Keywords
Sellar and parasellar region lesions, pituitary disease, pituitary adenoma, classification, epidemiology.
Sellar and parasellar region lesions, pituitary disease, pituitary adenoma, classification, epidemiology.
See the authors' detailed response to the review by Khaled Mohammed Al-Dahmani
Sellar and parasellar region lesions spectrum includes a wide variety of conditions ranging from adenoma to empty sella syndrome, apoplexy, congenital or acquired condition1–4. Other than adenoma, genetic causes of pituitary disease are increasingly recognized3.
Pituitary adenomas are not rare and account for 20% all intracranial tumors5,6. Half of these secrete hormones, and half are microadenoma2. Clinically non-functioning adenomas (NFPA) constitute 15–54% of all adenomas. Prolactinomas accounts for 32–66%, growth hormone secreting adenoma (acromegaly) account for 8–16%, adrenocorticotropic hormone (ACTH)-secreting adenoma (Cushing's disease) forms 2–6%, and TSHoma accounts for less than 1%2,7. These pituitary adenomas behave as typical or have a more aggressive to malignant behavior6,8. They can cause mass effect, in addition to hypersecretion or hypopituitarism7,9.
Advances in neuroradiology have opened the door for earlier and easier diagnosis of pituitary disease and other sellar and suprasellar lesions10.
The Faiha Specialized Diabetes, Endocrine, and Metabolism Center (FDEMC) in Basrah is a tertiary referral center receiving patients with pituitary diseases from most of Southern Iraq. The FDEMC is trying to adapt the three mission criteria of the pituitary center of excellence, which includes care and support for patients, fellowship training and contribution to pituitary disease research11. To our knowledge, there are no studies on sellar and parasellar region lesions in Iraq.
This study aimed at a comprehensive description of pituitary disorders for patients from FDEMC in Basrah (Southern Iraq).
Sequences of pituitary MRI imaging were classified according to the international standard12. Adenomas were classified as macroadenoma if these were 10 mm or more in size, while microadenoma if less than 10 mm and giant prolactinoma if these were 4 cm and above2.
Pituitary adenoma (NFPA, prolactinoma, growth hormone secreting adenoma [acromegaly], and adrenocorticotropic hormone (ACTH)-secreting adenoma) were defined according to the usual criteria2,8,12.
Hypopituitarism, whether postoperative or in those with or without adenoma, was considered according to the hormonal assessment with basal and dynamic hormonal tests13.
Empty sella syndrome, whether primary or secondary to surgery or apoplexy, were considered based on MRI findings14.
Craniopharyngioma diagnosis was based on clinical behavior with MRI and pathological diagnosis.
Analysis was done in July 2017. All patients with labeling diagnosis of pituitary disease were included. Data were included on an Excel spreadsheet and transferred to SPSS for Windows, Version 23.0 (SPSS Inc., Chicago, USA).
Continuous variables were summarized as number and percentage and dichotomous variables as mean ±SD.
The ethics committee of the Medical College in Basrah University approved the study design and the Center authorities agreed to review the patients data. At the time of registeration in the Center, all patients included in this study approved the use of their clinical information for research purposes.
A total of 232 patients were included in this study. Pituitary disorders were more common among women (Table 1). Those with macroadenoma were older than those with microadenoma with nearly equal gender prevalence of macroadenoma. Four patients died; two with growth hormone secreting adenoma (acromegaly) and advanced cardiovascular disease, and two with prolactinoma that caused hypopituitarism and adrenal failure.
N (%); mean ±SD | |||
---|---|---|---|
Gender | All pituitary disorders | Men | 84 (36.2) |
Women | 148 (63.8) | ||
Pituitary adenoma* | Men | 67 (43) | |
Women | 89 (57) | ||
Age at registration, years | All | 38.2±15.3 | |
Macroadenoma | 42.5±14.9 | ||
Microadenoma | 34.8±14.7 | ||
Macroadenoma** | Men | 51 (50.5) | |
Women | 50 (49.5) | ||
Died | 4 |
Table 2 shows that pituitary adenoma constituted the bulk of pituitary disorders in this registry (67.2%). Growth hormone secreting adenoma (acromegaly) were the commonest adenoma seen in 41.0% followed by NFPA in 31.4% than prolactinoma in 26.9%. Hypopituitarism due to various causes was observed in 24.5% in this series. Empty sella syndrome, whether primary or secondary, were seen in 9.4%. Craniopharyngioma and Sheehan syndrome were seen in 3.9% each. Meningioma based on MRI finding was been observed in 4 patients (1.7%).
