Hemorrhage associated with a pituitary tumor was first described by Bailey in 1898, but it was only in 1950 that Brougham et al. introduced the term "pituitary apoplexy" [7-8].

Pituitary apoplexy represents a rare medical emergency [6], even rarer in children and adolescents [9-15]; Due to the complexity and severity of the clinical presentation, requiring a multidisciplinary approach for the decision on conservative or surgical management. This involves emergency physicians, neuroradiologists, neurosurgeons, endocrinologists, and neuro-ophthalmologists.

Pituitary apoplexy has been found in 42.5% of pediatric and adolescent pituitary tumors [15]. Unlike in adult patients, where apoplexy is more common in non-functional adenomas, in children and adolescents, pituitary apoplexy is more frequently observed in functional adenomas, primarily prolactinomas (73.5%) [13, 15].

There is no gender predilection, it has been noted that apoplexy in prolactinomas is more frequent during puberty, with the average age at diagnosis being 16.6 ± 2.6 years in most cases reported in the literature, including the present case and the case of the team led by E. Culpin et al. [13] (average age of 14 years).

Prolactinomas present with both a tumor syndrome and endocrine symptoms, such as hyperprolactinemia, leading to decreased fertility, reduced libido, and galactorrhea [16-17]. However, diagnosing prolactinomas in children and adolescents presents unique challenges, as symptoms can be mistaken for age-related developmental variations. Their endocrinological symptoms are often overlooked or underestimated, which can delay diagnosis and treatment. In most cases, the diagnosis is made incidentally during an apoplexy of the adenoma [18].

The clinical presentation of pituitary apoplexy can be acute and severe, with significant neurological deficits, even leading to coma. Alternatively, it may be subacute, with milder symptoms developing over several days or weeks, depending on the speed of infarction or hemorrhage onset and the volume of bleeding. These factors influence the degree of compression on adjacent vascular and neuro-ophthalmological structures, as well as the pituitary gland itself [19].

The clinical picture supports the diagnosis of a pituitary tumor syndrome and an endocrine syndrome of hypersecretion or hypopituitarism. However, hypothalamo-pituitary imaging is essential to confirm the diagnosis.

MRI remains the gold standard for diagnosing pituitary apoplexy, with a sensitivity of approximately 90%. It detects hemorrhagic infarction, necrosis, optic chiasm compression, and adjacent vasculonervous structures. It also allows for assessing the degree of invasion according to the Knosp classification system [15].

MRI can also identify lesions that may mimic pituitary apoplexy, such as aneurysms, Rathke's pouch cysts, craniopharyngiomas, and mucoceles [20].

In the acute phase (0 to 7 days), a hypointense signal is observed on T2-weighted images, and an isointense or slightly hypointense signal is observed on T1-weighted images.

In the subacute phase (7 to 21 days), methemoglobin shortens T1 relaxation time, making the hemorrhage appear hyperintense on both T1 and T2 sequences.

In the chronic phase (> 21 days), the presence of hemosiderin and ferritin causes marked hypointensity on both T1 and T2 images [21].

CT imaging, although less sensitive, may be useful when MRI is unavailable or contraindicated, detecting sellar expansion in over 80% of cases, but can identifies only about 30% of pituitary apoplexies [22].

Ophthalmological evaluation, including visual acuity, visual field testing, and ocular motility, should be performed [23]. Additionally, a hormonal assessment should be conducted to detect any pituitary hormone deficiencies or hypersecretion conditions. A comprehensive metabolic workup is also necessary to assess electrolytes, renal function, liver function, coagulation, and complete blood count [23].

The ideal therapeutic approach for pituitary apoplexy in the young population is not clearly defined and remains controversial. There are very few case series of clinical pituitary apoplexy in the pediatric population that focus on treatment guidelines [24].

Applying adult treatment strategies to adolescent patients remains uncertain [25-26]. It is now widely acknowledged that a conservative approach using dopaminergic agonists should be the first-line treatment for the majority of adult prolactinomas presenting with apoplexy, given that surgery shows similar effectiveness to conservative management in terms of visual outcomes [13]. However, for children, no published guidelines exist for management.

Some authors advocate for early transsphenoidal surgical decompression in all patients, particularly those with neurological symptoms, visual disturbances, or those who do not tolerate the side effects of dopaminergic agonists. Others recommend a conservative approach in some cases, utilizing dopaminergic agonists [13-27].

There is, however, a correlation between the Ki-67 marker and the degree of tumor aggressiveness and proliferation, as well as resistance to treatment with dopaminergic agonists. Literature suggests that younger patients tend to have higher Ki-67 indices compared to older patients, indicating a higher proliferative potential and a considerable risk of tumor aggressiveness [28-31]. This underscores the potential benefits of prioritizing surgical intervention as the initial approach.

In our case, the decision for surgical decompression was made immediately due to the tumor size exceeding 2 cm, chiasmatic compression, and invasion of vascular structures, with a KNOPS score of 1 on the left and KNOPS 2 on the right. In the series by Culpin E et al. [13] and the series by Hiroki et al. [24], all patients underwent immediate pituitary surgery to preserve visual function primarily.

Surgical intervention as the first-line treatment [18] seems to be more effective in younger patients due to their higher Ki-67 indices and the difficulties associated with managing more aggressive and invasive tumors, in order to preserve both visual and vital functional prognosis due to vascular compression.

Moreover, surgical treatment often results in a faster normalization of hyperprolactinemia compared to treatment with dopaminergic agonists alone, providing rapid symptomatic relief and effective decompression [32]. In the Hiroki et al. series, normalization of prolactin levels was noted seven days postoperatively [24].

Given the well-established correlation between resistance to dopaminergic agonists and tumor aggressiveness, and the higher Ki-67 index observed in younger patients, as well as the favorable evolution in terms of hormonal profile normalization and visual improvement, we propose that surgical intervention should be preferred over conservative medical treatment as the first-line approach in patients presenting with progressive neurological symptoms, signs of visual pathway compression, and vascular compression, based on the KNOPS score.

However, further case studies or series involving the younger population are needed to establish better recommendations and guidelines for standardizing therapeutic approaches.