LOCALIZED LANGERHANS' CELL HISTIOCYTOSIS OF THE CHEST TREATED SURGICALLY

Langerhans' cell histiocytosis is characterized by a wide spectrum of clinical manifestations, depending on the localized or diffuse form of the disease. This report describes a 27-year-old male patient, a heavy smoker, who presented with a localized bone lesion in the anterior aspect of the right 9th rib. The cardinal symptom was pain in the right lower hemithorax, aggravated by respiratory movements, without any pulmonary or systemic manifestations. A CT scan of the thorax showed a lytic lesion of the right 9th rib. Lung parenchyma was normal. A bone scan showed increased uptake by the anterior aspect of the 9th rib, but no other abnormality. Surgical excision of the involved segment of the right 9th rib was performed. Histological examination of the operative sample showed the characteristic Langerhans' cells. The diagnosis of Langerhans' cell histiocytosis was confirmed by immunohistochemical stain of the bone tissue and detection of S-100 protein, which is considered diagnostic. During a 6-year follow-up period, the patient remained asymptomatic, without anyevidence of recurrence. Localized Langerhans' cell histiocytosis of the bone, if accessible as in our case, may be treated effectively by surgical excision.Key Words: Langerhans' cells, Bone lytic lesion, S-100 protein

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  • Beckstead JH, Wood QS, Turner RR. histiocytosis X cells and Langerhans' cell: enzyme, histochemical 'and immunologic similarities. Human Pathol 1984; 15:529-533.
  • Flint A, Loyd RV, Colby TV, Wilson BW.
  • Pulmonary histiocytosis X.
  • Immunoperoxidase staining Lor HLA-DR antigen and S-IOO protein. Arch Path Lab Med
  • ,1 10:930-933.
  • Weber D, Tron V, Askin F, Churg A. S-100 protein staining in the diagnosis of eosinophilic granuloma of lung. Am J Clin Path 1985;84:447-453.
  • Papadimitriou C. Textbook of Pathology. Athens: Litsas Editions, 1990:412-414.
  • Egeler RM, Favara BE, Van Meurs M. Laman SD, Claasen E. Differential in situ cytokine profiles of Langerhans' cell histiocytosis. Abundant expression of cytokines relevant to disease and treatment. Blood 1999:94:4195- 4201.
  • Mierau GW, Favara BE, Brenman JM. Electron microscopy in histiocytosis X. Ultrastruct Pathol 1982;3:137-142.
  • hance AJ, Bassat F. Langerhans cell granulomatosis. In: Stein JH, ed. Internal Medicine. St Louis: Mosby, 1994:1701-1704.
  • Fernandez-Lattore F, Menor-Serrano F, Alonso- Charterina S, Arenas-Jimenez J. Langerhans' cell histioi ytosis of the temporal bone in pediatric patients. Imaging and follow-up. Am. J Roentgenol 2000; 1 74:21 7-221.
  • Makley JT, Carter JR. Eosinophilic granuloma of bone. Clin Orthop 1986;204:37-44.
  • Beltran J, Aparisi F, Bonmati LM, Rosenberg ZS, Present D, Steiner GC. Eosinophilic granuloma. MR! manifestations. Skelet Radiol 1993;22:157-161.
  • De Schepper AM, Ramon F, Van Marek E. MR imaging of eosinophilic granuloma. Report of II cases. Skeletal Radiol 1993;22:163-166.
  • Elsheikh T, Silverman JF, Wakely PE, Holbrook CT, Joshi VV. Fine needle aspiration cytology of Langerhans' cell. Diagn Cytopathol 1991;7:261-266.
  • Shabb IT, Fanning C V, Carrasco CH, et al. Diagnosis of eosinophilic granuloma of bone by fine needle aspiration with concurrent institution of therapy. A cytologic, histologic clinical and radiographic study of 27 cases. Diagn Cytopathol 1993;9:3-12.
  • Greis PE, Hankin FM. Eosinophilic granuloma. The management of solitary lesions of bone. Clin Orth 1999;257:204-221.
  • Tomashefski JF, Rhiyami A, Rleinerman J. Tieoplasms associated with pulmonary eosinophilic granuloma. Arch Pathol Lab Med 1991,115:499-506.
  • Aguayo SM, Ring TE, Waldron JA, Sherritt RM, Rane MA, Miller YE. Increased pulmonary neuroendocrine cells with bombesin-like immunoreactivity in adult patients with eosinophilic granuloma. J Clin Invest 1990;86:838-844.
  • 7. Youkeles LH, Grizzanti JIT, Liao Z, Chang CJ, Rosenstreich DL. Decreased tobacco- glycoprotein-induced lymphocyte proliferation in vitro in pulmonary eosinophilic granuloma. Am. J Resp Crit Care Med 1995; 151:145-150.