Acta Neurol Scand 2014: 130: 18–26 DOI: 10.1111/ane.12209
© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd ACTA NEUROLOGICA SCANDINAVICA
Classification of hypothalamic hamartoma and prognostic factors for surgical outcome Li CD, Luo SQ, Tang J, Jia G, Ma ZY, Zhang YQ. Classification of hypothalamic hamartoma and prognostic factors for surgical outcome. Acta Neurol Scand 2014: 130: 18–26. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. Objectives – The main aims of this study were to classify patients with hypothalamic hamartoma (HH) based on neuroimaging features and describe the clinical manifestations of HH. Materials and methods – A retrospective review of 214 consecutive patients with HH treated in Beijing Tiantan Hospital was performed. Results – HH were diagnosed and divided into Types I–IV based on MRI. Types I and II were defined as the HH attached to the floor of the third ventricle with narrow (Type I) or broad (Type II) interfaces. Type III (‘straddling’) was defined by the HH extending into the third ventricle and interpeduncular cistern. Type IV was defined as the HH located totally within the third ventricle. The percent distribution of patients was 35.9% Type I, 12.1% Type II, 40.7% Type III, and 11.2% Type IV. The percentage of patients with precocious puberty was highest in Type I (81.8%). The percentage of patients with gelastic seizures was highest in Type IV (91.7%). After surgery, 20% (1/5) of patients with Type II HH, 48.8% (20/42) with Type III, and 91.7% (11/12) with Type IV were free of epileptic seizures. Significant prognostic factors for surgical outcome were HH size, surgical approach, and resection level. Conclusions – The clinical manifestations of HH are correlated with the topology of the HH in relation to the hypothalamus. Our results suggest that patients with Type IV HH have the best outcome from surgery and neurosurgeons should be cautious about performing surgery on patients with Type II and Type III HH.
Introduction
Hypothalamic hamartoma (HH) is a rare intracranial, non-neoplastic, congenital malformation resembling gray matter that is characterized by several peculiar symptoms, including precocious puberty (PP) and/or gelastic seizures (GS). These lesions have cytologically normal neurons and glia but the distribution of these cells is abnormal. In recent years, the reports of this disease have gradually increased as a result of advances in magnetic resonance imaging (MRI) (1–16). It has been estimated that the incidence of HH ranges from 1/100,000 to 1/1,000,000 (4). Some patients undergo gradual deterioration in intellectual abilities and behavior (1, 17– 23). The neuroradiological diagnosis of HH is 18
C. D. Li, S. Q. Luo, J. Tang, G. Jia, Z. Y. Ma, Y. Q. Zhang Department of Neurosurgery, Beijing Tiantan Hospital, Capital University of Medical Sciences, Beijing, China
Key words: classification; gelastic seizure; hypothalamic hamartoma; precocious puberty; prognosis; surgery; symptoms S. Q. Luo, Department of Neurosurgery, Beijing Tiantan Hospital, Capital University of Medical Sciences, Tiantan Xili 6, Beijing 100050, China Tel.: +86 10 6709 6519 Fax: +86 10 6705 1377 e-mail: [email protected] Accepted for publication November 7, 2013
based on the confirmation of the following criteria: an isointense lesion on T1-weighted MRI relative to normal gray matter, a pedunculated or sessile HH attached to the hypothalamus, no contrast enhancement on MRI or computed tomography (CT) scanning and an absence of radiological progress on repeated examinations (24–28). The morphology of HH found on MRI is closely related to the clinical presentation (1, 3, 28–31). A number of classification schemes have been developed to describe HH based on anatomical features seen on neuroimaging (19). In 2004, the senior author of this study (Luo SQ) published a neuroimaging classification of HH based on 76 initial cases (23). The main aims of this study were to classify HH using a large number of
1/77 4/26 32/87 1/24 38/214 (10.4%) (50.0%) (92.0%) (91.7%) (57.5%) 8/77 13/26 80/87 22/24 123/214 (3.9%) (26.9%) (73.6%) (91.7%) (44.9%) 3/77 7/26 64/87 22/24 96/214 (81.8%) (46.2%) (43.7%) (8.3%) (53.7%) 63/77 12/26 38/87 2/24 115/214 (9.1%) (19.2%) (1.1%) (4.2%) (6.5%) 7/77 5/26 1/87 1/24 14/214 Asy, asymptomatic; EP, epilepsy; GS, gelastic seizures; PP, precocious puberty. All EP (all kinds of epilepsy, include GS, drop attacks, focal seizures and generalized epilepsy, etc.); Max, Maximum; D, Diameter. *The mean onset age of 63 cases of PP in Type I was 1.5 1.9 years.
