Empty sella in somatotropic pituitary adenomas; a series of 23 cases.

Purpose: We aimed to investigate empty sella syndrome in somatotrophic pituitary adenoma for possible etiology, complications, and treatment options. Method: Among over 2,000 skull base masses that have been managed in our center since 2013, we searched for growth hormone-producing adenomas. Clinical, surgical, and imaging data were retrospectively collected from hospital records to check for sella that lacked pituitary tissue on routine imaging. Result: In 220 somatotrophic adenomas, 23 patients had an empty sella with surgical and follow-up data. The mean age of the sample was 46 years with the same male-to-female ratio. Five cases had partial empty sella and the rest were complete empty sellas. The most common simultaneous hormonal disturbance was high prolactin levels. Six had adenoma invasion into the clivus or sphenoid sinus and 10 had cavernous sinus intrusion. Peri-operative low-flow and high-flow cerebrospinal fluid (CSF) leaks were encountered in one and two patients, respectively, which were successfully sealed by abdominal fat. The majority of cases required growth hormone replacement therapy while it was controlled without any replacement therapy in nine patients. No pituitary hormonal disturbance occurred after transsphenoidal surgery except for hypothyroidism in one patient. Conclusion: An empty sella filled with fluid can be detected frequently in pituitary adenomas, especially in the setting of acromegaly. The pituitary gland may be pushed to the roof of the sella and might be visible as a narrow rim on imaging or may be detected in unusual places out of the sella. The pathophysiology behind such finding originates from soft and hard tissue changes and CSF pressure alternations during abundant growth hormone production.

A rare case of aneurysmal bone cyst of the anterior clinoid process in an 18-year-old female mimicking optic neuritis.

BACKGROUND: Aneurysmal bone cyst (ABC) is an uncommon, benign, vascular multicystic bony lesion that most frequently develops in the first two decades of life. The metaphysis of long bones, pelvic, and vertebral column are the most common locations. The precise underlying pathophysiology of ABCs formation remains unclear; however, it is believed that reactive processes subsequent to trauma or vascular disturbance may play an important role. Involvement of the skull base rarely occurs with a prevalence of up to 5% of intracranial ABCs. CASE PRESENTATIONS: An 18-year-old adolescent female with a history of progressive blurred vision since three months ago presented to our office. The brain and orbital MRI demonstrated no abnormal findings. After three months of glucocorticoid treatment with the diagnosis of multiple sclerosis, the visual impairment of the left eye deteriorated abruptly. The patient underwent an MRI and the imaging study demonstrated a well-defined 30 × 22 × 20-mm lesion at the anterior clinoid process with an extension to the optic canal and ethmoid sinus. The patient underwent pterional craniotomy, and the tumor was resected. The histopathological examination was suggestive of ABC. CONCLUSION: ABC and other conditions should be considered in young-age people with an early unilateral decline in vision and imaging studies should be obtained in early stages and during follow-ups.

Vertical triband flag sign for differential diagnosis of Rathke's cleft cyst.

Background: The Rathke cleft cyst (RCC) is a type of cystic growth that is benign, circular, and well-defined with an incidence rate of 4 %. This study aims to identify a useful diagnostic imaging sign that can aid in the differentiation of RCC from other cystic lesions. Methods: We retrospectively analyzed the records of 42 symptomatic RCC patients who were referred to our facility between 2016 and 2023. The data for the study were obtained from our electronic database. All magnetic resonance imaging (MRI) studies were performed using a 1.5-T superconducting magnetic scanner. All patients underwent endonasal transsphenoidal surgical resection. All MRIs were reviewed and evaluated by a neurosurgeon and a neuroradiologist. Results: There were 8 (19 %) males and 34 (81 %) females with a mean age of 37.2-years. Our study identified a distinct imaging characteristic in 38 of the cases, which we have named the "vertical triband flag sign", due to the growth of the cyst developing a specific appearance. The flag sign was mostly observed only in the T1-images (71.5 %), while in four cases the sign was spotted only in T2-images, and in four cases it appeared in both T1 and T2. In 4 cases, the flag sign was not observed in which further investigations revealed that these cases were suprasellar or small sellar RCCs. The dot sign, which is a characteristic finding in RCCs was only observed in one of our cases. Conclusion: Early diagnosis of RCCs may be facilitated by utilizing the vertical triband flag sign.

A case report of clubbing as the initial sign of systemic sclerosis sine scleroderma: A rare possible presentation.

Key Clinical Message: Even in the absence of characteristic cutaneous symptoms of scleroderma, systemic sclerosis should be considered in the differential diagnosis of patients initially diagnosed with idiopathic interstitial lung disease. Abstract: Systemic sclerosis (SSc) is an idiopathic connective tissue disorder characterized by multisystem involvement. Although skin thickening is a hallmark manifestation of SSc, a subset known as systemic sclerosis sine scleroderma (ssSSc) presents with internal organ involvement and positive serologic markers in the absence of significant cutaneous manifestations. We report the case of a 36-year-old Iranian woman who presented with clubbing as an initial symptom of ssSSc. Notably, clubbing as the sole initial sign of the disease has not been previously reported. Timely diagnosis of ssSSc is crucial to facilitate appropriate treatment and prevent disease progression. Physicians should adopt a comprehensive approach when evaluating patients presenting with limited clinical features, as they might be indicative of underlying ssSSc.

Waldenström's macroglobulinemia with necrotic extremities: A case with challenging diagnosis.