N (%) | ||
---|---|---|
Pituitary adenoma | 156 (67.2) | |
Growth hormone secreting adenoma (acromegaly) | 64 (41.0) | |
Clinically non-functioning pituitary adenoma (NFPA) | 49 (31.4) | |
Prolactinoma* | 42 (26.9) | |
GH-secreting adenoma with hyperprolactinemia* | 5 | |
ACTH- secreting pituitary adenoma | 2 (1.2) | |
Hypopituitarism | 57 (24.5) | |
Empty sella syndrome | All | 22 (9.4) |
Primary** | 9 | |
Secondary | 13 | |
Diabetes insipidus | 15 | |
Apoplexy | 3 | |
Hyperprolactinemia | All | 51 |
No adenoma | 12 | |
Pituitary enlargement | 3 | |
Stalk lesions | 1 | |
Miscellaneous | ***11 | |
Craniopharyngioma | 9 (3.9) | |
Sheehan syndrome | 9 (3.9) | |
Meningioma | 4 (1.7) | |
Rathke's cleft cyst | 3 | |
Total | 232 |
In this study, 64.8% of pituitary adenoma were macroadenomas (Table 3). Macroadenoma was seen in 73.4% of acromegaly, 61.2% in NFPA and 62.0% of prolactinoma (of them six were giant prolactinoma).
Microadenoma N (%) | Macroadenoma N (%) | Total | |
---|---|---|---|
Pituitary adenoma | 55 (35.2) | 101 (64.8) | 156 |
Growth hormone secreting adenoma (acromegaly) | 17 (26.5) | 47 (73.4) | 64 |
Clinically non- functioning pituitary adenoma (NFPA) | 19 (38.8) | 30(61.2) | 49 |
Prolactinoma | 16 (38) | 26 (62.0)* | 42 |
ACTH- secreting Pituitary adenoma | 2 (100.0) | 0 | 2 |
In Table 4 we see hypophysectomy whether transsphenoidal or transcranial or both was performed in 45 patients with pituitary adenoma (28.8%). Stereotactic radiosurgery is done in 5 patients (3.2%) with pituitary adenoma. Growth hormone secreting adenoma (acromegaly) and prolactinomas were treated primarily with medical therapy (71.4% and 76.1% respectively).
N (%) | ||
---|---|---|
Hypophysectomy-transsphenoidal | 33 (21.1) | |
Hypophysectomy-transcranial | 8 (5.1) | |
Hypophysectomy-transsphenoidal followed by transcranial or reverse or repeat same surgery,i.e., twice surgery | 4 (2.5) | |
Stereotactic radiosurgery | 5 (3.2) | |
Radiotherapy | 1 (0.6) | |
Primary medical treatment | Growth hormone secreting adenoma (acromegaly) | 46 (71.4)* |
Prolactinoma | 32 (76.1)** | |
Total | 156 |
All pituitary disorders and adenoma were more common among women in this study. The gender predominance among patients with pituitary adenoma is variable in the literature depending on hormone secretion and age of the patients, the size of the tumor and female dominance is not clear15,16. However, female dominance has been seen in Saudi Arabia17 and one series from Argentina18. Those with macroadenoma tend to be older in age with no difference in the prevalence between men or women.
Seen in about two-thirds of patients, pituitary adenoma constituted the main bulk of pituitary disease in this study, which is compatible with reports in the literature16.
The commonest pituitary adenoma was growth hormone secreting adenoma (acromegaly), followed by NFPA and prolactinoma. This is entirely different from the literature on the prevalence of pituitary adenoma2,16,17,19. This could be attributed to selection bias because only growth hormone secreting adenoma (acromegaly) patients are being referred, while NFPA and prolactinoma were treated by different specialties, such as neurosurgeons or gynecologists, without referral to a specialized Center like FDEMC. In Basrah, most cases of hyperprolactinemia were seen by a gynecologist because of amenorrhea and infertility, and the neurosurgeon follows patients with NFPA without referring them.
Hypopituitarism is prevalent in a quarter of this pituitary centre, from different causes, ranging from macroadenoma to hypophysectomy. Evaluation for hypopituitarism remains an integral part of the workup for any pituitary lesions because missing such diagnosis could be catastrophic9,13. This figure is far higher than that of Saudi Arabia, which was 1.2%17.
Empty sella syndrome was seen in 9.4% of patients in this study, which can be primary or secondary to surgery or apoplexy. Empty sella syndrome needs an extensive workup to assess pituitary function20.
Craniopharyngioma and Sheehan syndrome are two diseases with a different spectrum of age distribution, but they were seen at the same frequency in this cohort. Craniopharyngioma is a disease of childhood and adolescence21. Sheehan syndrome is supposed to be rare in developed countries, but is still seen in developing countries22.
