Update: Giant GH secreting pituitary adenoma

Giant GH secreting pituitary adenoma

Patients with acromegaly usually harbor pituitary macroadenomas measuring between 10 and 30 mm in maximal diameter. Giant (adenoma size ≥40 mm) GH secreting pituitary adenoma are rarely encountered.
They are invasive, uncontrolled by surgery, and respond poorly to medical treatment. Aggressive multimodal therapy is critical for their management, enhancing control rate and biochemical remission 1).

Case series

2017

Giordano et al., present the clinical, radiological and hormonal status of three patients affected by invasive GH-secreting pituitary adenomas without clinical signs and symptoms of acromegaly with elevation of serum IGF-1 from a series of 142 pituitary adenomas operated in the Department of Neurosurgery, International Neuroscience Institute-Hannover, Germany with the aid of intraoperative magnetic resonance imaging(MRI). Total tumor removal was possible in two of the three cases; the patients show normal hormonal status and no recurrence at long-term follow-up. In the third case, due to the different features of the tumor, complete resection was not possible and a multimodal treatment was performed that allowed regularization of the hormonal status and control of the residual tumor. GH-secreting adenomas without clinical manifestation of acromegaly are uncommon lesions. Total microsurgical excision can be curative. However, in case of partial removal, a tailored adjuvant treatment should be considered to preserve the quality of life of the patient and avoid regrowth of the lesion. In not resectable tumors, preoperative medical treatment with somatostatin analogues is always an option 2).

2015

Shimon et al. identified 34 patients (15 men and 19 females) with giant adenomas among 762 subjects (4.5%) with acromegaly, and characterized their clinical characteristics and response to treatment.
Mean age at diagnosis was 34.9±12.5 years (range, 16-67 years). Mean adenoma size was 49.4±9.4 mm (range, 40-80 mm); 30 adenomas showed cavernous sinus invasion and 32 had suprasellar extension. Twenty-nine (85%) patients had visual field defects. Mean baseline IGF1 was 3.4±1.8×ULN. All patients except one underwent pituitary surgery (one to three procedures), but none achieved hormonal remission following first surgery. Among the 28 subjects with visual disturbances, 14 recovered post-operatively and 13 improved. Treatment with somatostatin analogs was given to all patients after surgical failure. Six achieved remission, nine others were partially controlled (IGF1<1.5×ULN; 3/9 when combined with cabergoline), and 17 did not respond (two were lost). Nine patients were treated with pegvisomant, alone (n=4) or in combination with somatostatin analogs (n=5); five are in remission and two are partially controlled. Pasireotide-LAR achieved hormonal remission in one of the six patients. Currently, after a mean follow-up period of 8.9 years, 17 patients are in biochemical remission, eight are partially controlled, and seven are uncontrolled (two were lost to follow-up).
Giant GH-secreting adenomas are invasive, uncontrolled by surgery, and respond poorly to medical treatment. Aggressive multimodal therapy is critical for their management, enhancing control rate and biochemical remission 3).

Case reports

A 23-year-old male patient presented with continuous increase in height during the past 6 years due to a GH-secreting giant pituitary adenoma. Because of major intracranial extension and failure of octreotide treatment to shrink the tumour, the tumour was partially resected by a trans-frontal surgical approach. At immunohistochemistry, the tumour showed a marked expression of GH and a sparsely focal expression of prolactin. Somatostatin receptors (sst) 1-5 were not detected. Tumour tissue weakly expressed dopamine receptor type 2. The Gs alpha subunit was intact. Conversion from somatostatin analogue to pegvisomant normalized insulin-like-growth-factor-I (IGF-I) levels and markedly improved glucose tolerance.
Pegvisomant is a potent treatment option in patients with pituitary gigantism. In patients who do not respond to somatostatin analogues, knowledge of the SST receptor status may shorten the time to initiation of pegvisomant treatment 4).
1)

Shimon I, Jallad RS, Fleseriu M, Yedinak CG, Greenman Y, Bronstein MD. Giant GH-secreting pituitary adenomas: management of rare and aggressive pituitary tumors. Eur J Endocrinol. 2015 Jun;172(6):707-13. doi: 10.1530/EJE-14-1117. Epub 2015 Mar 19. PubMed PMID: 25792375.
2)

