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.

Anticipating Facial Nerve Position Using Three-Dimensional Tractography During the Preoperative Assessment of Cerebellopontine Angle Tumors.

OBJECTIVE: We aim to evaluate the usefulness of preoperative facial nerve tractography in determining the facial nerve position in cerebellopontine angle tumor resection and its value in helping to preserve facial nerve function during surgery. METHODS: A prospective study was designed to include patients presenting with cerebellopontine angle tumors. Three-dimensional reconstruction of facial nerve tractography was performed and added to the usual preoperative testing in all patients. Facial nerve position was compared between tractography results and surgical findings. Moreover, facial nerve function was evaluated at baseline and during follow-up. RESULTS: Fifteen patients were included for analysis. Complete resection was obtained in 5 patients, near-total resection was achieved in 8 patients, and partial resection in 2 patients. We found a strong statistically significant concordance between the preoperative facial nerve tractography reconstruction and the intraoperative findings (complete concordance in 86.66% of all the cases; κ = 0.784; P < 0.0001). Facial nerve anatomic structure was preserved in all patients during surgery. At 6 months follow-up, 66.66% of patients had a facial nerve normal function or a mild dysfunction. CONCLUSIONS: Preoperative facial nerve tractography reconstruction showed a high correlation with intraoperative findings. Preoperative tractography information regarding facial nerve position and its cisternal course is valuable information and could help the surgeon in increasing the safety of the procedure during cerebellopontine angle tumor surgery.

Effectiveness and safety of intraoperative radiotherapy (IORT) with low-energy X-rays (INTRABEAM) for accelerated partial breast irradiation (APBI)

PurposeTo evaluate treatment outcomes in patients with early-stage breast cancer (ESBC) treated with targeted intraoperative radiation therapy (IORT) administered as accelerated partial breast irradiation (APBI).MethodsBetween December 2014 and May 2019, 50 patients diagnosed with ESBC were treated with a 50 kilovoltage (kV) X-ray source with a single dose of 20 Gy using the Intrabeam® radiotherapy delivery system. All patients were followed prospectively to assess local control (LC), disease-free survival (DFS), cancer-specific survival (CSS), overall survival (OS), radiation-induced toxicity, and cosmetic outcomes. We also evaluated the prognostic implications of the neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR).ResultsMedian follow-up was 53 months. Mean patient age was 70 years. The mean duration of radiation delivery was 22.25 min. Two patients developed a recurrence. One death was recorded. Elevated pretreatment NLR levels were a significant risk factor for mortality (p = 0.0026). The most common treatment-related toxicities were breast induration (30%) and seroma (18%). Five-year LC, DFS, CSS, and OS rates were 97.1%, 93.9%, 100%, and 94.4%, respectively. Cosmesis was excellent or good in most cases (94%).ConclusionThese findings confirm the effectiveness of a single dose of 20 Gy of IORT with the Intrabeam device as APBI. The toxicity profile was good with excellent cosmesis. These results provide further support for the clinical use of APBI in well-selected patients. (AU)

High Myoinositol on Proton MR Spectroscopy Could Be a Potential Signature of Papillary Tumors of the Pineal Region-Case Report of Two Patients.

Papillary tumor of the pineal region (PTPR) is an uncommon entity in which a presurgical suspicion may be crucial for patient management. Maximal safe neurosurgical resection is of choice when PTPR is suspected, whereas non-surgical approaches can be considered in other tumors of the pineal region, such as pineocytoma or concrete subtypes of germ-cell tumors. In general terms, imaging features of tumors of the pineal region have been reported to be unspecific. Nevertheless, in this report, we describe two pathology-confirmed PTPRs in which presurgical proton MR spectroscopy demonstrated extremely high myoinositol, a pattern which drastically differs from that of other pineal tumors. We hypothesize that this high myoinositol may be related to PTPR's known ependymal component, and that it could be used as a specific non-invasive diagnostic signature.

Effectiveness and safety of intraoperative radiotherapy (IORT) with low-energy X-rays (INTRABEAM®) for accelerated partial breast irradiation (APBI).

PURPOSE: To evaluate treatment outcomes in patients with early-stage breast cancer (ESBC) treated with targeted intraoperative radiation therapy (IORT) administered as accelerated partial breast irradiation (APBI). METHODS: Between December 2014 and May 2019, 50 patients diagnosed with ESBC were treated with a 50 kilovoltage (kV) X-ray source with a single dose of 20 Gy using the Intrabeam® radiotherapy delivery system. All patients were followed prospectively to assess local control (LC), disease-free survival (DFS), cancer-specific survival (CSS), overall survival (OS), radiation-induced toxicity, and cosmetic outcomes. We also evaluated the prognostic implications of the neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR). RESULTS: Median follow-up was 53 months. Mean patient age was 70 years. The mean duration of radiation delivery was 22.25 min. Two patients developed a recurrence. One death was recorded. Elevated pretreatment NLR levels were a significant risk factor for mortality (p = 0.0026). The most common treatment-related toxicities were breast induration (30%) and seroma (18%). Five-year LC, DFS, CSS, and OS rates were 97.1%, 93.9%, 100%, and 94.4%, respectively. Cosmesis was excellent or good in most cases (94%). CONCLUSION: These findings confirm the effectiveness of a single dose of 20 Gy of IORT with the Intrabeam device as APBI. The toxicity profile was good with excellent cosmesis. These results provide further support for the clinical use of APBI in well-selected patients.

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.

Esclerosis tuberosa: degeneración grasa del rabdomioma / Tuberous sclerosis: Fatty degeneration of rhabdomyoma

No disponible