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Preoperative embolization as an adjunct to the operative management of mediastinal castleman disease
Preoperative Embolization as an Adjunct to the Operative Management of Mediastinal Castleman Disease By Shawn D. Safford, Anand S. Lagoo, and Samuel A. Mahaffey Durham, North Carolina
Castleman disease usually presents in children as a localized mass with prominent feeding vessels. The mainstay of treatment of Castleman disease is surgical resection; historically, resection is associated with excessive blood loss. These tumors are well known to have large feeding vessels and, thus, are amenable to preoperative arteriography with embolization. The authors present a case of Castleman disease
treated with preoperative embolization as an adjunct to operative management. J Pediatr Surg 38:E43. © 2003 Elsevier Inc. All rights reserved.
C
than 50 mL of blood loss. His hematocrit level (0.33) on the first postoperative day was not significantly different than his preoperative hematocrit level (0.34). He did not require any blood transfusions during his hospital stay. He was discharged on postoperative day 7 after removal of the chest tube. Final pathology showed hyaline vascular Castleman disease with pleural adhesions, but no lung involvement. There was no microscopic evidence of gross necrosis or degeneration, but multiple blood vessels were noted to contain embolized Ivalon particles (Fig 3). At 1 month follow-up the patient is clinically well; interestingly, his headaches and dizziness resolved after tumor resection.
ASTLEMAN DISEASE, or angiofollicular lymph node hyperplasia, is a rare disorder that usually presents as an asymptomatic, localized mediastinal mass.1,2 Although the treatment of this disease is primarily surgical, the operation is associated with excessive blood loss secondary to the hypervascular nature of the tumor.3 Castleman disease is characterized by dominant feeding vessels that are amenable to preoperative radiographic embolization.4 Two previous reports have used preoperative embolization as an adjunctive treatment to the surgical management of Castleman disease5,6; however, this has not been generally supported by further literature. We present an additional case of Castleman disease to support preoperative embolization as an adjunct to operative management. CASE REPORT An 11-year-old boy with no significant past medical history presented to his primary care provider with “sinus” complaints, headaches, and dizziness. He was evaluated with a chest x-ray that showed a mediastinal mass. At the referral hospital he underwent an open thoracotomy and biopsy of the mediastinal mass. The biopsy was complicated by significant intraoperative blood loss owing to the hypervascularity of the tumor. Pathology results showed Castleman disease— hyalinovascular type. The patient recovered from the procedure and was then transferred to our institution for further workup and definitive management. On physical examination there were no significant findings. Laboratory values were significant for a hematocrit level of 0.34. Magnetic resonance imaging (MRI) of the chest showed middle and superior mediastinal masses consistent with his diagnosis of Castleman disease (Fig 1). Aortic arteriogram with selective right and left internal mammary artery arteriograms showed large branches feeding the mediastinal tumor that originated from the right intercostal artery and the right internal mammary artery (Fig 2A). A large branch supplying the tumor was selected, and the tumor mass was embolized with Ivalon particles, 355 to 500 m, and 2 tracker coils 2 mm ⫻ 10 mm (Fig 2B). The following day, the patient was taken to the operating room where he underwent a right thoracotomy, right upper lobectomy, and resection of the mediastinal mass. Intraoperatively, the mass was noted to be gray and mobilized easily. He tolerated the surgery well with less Journal of Pediatric Surgery, Vol 38, No 9 (September), 2003: E43
INDEX WORDS: Castleman disease, angiofollicular lymph node hyperplasia, embolization, angiography.
DISCUSSION
Castleman disease is a lymphoproliferative disorder of unknown etiology.1 The condition was first described in 1954 by Castleman as a mediastinal lymph node hyperplasia resembling a thymoma.7,8 Castleman disease primarily has 2 pathologic variants: hyaline vascular, characterized by small, hyalinized follicles with interfollicular vascular proliferation and plasma cell type, dominated by plasma cells.1,9,10 The majority of those found in children are of the hyaline vascular subtype.2 Clinically, Castleman disease presents as a localized or mulitcentric mass occurring in the mediastinum, neck, or mesentery.11 As with our patient, the localized form usually presents as a slow-growing mass that has a relatively benign course. In children with Castleman disease, there is a recognized triad of anemia, hypergammaglobulinemia, and failure to thrive.2 Thus, anemia is a significant baseline problem before surgical treatment of disease. From the Departments of Pediatric Surgery and Pathology, Duke University Medical Center, Durham, NC. Address reprint requests to Shawn D. Safford, MD, Box 31291, Duke University Medical Center, Durham, NC 27710. © 2003 Elsevier Inc. All rights reserved. 1531-5037/03/3809-0043$30.00/0 doi:10.1016/S0022-3468(03)00421-4 1
2
SAFFORD ET AL
Fig 1.