(7.8%) (15.4%) (17.2%) (4.2%) (12.1%) 6/77 4/26 15/87 1/24 26/214 (1.3%) (19.2%) (62.1%) (83.3%) (37.4%) 1/77 5/26 54/87 20/24 80/214 (81.8%) (46.2%) (19.5%) (8.3%) (43.9%) 63/77 12/26 17/87 2/24 94/214 4–30 8–38 9–70 8–16 3–70 7.6* 5.0 4.4 3.0 5.7
13.6 18.7 22.1 11.3 17.4
6.3 7.2 9.8 2.2 8.8 3.3 4.0 2.4 2.0 2.9 39/38 14/12 58/29 18/6 129/85 (36.0%) (12.1%) (40.7%) (11.2%)
Max. D (mm)
Mean SD Gender(M/F) Num.
Patients were required to visit the outpatient department at 3, 6, 12, and 24 months after surgery. Subsequently, the patients were followed up with unscheduled outpatient department visits
Type
Follow-up
Age At onset (years)
The transcallosal interforniceal approach was used for 35 patients, the lamina terminalis approach for one patient, and the pterional approach for 57 patients. The surgical approach selected was based on location of the HH. The extent of resection was determined by postoperative MRI. Total resection (TR) was defined as 100% of the tumor removed, subtotal resection (STR) as >95% of the tumor removed, extensive partial resection (EPR) as between 51% and 95% of the tumor removed, and partial resection (PR) as
© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd ACTA NEUROLOGICA SCANDINAVICA
Classification of hypothalamic hamartoma and prognostic factors for surgical outcome Li CD, Luo SQ, Tang J, Jia G, Ma ZY, Zhang YQ. Classification of hypothalamic hamartoma and prognostic factors for surgical outcome. Acta Neurol Scand 2014: 130: 18–26. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. Objectives – The main aims of this study were to classify patients with hypothalamic hamartoma (HH) based on neuroimaging features and describe the clinical manifestations of HH. Materials and methods – A retrospective review of 214 consecutive patients with HH treated in Beijing Tiantan Hospital was performed. Results – HH were diagnosed and divided into Types I–IV based on MRI. Types I and II were defined as the HH attached to the floor of the third ventricle with narrow (Type I) or broad (Type II) interfaces. Type III (‘straddling’) was defined by the HH extending into the third ventricle and interpeduncular cistern. Type IV was defined as the HH located totally within the third ventricle. The percent distribution of patients was 35.9% Type I, 12.1% Type II, 40.7% Type III, and 11.2% Type IV. The percentage of patients with precocious puberty was highest in Type I (81.8%). The percentage of patients with gelastic seizures was highest in Type IV (91.7%). After surgery, 20% (1/5) of patients with Type II HH, 48.8% (20/42) with Type III, and 91.7% (11/12) with Type IV were free of epileptic seizures. Significant prognostic factors for surgical outcome were HH size, surgical approach, and resection level. Conclusions – The clinical manifestations of HH are correlated with the topology of the HH in relation to the hypothalamus. Our results suggest that patients with Type IV HH have the best outcome from surgery and neurosurgeons should be cautious about performing surgery on patients with Type II and Type III HH.
