Spontaneous regression of a clival chordoma. Case report.

In this case report, we present a rare and previously unreported case of spontaneous regression of a histologically consistent clival chordoma. At the time of diagnosis, imaging demonstrated a T2 hyperintense and T1 isointense midline skull base mass, centered in the nasopharynx, with scalloping of the ventral clivus consistent with a chordoma measuring 3.1 × 1.9 × 3.0 cm (8.84 cm3). On pre-operative imaging 2 months later, with no intervening therapy, the mass had regressed by 61.7% to a size of 2.3 × 2.1 × 1.4 cm (3.38 cm3). The patient self-administered several herbal supplements and animal oils which may have contributed to tumor regression. The purpose of this report is to document this rare occurrence and provide a comprehensive description of the case details and list of the various medications, herbs, and supplements used prior to this rare event.

Evidence-Based Optimization of Post-Treatment Surveillance for Skull Base Chordomas Based on Local and Distant Disease Progression.

BACKGROUND: There are no guidelines regarding post-treatment surveillance specific to skull base chordomas. OBJECTIVE: To determine an optimal imaging surveillance schedule to detect both local and distant metastatic skull base chordoma recurrences. METHODS: A retrospective review of 91 patients who underwent treatment for skull base chordoma between 1993 and 2017 was conducted. Time to and location of local and distant recurrence(s) were cataloged. Existing chordoma surveillance recommendations (National Comprehensive Cancer Network [NCCN], London and South East Sarcoma Network [LSESN], European Society for Medical Oncology [ESMO], Chordoma Global Consensus Group [CGCG]) were applied to our cohort to compare the number of recurrent patients and months of undiagnosed tumor growth between surveillances. These findings were used to inform the creation of a revised imaging surveillance protocol (MD Anderson Cancer Center Chordoma Imaging Protocol [MDACC-CIP]), presented here. RESULTS: Thirty-four patients with 79 local/systemic recurrences met inclusion criteria. Mean age at diagnosis and follow-up time were 45 yr and 79 mo, respectively. The MDACC-CIP imaging protocol significantly reduced the time to diagnosis of recurrence compared with the LSESN and CGCG/ESMO imaging protocols for surveillance of local disease with a cumulative/average of 576/16.9 (LSESN), 336/9.8 (CGCG), and 170/5.0 (MDACC-CIP) months of undetected growth, respectively. The NCCN and MDACC-CIP guidelines for distant metastatic surveillance identified a cumulative/average of 65/6.5 and 51/5.1 mo of undetected growth, respectively, and were not significantly different. CONCLUSION: The MDACC-CIP for skull base chordoma accounts for recurrence trends unique to this disease, including a higher rate of leptomeningeal spread than sacrococcygeal primaries, resulting in improved sensitivity and prompt diagnosis.

[Aspects of nutrition therapy of an infant with central nervous system tumour]. / A táplálásterápia alkalmazásának szempontjai egy központi idegrendszeri daganatos csecsemõ ellátása során.

The atypical teratoid/rhabdoid tumour (ATRT) is a rare type of central nervous system tumour appearing usually under 2 years of age. The survival of patients is insufficient despite the combined treatment (neurosurgical removal, intensive chemo- and radiotherapy). ATRT recurs one year after completion of treatment in 60% of cases. Maintaining appropriate nutritional status during treatment is of great importance in this young age group. Nutritional treatment of patients with ATRT is especially difficult due to young age and possible neurological sequelae. A successful case of a three-month-old female infant is presented, with special emphasis on the importance of feeding therapy.

125I brachytherapy alone for recurrent or locally advanced adenoid cystic carcinoma of the oral and maxillofacial region.

BACKGROUND AND PURPOSE: This retrospective study was to evaluate the local control and survival of (125)I brachytherapy for recurrent and/or locally advanced adenoid cystic carcinoma (ACC) of the oral and maxillofacial region. PATIENTS AND METHODS: A total of 38 patients with recurrent and/or locally advanced ACC of the oral and maxillofacial region received (125)I brachytherapy alone from 2001-2010. Twenty-nine were recurrent cases following previous surgery and radiation therapy. The other 9 cases involved primary tumors. Overall, 12 tumors were located in the major salivary glands, 12 in the minor salivary glands, and 14 in the paranasal region, the nasal cavity or the skull base. The prescribed dose was 100-160 Gy. RESULTS: Patients were followed for 12-122 months (median 51 months). The 2-, 5-, and 10-year local tumor control rates were 86.3, 59, and 31.5 %, respectively. The 2-, 5-, and 10-year overall survival rates were 92.1, 65 and 34.1 %, respectively. Tumors > 6 cm had significantly lower local control and survival rates. No severe complications were observed during follow-up. CONCLUSION: (125)I brachytherapy is a feasible and effective modality for the treatment of locally advanced unresectable or recurrent ACC.

