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Castleman's disease in children
Castleman's D i s e a s e in Children By Randall W. Powell, Alton L. Lightsey, William J. Thomas, and William L. Marsh San Diego, California 9 A three and one-half-year-old girl and a 12-year-old boy presented with features of the two clinical presentations of Castleman's disease or giant lymph node hyperplasia. The girl presented with anemia, fever, night sweats, hypergammaglobulinemia, and a palpable abdominal mass. Her symptoms w e r e consistent with those seen in the plasma-cell type of this disease. The boy presented with acute appendicitis and a left hilar mass was noted on his chest radiograph, His asymptomatic presentation was typical of the hyaline-vascular form. Both patients are well without evidence of recurrence four years following resection. Castleman's disease is a benign disorder of lymph nodes that occurs rarely in children. Since the original report in 1954 w e could find only 18 cases in the 16-year and younger age group and our patient with the symptomatic form represents the youngest patient reported in the English literature. The management requires surgical resection of the enlarged nodes both for diagnosis and therapy since the enlarged nodes can mimic malignant tumors of the lymphoid system. No recurrences have been reported in pediatric patients. T h i s is a U S g o v e r n m e n t its use.
work. There are no restrictions on
INDEX WORDS: Castleman's disease; adenopathy.
IANT LYMPH NODE hyperplasia, described by Castleman in 19547 occurs rarely in children. Two children presenting within a six-month period exhibited the two distinct clinical entities that have been described for this disease and represent the basis for this report.
G
CASE REPORT Case 1 This three and one-half-year-old white girl presented to an outpatient facility with a history of intermittent fever, night sweats, and abdominal pain. The child's symptoms began 6 months prior to presentation with night sweats. A month before admission she began to complain of postprandial epigastric pain and lost 1.8 kg over a six-
to eight-week period. During this same period, the child developed a decreased appetite, decreased level of activity, and pallor. Palpation of an abdominal mass by the pediatrician prompted admission. Her past history, review of symptoms, and social history were unremarkable except for a milk allergy. Physical examination revealed a well-developed, well-nourished but pale child with normal vital signs. The only abnormal finding was a mobile nontender 3 • 3 cm mass left of the umbilicus. Laboratory studies revealed HGB 9.1, HCT 26.4, mean corpuscular volume 61, WBC 15,500 (P42 Bd 2, L52, M3 El). The platelet count was 1,132,000, erythrocyte sedimentation rate 135/132 m/hr, retic count 0.8%, free erythrocyte protoporphyrin 73 meg/dL, U / A normal, 24 hour urine VMA 5.3 mg, normal electrolytes and liver function tests, TP 7.5, ALB 3.89 IgG 1020 mg%, IgA 124 rag%, IgM 220 mg% with a slight increase in the alpha-2 globulin fraction. Skin tests revealed PPD negative, Candida 3 x 3 cm. Radiographic studies revealed a normal abdominal film, normal IVP, and on abdominal ultrasound, a 3 cm spherical mass appearing cystic but with some internal echoes located anterior to and separate from the left kidney (Fig 1). After blood transfusion and mechanical bowel preparation, exploratory celiotomy revealed the mass to be a 4.0 x 3.5 • 2.5 cm lymph node in the jejunal mesentery with two smaller but definitely enlarged lymph nodes adjacent to it (Fig 2). Histopathologic examination of the large mass revealed a peripheral rim of lymphoid architecture with follicular hyperplasia and moderate interfollicular plasmacystosis. The remainder of the large mass contained numerous small abnormal follicles with epithelioid centers and hyalinized vessels (Fig 3). The interfollicular areas showed a prominent proliferation of small hyalinized vessels, numerous small lymphocytes, and scattered plasma cells. The two smaller nodes submitted revealed follicular hyperplasia, moderate interfollicular plasmacytosis, and foci of small abnormal follicles. Most of the large mass contained features of the hyaline-vascular form of giant lymph node hyperplasia but the periphery of the large mass and the smaller nodes exhibited features of the plasma-ceil type. The child's postoperative course was benign with resolution of her symptoms. Her anemia, elevated ESR, and mild hypergammaglobulinemia also resolved and she remains well 4 years following surgery.
