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Neuroendocrinologia | Trabalhos em Congressos

Intrasellar internal carotid aneurysm coexisting with GH-secreting pituitary adenoma in an acromegalic patient

Lauro Seda Jr., MD1
Arthur Cukiert, MD, PhD 1
Kátia C Nogueira, MD 2
Martha KP Huayllas, MD, PhD 2
Bernardo Liberman, MD, PhD 2

Departments of Neurosurgery1 and Endocrinology2, Hospital Brigadeiro
And Clínica Neuroendócrina de São Paulo, São Paulo SP, Brazil.


Resumo:
Aneurisma intraselar da carótida interna coexistindo com adenoma secretor de GH em paciente acromegálico.
Seda Jr L, Cukiert A, Nogueira KC, Huayllas MKP, Liberman B
Departamentos de Neurocirurgia e Endocrinologia, Hospital Brigadeiro, e Clínica Neuroendócrina de São Paulo, São Paulo SP, Brasil.

Objetivo e relevância: Relatamos condição extremamente rara em paciente acromegálico no qual um adenoma secretor de hormônio do crescimento (GH) coexistia com um aneurisma intraselar da artéria carótida interna (ACI).

Apresentação Clínica: Uma paciente de 58 anos apresentou-se com cefaléia e fenótipo acromegálico. A ressonância magnética da região selar mostrou um aneurisma de ACI, medindo 1.2 cm, ocupando a hemisela esquerda e um adenoma hipofisário de 0.7 cm logo abaixo do mesmo. A angiografia digital mostrou que o aneurisma emergia posteriormente à artéria oftálmica.

Intervenção: A paciente foi submetida à exclusão do aneurisma por meio de oclusão da ACI no pescoço e clipagem intracraniana da ACI supraclinoidea logo antes da emergência da artéria oftálmica. Um mês depois, ela foi submetida à ressecção completa do adenoma por via transesfenoidal. Durante a cirurgia, o aneurisma previamente excluído pôde ser visibilizado ocupando a hemisela esquerda. Apesar da redução significativa no pós-operatório inicial, os níveis de GH e IGF-1 mantiveram-se elevados no terceiro mês de seguimento. Não foi possível realizar ressonância magnética de controle, pois o clip usado na cirurgia não era compatível com a realização do exame. A paciente foi submetida a tratamento complementar com octreotide e radiocirurgia, resultando em bom controle clínico e laboratorial da doença.

Discussão: Aproximadamente 50% dos pacientes que possuem esta associação patológica possuem acromegalia, sugerindo que os níveis elevados de GH e IGF-1, ou seus efeitos biológicos, podem ser os responsáveis pelo surgimento dos aneurismas. Embora o tratamento destes pacientes represente um desafio, acreditamos que a patologia vascular deva ser tratada inicialmente.

Abstract:
Intrasellar internal carotid aneurysm coexisting with GH-secreting pituitary adenoma in an acromegalic patient.
Seda Jr. L, Cukiert A, Nogueira KC, Huayllas MKP, Liberman B.
Departments of Neurosurgery and Endocrinology, Hospital Brigadeiro, São Paulo SP and Clínica Neuroendócrina de São Paulo, São Paulo SP, Brazil

Objective and importance: We report on an extremely rare condition where a growth hormone (GH) -secreting adenoma coexisted with an intrasellar internal carotid artery (ICA) aneurysm in an acromegalic patient.

Clinical presentation: A 58 years-old female presented with headache and acromegalic phenotype. Magnetic resonance imaging (MRI) of the sellar region disclosed a 1.2 cm ICA aneurysm occupying the left hemisella and a 0.7 cm pituitary adenoma just bellow it. Angiography disclosed left ICA aneurysm posterior to the emergency of the ophthalmic artery.

Intervention: The patient was submitted to aneurysm exclusion through ICA occlusion in the neck and transcranial clipping of the supraclinoid ICA immediately bellow the ophthalmic artery’s exit. One month afterwards, she was submitted to complete adenoma removal through a transesphenoidal approach. The excluded aneurysm was easily seen occupying the entire left hemisella. Even though there was a significant initial reduction, GH and IGF-1 levels remained high after 3 months of follow-up. It was not possible to perform a follow-up MRI since the clip used in surgery was not MRI-compatible. The patient was submitted to a adjuvant therapy with octreotide and radiosurgery resulting in a good control of the disease.

Conclusion: Approximately 50% of patients with this dual-pathology have acromegaly, suggesting that high GH and IGF-1 levels or their biological effects might be implicated in aneurysm’s genesis. Although treatment of these patients might be challenging, approaching the vascular lesion first is usually the best choice.

Intrasellar internal carotid aneurysm coexisting with a GH-secreting pituitary adenoma in an acromegalic patient.

Seda Jr. L,Cukiert A, Zach PL, Silva FFA, Saleh A, Nogueira KC, Huayllas MKP, Silva MER, Jacob RS, Liberman B

Departments of Neurosurgery and Endocrinology, Hospital Brigadeiro São Paulo and Clínica Neuroendócrina de São Paulo, São Paulo SP, Brazil

Introduction: The coexistence of pituitary adenoma and cerebral aneurysm is rare, although its prevalence is higher then would be expected in general population1-5. We report on an extremely rare condition where growth hormone (GH) -secreting adenoma coexisted with an intrasellar internal carotid artery (ICA) aneurysm.

