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Renal lymphoma in castleman's disease
CLINICAL IMAGING1994;18:93-95
93
RENAL LYMPHOMA IN CASTLEMAN’S DISEASE KENNETH P. MORESCO, MD, ROBERT S. SHAPIRO, AND AGATA STANCATO-PASIK, MD
An unusual case of renal Iymphoma occurring in a patient with Castleman’s disease is presented. The Mdiogrophic features and the relationship to the Iymphoproliferative disorders, Castleman’s disease, and multicentric angiofollicular lymph node hyperplasia are described. KEY WORDS:
Castleman’sdisease; Lymphaticsystem,Computed tomography;Lymphatic system,Ultrasound; Kidney neoplasms. INTRODUCIlON Castleman’s disease is a benign disorder of lymphoid hyperplasia first described by Castleman et al. in 1956 (1). More recently, multicentric angiofollicular lymph node hyperplasia (MAFH), a multicentric lymphoproliferative disorder with associated systemic manifestations that resembles Castleman’s disease histologically, has been described (2,3). Whereas Castleman’s disease classically presents as a localized mass, usually in the chest, MAFH involves multiple lymph node regions and other organs, and is frequently fatal. While the development of malignant lymphoma has been observed in a limited number of patients with MAFH and a patient with Castleman’s disease (2-5), malignant lymphoma involving the kidney as a complication of Castleman’s disease or MAFH has not been previously reported.
MD,
The patient’s medical history was remarkable for the diagnosis of Castleman’s disease, made approximately 13 years ago by biopsy of axillary lymph nodes. On the most recent admission a right abdominal mass was palpated. The patient tested negative for human immunodeficiency virus (HIV) and hepatitis A and B. Abdominal ultrasound revealed a large mass in the right renal fossa with displacement of the interior vena cava (Figure 1). Contrast-enhanced computed tomography (CT) of the abdomen revealed a 22-cm mass of mixed attenuation inseparable from the right kidney, with extension into the retroperitoneum (Figure 2A). There was anterior displacement of the inferior vena cava and left lateral displacement of the aorta and celiac axis by associated retroperitoneal lymphadenopathy (Figure 2B). An ultrasound-guided needle biopsy yielded
CASE REPORT A 2%year-old African-American man was admitted with a history of fever, night sweats, and weight loss. Prom the Department of Radiology, Mount Sinai Medical Center of the Citv Universitv of New York, New York. New York. Address reprint requ&ts to: Robert s. Shapiro, MD, Department of Radiology- Box 1234, Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY 10029-6574. 0 1994 Elsevier Science Inc. 655 Avenue of the Americas, 0899-7071/94/$%00
New York, NY 10010
FIGURE 1. Dopplersonogram,in a longitudinalorientation, showsa large retroperitonealmass. The inferiorvena cava is displaced anteriorly,The arrow indicates the direction of flow in the IVC.M, mass; GB, gallbladder;IVCinferior vena cava.
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MORESCO ET AL.
B FIGURE 2. (A) CT scan shows a large mass involving the right kidney [small arrows]. The mass extends into the right side of the retroperitoneum, displacing the inferior vena cava anteriorly (curved white arrow) and the celiac axis and aorta laterally [curved open arrow). (B) CT scan showsthe non-
homogeneousfeaturesof the mass [whitearrow)as well as the associated retroperitoneal lymphadenopathy(curved arrows).
