SPR Unknown #45-- FINAL

Pinar Karakas, MD

Mustafa Gok, MD

Cleveland Clinic Foundation

Keywords

SPR unknown 45 LCH Langerhans cell histiocytosis


Publication Date: 2011-01-25

History

4 year old male with swelling in the left lateral and left frontal area of his head over the past two months.

Findings

There are two lytic lesions involving the inner and outer tables of the calvarium, one in the left lateral frontal bone with a large soft tissue component and the other one in the left frontal bone near the sagittal suture without significant soft tissue component. There are apparent fluid fluid levels within both lesions well seen on both CT and MRI (Figure 3 CT, Figure 6 MRI T2). The larger lesion has a few septations best seen on the MRI. Following contrast administration there is wall and septal enhancement (Figure 5 post gad T1).

Discussion

Langerhans Cell Histiocytosis is characterized by idiopathic abnormal proliferation of histiocytes in various parts of the reticuloendothelial system such as bone, lungs, central nervous system, skin and lymph nodes creating localized or systemic disease. It is most common in the first decade of life with male predominance. The localized form of disease is commonly referred to as Eosinophilic Granuloma (EG) and is limited to bone or lung. EG represents approximately %70 of cases of LCH and most frequently involves the skull; especially prevalent in the frontal and parietal bones. The radiographic features of LCH of the skull depend on the phase of the lesion. In the incipient phase, it consists of an osteolytic area with poorly delineated borders and lamellar periosteal reaction, mimicking malignant tumor. Later, the lesion becomes sharply delineated and a ring of sclerosis may appear, giving it a more benign appearance. Rare manifestations include cortical lesions, button sequestra, calcification in a soft tissue mass and fluid-fluid levels. For these lesions differential diagnosis included aneurysmal bone cyst and lymphatic malformation.

References

  1. Monroc M, Ducou le Pointe H, Haddad S, Josset P, Montagne JP. Soft tissue abnormality associated with eosinophilic granuloma. Correlation of MRI with pathologic findings. Pediatr Radiol 1994; 24: 328-332
  2. Chen HC, Shen WC, Chou DY, Chiang IP. Langerhans cell histiocytosis of the skull complicated with an epidural hematoma. AJNR Am J Neuroradiol 2002; 23: 493-495
  3. Fahad B. Albadr, Essam A. Elgamal, Ibrahim A. Fluid-fluid level in Langerhans Cell Histiocytosis of the skull. Neurosciences 2006; Vol.11(1): 47-49
  4. Ajay T, Ella O. Fluid-fluid levels in transcalvarial Langerhans' cell histiocytosis. Pediatr Radiol 2004; 34:363
  5. Bernard W, Robert D, Lionel w, Leisure Y. Langerhans cell histiocytosis:unusual skeletal manifestations observered in thirty-four cases. Skeletal Radiol 1998; 27: 177-181

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