Case Report: A 58 years old female presented with headache and acromegalic phenotype. She had hypertension for 10 years and galactorrhea for 3 years. Mean plasma GH was 8.1 ηg/ml and mean IGF-1 level was 703 ηg/ml (Reference: 78 – 258ηg/ml by immunoradiometric assay). Results obtained during pre- and postoperative Glucose Tolerance Tests (OGTT) and Octreotide Response Test (100μg administered subcutaneously every six hours) can be seen in table 1 and 2, respectively. Prolactin baseline level was 28.6 ηg/ml and pituitary function was otherwise intact. Magnetic resonance imaging (MRI) of the sellar region disclosed a 1.2 cm ICA aneurysm occupying the left hemisella and a 0.7 cm pituitary adenoma just bellow it (Fig. 1A). Angiography disclosed left ICA aneurysm posterior to the emergency of the ophthalmic artery (Fig. 1B). The patient tolerated well a balloon occlusion test and was submitted to aneurysm exclusion through ICA occlusion in the neck and transcranial clipping of the supraclinoid ICA immediately below the ophthalmic artery’s exit. One month afterwards, she was submitted to complete adenoma removal through a transsphenoidal approach (Fig 2). The excluded aneurysm was easily seen occupying the entire left hemisella. Immunohistochemical examination confirmed the presence of GH-secreting adenoma. Immediate postoperative GH and prolactin levels were 3.7 and 5.6 ηg/ml, respectively. Four days later, IGF-1 was 543 ηg/ml (78 – 258 ηg/ml). One month after adenoma removal, GH and IGF1 levels were 5.0 ηg/ml and 527 ηg/ml, respectively. After 3 months of follow-up, patient’s GH and IGF-1 levels were 4.1 ηg/ml and 413 ηg/ml, respectively. It was not possible to perform a follow-up MRI since the clip used in surgery was not MRI-compatible.

She was then started on Sandostatin® LAR 30 mg / month. Four months later, IGF-1 and GH levels were 107 ηg/ml (Reference: 81 - 225 ηg/ml) and 0.9 ηg/ml, respectively. By this time, patient underwent radiosurgery. After two years of follow-up, the patient remained in remussion and receiving Sandostatin LAR.

The case report was agreed by the local ethic committee.

Table 1: Pre- /  Postoperative Oral Glucose Tolerance Test

Time (minutes)

0

30

60

90

120

Glucose level (mg/dl)

93 / 92

193 / 169

181 / 204

150 / 174

108 / 111

GH (ηg/ml)

11.4 / 2.7

6.6 / 1.1

6.8 / 2.7

6.8 / 2.5

7.1 / 2.7


  Table 2: Octreotide Acute Response Test (100μg sc 6/6h)

Time (hours)

0

2

4

6

8

24

26

GH (ηg/ml)

5.5

0.44

0.52

0.63

0.71

0.37

0.22


Neuroendocrinologia
FIG. 1A - A gadollinium-enhanced MRI showing a round flow-void mass in the left hemisella with 1.2 cm (arrow head) associated to a subjadcend pituitary microadenoma (small arrow).
Neuroendocrinologia
FIG. 1B - A lateral-scan angiography showing a left paraclinoid ICA aneurysm directed down into the sella.


Neuroendocrinologia
FIG 2 - A intraoperative plain film showing a lateral view of the sellar region
depicting the relantionship between the previous clipped ICA and the curette
during the transphenoidal removal of adenoma.

Discussion: The prevalence of sellar-region’s aneurysm among others is 1-2% 2. The prevalence of the coexistence of pituitary adenoma and cerebral aneurysm is higher then that with other benign brain tumors in the general population5. Approximately 50% of these patients have acromegaly1,3,5-8, suggesting that high GH and IGF-1 levels or their biological effects might be implicated in the aneurysm’s genesis. High IGF-1 induces artery dilation8, atherosclerotic and degenerative changes of the artery wall8,9, tumor invasion and tumor-directed neovessels9. Hypertension and diabetes are very likely to be involved in the process5. Patients with intrasellar (not intracranial) aneurysms and pituitary adenomas are extremely rare.

Mostly, the diagnosis of such aneurysms is incidental, and occurs when performing the preoperative investigation for adenomas7. However, different clinical presentations may occur, such as fatal epistaxis10 or pituitary apoplexy11 , as a result of aneurismal bleeding into the adenoma. Misdiagnosis of this condition may have hazardous hemorrhagic consequences.

These two conditions must be treated. Although simultaneous microsurgical treatment of the aneurysm and the adenoma through a pterional or a supraorbital keyhole approach has been advocated in different reports6,12,13, approaching the vascular lesion first is usually the best choice. In our case, we performed an ICA entrapment by endovascular occlusion in the neck and transcranial clipping of ICA just bellow the ophthalmic artery’s emergence. This treatment was possible because the patient tolerated well a previous balloon occlusion test. One month later, the adenoma was completely removed through a transsphenoidal route. Nevertheless, the patient did not achieved endocrinological remission and needed adjuvant therapy with Octreotide and radiosurgery. Clinical and endocrinological control were then obtained.

Although the results obtained while treating this patient were good, a minimally invasive therapeutic option would also be available for such patients: the vascular lesion could be treated by endovascular trapping of the internal carotid artery and the GH-secreting tumor could have been treated primarily with somatostatin analogs or transsphenoidal surgery. Primary clinical treatment with somatostatin should be considered especially in patients with known cavernous sinus invasion by the tumor and no mass effect directed to the optic apparatus 14-15.

We believe that the treatment of this dual-pathology should be carried out in two steps: vascular pathology should be treated first to avoid potential future catastrophic hemorrhage and the pituitary pathology afterwards.

References:

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14. Vieira JO, Cukiert A, Liberman B. Evaluation of MRI criteria for cavernous sinus invasion in patients with pituitary adenoma: logistic regression analysis and correlation with surgical findings. Surg Neurol 65: 130-5, 2006.
15. Vieira JO, Cukiert A, Liberman B. MRI of cavernous sinus invasion by pituitary adenoma: diagnostic criteria and surgical findings. Arq Neuropsiquiatr 62: 437-43, 2004.