cells positive for leukocyte common antigen, consistent with malignant lymphoma. T- and B-cell antibody analysis did not define a phenotype. DISCUSSION
The process known as Castleman’s disease, as originally described, is a localized tumor-like mass most often involving the mediastinum (1). Extrathoracic and extranodal sites of involvement have been described
CLINICAL IMAGING VOL. 18, NO. 2
(6). Castleman’s description of “hyperplasia of lymphoid follicles” (1) and “marked capillary proliferation with endothelial proliferation”(1) resembling the “Hassall corpuscle of the thymus” (1) defines the hyalinevascular type (type II) (7) of the disease. This is the most common variant, representing 91% of the reported cases (6). In 1969, Flendrig (7) described a new morphological type (plasma cell type, type I) characterized by sheets of mature plasma cells associated with large hyperplastic follicles. This type:is typically associated with a clinical syndrome consisting of fever, sweats, fatigue, anemia, elevated erythrocyte sedimentation rate, polyclonal hypergammaglobulinemia, and bone marrow plasmacytosis (3). He also described a form with intermediate morphological features, suggesting a spectrum of abnormalities linking the varying histological subtypes. Morphologically similar to classic Castleman’s disease is the entity MAFH (2-4). This systemic disease is characterized by occurrence in late adulthood, a predilection for males, systemic symptoms, and multiple and mainly peripheral sites of involvement with associated organomegaly and hypergammaglobulinemia. The course of the disease may be chronic, with recurrent exacerbations and remissions, or it may have an aggressive course. In addition to the increased incidence of systemic abnormalities, there are higher rates of mortality and predisposition toward the development of malignancies. Approximately 73% of these patients have infectious complications, with 27 to 32% developing malignancies, especially Kaposi’s sarcoma and lymphoid neoplasms (4). Despite the tendency toward severe systemic abnormalities, the existence of MAFH as a distinct entity from classic Castleman’s disease has been debated (2-4). There are a number of features of MAFH that suggest a unique entity: multicentricity, older age of occurrence, increased severity of systemic manifestations, and progressive clinical course. The short and progressive clinical course, the associations with Kaposi sarcoma, and the severe infectious complications have not been reported for classic Castleman’sdisease. Only one additional case of the development of malignant lymphoma has been reported (5). It appears that classic Castleman’s disease and MAFH are closely related, with MAFH associated with significant morbidity and mortality (2-4). Our patient was diagnosed with Castleman’sdisease involving axillary lymph nodes at the age of 10. The patient was then lost to follow-up until he presented with a renal mass that proved to be malignant lymphoma on biopsy. The previously reported case of malignant lymphoma developing in a patient with Castleman’s disease was localized to the mediastinum.
RENAL LYMPHOMA IN CASTLEMAN’S DISEASE
APRIL-JUNE 1994
This is the first case of renal lymphoma developing in a patient with Castleman’s disease. The predisposition for the development of malignant lymphomas in MAFH and now Castleman’s disease may be explained by the proposed abnormality in immune regulation, with the clinical course and evolution of the disease determined by the severity of the immunological defect. Imaging characteristics in Castleman’s disease and MAFH are in part determined by the morphological subtypes (8,9). The hyaline-vascular type tends to present as a homogeneously enhancing mass, possibly due to the marked endothelial proliferation. The plasma cell type has a less consistent appearance, again presumed secondary to the varying vascular proliferative component. The intermediate form varies in its imaging appearance according to the amount of endothelial proliferation demonstrated on histological section. In MAFH, CT and ultrasound findings include splenomegaly, generalized lymphadenopathy, and ascites (9). The findings are nonspecific and are compatible with lymphoma. The diagnosis usually can only be established after lymph node biopsy. In our patient the sonographic findings of a uniformly hypoechoic mass in the region of the right renal fossa were nonspecific. The differential for this mass included hypernephroma, renal lymphoma, retroperitoneal leiomyoma and sarcoma, and an inflammatory mass involving the right kidney. The CT appearance was that of a heterogeneously enhancing right renal mass. This again was nonspecific, but in a patient with a known history of Castleman’s disease
95
diagnosis of malignant lymphoma was suspected. To our knowledge, no previous description of renal lymphoma complicating Castleman’s disease has been reported.
the
REFERENCES 1.
Castleman B, Iverson L, Menendez V. Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 1956; 9:822-830.
G, Peterson B, Bayrd E, Goldman A. A systemic lym2. Frizzera phoproliferative disorder with morphologic features of Castleman’sdisease: clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol 1985;3:1202-1216. 3. Weisenburger D, Nathwani B, Winberg C, Rappaport H. Multicentric angiofollicularlymph node hyperplasia:a clinicopathologic study of 16 cases. Hum Path01 1985;2:162-172. 4. Frizzera G, Banks PM, Massarelli G, Rosai J. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease. Pathologic findings in 15 patients. Am J Surg Path01 1983;7:211-231. 5. Vasef M, Katzen W, Mendelsohn G, Reydman M. Report of a case of Castleman’sdisease with progression to malignant lymphoma. Am J Clin Path01 1992;98:633-636. 6. Keller A, Hochholzer L, Castleman B. Hyaline-vascular and plasma cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1976;29:679-683. 7. Flendrig JA. Benign giant lymphoma: clinicopathologic correlation study. In Clark RL, Cumley RW (eds): The Year Book of Cancer. Chicago: Year Book Medical, 1970; 296. 8. Joseph N, VogelzangR, Hidvegi D, Neiman H. Computed tomography of retroperitoneal Castleman disease (plasma type) with sonographic and angiographic correlation. J Comput Assist Tomogr 1985;9:570-572. 9. 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.
