Clinical features and therapeutic outcomes of patients with acromegaly in Saudi Arabia: a retrospective analysis.

BACKGROUND: Acromegaly is a rare disease resulting in clinical sequelae with significant morbidity and mortality due to the central tumor mass effect and prolonged growth hormone (GH) hypersecretion. OBJECTIVES: The goal is to describe the epidemiology, clinical features, presence of comorbidities, and treatment outcomes of acromegaly in Saudi Arabia. METHODS: Data was collected through a retrospective review of the charts of all patients diagnosed with acromegaly from nine major hospitals in Saudi Arabia over a period of more than 25 years. RESULTS: A total of 195 patients (116 males and 79 females), with a mean age at diagnosis of 43 ± 12 (males) and 46 ± 14 years (females), from nine major hospitals were identified and included in the analysis. All cases were caused by pituitary adenomas, of which 92.4% were macroadenomas. Headache, coarse facial features, acral growth, and sweating/oily skin were by far the most frequent presenting complaints. The most common comorbidities were diabetes mellitus (51.7%), followed by hypertension (50%) and visual field defect (30.5%). The vast majority (95%) of patients were treated surgically (98%). Twenty-four percent also received radiotherapy, and 74.4% received medical therapy. When stringent criteria were applied for assessment of outcomes of therapy, 28.7% of the patients were cured and 30.1% had their disease under control, while 28.7% were found to have active disease despite receiving multimodal therapy. CONCLUSIONS: Our findings highlight the need for a national acromegaly registry to enable early identification, evaluation, and selection of the best therapeutic approaches to improve the outcome and remission rate of the disease.

Cabergoline-Induced Pneumocephalus Following Treatment for Giant Invasive Macroprolactinoma Presenting With Spontaneous Cerebrospinal Fluid Rhinorrhea.

Cerebrospinal fluid (CSF) rhinorrhea is rarely reported as the first presenting feature of giant invasive macroprolactinomas. Cerebrospinal fluid rhinorrhea is usually reported as a complication of trauma, neurosurgical, and skull-based procedures (such as pituitary surgery or radiations), and less frequently after medical treatment with dopamine agonists (DAs) for macroprolactinomas. This phenomenon results from fistula creation that communicates between the subarachnoid space and the nasal cavity. Meanwhile, pneumocephalus is another well-recognized complication after transsphenoidal surgery for pituitary macroadenomas. This entity may present with nausea, vomiting, headache, dizziness, and more seriously with seizures and/or a decreased level of consciousness if tension pneumocephalus develops. Case reports about the occurrence of spontaneous pneumocephalus after medical treatment with DAs without prior surgical interventions are scarce in the literature. Our index case is a young man who was recently diagnosed with a giant invasive prolactin-secreting pituitary macroadenoma with skull base destruction. A few months before this diagnosis, he presented with spontaneous CSF rhinorrhea with no history of previous medical or surgical treatment. In this case report, we report an uncommon presentation for giant invasive macroprolactinoma with a CSF leak treated with cabergoline that was subsequently complicated by meningitis and pneumocephalus. This is a very rare complication of cabergoline therapy, which occurred approximately 1 month after treatment initiation.

Atypical Teratoid/Rhabdoid Tumor of the Sellar Region in an Adult With Long Survival: Case Report and Review of the Literature.

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm usually diagnosed in young children, although it can occur in adults. Prognosis for AT/RT is poor, with a median survival of 10 - 11 months. We report a rare case of adult sellar and suprasellar AT/RT in a 36-year-old female patient. She was treated with multi-modalities including surgery, chemotherapy and radiation. She markedly improved following treatment with no recurrence in 3 years follow-up. To our knowledge, this is the 11th case of an adult-onset AT/RT in the sellar or suprasellar region with favorable long-term outcome.

Managing Prolactinomas during Pregnancy.

Prolactinomas are the most prevalent functional benign pituitary tumors due to a pituitary micro- or macroadenoma. The majority of patients presents with infertility and gonadal dysfunction. A dopamine agonist (DA) (bromocriptine or cabergoline) is the treatment of choice that can normalize prolactin levels, reduce tumor size, and restore ovulation and fertility. Cabergoline generally preferred over bromocriptine because of its higher efficacy and tolerability. Managing prolactinomas during pregnancy may be challenging. During pregnancy, the pituitary gland undergoes global hyperplasia due to a progressive increase in serum estrogens level that may lead to increase of the tumor volume with potential mass effect and visual loss. The risk of tumor enlargement may occur in 3% of those with microadenomas, 32% in those with macroadenomas that were not previously operated on, and 4.8% of those with macroadenomas with prior ablative treatment. Though both drugs appear to be safe during pregnancy, the data on fetal exposure to DAs during pregnancy have been reported with bromocriptine far exceeds that of cabergoline with no association of increased risk of pregnancy loss and premature delivery. It is advisable to stop the use of DAs immediately once pregnancy is confirmed, except in the case of women with invasive macroprolactinomas or pressure symptoms. This review outlines the therapeutic approach to prolactinoma during pregnancy, with emphasis on the safety of available DA therapy.