MRI of the chest shows middle and superior mediastinal masses.
Fig 2. Selective right internal mammary arteriogram. (A) Large branches feed the mediastinal tumor that originated from the right intercostal artery and the right internal mammary artery. (B) A large branch supplying the tumor is embolized with Ivalon particles and 2 tracker coils. Arrows identify the 2 dominant feeding vessels with tracker coils deployed.
Radiographically, Castleman disease appears on computed tomography scan (CT) as a solid mass, occasionally with calcifications.11 Specifically, the hyaline vascular subtype shows an abundant vascular component.4 These lesions generally appear on arteriogram as a hypervascular tumor. Radiographic biopsy of these tumors is potentially dangerous because of the hypervascular nature of the tumor, and, thus, biopsy should be reserved to open surgical biopsy. The mainstay of treatment of localized Castleman disease is surgical resection of the mass.2,3 In contrast, multicentric Castleman’s disease, which usually is of the plasma cell type, is more aggressive and is most effectively treated with combination chemotherapy. Surgical resection of localized Castleman disease is commonly complicated by excessive blood loss owing to the vascular nature of the tumor. We have addressed this potential complication using arterial embolization as a preoperative adjunct to surgery. Our experience supports preoperative embolization to control the vascular supply of the tumor. This aided in the hemostatic control and limited the amount of intraoperative bleed-
Fig 3. Embolized blood vessel. An artery in the wall of the lesion shows the embolized material (Ivalon particles) in the form of short rods and spheres of an amorphous, pale basophilic material with a brisk giant cell reaction. Arrows identify the Ivalon particles within the blood vessel. The particles have an average diameter of 35 to 40 m and are seen longitudinally and in cross section.
PREOPERATIVE EMBOLIZATION OF CASTLEMAN DISEASE
3
ing in our patient. No blood was administered during the patient’s hospitalization; thus, preoperative embolization decreased the risks of exposure to blood borne pathogens.
Our experience supports preoperative selective arteriography and embolization of dominant vessels as an adjunct to the operative management of mediastinal Castleman disease.
REFERENCES 1. Frizzera G: Castleman disease and related disorders. Semin Diagn Pathol 5:346, 1988 2. Parez N, Bader-Meunier B, Roy C, et al: Paediatric Castleman disease: Report of seven cases and review of the literature. Eur J Pediatr 158:631-637, 1999 3. Chronowski G, Ha C, Wilder R, et al: Treatment of unicentric and multicentric Castleman disease and the role of radiotherapy. Cancer 92:670-676, 2001 4. Ecklund K, Hartnell G: Mediastinal Castleman disease: MR and MRA features. J Thoracic Surg 9:156-159, 1994 5. Williams HR, Millner PA, Coral A: Castleman’s disease of the erector spinae muscle. Skeletal Radiol 27:637-640, 1998 6. Walter JF, Rottenberg RW, Cannon WB, et al: Giant mediastinal lymph node hyperplasia (Castleman’s disease): Angiographic and clinical features. Am J Roentgenol 130:447-450, 1978
7. Castleman B, Towne V: Case records of the Massachusetts general hospital: Case 40011 (hyperplasia of mediastinal lymph nodes). N Engl J Med 250:26-30, 1954 8. Castleman B, Iverson L, Mendeudex V: Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 9:822-830, 1956 9. Buys L, Wijermans P, Groningen KY, et al: Hyalinc- vascular type Castleman disease with concomitant malignant B-cell lymphoma. Acta Hematol 87:160-162, 1992 10. Frizzera G, Banks P, Massarelli G, et al: A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease. Am J Surg Pathol 7:211-231, 1983 11. Taura T, Takashima S, Shakudo M, et al: Castleman’s disease of the spleen: CT, MR imaging and angiographic findings. Eur J Radiol 6:11-15, 2000
Castleman disease usually presents in children as a localized mass with prominent feeding vessels. The mainstay of treatment of Castleman disease is surgical resection; historically, resection is associated with excessive blood loss. These tumors are well known to have large feeding vessels and, thus, are amenable to preoperative arteriography with embolization. The authors present a case of Castleman disease
treated with preoperative embolization as an adjunct to operative management. J Pediatr Surg 38:E43. © 2003 Elsevier Inc. All rights reserved.