Case 2
From the Departments o f Surgery, Pediatrics, Laboratory Medicine, and Clinical Investigation, Naval Hospital, San Diego, Calif. Presented at the 34th Annual Meeting o f the Surgical Section o f the American Academy o f Pediatrics, San Antonio, Texas, October 19-20, 1985. The opinions or assertions expressed in this paper are those o f the authors and are not to be construed as official or as necessarily reflecting the views o f the Department o f Navy or the naval service at large. Address reprint request to Randall IV. Powell, MD, Division o f Pediatric Surgery, University o f South Alabama Medical Center, 2451 Fillingim St, Mobile, AL 36617. This is a US government work. There are no restrictions on its use. 0022-3468/86/2108~00450.00/0
This 12-year-old white boy presented with signs and symptoms consistent with acute appendicitis. A chest radiograph obtained in the emergency department prior to admission revealed a left hilar mass. He did give a history of an intermittent dry cough for a year but denied any other pulmonary or cardiac symptoms. After his appendectomy, he underwent computed tomography of the chest, which revealed a 4 x 3 cm left hilar mass with a mixed tissue density with no enhancement after IV contrast was administered (Fig 4). After recovering from his appendectomy, he was readmitted for removal of the left hilar mass. Physical examination revealed a well-developed well-nourished boy with normal vital signs. The entire physical examination was normal and no peripheral adenopathy was found. He underwent left thoracotomy and a lemon-sized mass was found in the anterior portion of the left hilum. Upon initial dissection, the mass was densely adherent to branches of the pulmonary artery and superior pulmonary vein. An incisional biopsy for frozen section revealed a lymphoid hyperplastic disorder. With a benign diagnosis, the mass was then reseeted as completely as possible. This was accomplished with difficulty due to the dense
678
Journal of PediatricSurgery, Vol 21, No 8 (August), 1986: pp 678-682
CASTLEMAN'S DISEASE
679
Fig 1. Case 1. This transverse abdominal ultrasound reveals a 3 cm spherical cystic-appearing mass with some internal echoes lying just beneath the abdominal wall.
adherence of the mass to the hilar structures. In some areas, thin layers of the mass were left on the pulmonary artery, pulmonary vein, and left bronchus. The child experienced a benign postoperative course and remains well without evidence of disease 4 years following the resection of the left hilar mass. Histopathology of the hilar mass revealed a well-circumscribed lymphoid lesion containing numerous evenly distributed abnormal lymphoid follicles (Fig 5A). Most of the abnormal follicles had a wide peripheral mantle of small lymphocytes often concentrically arranged. Germinal centers were small with epithelioid foci, hyaline material, and occasional penetrating vessels with hyaline walls (Fig 5B). The interfollicular areas showed fibrosis, prominent vessels with hyalinization, and scattered plasma cells. A small node from the
Fig 2. Case 1. An intraoperative photograph shows the enlarged lymph node located in the jejunal mesentery.
aortic window revealed follicular hyperplasia with moderate plasmacytosis. The findings were those of giant lymph node hyperplasia of the hyaline-vascular type. DISCUSSION
Benjamin Castleman first described this syndrome in 1954 in the Case Records of the Massachusetts General Hospital, ~and then reported 13 cases in 1956. 2 This latter report described 13 patients aged 19 to 54 years usually presenting with asymptomatic mediastinal masses with two distinct microscopic features: (1) lymphoid follicle hyperplasia with and without germinal center formation; and (2) marked capillary proliferation with endothelial hyperplasia. T u n g and M c C o r m a c k collected 57 cases from the literature and added five of their own. 3 In 1965, Lee et al reported an eight-year-old male with hyperplastic mediastinal lymph nodes with associated iron-deficiency anemia, fever, hyperglobulinemia, splenomegaly, and bone marrow plasmacytosis, which resolved following resection of the enlarged nodes. 4 Keller et al collected 81 cases of giant lymph node hyperplasia and described two distinct histopathologic forms of the disease. 5 The hyaline-vascular type (91%) was characterized microscopically by prominent vascular proliferation and hyalinization, These proliferating vessels tend to radially transverse follicle centers and m a n y contain hyaline material in the vessel wall. The cells of the germinal centers often assume a squamoid appearance and the centers resemble the Hassall's corpuscle found in the thymus. In larger
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POWELL ET AL
Fig 3. Case 1. Photomicrograph from the mesenteric mass shows an abnormal follicle on the left with a penetrating small vessel (H&E • 125).
