11-months-old male showed signs of tumefaction in the left frontotemporal region. An ultrasound showed a solid noncalcified neoformation, and a brain CAT scan showed a small solid lesion resulting in erosion of the cranial theca both in the external and internal table. Based on clinical status and tomodensitometric appearance, the diagnosis of suspected eosinophilic granuloma or hystiocytosis X was made, and a surgical curettage of the craniolacunia was recommended in order to comfirm the diagnosis.
1) What is your opinion about the diagnosis of this condition?
2) What is your opinion about the requirement to perform the indicated surgical procedure?
3) What is the risk of relapse, following the procedure, and are there any therapies that should be undertaken?
4) Which centres of excellence in Italy would be indicated for the procedure and follow-up?
Sex: M,Age: 11 months
Diagnosis: Left frontotemporal craniolacunia.
At the beginning of March 2008, there were signs of tumefaction not due to trauma in the left frontotemporal region.
On the advice of a pediatrician, an ultrasound was carried out initially on 03/2008 which showed a solid, noncalcified, neoformation associated with erosion of the cranial theca. The dimensions of this lesion are as follows: 13 x 7 mm.
In addition, a brain CAT scan with no contrast medium was carried out on 03/2008, which showed “a small solid lesion of 2 cm diameter resulting in erosion of the cranial theca both in the external and internal table. No observed encephalic abnormalities. No other thecal abnormalities.”
On the same day, the patient attended a specialist neurosurgical visit, during which it was observed that the lesion had increased in the last 10 days.
Based on clinical status and tomodensitometric appearance, the specialist diagnosed a suspected eosinophilic granuloma or hystiocytosis X, referring the patient for a further neurosurgical visit to Professor R.
Professor R. confirmed that the anamnesis and neuroradiology (CAT scan) information are compatible with a thecal lesion that is likely attributable to hystiocytosis (clinical features, bone erosion, tendency to spontaneous repair). In addition, Professor R. indicated that the only way to make a diagnosis is to perform a surgical curettage of the craniolacunia.
This surgery is scheduled for April 2008.
From the medical history, about six weeks ago appeared a swollen of the skin in the left temporal region. On the CT scan of the skull the lesion of about 14x7 mm seems to invade the temporal bone. The invasion is more of the external table and less of the internal table of the bone. There is no difference between the subcutaneous and the tumor soft tissue. Usually those lesions are Histiocytosis X – Eosinophillic granuloma . For such lesions it is recommended a surgical excision. If one wants to see better the tumor soft tissue a MRI scan can be done.
Eosinophillic granuloma can present in one location or multiple location.
In case of one location the therapy is surgical excision only. Usually if the surgery has been done properly there are no local recurrences. In case of multiple lesions biopsy should confirm the diagnosis and the patient should get chemotherapy.
In the patient's case the lesion is a single one in the skull and therefore the only therapy should be a surgical excision of the lesion. A local recurrence of the lesion is unlikely.
I recommended that after surgery the patient should get isotope bone scan.