Sinus Histiocytosis and Massive Lymphadenopathy (Rosai-Dorfman Disease) Mimicking Cervical Lymphadenitis
A
n 8-month-old African-American male was admitted with a 2-week history of right-sided neck swelling and fever, treated unsuccessfully with cephalexin. He received intravenous clindamycin with resolution of fever, reduction in size of swelling, and was discharged on oral clindamycin, only to be readmitted 4 weeks later for the same complaint. Examination revealed 2 overlapping firm, tender, and matted masses 5 5 and 5 4 cm in size, in the right anterior cervical and supraclavicular areas respectively, confirmed to be lymph nodes by ultrasound and computed tomography (CT) scan (Figure 1). He had no enlarged glands elsewhere, showed no sign of upper airway obstruction, but was febrile with elevated white cell counts (26 600 and 18 600 cells/mm3) and a left shift on both occasions. Histology from lymph node biopsy is shown (Figure 2; available at www.jpeds.com). Biopsy cultures were negative. First described in 1969,1 Rosai-Dorfman disease is a systemic illness characterized by large, painless bilateral cervical lymphadenopathy, overproduction and accumulation of non-Langerhans sinus histiocytes, and fever with leucocytosis. It usually occurs in children and young adults, mostly with head and neck nodal involvement, and less often in other sites such as mediastinum, inguinal and retroperitoneum.2 Chest CT showed no intrathoracic node
involvement in our patient, among the youngest reported. He had 2 other atypical features: unilateral presentation and tender nodes, both suggestive of lymphadenitis. Extranodal sites can be affected, the commonest being skin, and a purely cutaneous form has been described.2 Of unknown etiology, altered immune responses to infectious agents such as human herpes virus-6 and -8, and Epstein-Barr virus have been suspected.3,4 Our patient tested negative to Epstein-Barr virus, cytomegalovirus, toxoplasmosis, and bartonella by serology. The clinical course is usually benign, with remissions and exacerbations followed eventually by spontaneous resolution within a few years. It may occasionally be complicated by vital organ or structure compression. n This case was an oral presentation at the annual meeting of the Eastern Society for Pediatric Research, Philadelphia, PA, March 22-23, 2013.
Kirti Sivakoti, MD Deepa Doraisamy, MD Department of Pediatrics
Masooma Niazi, MD Department of Pathology
Gary Kronfeld, MD Department of Radiology
Stefan Hagmann, MD, MSc Division of Pediatric Infectious Disease Bronx-Lebanon Hospital Center
Kara Kelly, MD
Figure 1. A, Axial CT image of neck with intravenous contrast at level of hyoid shows bilateral hypervascular lymph nodes in the internal jugular chain. A hypervascular 3.3-cm mass in right submandibular space is consistent with enlarged lymph node (arrow). There is mass effect on the neurovascular structures and upon the airway. B, Axial CT image of neck with intravenous contrast at level of thoracic inlet shows a supraclavicular lymph node measuring 2.4 cm (arrow).
Department of Pediatrics Division of Pediatric Oncology Morgan Stanley Children’s Hospital of New York-Presbyterian Columbia University New York, New York
Murli Purswani, MD Division of Pediatric Infectious Disease Bronx-Lebanon Hospital Center Albert Einstein College of Medicine Bronx, New York
References available at www.jpeds.com
J Pediatr 2014;164:943. 0022-3476/$ - see front matter. Copyright ª 2014 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.12.001
943
THE JOURNAL OF PEDIATRICS
www.jpeds.com
References 1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969; 87:63-70. 2. Kutlubay Z, Bairamov O, Sevim A, Demirkesen C, Mat MC. Rosai-Dorfman disease: a case report with nodal and cutaneous involvement and review of the literature. Am J Dermatopathol 2013. in press.
Vol. 164, No. 4 3. O’Malley DP, Grimm KE. Reactive lymphadenopathies that mimic lymphoma: entities of unknown etiology. Semin Diagn Pathol 2013;30:137-45. 4. Harley EH. Sinus histiocytosis with massive lymphadenopathy (RosaiDorfman disease) in a patient with elevated Epstein-Barr virus titers. J Natl Med Assoc 1991;83:922-4.
