Rituximab for corticosteroid-resistant relapsing IgG4-related intracranial pachymeningitis: report of two cases.

IgG4-related disease is now recognised as an important cause of intracranial and spinal hypertrophic pachymeningitis. Treatment with corticosteroids generally leads to significant clinical improvement. We present two cases of IgG4 pachymeningitis unresponsive to corticosteroids who improved with rituximab.

Transoral robotic surgery for sellar tumors: first clinical study.

OBJECTIVE The aim of this study was to confirm the feasibility of an innovative transoral robotic surgery (TORS), using the da Vinci Surgical System, for patients with sellar tumors. This technique was designed to offer a new minimally invasive approach, without soft-palate splitting, that avoids the rhinological side effects of classic endonasal approaches. METHODS The authors performed a prospective study of TORS in patients with symptomatic sellar tumors. Specific anatomical features were required for inclusion in the study and were determined on the basis of preoperative open-mouth CT scans of the brain. The main outcome measure was sellar accessibility using the robot. Resection quality, mean operative time, postoperative changes in patients' vision, side effects, and complications were additionally reported. RESULTS Between February and May 2016, 4 patients (all female, mean age 49.5 years) underwent TORS for resection of sellar tumors as participants in this study. All patients presented with symptomatic visual deficits confirmed as bitemporal hemianopsia. All tumors had a suprasellar portion and a cystic part. In all 4 cases, the operation was performed via TORS, without the need for a second surgery. Sella turcica accessibility was satisfactory in all cases. In 3 cases, tumor resection was complete. The mean operative time was 2 hours 43 minutes. Three patients had a significant visual improvement at Day 1. No rhinological side effects or complications in patients occurred. No pathological examination was performed regarding the fluid component of the tumors. There was 1 postoperative delayed CSF leak and 1 case of transient diabetes insipidus. Side effects specific to TORS included minor sore throat, transient hypernasal speech, and 1 case of delayed otitis media. The mean length of hospital stay and mean follow up were 8.25 days and 82 days, respectively. CONCLUSIONS To our knowledge, this is the first report of the surgical treatment of sellar tumors by means of a minimally invasive TORS. This approach using the da Vinci Surgical System seems feasible and constitutes an innovative neurosurgical technique that may avoid the adverse side effects and technical disadvantages of the classic transsphenoidal route. Moreover, TORS allows an inferosuperior approach to the sella turcica, which is a key point, as the tumor is approached in the direction of its growth.

[Specificities of carcinomas in adolescents and young adults]. / Carcinomes des adolescents et jeunes adultes : quelles spécificités ?

Carcinomas are rare tumors of the adolescent-young adult (AYA) with a different spectrum from those of adults. The most common sites outside of the thyroid is the nasopharynx, salivary gland, colon-rectum and ovaries. If nasopharyngeal carcinoma or salivary gland tumors are good prognosis, others are more reserved prognosis, such as digestive carcinomas, gynecological or midline. The revelation modes are non-specific and depend on the location: mass, tumor syndrome, pain, impaired general condition. The unusual of pediatric carcinomas led to propose a systematic oncogenetic exploration. The medical history of the family, the symptoms and the type of the carcinoma should guide the analysis. In the absence of guidance, analysis of TP53 gene and, for carcinomas of the expanded spectrum of the hereditary non-polyposis colorectal cancer (HNPCC) syndrome, the search for mutation of MMR genes (mismatch repair) seems essential. Because of the rarity of these diseases and the absence of homogeneous recommendations, members of the rare tumors committee of the SFCE recommended for the management of these AJA, an histological review by a pathologist familiar with carcinomas of the adult, a double discussion between pediatric and adult oncologists, analysis of adult standards with adaptation to pediatric data, especially in terms of risk of side effects. An advice to a national opinion (by a member of FRACTURE group) or European (group EXPERT) is sometimes necessary in the most complex situations.

Pediatric salivary gland carcinomas: Diagnostic and therapeutic management.

OBJECTIVES/HYPOTHESIS: To analyze clinical presentations, treatment modalities, and evolution of pediatric cases of salivary gland carcinomas to standardize care for these rare diseases. STUDY DESIGN: Multicentric, retrospective study. METHODS: We included in this retrospective study all children and adolescents (aged ≤18 years) treated from 1992 to 2012 in six pediatric centers in Paris, France. Pathological tumor specimens of these cases were reviewed. RESULTS: Forty-three children were included (sex ratio male/female = 19/24, median age = 13 years). The parotid gland was the most common (37 cases) location for tumors. Histological subtypes were mucoepidermoid carcinomas (n = 20), acinic cell carcinomas (n = 14), and other (n = 9). Initial fine-needle aspiration was performed in 15 cases (33%), and was concordant to final diagnosis in three cases (20%). Primary surgery was performed in 42 patients, leading to a complete microscopic resection in 80%. Associated lymph node dissection was performed in 28 patients (homolateral: 27, bilateral: 1) and showed lymph node metastases in only two patients. One patient had distant metastases. Adjuvant irradiation was delivered to 11 patients (median: 60 Gy; range: 50-65) and chemotherapy in five cases. After a median follow-up of 5 years, six tumors relapsed, but no deaths occurred. CONCLUSIONS: Childhood salivary gland carcinomas have a good prognosis despite possible recurrences. Treatment is mainly based on surgery, with simultaneous node dissection in cases of clinical or radiologic node enlargement. Radiation therapy seems to be restricted to inoperable high-grade tumors or after recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:140-147, 2017.