55-year follow-up of the first adult patient with craniopharyngioma treated with gamma knife radiosurgery
This is a report on the 55-year follow-up of the first case of a craniopharyngioma patient who underwent gamma knife radiosurgery. The article details the medical history, diagnosis process, gamma knife surgery details, as well as the 55-year follow-up with multiple treatments, providing a comprehensive overview of the patient’s long medical journey.
I. Introduction
The article begins by briefly reviewing the immense challenges of surgically removing craniopharyngiomas before the 1960s and the limited treatment options available at the time. In 1968, Professor Leksell and Professor Backlund at the Karolinska Institute in Sweden successfully developed the gamma knife radiosurgery system at the Sophiahemmet Hospital and performed the first surgery on a 21-year-old female craniopharyngioma patient in May of that year, pioneering this milestone new therapy. Now, 55 years after that historic moment, this patient remains under continuous follow-up, becoming the longest recorded case of gamma knife treatment for craniopharyngioma worldwide.
II. Patient Presentation and Diagnosis
In March 1966, this 19-year-old female patient presented with amenorrhea and weight gain. Physical examination revealed abdominal striae, reduced axillary and pubic hair. Laboratory tests showed low total sex hormone levels. In September 1967, an X-ray revealed an enlarged and calcified sella turcica, suggesting a possible tumor. In February 1968, a pneumoencephalogram revealed a 2x2.5cm calcified oval mass superior to the sella, leading to an initial diagnosis of a craniopharyngioma causing hypogonadism.
III. Neurosurgical Procedure
Professor Backlund attempted a stereotactic biopsy to explore the tumor composition but unexpectedly found it to be a solid tumor rather than the typical cystic type. Therefore, he implanted two silver clips at the tumor’s apex to aid in subsequent imaging localization. Given the tumor’s moderate size, symmetrical shape, and predominantly solid component, the doctors decided to proceed with gamma knife radiosurgery.
IV. Gamma Knife Surgery Details
On May 23, 1968, Professor Leksell, Professor Backlund, and two physicists formed the surgical team and performed the first gamma knife treatment for an adult craniopharyngioma patient in human history.
They first fixed the Leksell stereotactic frame to the patient’s head and determined the spatial coordinates of the tumor center through X-ray imaging. The physicists then manually planned the radiation dose distribution, setting 179 beams of cobalt-60 radiation to deliver a total dose of 50 Gy to the solid tumor portion while avoiding the visual and endocrine areas of the brain. The entire surgery took approximately 30 minutes, and the patient was discharged the following day.
V. Long-term Follow-up and Management of Complications
1) Tumor Control
Routine imaging and clinical follow-up continued for 55 years after the surgery. Initially, the tumor volume decreased significantly within a few months. However, in September 1969, signs of recurrence appeared, and a biopsy revealed a cystic component.
Between 1969 and 1978, the cystic portion of the tumor recurred four times. The doctors performed stereotactic aspiration of the cystic fluid and administered intracavitary radiation therapy with yttrium-90 each time. After the fourth recurrence and meningitis complication in 1978, the cystic component was completely controlled, and no further recurrences occurred for decades. However, the solid tumor tissue persisted but remained stable, and no additional gamma knife treatment was performed. A CT scan in 2021 confirmed no residual tumor.
2) Endocrine Outcomes
The preoperative low sex hormone state was temporarily corrected, but in 1970, hypercortisolism developed. After the cyst recurred in 1976, panhypopituitarism occurred, necessitating long-term replacement therapy with glucocorticoids and thyroid hormone. In 1994, type 2 diabetes mellitus developed, and in 1999, diabetes insipidus occurred, both of which were treated accordingly. The various endocrine disorders were gradually brought under control.
3) Visual Outcomes
Visual acuity remained stable for the first eight years. In 1976, there was a temporary visual decline during the cyst recurrence but recovered after treatment. In 1978, a visual field defect occurred but also resolved with treatment. In 1983, a persistent superolateral quadrantanopia developed, with no clear cause identified on imaging studies. Additionally, diabetic retinopathy developed in later years.
Currently, at 76 years old, the patient can perform daily activities with the assistance of family members, serving as the best evidence of the safety and efficacy of gamma knife treatment.
VI. Discussion
The purpose of this article is to summarize this rare case and evaluate the long-term efficacy and complications of gamma knife surgery for craniopharyngioma. The results indicate that even in an era with limited imaging capabilities, gamma knife treatment can achieve excellent long-term tumor control for moderately sized solid tumors. However, recurrence of the cystic component is unavoidable, and the stereotactic aspiration combined with isotope treatment employed at the time was a viable approach. Endocrine and visual function deficits could not be completely reversed, which were expected complications.
Although the results of a case study cannot be directly generalized, this remarkably long 55-year follow-up strongly demonstrates the safety and efficacy of gamma knife radiosurgery in the treatment of craniopharyngioma, reflecting the pioneering spirit of Professors Leksell and Backlund at the time. It also laid the foundation for the current multidisciplinary treatment approach.
This report comprehensively presents the lengthy medical journey of the first craniopharyngioma patient treated with gamma knife, documenting the clinical application and long-term outcomes of the innovative technology at the time. It holds significant historical and clinical reference value.