Introduction
Hypothalamic hamartoma (HH) is a rare intracranial, non-neoplastic, congenital malformation resembling gray matter that is characterized by several peculiar symptoms, including precocious puberty (PP) and/or gelastic seizures (GS). These lesions have cytologically normal neurons and glia but the distribution of these cells is abnormal. In recent years, the reports of this disease have gradually increased as a result of advances in magnetic resonance imaging (MRI) (1–16). It has been estimated that the incidence of HH ranges from 1/100,000 to 1/1,000,000 (4). Some patients undergo gradual deterioration in intellectual abilities and behavior (1, 17– 23). The neuroradiological diagnosis of HH is 18
C. D. Li, S. Q. Luo, J. Tang, G. Jia, Z. Y. Ma, Y. Q. Zhang Department of Neurosurgery, Beijing Tiantan Hospital, Capital University of Medical Sciences, Beijing, China
Key words: classification; gelastic seizure; hypothalamic hamartoma; precocious puberty; prognosis; surgery; symptoms S. Q. Luo, Department of Neurosurgery, Beijing Tiantan Hospital, Capital University of Medical Sciences, Tiantan Xili 6, Beijing 100050, China Tel.: +86 10 6709 6519 Fax: +86 10 6705 1377 e-mail: [email protected] Accepted for publication November 7, 2013
based on the confirmation of the following criteria: an isointense lesion on T1-weighted MRI relative to normal gray matter, a pedunculated or sessile HH attached to the hypothalamus, no contrast enhancement on MRI or computed tomography (CT) scanning and an absence of radiological progress on repeated examinations (24–28). The morphology of HH found on MRI is closely related to the clinical presentation (1, 3, 28–31). A number of classification schemes have been developed to describe HH based on anatomical features seen on neuroimaging (19). In 2004, the senior author of this study (Luo SQ) published a neuroimaging classification of HH based on 76 initial cases (23). The main aims of this study were to classify HH using a large number of
1/77 4/26 32/87 1/24 38/214 (10.4%) (50.0%) (92.0%) (91.7%) (57.5%) 8/77 13/26 80/87 22/24 123/214 (3.9%) (26.9%) (73.6%) (91.7%) (44.9%) 3/77 7/26 64/87 22/24 96/214 (81.8%) (46.2%) (43.7%) (8.3%) (53.7%) 63/77 12/26 38/87 2/24 115/214 (9.1%) (19.2%) (1.1%) (4.2%) (6.5%) 7/77 5/26 1/87 1/24 14/214 Asy, asymptomatic; EP, epilepsy; GS, gelastic seizures; PP, precocious puberty. All EP (all kinds of epilepsy, include GS, drop attacks, focal seizures and generalized epilepsy, etc.); Max, Maximum; D, Diameter. *The mean onset age of 63 cases of PP in Type I was 1.5 1.9 years.
(7.8%) (15.4%) (17.2%) (4.2%) (12.1%) 6/77 4/26 15/87 1/24 26/214 (1.3%) (19.2%) (62.1%) (83.3%) (37.4%) 1/77 5/26 54/87 20/24 80/214 (81.8%) (46.2%) (19.5%) (8.3%) (43.9%) 63/77 12/26 17/87 2/24 94/214 4–30 8–38 9–70 8–16 3–70 7.6* 5.0 4.4 3.0 5.7
13.6 18.7 22.1 11.3 17.4
6.3 7.2 9.8 2.2 8.8 3.3 4.0 2.4 2.0 2.9 39/38 14/12 58/29 18/6 129/85 (36.0%) (12.1%) (40.7%) (11.2%)
Max. D (mm)
Mean SD Gender(M/F) Num.
Patients were required to visit the outpatient department at 3, 6, 12, and 24 months after surgery. Subsequently, the patients were followed up with unscheduled outpatient department visits
Type
Follow-up
Age At onset (years)
The transcallosal interforniceal approach was used for 35 patients, the lamina terminalis approach for one patient, and the pterional approach for 57 patients. The surgical approach selected was based on location of the HH. The extent of resection was determined by postoperative MRI. Total resection (TR) was defined as 100% of the tumor removed, subtotal resection (STR) as >95% of the tumor removed, extensive partial resection (EPR) as between 51% and 95% of the tumor removed, and partial resection (PR) as