Less than two thirds of adenoma in this study were macroadenomas. While in most series macroadenomas constitute 50% of the pituitary adenomas2; however in Canada, a similar finding has been seen compared with this study23. Again this could be explained by referral bias in this study. In Saudi Arabia, microadenomas were more prevalent17.
For growth hormone secreting adenoma (acromegaly), more than two thirds were macroadenomas, which is established fact for all acromegaly2,24,25.
NFPA was a macroadenoma and seen at around 60% in this study. A similar finding was seen in a previous series2.
Prolactinomas were macroadenoma in around 60% of cases in this study. This differs from the literature, where more than 90% of prolactinomas were microadenomas2,18.
Hypophysectomy-transsphenoidal as surgical treatment was done in one third of pituitary adenomas, while transcranial approach or stereotactic radiosurgery was contemplated in the minority. This is a typical approach for most of the pituitary adenomas2,26. For growth hormone secreting adenoma (acromegaly), the primary treatment in this study was medical treatment in about two thirds of individuals. This is contrary to literature where surgery is the main mode of therapy26. The explanation is that we are just building a new neurosurgery unit for pituitary glands over the last few years, and in the future, surgery of pituitary is supposed to improve, and early referral will be the best.
For prolactinoma, primary medical treatment was done in two thirds of patients, while it should be the main treatment of choice in more than 90%, as seen in previous literature26.
Malignant disease metastasizing to the pituitary is not observed in this study because they are not referred from Oncology Center in Basrah.
This study supposes to involve most of the pituitary disease patients in Basrah because the Center is a tertiary referral center. However, due to referral bias among some neurosurgeons and gynecologologists, we cannot guarantee that the data includes all patients with this condition in Basrah.
Pituitary adenomas constituted the bulk of pituitary disorders in Basrah. Growth hormone secreting adenoma (acromegaly) is the most frequent adenoma followed by NFPA and prolactinoma due to referral bias. A change in the practice of pituitary adenoma treatment is needed.
Dataset 1: Description of patients included in the study 10.5256/f1000research.13632.d19743927
All authors are thankful to the medical staff of FDEMC for their contribution and support.
Views | Downloads | |
---|---|---|
F1000Research | - | - |
PubMed Central
Data from PMC are received and updated monthly.
|
- | - |
Competing Interests: No competing interests were disclosed.
Reviewer Expertise: Pituitary, thyroid
Is the work clearly and accurately presented and does it cite the current literature?
Partly
Is the study design appropriate and is the work technically sound?
Yes
Are sufficient details of methods and analysis provided to allow replication by others?
Yes
If applicable, is the statistical analysis and its interpretation appropriate?
Yes
Are all the source data underlying the results available to ensure full reproducibility?
Yes
Are the conclusions drawn adequately supported by the results?
Partly
Competing Interests: No competing interests were disclosed.
Reviewer Expertise: Pituitary, thyroid
Is the work clearly and accurately presented and does it cite the current literature?
Yes
Is the study design appropriate and is the work technically sound?
Yes
Are sufficient details of methods and analysis provided to allow replication by others?
Yes
If applicable, is the statistical analysis and its interpretation appropriate?
Yes
Are all the source data underlying the results available to ensure full reproducibility?
Yes
Are the conclusions drawn adequately supported by the results?
Yes
Competing Interests: No competing interests were disclosed.
Alongside their report, reviewers assign a status to the article:
Invited Reviewers | ||
---|---|---|
1 | 2 | |
Version 2 (revision) 22 Jun 18 |
read | |
Version 1 06 Apr 18 |
read | read |
Click here to access the data.
Spreadsheet data files may not format correctly if your computer is using different default delimiters (symbols used to separate values into separate cells) - a spreadsheet created in one region is sometimes misinterpreted by computers in other regions. You can change the regional settings on your computer so that the spreadsheet can be interpreted correctly.
Provide sufficient details of any financial or non-financial competing interests to enable users to assess whether your comments might lead a reasonable person to question your impartiality. Consider the following examples, but note that this is not an exhaustive list:
Sign up for content alerts and receive a weekly or monthly email with all newly published articles
Already registered? Sign in
The email address should be the one you originally registered with F1000.
You registered with F1000 via Google, so we cannot reset your password.
To sign in, please click here.
If you still need help with your Google account password, please click here.
You registered with F1000 via Facebook, so we cannot reset your password.
To sign in, please click here.
If you still need help with your Facebook account password, please click here.
If your email address is registered with us, we will email you instructions to reset your password.
If you think you should have received this email but it has not arrived, please check your spam filters and/or contact for further assistance.
Comments on this article Comments (0)