Quantitative analysis of exposure of staged orbitozygomatic and retrosigmoid craniotomies for lesions of the clivus with supratentorial extension.

OBJECTIVE: A two-stage approach using orbitozygomatic (OZ) and retrosigmoid (RS) craniotomies is one option for the management of petroclival lesions with supratentorial extension. The goal of this study was to investigate the supratentorial and infratentorial exposures of the clivus obtained through this staged approach. METHODS: Formalin-fixed, silicon-injected specimens underwent stereotactic imaging. Six paired OZ and RS craniotomies were performed. Neuronavigation was used to determine the areas and limits of exposure and to plot these areas on three-dimensional reconstructions of the skull base. RESULTS: The mean area of exposure of the parasellar region and clivus through the OZ craniotomy was 640 +/- 75 mm. Visualization of the parasellar region, cavernous sinus, and upper cranial nerves was achieved. The ventral brainstem corresponding to the cranial quarter of the clivus was visualized. The mean area of exposure of the clivus and petrous bone through the RS was 1930 +/- 250 mm. In the cranial quarter of the clivus, there was a small region of overlap in exposure between the two craniotomies. The limits of exposure are described. CONCLUSION: OZ and RS craniotomies provide complementary exposure with limited redundancy. Significant visualization of the parasellar region, clivus, and surrounding bony landmarks is obtained. The primary limitation is exposure of the contralateral half of Zones II and III of the clivus. This strategy represents a reasonable option for accessing paracentral petroclival lesions with a supratentorial extension.

Maffucci's syndrome associated with a cranial base chondrosarcoma: case report and literature review.

OBJECTIVE AND IMPORTANCE: Our objective was to study the diagnosis and management of this rare condition. A review of the literature concerning chondrosarcomas related to Maffucci's syndrome is reported. Cause and management are discussed. CLINICAL PRESENTATION: We report a case of Maffucci's syndrome associated with a cranial base chondrosarcoma. To our knowledge, only five similar cases have been reported in the literature. The differential diagnosis between Ollier's disease and Maffucci's syndrome and the causes of these conditions are not clear. INTERVENTION: An 18-year-old female patient presented with a giant tumor involving the posterior fossa, clivus, middle fossa, and cavernous sinus. The lesion could be totally removed through a transzygomatic approach. The histological diagnosis was chondrosarcoma. It was confirmed by immunohistochemical studies. There were no postoperative complications. CONCLUSION: Maffucci's syndrome is a rare clinical condition that presents difficulties concerning its diagnosis and management. It is characterized by the presence of multiple enchondromas and cutaneous hemangiomas. Intracranial chondrosarcomas may be associated with this syndrome. Immunohistochemical studies are necessary to differentiate chondrosarcomas from chordomas. The treatment of choice for cranial base chondrosarcomas is total removal of the lesion. Total removal may be very difficult to achieve because of the involvement of neurovascular structures. Alternative therapies, such as proton beam radiosurgery, should be considered. In this case, radical removal of the tumor was possible using a transzygomatic approach. Gross total removal of large cranial base chondrosarcomas is possible, but a longer follow-up period is necessary to ascertain that radical resection was achieved.

Chondrosarcoma of the skull base: CT, MR and pathological features.

We report on CT and MR image findings of a skull base chondrosarcoma. Chondroid mineralisation, a histological feature of chondrosarcoma, can be recognised on CT and is, together with the off-midline position of the tumour, the most characteristic finding. Differentiation is usually made by histological and immunocytochemical staining. Surgery, proton radiation therapy and stereotactic single high-dose irradiation have been used to treat patients with chondrosarcoma. It is worth mentioning that it was likely that the chondrosarcoma in our patient represented a malignant degeneration of an enchondroma that had been resected 30 years earlier.