93
RENAL LYMPHOMA IN CASTLEMAN’S DISEASE KENNETH P. MORESCO, MD, ROBERT S. SHAPIRO, AND AGATA STANCATO-PASIK, MD
An unusual case of renal Iymphoma occurring in a patient with Castleman’s disease is presented. The Mdiogrophic features and the relationship to the Iymphoproliferative disorders, Castleman’s disease, and multicentric angiofollicular lymph node hyperplasia are described. KEY WORDS:
Castleman’sdisease; Lymphaticsystem,Computed tomography;Lymphatic system,Ultrasound; Kidney neoplasms. INTRODUCIlON Castleman’s disease is a benign disorder of lymphoid hyperplasia first described by Castleman et al. in 1956 (1). More recently, multicentric angiofollicular lymph node hyperplasia (MAFH), a multicentric lymphoproliferative disorder with associated systemic manifestations that resembles Castleman’s disease histologically, has been described (2,3). Whereas Castleman’s disease classically presents as a localized mass, usually in the chest, MAFH involves multiple lymph node regions and other organs, and is frequently fatal. While the development of malignant lymphoma has been observed in a limited number of patients with MAFH and a patient with Castleman’s disease (2-5), malignant lymphoma involving the kidney as a complication of Castleman’s disease or MAFH has not been previously reported.
MD,
The patient’s medical history was remarkable for the diagnosis of Castleman’s disease, made approximately 13 years ago by biopsy of axillary lymph nodes. On the most recent admission a right abdominal mass was palpated. The patient tested negative for human immunodeficiency virus (HIV) and hepatitis A and B. Abdominal ultrasound revealed a large mass in the right renal fossa with displacement of the interior vena cava (Figure 1). Contrast-enhanced computed tomography (CT) of the abdomen revealed a 22-cm mass of mixed attenuation inseparable from the right kidney, with extension into the retroperitoneum (Figure 2A). There was anterior displacement of the inferior vena cava and left lateral displacement of the aorta and celiac axis by associated retroperitoneal lymphadenopathy (Figure 2B). An ultrasound-guided needle biopsy yielded
CASE REPORT A 2%year-old African-American man was admitted with a history of fever, night sweats, and weight loss. Prom the Department of Radiology, Mount Sinai Medical Center of the Citv Universitv of New York, New York. New York. Address reprint requ&ts to: Robert s. Shapiro, MD, Department of Radiology- Box 1234, Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY 10029-6574. 0 1994 Elsevier Science Inc. 655 Avenue of the Americas, 0899-7071/94/$%00
New York, NY 10010
FIGURE 1. Dopplersonogram,in a longitudinalorientation, showsa large retroperitonealmass. The inferiorvena cava is displaced anteriorly,The arrow indicates the direction of flow in the IVC.M, mass; GB, gallbladder;IVCinferior vena cava.
94
MORESCO ET AL.
B FIGURE 2. (A) CT scan shows a large mass involving the right kidney [small arrows]. The mass extends into the right side of the retroperitoneum, displacing the inferior vena cava anteriorly (curved white arrow) and the celiac axis and aorta laterally [curved open arrow). (B) CT scan showsthe non-
homogeneousfeaturesof the mass [whitearrow)as well as the associated retroperitoneal lymphadenopathy(curved arrows).
