawa T. Aetroperitoneal Castleman’s disease: a case report and brief review of tumors of the pararenal area. Urol Int 1989;44: 169-172 8. Seniuta P, Cazenave-Mahe JP, Le Treut A, Trojani M. Adrenal myelolipoma and Castleman’s pseudotumor: a case of association in a retroperitoneal tumor (review). J Urol (Paris) 1989:95:511-514
American Come
Roentgen to the
Ray Society Scientific
Program
Instructional
(200
Course Neuroradiologv
Categorical
The Caldwell Award Scientific Social,
Golf,
hours) Ofl
Lecture
ANNUAL MEETING
Papers
Orlando,
Exhibits
and Tennis
Guest
92’
tapers)
( 60
Courses
Programs
Programs
Marriott’s
FL
OrlandoWorid May 10-15, 1992
Center
