Susanne Oechsle (Klinikum Augsburg/Abteilung Kinderradiologie/Augsburg/Germany), Kurt Vollert (Klinikum Augsburg/Abteilung Kinderradiologie/Augsburg/Germany)  

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5 Years  

Female  

Head-Bones  

neoplastic  

History: Fall on the back of the head 11 days ago. According to mother she developed increased swelling in the left occipital area. There are no neurological deficits.
Physical examination: Painful palpable left occipital swelling.  

Eosinophilic granuloma is the most common and the least severe varient of the Langerhans Cell histiocytosis. Most commonly found in the skull, femur pelvis, ribs and vertebral column. Most monostotic - in 20% of the cases there are multiple masses. Included in the Langerhans Cell Histiocytosis spectrum are the multisystemic forms - Abt-Letterer-Siwe and the Hand-Schüller-Christian illnesses. (I)

The eitology of the Langerhans Cell Histiocytosis is still unclear. There is a reactice proliferation and/or accumulation of dendritic cells. Assumingly, there is an intercellular communication defect with cytokine-disregulation. As of now, there is no proof of malignancy. (II)

X-ray morphology is mainly dependent on the stage of the eosinophilic granuloma. Morphology is very diverse, including aggressive as well as latent and active growth patterns possible.
In the calvarium, there are usually round or oval osteolyses with diameters up to 3 cm. Most are sharply demarcated, as if "punched" through, but in the acute phase there are also unsharp borders possible. In the healing phase, one may find border sclerosis (50%). Bony leftovers in the mid-section of the lesion are seen as button sequesters. In a severe case, there may be a "map-like" picture. (I)  

<- view Ultrasound 1

Ultrasound 1: Ultrasound of the back of the head
Left occipital identified is a 2 cm, subcutaneous, inhomogeneous and hypoechoic mass lesion. In this area, there is about 12 mm cortical discontinuity. Dura appears deviated inferiorly.
Color coded Duplex sonography: Increased vascularization in the periphery of the lesion. (Images not shown)
Ultrasound of the abdomen is unremarkable. (Images not shown)




<- view CT 1

CT 1: CCT Brain window
Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






<- view CT 2

CT 2: CCT Bone window
Left occipital osteolytic lesion with soft tissue components, no further osteodestructive findings.






<- view MRI 1

MRI 1: MRI of the head - T1 axial
Left occipital soft tissue mass with marked marginal contrast enhancement, size ca. 1,8 x 2,8 x 1,5 cm. It causes the known defect in the posterior skull and mild dural displacement to the inside.
There is surrounding leptomeningeal contrast enhancement. There is increased T2 signal and fluid level in the center of the lesion, which represents a trauma related secondary hemorrhage.





<- view MRI 2

MRI 2: MRI of the head - T2 axial






<- view MRI 3

MRI 3: MRI of the head - T1 axial with contrast






<- view MRI 4

MRI 4: MRI of the head - T1 spir coronal (oblique coronal with surface coil) with contrast



 

Surgery / Histo  

Lymphoma, solitary bone metastasis of a Neuroblastoma  

Treatment options in Langerhans Cell Histiocytosis: Surgical excision in a solitary skeletal mass (II). Intralesional steroid injection have been tried with questionable results (I). In multisystemic masses and multiple bony lesions, a systemic treatment with corticosteroids, cytostatics and immunosuppressives. Radiation only as a last resort (II).

The course and prognosis of Langerhans Cell Histiocytosis:
Monosystemic lesions independent of the treatment have a good prognosis. Multisystemic lesions with organ dysfunction in particularly small children is seen as having a poor prognosis, with possible lethal course. (II)

Eosinophilic granulomas are mostly seen between the ages of 5 and 10 years. Male gender predominates (makes up of about 60-80% of all Langerhans Cell Histiocytoses). (II)  

In this case, there was an unnoticed development of a eosinophilic granuloma in the skull. After trauma, there was hemorrhaging and pain, then the noticed swelling on the left, occipital side.
Final diagnosis and treatment was accomplished through resection of the mass.  

N/A  

(I) Bohndorf, Imhof, Fischer:
Radiologische Diagnostik der Knochen und Gelenke.
Thieme Verlag 2006. S. 208-209.
(II) Staatz, Honnef, Piroth, Radkow:
Kinderradiologie. In: Pareto-Reihe Radiologie.
Thieme Verlag 2007. S. 257-262.
 

solitary osteolysis of the skull, langerhans cell histiocytosis, LCH, eosinophilic granuloma, lymphoma, neuroblastoma, swelling of head, child, childhood, pediatric radiology  

Susanne Oechsle, Kurt Vollert. Eosinophilic granuloma / monostotic bone lesion of a langerhans cell histiocytosis (LCH). PedRad [serial online] vol 7, no. 6.
URL: www.PedRad.info/?search=20070611231443