Plasma cell myeloma of the skull base: Report of two cases

Plasma cell myeloma (PCM) of the skull base is rarely encountered in neurosurgical practice. PCM has a wide spectrum of pathology, including a quite benign, solitary plasmacytoma (SPC), and an extremely malignant, multiple myeloma (MM) at the two ends of the spectrum. We have described two patients with PCM of the skull base, of which one harbored SPC, and the other progressed to MM. In case 1, a 46-year-old man presented with left multiple cranial nerve impairments and had a large skull base tumor. Subtotal removal was performed. The specimen and general examination, including bone marrow aspiration, revealed SPC. Postoperatively 50 Gy of external radiotherapy was administered. The patient has no manifestation of MM 24 months after the initial presentation. In case 2, a 53-year-old woman presented with left abducens palsy and had a left petroclival osteolytic mass. Gross total resection was performed. The specimen revealed a plasmablastic tumor, i.e., myeloma. General examination established the diagnosis of MM. She was administrated adjuvant chemotherapy and autologous bone marrow transplantation. She is alive without local recurrence 30 months after the initial presentation.     

Detection of liver metastases from endocrine tumors: a prospective comparison of somatostatin receptor scintigraphy, computed tomography, and magnetic resonance imaging.

PURPOSE: To compare the respective sensitivity of somatostatin receptor scintigraphy (SRS), computed tomography (CT), and magnetic resonance imaging (MRI) in the detection of liver metastases from well-differentiated gastroenteropancreatic endocrine tumor (WDGEP ET) patients. To define predictive factors for "high-sensitivity SRS." PATIENTS AND METHODS: Sixty-four patients with WDGEP ET underwent SRS with abdominal single-photon emission computed tomography (SPECT), spiral CT, and 1.5-T MRI within a 15-day interval, the order of which was randomized. Two readers analyzed images of each modality, blindly and independently. RESULTS: Hepatic metastases were present in 40 of the 64 patients and confirmed by pathology after liver biopsy or surgery in 32 and eight patients, respectively. SRS, CT, and MRI detected a total of 204, 325, and 394 metastases, respectively. The number of detected metastases was significantly higher with MRI than with CT (P = .02) and SRS (P < 10(-4)) and higher with CT than with SRS (P < 10(-4)). SRS was negative in seven patients with a positive CT and/or MRI. More lesions were detected in 10 patients by SPECT compared with static views. The median metastasis size was significantly correlated (P = .04) with the sensitivity of SRS. CONCLUSION: MRI seems to have an edge over CT and SRS for the detection of liver metastases from endocrine tumors. We recommend the systematic performance of liver MRI at WDGEP ET initial staging and before major therapeutic events. The low performance of SRS was mainly explained by the impact of the metastasis size on the detection capacity of SRS.     

Giant cell tumor of the base of the skull: a report of two cases and review of the literature]

Giant cell tumors of the skull base are rare neoplasms. This report reviews two cases of patients presenting with aggressive giant cell tumors that were irradiated by a combination of photons and protons. Two females 29 and 14 years old were initially managed with one and three extensive surgical resections respectively. Radiation therapy was recommended in respect to tumor aggressiveness. Combined proton and photon radiation therapy was performed based on a three-dimensional planning, and delivered a total dose of 59.4 CGE to 65.2 CGE respectively, administered in 5 sessions per week of 1.8-2 Gy/CGE (Cobalt Gray Equivalent). With 8 and 83 months follow-up, respectively, the youngest patient relapsed marginally 4 months post irradiation, while the second remained with NED. No complication developed in any of them. In conclusion, we have reviewed a total of 116 cases (114 previously published cases+2 new cases) and discuss the role and modalities of radiation therapy in the management of giant cell skull base tumors.     