Giordano M, Samii A, Fahlbusch R. Aggressive somatotrophinomas lacking clinical symptoms: neurosurgical management. Neurosurg Rev. 2017 Dec 30. doi: 10.1007/s10143-017-0940-y. [Epub ahead of print] PubMed PMID: 29290044.
3)

Shimon I, Jallad RS, Fleseriu M, Yedinak CG, Greenman Y, Bronstein MD. Giant GH-secreting pituitary adenomas: management of rare and aggressive pituitary tumors. Eur J Endocrinol. 2015 Jun;172(6):707-13. doi: 10.1530/EJE-14-1117. Epub 2015 Mar 19. PubMed PMID: 25792375.
4)

Müssig K, Gallwitz B, Honegger J, Strasburger CJ, Bidlingmaier M, Machicao F, Bornemann A, Ranke MB, Häring HU, Petersenn S. Pegvisomant treatment in gigantism caused by a growth hormone-secreting giant pituitary adenoma. Exp Clin Endocrinol Diabetes. 2007 Mar;115(3):198-202. PubMed PMID: 17427111.

Update: Cerebellopontine angle pilocytic astrocytoma

Cerebellopontine angle pilocytic astrocytoma

A rare case of a 55-yr old patient of pilocytic astrocytoma of the cerebellopontine angle mimicking a vestibular schwannoma. The tumor protruded into the porus acusticus causing enlargement of the internal auditory meatus, which is quite an unusual feature of glial tumor 1).


Schneider et al. report a pilocytic astrocytoma of the cerebellopontine angle in a child presenting with auditory neuropathy spectrum disorder 2).


Mirone et al. describe a rare case of pediatric pilocytic astrocytoma presented as a right cerebellopontine angle (CPA) mass, completely separated from the brainstem and arising from the proximal VIII cranial nerve portion.
A 12-year-old boy, with no evidence of neurofibromatosis type 2, presented with progressive hearing loss at the right ear and headache. An initial enhanced magnetic resonance examination suggested the diagnosis of schwannoma. The tumor was resected by a suboccipital retrosigmoid approach.
The case seems to be the first report of a primary pediatric CPA pylocitic astrocytoma arising from the VIII nerve complex and presenting internal auditory canal enlargement. It represents the third reported case of a primary CPA pilocytic astrocytoma (the second pediatric case with the first arising from V nerve) and the eighth report of primary CPA glioma, overall 3).


A case of pilocytic astrocytoma of the cerebellum mimicking an acoustic schwannoma. The tumour protruded into the porus acusticus and enlarged the internal auditory meatus, which is a quite unusual characteristic of glial tumours 4).
1)

Dutta G, Singh D, Singh H, Sachdeva D, Kumar V, Chaturvedi A. Pilocytic astrocytoma of the cerebellopontine angle mimicking vestibular schwannoma: report of a rare entity. Br J Neurosurg. 2017 Dec 26:1-3. doi: 10.1080/02688697.2017.1419163. [Epub ahead of print] PubMed PMID: 29278012.
2)

Schneider F, Kompis M, Ozdoba C, Beck J, Caversaccio M, Senn P. Pilocytic astrocytoma of the cerebellopontine angle in a child presenting with auditory neuropathy spectrum disorder. Otol Neurotol. 2015 Apr;36(4):e101-3. doi: 10.1097/MAO.0000000000000355. PubMed PMID: 24781101.
3)

Mirone G, Schiabello L, Chibbaro S, Bouazza S, George B. Pediatric primary pilocytic astrocytoma of the cerebellopontine angle: a case report. Childs Nerv Syst. 2009 Feb;25(2):247-51. doi: 10.1007/s00381-008-0690-9. Epub 2008 Aug 9. PubMed PMID: 18690462.
4)

Takada Y, Ohno K, Tamaki M, Hirakawa K. Cerebellopontine angle pilocytic astrocytoma mimicking acoustic schwannoma. Neuroradiology. 1999 Dec;41(12):949-50. PubMed PMID: 10639675.

