Xanthogranuloma of the sellar region: a systematic review.

Knowledge of xanthogranuloma (XG) of the sellar region comes from short series or single cases. We performed a systematic review, using the PubMed, Web of Science, Embase, Scopus, eLibrary, and BIOSIS Preview databases, of all cases reported from 2000 to the present. We also describe one unreported patient treated in our institution. A search of the literature revealed that of 71 patients 50.7% were male and that mean age at diagnosis was 34.7 ± 19.2 years old. Median time from clinical onset until diagnosis was 7 (3-21) months. Hypopituitarism (70.4%), visual disorders (64.7%), headache (53.5%), and polyuria-polydipsia (28.2%) were the most common symptoms. On MRI, median tumor size was 20 (16-29) mm, while 71.8% were sellar/suprasellar and less frequently exclusively suprasellar (15.5%) or sellar (12.7%). On T1-weighted imaging, XG was hyperintense in 76.3% of patients, while it showed variable appearance on T2-weighted imaging. The tumor showed cystic features in 50.7%, gadolinium enhancement in 45.1%, and calcification in 22.5% of patients. All patients underwent surgery (77.4% transphenoidal approach and 18.3% craniotomy), with hypopituitarism (56.4%), diabetes insipidus (34.5%), and visual defects (7.3%) being the most common complications. Total/subtotal resection was achieved in 93.5%, while the tumor was partially removed in 6.6%. Median follow-up was 24 (6-55) months and no tumor recurrence or remnant growth was reported in 97.5% of cases. In conclusion, XG affects the younger population, manifested by hormonal deficit and mass effect symptoms. Surgery is safe and offers excellent outcomes, though hypopituitarism is frequent post-surgery. Tumor recurrence or remnant growth is rare and radiological surveillance is a good option for patients with remnant lesions.

Sarcomas of the sellar region: a systematic review.

PURPOSE AND METHODS: Sarcomas of the sellar region are uncommon and unexpected tumors. Here, we review the cases reported in literature via a systematic search. RESULTS: Ninety-four patients, 58.5% male with mean age of 39.2 ± 17.2 years were included. Fifty-seven (62%) had soft tissue sarcomas (STS) and 35 (38%) bone sarcomas (BS). Sarcoma was a primary tumor in 66%, developed after radiotherapy in 31.9% and 7.4% were metastatic. Median time between radiotherapy and sarcoma development was 10.5 (11) years. Main presentation symptoms were visual disorders (87.9%), headache (61.5%) and III cranial nerve palsy (24.1%). After surgery, sarcoma persisted or recurred in 82.3% and overall mortality reported was 44.6% with 6.5 (14) months of median survival. Tumor appeared earlier in BS compared to STS (34.4 ± 15.1 vs. 42.6 ± 17.6 years), p = 0.034 and complete tumor resection was achieved more often (41.3% vs. 4.4%), p = < 0.001. Condrosarcoma and rhabdomyosarcoma were more frequent subtypes among primary tumors while fibrosarcoma was among post-radiation sarcomas. Tumor size was larger in radiation associated sarcomas (mean maximum diameter 46.3 ± 9.3 vs. 29.1 ± 8.0 mm, p = 0.004) and persistency/recurrence was similar in both groups (70.1 vs. 73.3%, p = 0.259). CONCLUSION: Sarcomas appear as mass effect symptoms in the middle aged population, mainly as primary tumors, but one third is associated with radiotherapy. Surgery is commonly not curative, mortality rate is high and death ensues shortly after diagnosis.

Radiologic Predictors for Extent of Resection in Pituitary Adenoma Surgery. A Single-Center Study.