lesions, sclerosis occurred in a perivascular location and calcification was occasionally seen. In some cases, the lymphocytes surrounding the germinal center were arranged in onion-skin layers. 5 The plasma-cell type (9%), characterized by sheets of mature plasma cells in the interfollicular spaces, exhibits much less of the vascular proliferation. Geminal centers tend to be normal or large in size but frequent hyaline-vascular type follicles may be seen. In some of the hyalinevascular lesions, small areas exhibiting features of the plasma-cell type may be seen. 5 The clinical presentation of giant lymph node hyperplasia usually follows one of two patterns. The majority of patients present with an asymptomatic mediastinal mass. These masses are usually discovered serendipitously on routine chest radiographs as in our second case. Like our patient, occasionally one can elicit symptoms referable to tracheobronchial compression. The enlarged node may also present in the
neck, axilla, or abdomen. On removal of the asymptomatic mass or the mass causing symptoms due to pressure on adjacent structures, the hyaline-vascular histologic picture is found. 5 The other clinical picture as seen in our case 1 includes anemia, fever, weight loss, and hypergammaglobulinemia. 4-]~The anemia possesses all the features of an iron-deficiency anemia (microcytic, hypochromic indices with hypoferremia) but does not respond to exogenous iron therapy. Other abnormal laboratory features that have been reported include an elevated ESR, thrombocytosis, and hypergammaglobulinemia. 5 Unusual associated clinical features reported in the literature include amyloidosis, nephrotic syndrome, thrombocytopenia, myasthenia gravis, peripheral neuropathy, erosive stomatitis and keratitis, thrombotic thrombocytopenic purpura, and myelofibrosis. 1] In 1972, 4 of the 13 patients aged 16 or less reported in the literature presented with this form of the disease.
!
/
Fig 4. Case 2. Computed tomography of the chest reveals a mixed tissue density in the left hilum measuring 4 • 3 cm. The mass did not enhance after IV contrast.
J
CASTLEMAN'S DISEASE
681
Fig 5. Case 2. (A) This lowp o w e r photomicrograph of the hilar mass shows four abnormal follicles, Notable features are the hyalinized vessels in the follicle at the upper left and concentric layering of small lymphocytes in the follicle at the lower right (HF x 60 x ) . (B) A highp o w e r view reveals an abnormal follicle at the left w i t h small epitheloid center and vessels with partially hyalinized walls (H&E x 125),
All responded with complete resolution of their symptoms when the abnormal lymph nodes were resected. 4 All five pediatric patients reported since 1972 have had the symptomatic plasma-cell variety of the disease. ~ 4 Radiographs of the mediastinal form of the disease reveal a rounded mediastinal or hilar mass with welldefined margins. 5'~5The hyaline-vascular form usually enhances well on computed tomography ~5 due to the increased vascularity, which has also been noted on angiographic studies) 6 Ultrasound examination as in our first case usually will reveal a mixed mass that mimics the findings in retroperitoneal lymph nodes involved in lymphomatous processes.
The management of both clinical forms of Castleman's disease requires surgical removal of these lesions both for diagnosis and therapy. The hyaline-vascular form, due to its increased vascularity, must be approached carefully due to its potential to create bleeding problems. 16 The plasma-cell type is usually less vascular. Complete resection is the goal but portions of nodes can be left behind if attached to vital structures (case 2). In children, no recurrences have been reported and all symptomatic cases resolve their symptoms after the abdominal nodes are removed. Limited success with radiation treatment 17and chemotherapy u has been reported in adult patients.
682
POWELL ET AL
REFERENCES
1. Castleman B: Case 40011 [Hyperplasia of mediastinal lymph nodes] N Eng J Med 250:26-30, 1954 2. Castleman B, Iverson L, Menendez VP: Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 9:822-830, 1956 3. Tung KSK, McCormack L J: Angiomatous lymphoid hamartoma: Report of five cases with a review of the literature. Cancer 20:525-536, 1967 4. Lee SL, Rosner F, Rivero I, et al: Refractory anemia with abnormal iron metabolism: Its remission after resection of hyperplastic mediastinal lymph nodes. N Engl J Med 272:761-766, 1965 5. Keller AR, Hochholzer L, Castleman B: Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 29: 670-683, 1972 6. Neerhout RC, Larson W, Mansur P: Mesenteric lymphoid hamartoma associated with chronic hypoferremia, anemia, growth failure and hyperglobulinemia. N Engl J Med 280:922-925, 1969 7. Boxer LA, Boxer G J, Flair RC, et al: Angiomatous lymphoid hamartoma associated with chronic anemia, hypoferremia and hypergammaglobulinemia. J Pediatr 81:66-70, 1972 8. Kahn LB, Ranchod M, Stables DP, et al: Giant lymph node hyperplasia with haematological abnormalities. S Aft Med J 47:811-816, 1973 9. Burgert EO Jr, Gilchrist GS, Fairbanks VF et al: Intraabdominal, angiofollicular lymph nodes hyperplasia (plasma-cell variant)
with an antierythropoietic factor. Mayo Clin Proc 50:542-546, 1975 10. Buchanan GR, Chipman J J, Hamilton BL, et al: Angiomatous lymphoid hamartoma: Inhibitory effects on erythropoiesis, growth and primary hemastasis. J Pediatr 99:382-388, 1981 11. Bjarnason I, Cotes PM, Knowles S, et al: Giant lymph node hyperplasia (Castleman's disease) of the mesentary: Observations on the associated anemia. Gastroenterology 87:216-223, 1984 12. Chan WC, Hargreaves H, Keller J: Giant lymph node hyperplasia with unusual clinicopathologic features. Cancer 53:2135-2139, 1984 13. Ballow M, Park BH, Dupont B, et al: Benign giant lymphoid hyperplasia of the mediastinum with associated abnormalities of the immune system. J Pediatr 84:418-420, 1974 14. Miller JS, Miller JJ III: Benign giant lymph node hyperplasia presenting as fever of unknown origin. J Pediatr 87:237-239, 1975 15. Onik G, Goodman PC: CT of Castleman's disease. AJR 140:691-692, 1983 16. Walter JF, Rottenberg RW, Cannon WB, et al: Giant mediastinal lymph node hyperplasia (Castleman's disease): Angiographic and clinical features. A JR 130:447-450, 1978 17. Nordstrom DG, Tewfik HH, Latourette HB: Plasma cell giant lymph node hyperplasia responding to radiation therapy. A JR 130:169-171, 1978
work. There are no restrictions on
INDEX WORDS: Castleman's disease; adenopathy.
IANT LYMPH NODE hyperplasia, described by Castleman in 19547 occurs rarely in children. Two children presenting within a six-month period exhibited the two distinct clinical entities that have been described for this disease and represent the basis for this report.
G
CASE REPORT Case 1 This three and one-half-year-old white girl presented to an outpatient facility with a history of intermittent fever, night sweats, and abdominal pain. The child's symptoms began 6 months prior to presentation with night sweats. A month before admission she began to complain of postprandial epigastric pain and lost 1.8 kg over a six-
to eight-week period. During this same period, the child developed a decreased appetite, decreased level of activity, and pallor. Palpation of an abdominal mass by the pediatrician prompted admission. Her past history, review of symptoms, and social history were unremarkable except for a milk allergy. Physical examination revealed a well-developed, well-nourished but pale child with normal vital signs. The only abnormal finding was a mobile nontender 3 • 3 cm mass left of the umbilicus. Laboratory studies revealed HGB 9.1, HCT 26.4, mean corpuscular volume 61, WBC 15,500 (P42 Bd 2, L52, M3 El). The platelet count was 1,132,000, erythrocyte sedimentation rate 135/132 m/hr, retic count 0.8%, free erythrocyte protoporphyrin 73 meg/dL, U / A normal, 24 hour urine VMA 5.3 mg, normal electrolytes and liver function tests, TP 7.5, ALB 3.89 IgG 1020 mg%, IgA 124 rag%, IgM 220 mg% with a slight increase in the alpha-2 globulin fraction. Skin tests revealed PPD negative, Candida 3 x 3 cm. Radiographic studies revealed a normal abdominal film, normal IVP, and on abdominal ultrasound, a 3 cm spherical mass appearing cystic but with some internal echoes located anterior to and separate from the left kidney (Fig 1). After blood transfusion and mechanical bowel preparation, exploratory celiotomy revealed the mass to be a 4.0 x 3.5 • 2.5 cm lymph node in the jejunal mesentery with two smaller but definitely enlarged lymph nodes adjacent to it (Fig 2). Histopathologic examination of the large mass revealed a peripheral rim of lymphoid architecture with follicular hyperplasia and moderate interfollicular plasmacystosis. The remainder of the large mass contained numerous small abnormal follicles with epithelioid centers and hyalinized vessels (Fig 3). The interfollicular areas showed a prominent proliferation of small hyalinized vessels, numerous small lymphocytes, and scattered plasma cells. The two smaller nodes submitted revealed follicular hyperplasia, moderate interfollicular plasmacytosis, and foci of small abnormal follicles. Most of the large mass contained features of the hyaline-vascular form of giant lymph node hyperplasia but the periphery of the large mass and the smaller nodes exhibited features of the plasma-ceil type. The child's postoperative course was benign with resolution of her symptoms. Her anemia, elevated ESR, and mild hypergammaglobulinemia also resolved and she remains well 4 years following surgery.
Case 2
From the Departments o f Surgery, Pediatrics, Laboratory Medicine, and Clinical Investigation, Naval Hospital, San Diego, Calif. Presented at the 34th Annual Meeting o f the Surgical Section o f the American Academy o f Pediatrics, San Antonio, Texas, October 19-20, 1985. The opinions or assertions expressed in this paper are those o f the authors and are not to be construed as official or as necessarily reflecting the views o f the Department o f Navy or the naval service at large. Address reprint request to Randall IV. Powell, MD, Division o f Pediatric Surgery, University o f South Alabama Medical Center, 2451 Fillingim St, Mobile, AL 36617. This is a US government work. There are no restrictions on its use. 0022-3468/86/2108~00450.00/0
This 12-year-old white boy presented with signs and symptoms consistent with acute appendicitis. A chest radiograph obtained in the emergency department prior to admission revealed a left hilar mass. He did give a history of an intermittent dry cough for a year but denied any other pulmonary or cardiac symptoms. After his appendectomy, he underwent computed tomography of the chest, which revealed a 4 x 3 cm left hilar mass with a mixed tissue density with no enhancement after IV contrast was administered (Fig 4). After recovering from his appendectomy, he was readmitted for removal of the left hilar mass. Physical examination revealed a well-developed well-nourished boy with normal vital signs. The entire physical examination was normal and no peripheral adenopathy was found. He underwent left thoracotomy and a lemon-sized mass was found in the anterior portion of the left hilum. Upon initial dissection, the mass was densely adherent to branches of the pulmonary artery and superior pulmonary vein. An incisional biopsy for frozen section revealed a lymphoid hyperplastic disorder. With a benign diagnosis, the mass was then reseeted as completely as possible. This was accomplished with difficulty due to the dense
678
Journal of PediatricSurgery, Vol 21, No 8 (August), 1986: pp 678-682
CASTLEMAN'S DISEASE
679
Fig 1. Case 1. This transverse abdominal ultrasound reveals a 3 cm spherical cystic-appearing mass with some internal echoes lying just beneath the abdominal wall.
adherence of the mass to the hilar structures. In some areas, thin layers of the mass were left on the pulmonary artery, pulmonary vein, and left bronchus. The child experienced a benign postoperative course and remains well without evidence of disease 4 years following the resection of the left hilar mass. Histopathology of the hilar mass revealed a well-circumscribed lymphoid lesion containing numerous evenly distributed abnormal lymphoid follicles (Fig 5A). Most of the abnormal follicles had a wide peripheral mantle of small lymphocytes often concentrically arranged. Germinal centers were small with epithelioid foci, hyaline material, and occasional penetrating vessels with hyaline walls (Fig 5B). The interfollicular areas showed fibrosis, prominent vessels with hyalinization, and scattered plasma cells. A small node from the
Fig 2. Case 1. An intraoperative photograph shows the enlarged lymph node located in the jejunal mesentery.
aortic window revealed follicular hyperplasia with moderate plasmacytosis. The findings were those of giant lymph node hyperplasia of the hyaline-vascular type. DISCUSSION
Benjamin Castleman first described this syndrome in 1954 in the Case Records of the Massachusetts General Hospital, ~and then reported 13 cases in 1956. 2 This latter report described 13 patients aged 19 to 54 years usually presenting with asymptomatic mediastinal masses with two distinct microscopic features: (1) lymphoid follicle hyperplasia with and without germinal center formation; and (2) marked capillary proliferation with endothelial hyperplasia. T u n g and M c C o r m a c k collected 57 cases from the literature and added five of their own. 3 In 1965, Lee et al reported an eight-year-old male with hyperplastic mediastinal lymph nodes with associated iron-deficiency anemia, fever, hyperglobulinemia, splenomegaly, and bone marrow plasmacytosis, which resolved following resection of the enlarged nodes. 4 Keller et al collected 81 cases of giant lymph node hyperplasia and described two distinct histopathologic forms of the disease. 5 The hyaline-vascular type (91%) was characterized microscopically by prominent vascular proliferation and hyalinization, These proliferating vessels tend to radially transverse follicle centers and m a n y contain hyaline material in the vessel wall. The cells of the germinal centers often assume a squamoid appearance and the centers resemble the Hassall's corpuscle found in the thymus. In larger
680
POWELL ET AL
Fig 3. Case 1. Photomicrograph from the mesenteric mass shows an abnormal follicle on the left with a penetrating small vessel (H&E • 125).
lesions, sclerosis occurred in a perivascular location and calcification was occasionally seen. In some cases, the lymphocytes surrounding the germinal center were arranged in onion-skin layers. 5 The plasma-cell type (9%), characterized by sheets of mature plasma cells in the interfollicular spaces, exhibits much less of the vascular proliferation. Geminal centers tend to be normal or large in size but frequent hyaline-vascular type follicles may be seen. In some of the hyalinevascular lesions, small areas exhibiting features of the plasma-cell type may be seen. 5 The clinical presentation of giant lymph node hyperplasia usually follows one of two patterns. The majority of patients present with an asymptomatic mediastinal mass. These masses are usually discovered serendipitously on routine chest radiographs as in our second case. Like our patient, occasionally one can elicit symptoms referable to tracheobronchial compression. The enlarged node may also present in the
neck, axilla, or abdomen. On removal of the asymptomatic mass or the mass causing symptoms due to pressure on adjacent structures, the hyaline-vascular histologic picture is found. 5 The other clinical picture as seen in our case 1 includes anemia, fever, weight loss, and hypergammaglobulinemia. 4-]~The anemia possesses all the features of an iron-deficiency anemia (microcytic, hypochromic indices with hypoferremia) but does not respond to exogenous iron therapy. Other abnormal laboratory features that have been reported include an elevated ESR, thrombocytosis, and hypergammaglobulinemia. 5 Unusual associated clinical features reported in the literature include amyloidosis, nephrotic syndrome, thrombocytopenia, myasthenia gravis, peripheral neuropathy, erosive stomatitis and keratitis, thrombotic thrombocytopenic purpura, and myelofibrosis. 1] In 1972, 4 of the 13 patients aged 16 or less reported in the literature presented with this form of the disease.
!
/
Fig 4. Case 2. Computed tomography of the chest reveals a mixed tissue density in the left hilum measuring 4 • 3 cm. The mass did not enhance after IV contrast.
J
CASTLEMAN'S DISEASE
681
Fig 5. Case 2. (A) This lowp o w e r photomicrograph of the hilar mass shows four abnormal follicles, Notable features are the hyalinized vessels in the follicle at the upper left and concentric layering of small lymphocytes in the follicle at the lower right (HF x 60 x ) . (B) A highp o w e r view reveals an abnormal follicle at the left w i t h small epitheloid center and vessels with partially hyalinized walls (H&E x 125),
All responded with complete resolution of their symptoms when the abnormal lymph nodes were resected. 4 All five pediatric patients reported since 1972 have had the symptomatic plasma-cell variety of the disease. ~ 4 Radiographs of the mediastinal form of the disease reveal a rounded mediastinal or hilar mass with welldefined margins. 5'~5The hyaline-vascular form usually enhances well on computed tomography ~5 due to the increased vascularity, which has also been noted on angiographic studies) 6 Ultrasound examination as in our first case usually will reveal a mixed mass that mimics the findings in retroperitoneal lymph nodes involved in lymphomatous processes.
The management of both clinical forms of Castleman's disease requires surgical removal of these lesions both for diagnosis and therapy. The hyaline-vascular form, due to its increased vascularity, must be approached carefully due to its potential to create bleeding problems. 16 The plasma-cell type is usually less vascular. Complete resection is the goal but portions of nodes can be left behind if attached to vital structures (case 2). In children, no recurrences have been reported and all symptomatic cases resolve their symptoms after the abdominal nodes are removed. Limited success with radiation treatment 17and chemotherapy u has been reported in adult patients.
682
POWELL ET AL
REFERENCES
1. Castleman B: Case 40011 [Hyperplasia of mediastinal lymph nodes] N Eng J Med 250:26-30, 1954 2. Castleman B, Iverson L, Menendez VP: Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 9:822-830, 1956 3. Tung KSK, McCormack L J: Angiomatous lymphoid hamartoma: Report of five cases with a review of the literature. Cancer 20:525-536, 1967 4. Lee SL, Rosner F, Rivero I, et al: Refractory anemia with abnormal iron metabolism: Its remission after resection of hyperplastic mediastinal lymph nodes. N Engl J Med 272:761-766, 1965 5. Keller AR, Hochholzer L, Castleman B: Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 29: 670-683, 1972 6. Neerhout RC, Larson W, Mansur P: Mesenteric lymphoid hamartoma associated with chronic hypoferremia, anemia, growth failure and hyperglobulinemia. N Engl J Med 280:922-925, 1969 7. Boxer LA, Boxer G J, Flair RC, et al: Angiomatous lymphoid hamartoma associated with chronic anemia, hypoferremia and hypergammaglobulinemia. J Pediatr 81:66-70, 1972 8. Kahn LB, Ranchod M, Stables DP, et al: Giant lymph node hyperplasia with haematological abnormalities. S Aft Med J 47:811-816, 1973 9. Burgert EO Jr, Gilchrist GS, Fairbanks VF et al: Intraabdominal, angiofollicular lymph nodes hyperplasia (plasma-cell variant)
with an antierythropoietic factor. Mayo Clin Proc 50:542-546, 1975 10. Buchanan GR, Chipman J J, Hamilton BL, et al: Angiomatous lymphoid hamartoma: Inhibitory effects on erythropoiesis, growth and primary hemastasis. J Pediatr 99:382-388, 1981 11. Bjarnason I, Cotes PM, Knowles S, et al: Giant lymph node hyperplasia (Castleman's disease) of the mesentary: Observations on the associated anemia. Gastroenterology 87:216-223, 1984 12. Chan WC, Hargreaves H, Keller J: Giant lymph node hyperplasia with unusual clinicopathologic features. Cancer 53:2135-2139, 1984 13. Ballow M, Park BH, Dupont B, et al: Benign giant lymphoid hyperplasia of the mediastinum with associated abnormalities of the immune system. J Pediatr 84:418-420, 1974 14. Miller JS, Miller JJ III: Benign giant lymph node hyperplasia presenting as fever of unknown origin. J Pediatr 87:237-239, 1975 15. Onik G, Goodman PC: CT of Castleman's disease. AJR 140:691-692, 1983 16. Walter JF, Rottenberg RW, Cannon WB, et al: Giant mediastinal lymph node hyperplasia (Castleman's disease): Angiographic and clinical features. A JR 130:447-450, 1978 17. Nordstrom DG, Tewfik HH, Latourette HB: Plasma cell giant lymph node hyperplasia responding to radiation therapy. A JR 130:169-171, 1978