Update: Medulloblastoma epidemiology

Medulloblastoma epidemiology

In children medulloblastoma comprise 15-20 % of intracranial tumor1).
30-55 % of posterior fossa tumors.
Medulloblastoma is the most common malignant pediatric intracranial tumor 2).
Medulloblastomas comprise < 1 % of adult brain neoplasms. Peak incidence during 1st. decade. Median age at diagnosis 5-7 years (75 % are diagnosed by age 15). Male:female ratio is 2:1. Familial cancer syndromes that include medulloblastoma: Gorlin syndromeTurcot syndrome.
Population-based data examining recent epidemiological trends in medulloblastoma, are limited. Therefore, Khanna et al. sought to examine recent population-level trends in medulloblastoma incidence and survival. Central Brain Tumor Registry of the United States (CBTRUS) data were analyzed from 2001 to 2013. Age-adjusted incidence rates (IR) and annual percent changes (APCs) with 95% confidence intervals (CI) were calculated by age, sex, and race. Relative survival rates were calculated by age, sex, and race using Surveillance, Epidemiology and End-Results (SEER) registries; subsets of CBTRUS data. Kaplan-Meier and Cox proportional hazards models were used to examine survival differences. Medulloblastoma incidence remained relatively stable from 2001 to 2013, with minor fluctuations from 2001 to 2009 (APC = 2.2, 95% CI 0.8, 3.5) and 2009-2013 (APC = -4.1, 95% CI -7.5, -0.6). Incidence was highest in patients aged 1-4 years at diagnosis, but patients aged 10-14 years showed increased incidence from 2000 to 2013 (APC = 3.2, 95% CI 0.6, 5.8). Males displayed higher IR relative to females (males: 0.16 vs. females: 0.12), except in patients <1 year-old. Compared to Whites, Blacks displayed a non-significant increase in incidence (APC = 1.7, 95% CI -0.4, 4.0) and in mortality risk (hazard ratio for survival = 0.74; p = 0.09). The current study reports no overall change in medulloblastoma incidence from 2001 to 2013. Male and female patients <1 year-old had equal medulloblastoma incidence rates and poor 5-year relative survival compared to other ages. Non-significant trends in the data suggest disparities in medulloblastoma incidence and survival by race. Thus, analysis of tumor-specific trends by demographic variables can uncover clinically informative trends in cancer burden 3).


A report aims to provide accurate nationwide epidemiologic data on primary brain and central nervous system (CNS) tumors in the Republic of Korea. Dho et al. updated the data by analyzing primary brain and CNS tumors diagnosed in 2013 using the data from the national cancer incidence database.
Data on primary brain and CNS tumors diagnosed in 2013 were collected from the Korean Central Cancer Registry. Crude and age-standardized rates were calculated in terms of gender, age, and histological type.
A total of 11,827 patients were diagnosed with primary brain and CNS tumors in 2013. Brain and CNS tumors occurred in females more often than in males (female:male, 1.70:1). The most common tumor was meningioma (37.3%). Pituitary tumors (18.0%), gliomas (12.7%), and nerve sheath tumors (12.3%) followed in incidence. Glioblastomas accounted for 41.8% of all gliomas. In children (<19 years), sellar region tumors (pituitary and craniopharyngioma), embryonal/primitive/medulloblastoma, and germ cell tumors were the most common tumors 4).
1)

Laurent JP, Cheek WR. Brain tumours in children. J Pediatr Neurosci. 1985;1:15–32
2)

Allen JC. Childhood brain tumors: current status of clinical trials in newly diagnosed and recurrent disease. Pediatr Clin North Am. 1985 Jun;32(3):633-51. Review. PubMed PMID: 3889800.
3)

Khanna V, Achey RL, Ostrom QT, Block-Beach H, Kruchko C, Barnholtz-Sloan JS, de Blank PM. Incidence and survival trends for medulloblastomas in the United States from 2001 to 2013. J Neurooncol. 2017 Dec;135(3):433-441. doi: 10.1007/s11060-017-2594-6. Epub 2017 Aug 21. PubMed PMID: 28828582.
4)

Dho YS, Jung KW, Ha J, Seo Y, Park CK, Won YJ, Yoo H. An Updated Nationwide Epidemiology of Primary Brain Tumors in Republic of Korea, 2013. Brain Tumor Res Treat. 2017 Apr;5(1):16-23. doi: 10.14791/btrt.2017.5.1.16. Epub 2017 Apr 30. PubMed PMID: 28516074; PubMed Central PMCID: PMC5433946.
× How can I help you?
WhatsApp WhatsApp us
%d bloggers like this: