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LETTER TO EDITOR
Year : 2013  |  Volume : 61  |  Issue : 2  |  Page : 186-187

Clival tumor presenting with acromegaly


1 Department of Neurosurgery, NIMHANS (National Institute of Mental Health and Neurosciences), Bangalore - 560 029, India
2 Department of Neuropathology, NIMHANS (National Institute of Mental Health and Neurosciences), Bangalore - 560 029, India

Date of Submission28-Oct-2012
Date of Decision29-Oct-2012
Date of Acceptance07-Mar-2013
Date of Web Publication29-Apr-2013

Correspondence Address:
Dhaval P Shukla
Department of Neurosurgery, NIMHANS (National Institute of Mental Health and Neurosciences), Bangalore - 560 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.111150

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How to cite this article:
Konar S, Devi BI, Yasha T C, Shukla DP. Clival tumor presenting with acromegaly. Neurol India 2013;61:186-7

How to cite this URL:
Konar S, Devi BI, Yasha T C, Shukla DP. Clival tumor presenting with acromegaly. Neurol India [serial online] 2013 [cited 2019 Aug 23];61:186-7. Available from: http://www.neurologyindia.com/text.asp?2013/61/2/186/111150


Sir,

A 51-years-old lady presented with coarsening of facial features and amenorrhea since 20 years. She presented with voice change and visual blurring of three months duration. She had features of acromegaly. Examination of ocular fundi showed bilateral optic atrophy and visual acuity was 4/60 in right eye and 6/36 in left eye. Serum growth hormone (GH) was 96 ng/ml and other hormonal profile was normal. Magnetic resonance imaging (MRI) of brain showed a tumor in clivus, sphenoid and left ethmoid sinuses with extension into cavernous sinus till orbital apex, and sella. The tumor was isointense on T1-weighted and T2-weighted images with some areas of blooming on gradient sequences. Pituitary gland was not seen separately [Figure 1]. As she had clinical features of acromegaly for 20 years without visual dysfunction, we presume that the tumor origin was primarily in clivus and then invaded all around. She underwent microscopic transnasal-transsphenoidal decompression of tumor. The tumor could not be removed totally because of multi-compartmental extension. Serum GH level fell to 48 ng/ml in immediate post-operative period. Histology and immunohistochemistry of the excised lesion revealed GH secreting pituitary adenoma [Figure 2]. Except for CSF rhinorrhea, which was managed with lumbar drain, her post-operative period was uneventful and she was sent for radiotherapy for residual tumor.
Figure 1: Postcontrast MRI of head (a) Sagittal section showing the tumor in clivus, sphenoid sinus, ethmoid sinus, nasopharynx, nasal cavity and sella. (b) Axial section showing the tumor in sphenoid, cavernous sinus, and orbit apex

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Figure 2: Monomorphous pituitary adenoma cells with diffuse cytoplasmic positivity for Growth hormone. (Immunostaining for Growth hormone, Original magnification 200x)

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The location of majority of ectopic pituitary tumors is in the sphenoid sinus, cavernous sinus or nasopharynx. [1],[2],[3] Primary clival location is described in only few cases. The majority of ectopic pituitary tumors are biochemically active, secreting adrenocorticotrophic hormone (ACTH) or prolactin (PRL). [1] The GH secreting clival pituitary adenoma is rare [Table 1]. [1],[2],[3],[4] Surgery is required to confirm diagnosis and reduce GH burden in ectopic tumors, which was performed in the majority of reported patients, as well as in our patient. In one report, the ectopic GH secreting adenoma was managed successfully with medical therapy. [4] However, in the absence of clinical and laboratory evidence of hormone hypersecretion, the location and radiologic appearance of ectopic pituitary adenomas can mimic a variety of skull base lesions including chordomas, meningiomas, germ cell tumors, lymphoma and metastases. In such cases surgery should always be done, and tissue should be subjected to immunohistochemistry for neuroendocrine markers and pituitary markers for diagnosis of ectopic pituitary adenomas.
Table 1: Clinical characteristics of reported cases of clival GH secreting pituitary adenomas

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  References Top

1.Choi JH, Park MK, Choi SS, Kim KU. Ectopic Growth Hormone-Secreting Pituitary adenoma of the Clivus. J Korean Neurosurg Soc 2006;39:306-9.  Back to cited text no. 1
    
2.Bhatoe HS, Kotwal N, Badwal S. Clival pituitary adenoma with acromegaly: Case report and review of literature. Skull Base 2007;17:265-8.  Back to cited text no. 2
    
3.Appel JG, Bergsneider M, Vinters H, Salamon N, Wang MB, Heaney AP. Acromegaly due to an ectopic pituitary adenoma in the clivus: case report and review of literature. Pituitary 2012;15(Suppl 1):S53-6.  Back to cited text no. 3
    
4.Ferraz-Filho JR, Torres US, Teixeira AC, Castro ML, Dias MA. Ectopic growthhormone-secreting pituitary adenoma involving the clivus treated with octreotide: Role of magnetic resonance imaging in the diagnosis and clinical follow-up. Arq Neuropsiquiatr 2012;70:744-5.  Back to cited text no. 4
    


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This article has been cited by
1 A case report of an ectopic clival growth hormone adenoma associated with an empty sella and a review of the literature
Bin Liu,Zerui Zhuang,Jianming Luo,Yong Wang
Clinical Neurology and Neurosurgery. 2013; 115(12): 2533-2536
[Pubmed] | [DOI]



 

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