Figure 2. A, Low-power view of biopsy showing lymph node involved by Rosai-Dorfman Disease. The sinuses are markedly dilated with abundant pale histiocytes. B, High-power view of biopsy showing Rosai-Dorfman histiocyte (center) with abundant cytoplasm engulfing lymphocytes, termed emperipolesis. 943.e1
Sivakoti et al
A
n 8-month-old African-American male was admitted with a 2-week history of right-sided neck swelling and fever, treated unsuccessfully with cephalexin. He received intravenous clindamycin with resolution of fever, reduction in size of swelling, and was discharged on oral clindamycin, only to be readmitted 4 weeks later for the same complaint. Examination revealed 2 overlapping firm, tender, and matted masses 5 5 and 5 4 cm in size, in the right anterior cervical and supraclavicular areas respectively, confirmed to be lymph nodes by ultrasound and computed tomography (CT) scan (Figure 1). He had no enlarged glands elsewhere, showed no sign of upper airway obstruction, but was febrile with elevated white cell counts (26 600 and 18 600 cells/mm3) and a left shift on both occasions. Histology from lymph node biopsy is shown (Figure 2; available at www.jpeds.com). Biopsy cultures were negative. First described in 1969,1 Rosai-Dorfman disease is a systemic illness characterized by large, painless bilateral cervical lymphadenopathy, overproduction and accumulation of non-Langerhans sinus histiocytes, and fever with leucocytosis. It usually occurs in children and young adults, mostly with head and neck nodal involvement, and less often in other sites such as mediastinum, inguinal and retroperitoneum.2 Chest CT showed no intrathoracic node
involvement in our patient, among the youngest reported. He had 2 other atypical features: unilateral presentation and tender nodes, both suggestive of lymphadenitis. Extranodal sites can be affected, the commonest being skin, and a purely cutaneous form has been described.2 Of unknown etiology, altered immune responses to infectious agents such as human herpes virus-6 and -8, and Epstein-Barr virus have been suspected.3,4 Our patient tested negative to Epstein-Barr virus, cytomegalovirus, toxoplasmosis, and bartonella by serology. The clinical course is usually benign, with remissions and exacerbations followed eventually by spontaneous resolution within a few years. It may occasionally be complicated by vital organ or structure compression. n This case was an oral presentation at the annual meeting of the Eastern Society for Pediatric Research, Philadelphia, PA, March 22-23, 2013.
Kirti Sivakoti, MD Deepa Doraisamy, MD Department of Pediatrics
Masooma Niazi, MD Department of Pathology
Gary Kronfeld, MD Department of Radiology
Stefan Hagmann, MD, MSc Division of Pediatric Infectious Disease Bronx-Lebanon Hospital Center
Kara Kelly, MD
Figure 1. A, Axial CT image of neck with intravenous contrast at level of hyoid shows bilateral hypervascular lymph nodes in the internal jugular chain. A hypervascular 3.3-cm mass in right submandibular space is consistent with enlarged lymph node (arrow). There is mass effect on the neurovascular structures and upon the airway. B, Axial CT image of neck with intravenous contrast at level of thoracic inlet shows a supraclavicular lymph node measuring 2.4 cm (arrow).
Department of Pediatrics Division of Pediatric Oncology Morgan Stanley Children’s Hospital of New York-Presbyterian Columbia University New York, New York
Murli Purswani, MD Division of Pediatric Infectious Disease Bronx-Lebanon Hospital Center Albert Einstein College of Medicine Bronx, New York
References available at www.jpeds.com
J Pediatr 2014;164:943. 0022-3476/$ - see front matter. Copyright ª 2014 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.12.001
943
THE JOURNAL OF PEDIATRICS
www.jpeds.com
References 1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969; 87:63-70. 2. Kutlubay Z, Bairamov O, Sevim A, Demirkesen C, Mat MC. Rosai-Dorfman disease: a case report with nodal and cutaneous involvement and review of the literature. Am J Dermatopathol 2013. in press.
Vol. 164, No. 4 3. O’Malley DP, Grimm KE. Reactive lymphadenopathies that mimic lymphoma: entities of unknown etiology. Semin Diagn Pathol 2013;30:137-45. 4. Harley EH. Sinus histiocytosis with massive lymphadenopathy (RosaiDorfman disease) in a patient with elevated Epstein-Barr virus titers. J Natl Med Assoc 1991;83:922-4.
Figure 2. A, Low-power view of biopsy showing lymph node involved by Rosai-Dorfman Disease. The sinuses are markedly dilated with abundant pale histiocytes. B, High-power view of biopsy showing Rosai-Dorfman histiocyte (center) with abundant cytoplasm engulfing lymphocytes, termed emperipolesis. 943.e1
Sivakoti et al