BACKGROUND: Preoperative radiologic evaluation of pituitary adenomas is essential. Despite the efforts made to determine the achieved resection grade after pituitary adenoma surgery, there is a high level of disagreement among all the available classifications and measurement methods used. Our study aimed to determine pituitary adenoma imaging features, easily obtained from preoperative magnetic resonance, which could be used as resection predictor variables. Second, we analyzed the usefulness of the ellipsoid method in pituitary adenoma volume determination. METHODS: Two-hundred and ninety-four pituitary adenomas, which were surgically treated in our department, were retrospectively analyzed. Age, gender, surgical approach, hormonal status, greater tumor diameter, volume, cavernous sinus invasion, and extent of resection were evaluated. RESULTS: One-hundred and forty-eight surgical procedures were conducted with a microsurgical transsphenoidal approach whereas 146 were conducted with an endoscopic endonasal approach. Gross total resection was achieved in 54.08% of cases. There were no significant differences in the extent of resection regarding the approach used, age, gender, or hormonal production by the tumor. Only Knosp grade (P < 0.001) and tumor volume (P < 0.05) had a statistically and independent significant relationship with the extent of resection. Furthermore, we found a high correlation between the calculated volume, using the ellipsoid method, and the volume measurement obtained with complex planimetry methods. CONCLUSIONS: Pituitary adenoma volume and cavernous sinus invasion, graded with the Knosp scale, are 2 pituitary tumor features that, when used in combination, predict the complexity of the surgery and the difficulty of achieving gross total resection in pituitary adenoma surgery.

Alteraciones endocrinas en las histiocitosis de la región hipotálamo hipofisaria / Endocrine changes in histiocytosis of the hypothalamic-pituitary axis

Las histiocitosis son cuadros caracterizados por la proliferación de células del sistema mononuclear fagocítico. Incluyen la histiocitosis de células de Langerhans (HCL) y las histiocitosis de células no Langerhans (entre ellas la enfermedad de Chester-Erdheim [ECE]). Aunque la diabetes insípida (DI) es la alteración hipotálamo hipofisaria (HH) más frecuente, están menos estudiados los déficits hipofisarios anteriores. Se analiza la frecuencia y la progresión de los déficits hormonales hipofisarios y los hallazgos radiológicos de 9 pacientes (7 HCL y 2 ECE) con afectación de la región HH. El 89% de los pacientes presentaba DI (62% al diagnóstico) y el 78%, uno o más déficits anteriores (71% al diagnóstico). Dado que la afectación HH es relativamente frecuente en pacientes con diagnóstico de histiocitosis y que los déficits hormonales pueden estar presentes al diagnóstico o aparecer de forma paulatina durante el curso de la enfermedad, es recomendable monitorizar de manera regular a este tipo de pacientes

Histiocytosis is characterized by proliferation of cells from the mononuclear phagocyte system, and may be divided into Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (including Erdheim-Chester disease [ECD]). While diabetes insipidus (DI) is the most common hypothalamic-pituitary consequence, anterior pituitary deficiencies are less known. This study analyzed the frequency and progression of pituitary hormone deficiencies and the radiographic findings in 9 patients (7 with LCH and 2 with ECD) with hypothalamic-pituitary (HP) axis. Eighty-nine percent of patients had DI (62% at diagnosis), and 78% had one or more anterior pituitary deficiencies (71% at diagnosis). HP involvement is relatively common in patients diagnosed with histiocytosis and hormone deficiencies may be present at diagnosis or appear gradually during the course of disease. Regular monitoring of these patients is recommended

Endocrine changes in histiocytosis of the hypothalamic-pituitary axis.

Histiocytosis is characterized by proliferation of cells from the mononuclear phagocyte system, and may be divided into Langerhans cell histiocytosis (LCH) and non-Langerhans cell histiocytosis (including Erdheim-Chester disease [ECD]). While diabetes insipidus (DI) is the most common hypothalamic-pituitary consequence, anterior pituitary deficiencies are less known. This study analyzed the frequency and progression of pituitary hormone deficiencies and the radiographic findings in 9 patients (7 with LCH and 2 with ECD) with hypothalamic-pituitary (HP) axis. Eighty-nine percent of patients had DI (62% at diagnosis), and 78% had one or more anterior pituitary deficiencies (71% at diagnosis). HP involvement is relatively common in patients diagnosed with histiocytosis and hormone deficiencies may be present at diagnosis or appear gradually during the course of disease. Regular monitoring of these patients is recommended.