cells positive for leukocyte common antigen, consistent with malignant lymphoma. T- and B-cell antibody analysis did not define a phenotype. DISCUSSION
The process known as Castleman’s disease, as originally described, is a localized tumor-like mass most often involving the mediastinum (1). Extrathoracic and extranodal sites of involvement have been described
CLINICAL IMAGING VOL. 18, NO. 2
(6). Castleman’s description of “hyperplasia of lymphoid follicles” (1) and “marked capillary proliferation with endothelial proliferation”(1) resembling the “Hassall corpuscle of the thymus” (1) defines the hyalinevascular type (type II) (7) of the disease. This is the most common variant, representing 91% of the reported cases (6). In 1969, Flendrig (7) described a new morphological type (plasma cell type, type I) characterized by sheets of mature plasma cells associated with large hyperplastic follicles. This type:is typically associated with a clinical syndrome consisting of fever, sweats, fatigue, anemia, elevated erythrocyte sedimentation rate, polyclonal hypergammaglobulinemia, and bone marrow plasmacytosis (3). He also described a form with intermediate morphological features, suggesting a spectrum of abnormalities linking the varying histological subtypes. Morphologically similar to classic Castleman’s disease is the entity MAFH (2-4). This systemic disease is characterized by occurrence in late adulthood, a predilection for males, systemic symptoms, and multiple and mainly peripheral sites of involvement with associated organomegaly and hypergammaglobulinemia. The course of the disease may be chronic, with recurrent exacerbations and remissions, or it may have an aggressive course. In addition to the increased incidence of systemic abnormalities, there are higher rates of mortality and predisposition toward the development of malignancies. Approximately 73% of these patients have infectious complications, with 27 to 32% developing malignancies, especially Kaposi’s sarcoma and lymphoid neoplasms (4). Despite the tendency toward severe systemic abnormalities, the existence of MAFH as a distinct entity from classic Castleman’s disease has been debated (2-4). There are a number of features of MAFH that suggest a unique entity: multicentricity, older age of occurrence, increased severity of systemic manifestations, and progressive clinical course. The short and progressive clinical course, the associations with Kaposi sarcoma, and the severe infectious complications have not been reported for classic Castleman’sdisease. Only one additional case of the development of malignant lymphoma has been reported (5). It appears that classic Castleman’s disease and MAFH are closely related, with MAFH associated with significant morbidity and mortality (2-4). Our patient was diagnosed with Castleman’sdisease involving axillary lymph nodes at the age of 10. The patient was then lost to follow-up until he presented with a renal mass that proved to be malignant lymphoma on biopsy. The previously reported case of malignant lymphoma developing in a patient with Castleman’s disease was localized to the mediastinum.
RENAL LYMPHOMA IN CASTLEMAN’S DISEASE
APRIL-JUNE 1994
This is the first case of renal lymphoma developing in a patient with Castleman’s disease. The predisposition for the development of malignant lymphomas in MAFH and now Castleman’s disease may be explained by the proposed abnormality in immune regulation, with the clinical course and evolution of the disease determined by the severity of the immunological defect. Imaging characteristics in Castleman’s disease and MAFH are in part determined by the morphological subtypes (8,9). The hyaline-vascular type tends to present as a homogeneously enhancing mass, possibly due to the marked endothelial proliferation. The plasma cell type has a less consistent appearance, again presumed secondary to the varying vascular proliferative component. The intermediate form varies in its imaging appearance according to the amount of endothelial proliferation demonstrated on histological section. In MAFH, CT and ultrasound findings include splenomegaly, generalized lymphadenopathy, and ascites (9). The findings are nonspecific and are compatible with lymphoma. The diagnosis usually can only be established after lymph node biopsy. In our patient the sonographic findings of a uniformly hypoechoic mass in the region of the right renal fossa were nonspecific. The differential for this mass included hypernephroma, renal lymphoma, retroperitoneal leiomyoma and sarcoma, and an inflammatory mass involving the right kidney. The CT appearance was that of a heterogeneously enhancing right renal mass. This again was nonspecific, but in a patient with a known history of Castleman’s disease
95
diagnosis of malignant lymphoma was suspected. To our knowledge, no previous description of renal lymphoma complicating Castleman’s disease has been reported.
the
REFERENCES 1.
Castleman B, Iverson L, Menendez V. Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 1956; 9:822-830.
G, Peterson B, Bayrd E, Goldman A. A systemic lym2. Frizzera phoproliferative disorder with morphologic features of Castleman’sdisease: clinical findings and clinicopathologic correlations in 15 patients. J Clin Oncol 1985;3:1202-1216. 3. Weisenburger D, Nathwani B, Winberg C, Rappaport H. Multicentric angiofollicularlymph node hyperplasia:a clinicopathologic study of 16 cases. Hum Path01 1985;2:162-172. 4. Frizzera G, Banks PM, Massarelli G, Rosai J. A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease. Pathologic findings in 15 patients. Am J Surg Path01 1983;7:211-231. 5. Vasef M, Katzen W, Mendelsohn G, Reydman M. Report of a case of Castleman’sdisease with progression to malignant lymphoma. Am J Clin Path01 1992;98:633-636. 6. Keller A, Hochholzer L, Castleman B. Hyaline-vascular and plasma cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1976;29:679-683. 7. Flendrig JA. Benign giant lymphoma: clinicopathologic correlation study. In Clark RL, Cumley RW (eds): The Year Book of Cancer. Chicago: Year Book Medical, 1970; 296. 8. Joseph N, VogelzangR, Hidvegi D, Neiman H. Computed tomography of retroperitoneal Castleman disease (plasma type) with sonographic and angiographic correlation. J Comput Assist Tomogr 1985;9:570-572. 9. 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.