Answer for BIR CoW 23 Sep 2018
Eosiniphilic granuloma
Findings
Plain Radiograph Skull shows a well defined minimally expansile lytic lesion with no sclerotic rim in the right parietal bone. MRI -Relatively well defined expansile intradiploic lesion noted in right parietal bone with destruction of inner and outer table of skull. The lesion appears iso to hypointense in TIWI and hyperintense in T2WI , with no restricted diffusion. On contrast the lesion shows peripheral rim enhancement. Geographic Lytic lesion in the parietal bone in a child must immediately prompt one to suggest Eosinophilic Granuloma, the other possibility being Osteomyelitis Biopsy of the lesion confirmed the diagnosis.
Discussion
Langerhans cell histiocytosis, previously called histio cytosis X, encompasses a spectrum of multi-system lymphoreticular disorders that predominantly affect children and young adults . Three overlapping clinical variants have been recognized. Letterer-Siwe disease (about 10%) is the acute fulminant form with poor prognosis. It involves skin, liver, spleen, lymph nodes, and bone primarily in children under two years of age Hand-Schuller-Christian disease (15-40%) is the chronic form with variable prognosis; it involves skeletal, reticuloendothelial, and other visceral sites predominantly in children between five and ten years of age. Eosinophilic granuloma (EG) is the mildest form of Langerhans cell histiocytosis (LCH), a rare proliferative disorder of Langerhans type histiocytes with an unknown etiology . It occurs during the first three decades of life with peak incidence between five and ten years The skeleton is the most commonly involved organ system in Langerhans cell histiocytosis (LCH) and is by far the most common location for single-lesion LCH, often referred to as eosinophilic granuloma (EG) Especially in the skull, Langerhans cell histiocytosis appears to most commonly involve the parietal bone, followed by the frontal bone Plain radiographs of Skull solitary or multiple punched out lytic lesions without sclerotic rim double contour or beveled edge appearance may be seen due to asymmetrical involvement of the inner and outer tables (hole within a hole) sign button sequestrum representing residual bone geographic skull MRI: T1: typically hypointense to isointense T2: hyperintense STIR: hyperintense T1 C+ (Gd): often shows diffuse contrast enhancement. In cases of central necrosis peripheral rim enhancement noted. General imaging differential considerations include: osteomyelitis round cell tumours metastases primary bone tumours lymphoma leukaemia
Findings
Plain Radiograph Skull shows a well defined minimally expansile lytic lesion with no sclerotic rim in the right parietal bone. MRI -Relatively well defined expansile intradiploic lesion noted in right parietal bone with destruction of inner and outer table of skull. The lesion appears iso to hypointense in TIWI and hyperintense in T2WI , with no restricted diffusion. On contrast the lesion shows peripheral rim enhancement. Geographic Lytic lesion in the parietal bone in a child must immediately prompt one to suggest Eosinophilic Granuloma, the other possibility being Osteomyelitis Biopsy of the lesion confirmed the diagnosis.
Discussion
Langerhans cell histiocytosis, previously called histio cytosis X, encompasses a spectrum of multi-system lymphoreticular disorders that predominantly affect children and young adults . Three overlapping clinical variants have been recognized. Letterer-Siwe disease (about 10%) is the acute fulminant form with poor prognosis. It involves skin, liver, spleen, lymph nodes, and bone primarily in children under two years of age Hand-Schuller-Christian disease (15-40%) is the chronic form with variable prognosis; it involves skeletal, reticuloendothelial, and other visceral sites predominantly in children between five and ten years of age. Eosinophilic granuloma (EG) is the mildest form of Langerhans cell histiocytosis (LCH), a rare proliferative disorder of Langerhans type histiocytes with an unknown etiology . It occurs during the first three decades of life with peak incidence between five and ten years The skeleton is the most commonly involved organ system in Langerhans cell histiocytosis (LCH) and is by far the most common location for single-lesion LCH, often referred to as eosinophilic granuloma (EG) Especially in the skull, Langerhans cell histiocytosis appears to most commonly involve the parietal bone, followed by the frontal bone Plain radiographs of Skull solitary or multiple punched out lytic lesions without sclerotic rim double contour or beveled edge appearance may be seen due to asymmetrical involvement of the inner and outer tables (hole within a hole) sign button sequestrum representing residual bone geographic skull MRI: T1: typically hypointense to isointense T2: hyperintense STIR: hyperintense T1 C+ (Gd): often shows diffuse contrast enhancement. In cases of central necrosis peripheral rim enhancement noted. General imaging differential considerations include: osteomyelitis round cell tumours metastases primary bone tumours lymphoma leukaemia
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!