C
than 50 mL of blood loss. His hematocrit level (0.33) on the first postoperative day was not significantly different than his preoperative hematocrit level (0.34). He did not require any blood transfusions during his hospital stay. He was discharged on postoperative day 7 after removal of the chest tube. Final pathology showed hyaline vascular Castleman disease with pleural adhesions, but no lung involvement. There was no microscopic evidence of gross necrosis or degeneration, but multiple blood vessels were noted to contain embolized Ivalon particles (Fig 3). At 1 month follow-up the patient is clinically well; interestingly, his headaches and dizziness resolved after tumor resection.
ASTLEMAN DISEASE, or angiofollicular lymph node hyperplasia, is a rare disorder that usually presents as an asymptomatic, localized mediastinal mass.1,2 Although the treatment of this disease is primarily surgical, the operation is associated with excessive blood loss secondary to the hypervascular nature of the tumor.3 Castleman disease is characterized by dominant feeding vessels that are amenable to preoperative radiographic embolization.4 Two previous reports have used preoperative embolization as an adjunctive treatment to the surgical management of Castleman disease5,6; however, this has not been generally supported by further literature. We present an additional case of Castleman disease to support preoperative embolization as an adjunct to operative management. CASE REPORT An 11-year-old boy with no significant past medical history presented to his primary care provider with “sinus” complaints, headaches, and dizziness. He was evaluated with a chest x-ray that showed a mediastinal mass. At the referral hospital he underwent an open thoracotomy and biopsy of the mediastinal mass. The biopsy was complicated by significant intraoperative blood loss owing to the hypervascularity of the tumor. Pathology results showed Castleman disease— hyalinovascular type. The patient recovered from the procedure and was then transferred to our institution for further workup and definitive management. On physical examination there were no significant findings. Laboratory values were significant for a hematocrit level of 0.34. Magnetic resonance imaging (MRI) of the chest showed middle and superior mediastinal masses consistent with his diagnosis of Castleman disease (Fig 1). Aortic arteriogram with selective right and left internal mammary artery arteriograms showed large branches feeding the mediastinal tumor that originated from the right intercostal artery and the right internal mammary artery (Fig 2A). A large branch supplying the tumor was selected, and the tumor mass was embolized with Ivalon particles, 355 to 500 m, and 2 tracker coils 2 mm ⫻ 10 mm (Fig 2B). The following day, the patient was taken to the operating room where he underwent a right thoracotomy, right upper lobectomy, and resection of the mediastinal mass. Intraoperatively, the mass was noted to be gray and mobilized easily. He tolerated the surgery well with less Journal of Pediatric Surgery, Vol 38, No 9 (September), 2003: E43
INDEX WORDS: Castleman disease, angiofollicular lymph node hyperplasia, embolization, angiography.
DISCUSSION
Castleman disease is a lymphoproliferative disorder of unknown etiology.1 The condition was first described in 1954 by Castleman as a mediastinal lymph node hyperplasia resembling a thymoma.7,8 Castleman disease primarily has 2 pathologic variants: hyaline vascular, characterized by small, hyalinized follicles with interfollicular vascular proliferation and plasma cell type, dominated by plasma cells.1,9,10 The majority of those found in children are of the hyaline vascular subtype.2 Clinically, Castleman disease presents as a localized or mulitcentric mass occurring in the mediastinum, neck, or mesentery.11 As with our patient, the localized form usually presents as a slow-growing mass that has a relatively benign course. In children with Castleman disease, there is a recognized triad of anemia, hypergammaglobulinemia, and failure to thrive.2 Thus, anemia is a significant baseline problem before surgical treatment of disease. From the Departments of Pediatric Surgery and Pathology, Duke University Medical Center, Durham, NC. Address reprint requests to Shawn D. Safford, MD, Box 31291, Duke University Medical Center, Durham, NC 27710. © 2003 Elsevier Inc. All rights reserved. 1531-5037/03/3809-0043$30.00/0 doi:10.1016/S0022-3468(03)00421-4 1
2
SAFFORD ET AL
Fig 1.
