gms | German Medical Science

Objective: Intracranial granular cell tumours (GCT) of the sellar or suprasellar region are very rare, arising from the pituitary stalk or the neurohypophysis. Corresponding to WHO grade I tumour, GCT is generally considered a benign condition. However, data on large patient series and long-term follow-up data are lacking. As the natural history of GCT is unknown, validated treatment strategies are lacking. We here present the first case of GCT, which has been followed up over more than two decades.

Methods: Clinical, imaging and histological data of a 59-year-old female patient were evaluated, who was first diagnosed having GCT at the age of 38. Histological data was reanalyzed over two decades. In a PubMed search of the literature (until November 2011), only 77 clinically documented cases of neurohypophysial GCT were found. Clinical characteristics, treatment strategies and outcome measures were analyzed.

Results: In our patient, first symptoms of endocrinological dysfunction preceded diagnosis of GCT by 7 years, indicating a clinical course of more than 28 years. Clinical symptoms were unspecific and related to the tumour mass effect, consisting in headache, visual and endocrinological disturbances. Later in the disease course, depression, addictive behaviour and psychosis occurred. In three different operations, three different surgical approaches were applied. However, total removal was always prevented by the invasive and firm, highly vascularized nature of the tumour mass. Histological analysis confirmed stable histological characteristics of GCT over more than two decades. While in our case, the potential benefit of additional radiotherapy remains unclear, a review of the literature indicates a possible advantage of combined surgical and radiation therapy in cases of subtotal resection.

Conclusions: To our knowledge, this is the first case of GCT in the literature, which has been followed-up over more than two decades. It shows that even though the histological characteristics of GCT remain stable, progressive growth converts the so-called benign tumour into a potentially fatal condition. It suggests that after subtotal resection, regrowth needs to be carefully monitored by long-term follow-up. Timely repeated tumour resection may prevent irreversible clinical sequelae. The therapeutic value of adjunctive radiotherapy remains to be investigated. Larger patient series with long-term follow up are needed to evaluate optimal treatment strategies in GCT.