Key Clinical Message: Waldenström's macroglobulinemia may begin with constitutional symptoms that are common in primary care settings and it is crucial for physicians to be aware of the potential complications of hyperviscosity syndrome and to employ the appropriate diagnostic methods in order to achieve better outcomes. Abstract: Waldenström's macroglobulinemia (WM) refers to a type of lymphoplasmacytic lymphoma distinguished by the hyperproliferation of plasma cells, lymphocytes, and plasmacytoid lymphocytes. The disease is primarily diagnosed by increased monoclonal immunoglobulin M (IgM) levels and lymphoplasmacytic cell infiltration into the bone marrow. Individuals exhibit a high risk for hyperviscosity syndrome (HVS) as immunoglobulin levels increase. In addition to constitutional symptoms (fever, night sweats, and unintentional weight loss), clinical findings such as cytopenia, hepatosplenomegaly, and lymphadenopathy, this condition may cause hyperviscosity-related organ failures. Here we discuss a patient with WM who presented with neurological complaints and blurry vision and developed necrosis at distal portions of his body during the 6-month course of the disease.

Heparin induced thrombocytopenia in pregnancy: A therapeutic challenge case report and literature review.

The anticoagulants of choice for the prevention and treatment of venous thromboembolic disease during pregnancy are unfractionated heparin and low-molecular-weight heparin. Heparin-induced thrombocytopenia (HIT) is introduced as a rare but critical side effect of heparin products raising the thromboembolic event paradoxically. Here, we present a case of HIT in pregnancy with challenging management due to coincidence of lupus anticoagulant (LA) and limited anticoagulant options in the pharmaceutical market of our country of residence. We describe a 6-week pregnant patient with deep venous thrombosis (DVT) and pulmonary thromboembolism (PTE), which developed HIT during antenatal care. Therapeutic anticoagulation was initiated with argatroban, then switched to apixaban due to limited access to argatroban. Another therapeutic challenge was the concurrent incidence of LA. The interdisciplinary care team decided on adding up warfarin and scheduled termination at 12 weeks regarding the hazardous condition of the patient. We also reviewed related case literature to convey a new insight into managing pregnancy-related HIT. HIT is a pro-coagulatory and lethal complication associated with heparin therapy that can be diagnosed by clinical suspicion, the 4T score system, and confirmatory laboratory analyses. Alternative anticoagulation is the cornerstone of the treatment and an interdisciplinary plan will be worthwhile to make the best clinical decision regarding the critical situation and least the thromboembolic events mortality during pregnancy.

Paraneoplastic acute eosinophilic pneumonia due to carotid angiosarcoma: A rare case.

Key Clinical Message: This case report emphasizes that we should analyze a patient's signs and symptoms as a whole rather than relying exclusively on a common pattern to diagnose the condition and indicates that thorough histological investigation and sample collection are needed to accurately diagnose this malignancy. Abstract: Angiosarcoma is a rare, fatal, and poorly understood malignant tumor of vascular endothelial cells which is a challenging disease to diagnose in the clinical settings and requires early diagnosis to achieve a favorable prognosis. Paraneoplastic syndromes associated with angiosarcoma can include hypercoagulability, thrombocytopenia, anemia, fever, weight loss, and night sweats. In some cases, the paraneoplastic syndrome can be the first sign of the underlying malignancy. Here, we present a 47-year-old individual with angiosarcoma over the right scapula accompanied by hemoptysis and other pulmonary complaints whom at first was thought to be metastatic polmunary involvement. However, the patient's dramatic response to corticosteroids, in addition to further imaging and paraclinical studies, led us to an acute eosinophilic pneumonia (AEP) diagnosis which is an eosinophilic infiltrations of alveolar spaces. The patient received chemotherapy for angiosarcoma and radiation, since the brachial nerve network was disrupted, leaving the tumor unresectable. After 3 years of continuous follow-up, the patient is now completely cured.

Endoscopic versus Microsurgical Resection of Third Ventricle Colloid Cysts: A Single-Center Case Series of 140 Consecutive Patients.

BACKGROUND: Both endoscopic and microsurgery transcortical resection methods are used for colloid cysts of the third ventricle but they have not been compared regarding benefits and pitfalls. METHODS: Data of patients who underwent surgical resection of third ventricle colloid cyst via either endoscopic or microsurgery approach by a single surgeon from 2005 to 2020 were retrospectively collected. After administration of criteria, 140 records were retrieved (60 patients through endoscopic resection and 80 patients by a transcranial microsurgery approach). Clinical and surgical measures were compared between the 2 types of surgery after adjustment for confounders. RESULTS: Length of hospital stay, postoperative meningitis, operation time, cyst size, and baseline comorbidities were similar between two groups. Gross total resection (GTR) was achieved for all patients in the microsurgery group, whereas in the endoscopic group, resection was lower (90% vs. 100%; P = 0.005). Intraoperative hemorrhage occurred in 14 endoscopic patients (23.3%), whereas for the microscopic group, it was zero (P < 0.001). Postoperative shunt was required for 2 patients (one in the endoscopic group and the other in the microscopic group). Two patients had tumor recurrence, both of whom were in the endoscopic group. No mortality was detected in either group. Multivariate analyses were insignificant for confounding effects of clinical and demographic factors in occurrence of worse surgical outcomes (non-GTR and hemorrhage). CONCLUSIONS: In our series, the rate of intraoperative hemorrhage was higher with the endoscopic method and GTR was lower, even after adjustment for other factors. This situation could be caused by technological shortcomings and limited space for resection maneuvers and management of complications.