MRI of the chest shows middle and superior mediastinal masses.
Fig 2. Selective right internal mammary arteriogram. (A) Large branches feed the mediastinal tumor that originated from the right intercostal artery and the right internal mammary artery. (B) A large branch supplying the tumor is embolized with Ivalon particles and 2 tracker coils. Arrows identify the 2 dominant feeding vessels with tracker coils deployed.
Radiographically, Castleman disease appears on computed tomography scan (CT) as a solid mass, occasionally with calcifications.11 Specifically, the hyaline vascular subtype shows an abundant vascular component.4 These lesions generally appear on arteriogram as a hypervascular tumor. Radiographic biopsy of these tumors is potentially dangerous because of the hypervascular nature of the tumor, and, thus, biopsy should be reserved to open surgical biopsy. The mainstay of treatment of localized Castleman disease is surgical resection of the mass.2,3 In contrast, multicentric Castleman’s disease, which usually is of the plasma cell type, is more aggressive and is most effectively treated with combination chemotherapy. Surgical resection of localized Castleman disease is commonly complicated by excessive blood loss owing to the vascular nature of the tumor. We have addressed this potential complication using arterial embolization as a preoperative adjunct to surgery. Our experience supports preoperative embolization to control the vascular supply of the tumor. This aided in the hemostatic control and limited the amount of intraoperative bleed-
Fig 3. Embolized blood vessel. An artery in the wall of the lesion shows the embolized material (Ivalon particles) in the form of short rods and spheres of an amorphous, pale basophilic material with a brisk giant cell reaction. Arrows identify the Ivalon particles within the blood vessel. The particles have an average diameter of 35 to 40 m and are seen longitudinally and in cross section.
PREOPERATIVE EMBOLIZATION OF CASTLEMAN DISEASE
3
ing in our patient. No blood was administered during the patient’s hospitalization; thus, preoperative embolization decreased the risks of exposure to blood borne pathogens.
Our experience supports preoperative selective arteriography and embolization of dominant vessels as an adjunct to the operative management of mediastinal Castleman disease.
REFERENCES 1. Frizzera G: Castleman disease and related disorders. Semin Diagn Pathol 5:346, 1988 2. Parez N, Bader-Meunier B, Roy C, et al: Paediatric Castleman disease: Report of seven cases and review of the literature. Eur J Pediatr 158:631-637, 1999 3. Chronowski G, Ha C, Wilder R, et al: Treatment of unicentric and multicentric Castleman disease and the role of radiotherapy. Cancer 92:670-676, 2001 4. Ecklund K, Hartnell G: Mediastinal Castleman disease: MR and MRA features. J Thoracic Surg 9:156-159, 1994 5. Williams HR, Millner PA, Coral A: Castleman’s disease of the erector spinae muscle. Skeletal Radiol 27:637-640, 1998 6. Walter JF, Rottenberg RW, Cannon WB, et al: Giant mediastinal lymph node hyperplasia (Castleman’s disease): Angiographic and clinical features. Am J Roentgenol 130:447-450, 1978
7. Castleman B, Towne V: Case records of the Massachusetts general hospital: Case 40011 (hyperplasia of mediastinal lymph nodes). N Engl J Med 250:26-30, 1954 8. Castleman B, Iverson L, Mendeudex V: Localized mediastinal lymph node hyperplasia resembling thymoma. Cancer 9:822-830, 1956 9. Buys L, Wijermans P, Groningen KY, et al: Hyalinc- vascular type Castleman disease with concomitant malignant B-cell lymphoma. Acta Hematol 87:160-162, 1992 10. Frizzera G, Banks P, Massarelli G, et al: A systemic lymphoproliferative disorder with morphologic features of Castleman’s disease. Am J Surg Pathol 7:211-231, 1983 11. Taura T, Takashima S, Shakudo M, et al: Castleman’s disease of the spleen: CT, MR imaging and angiographic findings. Eur J Radiol 6:11-15, 2000