Sacrectomy for primary sacral tumors can be safely conducted, achieving tumor-free margins and acceptable functional and long-term outcomes.
Ganglioglioma (GG) is an uncommon primary lesion of the central nervous system that is typically located supratentorially. There are only a few reports of GG arising from the cerebellum. To the best of our knowledge this is the first case of a cerebellar GG with supratentorial extension and a longstanding history before its recognition. In fact, this 29-year-old male presented with an 11-year history of intermittent headaches. A cranial computerized tomography (CT) performed at the onset of his complaints failed to reveal the tumor. After a particularly longstanding cephalalgic episode, the patient underwent a new CT scan that was also negative. However, magnetic resonance (MR) imaging of the brain revealed a space-occupying lesion in the right cerebellar hemisphere with extension to the level of the superior colliculi and pineal recess. The tumor was partially removed through a midline suboccipital craniotomy and supracerebellar approach. Pathological examination of the tumor showed composition of atypical ganglion cells and astrocytes, indicating the diagnosis of cerebellar GG. At last follow-up, 24 months after surgery, the patient reported a marked improvement of his clinical condition with significant reduction of intensity and frequency of the headache. The present report illustrates how cerebellar GG may remain undetectable by CT and may therefore present with a longstanding history and nonspecific signs and symptoms. MR investigation can lead to the proper diagnosis. Even after partial removal the prognosis remains good and remission of the symptoms may be achieved. In this article, we review the literature and summarize the current understanding of infratentorial GGs.
Background. Sacrectomy with adequate margins is challenging because of the complexity of the surgical approach and morbidities. Two-staged sequential approach, therefore, has been advocated. This study was designed to demonstrate the modification of this technique. Methods. This is a case presentation of a 45-year-old man with chordoma involving the lower border of S2, who underwent one-staged subtotal sacrectomy. The technique involved the following: midline incision, mobilization of the rectum, construction of a colostomy and the modified Hartmann stump with intact superior rectal vessels, ligation of internal iliac arteries, ligation of all branches connecting to external iliac veins resulting in ''complete isolation'' of the external iliac veins, dissection of presacral tissue, anterior osteotomy at the S1-S2 junction and the sacroiliac joints, and abdominal closure. The posterior approach involved a threelimbed incision, dissection of the gluteus muscle and ligaments from the sacrum, subperiosteal dissection, S1 laminectomy, posterior osteotomy corresponding with the anterior osteotomy line with preservation of S1 nerves, division of S2-S4 nerves from sciatic nerves, and specimen removal. Closure of the large sacral defect was undertaken using the Hartmann stump and bilateral gluteus maximus flaps. Results. En bloc resection with free margins without tumor rupture was accomplished. Operative time was 12 hours. Blood transfusion was 6 units. This patient had a good recovery without complications. He was able to ambulate within 1 week and walk normally within 1 month. No recurrence was found at a 24-month-followup. Conclusions. One-staged sacrectomy can be safely performed, obtaining the satisfactory outcomes.
Introduction acromegaly, an overproduction of growth hormone (GH), is associated with high rate of morbidity and mortality particularly in case of delayed in diagnosis and treatment. A wide variation of clinical presentations, treatment outcomes and morbidities have been reported. Methods a retrospective study was conducted to review clinical characteristics and treatment outcomes of patients with acromegaly treated in King Chulalongkorn Memorial Hospital, Bangkok, Thailand, between 2006 and 2018. Results eighty-four patients (31 males and 53 females) were reviewed, mean age at diagnosis was 45.7 ± 12.6 years (±SD), mean time of disease onset was 7.6 ± 6.4 years and mean follow-up period was 7.8 ± 5.3 years. The most common presenting symptoms were maxillofacial change (96.8%) and acral enlargement (94.7%). Hypertension (39.3%), diabetes mellitus (28.6%) and dyslipidemia (23.8%) were prevalent co-existing conditions. Four patients were identified having cancer at presentation; however, no additional malignancy was reported during the follow up. Most patients harbored macroadenomas, only 10 were found to have microadenomas. The outcomes of treatment were controlled disease in 70% of microadenoma and 64.9% of macroadenoma. Permanent loss of pituitary function was found in about 21.3% and there was one case reported of mortality. The logistic regression analysis for controlled disease outcome showed the IGF-I index after surgery was associated with controlled disease outcome with statistically significant result (P-value=0.006). Conclusion our study offers descriptive clinical data of case series of acromegalic patients, which had favorable outcomes comparable with previous reports. In addition, IGF-I index after surgery is a predictive parameter for outcome of treatment.
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