MRI、增强CT和PET-CT对鼻咽癌颅底侵犯诊断价值的对比分析

目的：比较MRI、增强CT和18F-氟代脱氧葡萄糖正电子发射体层显影术（positron emission tomography，PET）-CT对鼻咽癌颅底侵犯的诊断价值，为鉴别诊断鼻咽癌病灶是否有颅底侵犯提供诊断依据。方法：对57例鼻烟癌患者同时行鼻咽MRI、增强CT和PET—CT，3种检查均在20d内完成。以组织病理学或至少6个月的临床和影像学随访结果为诊断病灶性质的标准，比较这3种方法诊断鼻咽癌颅底侵犯的敏感度、特异度、准确度、阳性预测值（positive predictive value，PPV）及阴性预测值（negative predictive value，NPV）。结果：增强CT、MRI和PET-CT诊断鼻咽癌颅底侵犯的敏感度分别为68．18％、84．09％和97．67％；特异度分别为76．92％、69．23％和57．14％；准确度分别为70．18％、80．70％和87．72％；PPV分别为90．90％、90．24％和87．50％；NPV分别为41．67％、56．25％和88．89％。PET—CT在敏感度方面较增强CT和MRI有明显优势（P〈0．05），准确度和NPV等方面优于增强CT（P〈0．05），其NPV也优于MRI。结论：在3种影像学方法中，PET—CT对初治鼻咽癌患者颅底是否累及的判定可能更具价值。     

Management of advanced adenoid cystic carcinoma infiltrating the skull base: a contemporary review

Journal of Neuro-Oncology - Due to the infiltrative nature of glioblastoma (GBM) outside of the contrast-enhancing region on MRI, there is interest in exploring supratotal resections (SpTR) that...     

Cancer of the prostate: value of bone scintigraphy. Point of view

Radionuclide bone scanning carried out with technetium radiopharmaceutics detects almost all prostatic carcinoma osseous metastases. It is easy to recognize focal areas of increased tracer uptake or a diffuse increased uptake, and the test provides a synthetic view of the entire skeleton. Complementary bone radiographs are necessary if the diagnosis remains doubtful, if mechanical complications are searched and if there is a post-radiotherapeutic decrease of the tracer uptake. A bone scan is necessary before the radical treatment of the primary tumour, in order to rule out the possibility of bone metastases. The initial bone scan has also a pronostic value. However, in the follow-up of initially non-metastatic patients, serial bone scans should not be realized when clinical symptoms or biological abnormalities lack. Bone scintigraphy is also useful to monitor the course of bone metastases under treatment, especially when the value of new therapeutic agents is investigated.     

Small islet cell tumour in the pancreas: diagnostic value of diffusion-weighted MRI

We present a case of a small islet cell tumour that was clearly depicted on diffusion-weighted imaging using a free breathing approach and discuss the diagnostic value of this sequence.     

Intensity-modulated radiotherapy for complex-shaped meningioma of the skull base: long-term experience of a single institution

PURPOSE: We analyzed our long-term experience with intensity-modulated radiotherapy (IMRT) in patients with complex-shaped meningioma of the skull base. PATIENTS AND METHODS: Between January 1998 and December 2004, 94 patients with complex-shaped meningioma were treated using IMRT at our institution. Tumor distribution was: World Health Organization (WHO) Grade 1 in 54.3%, WHO Grade 2 in 9.6%, and WHO Grade 3 in 4.2%. In 31.9% of patients, the clinical and radiologic characteristics of the tumor were consistent with the diagnosis of meningioma. Twenty-six patients received radiotherapy as primary treatment and 14 patients postoperative for residual disease. Fifty-four patients were treated after local recurrence. Median target volume was 81.4 mL, median total dose was 57.6 Gy given in 32 fractions. RESULTS: Median follow-up was 4.4 years. Overall local control was 93.6%. Sixty-nine patients had stable disease based on computed tomography/magnetic resonance imaging (MRI), whereas 19 had a tumor volume reduction after IMRT. Six patients showed local tumor progression on MRI 22.3 months' median after IMRT. Three patients died from non-treatment-related conditions after IMRT. In 39.8% of the patients, preexisting neurologic deficits improved. Worsening of preexisting neurologic symptoms was seen in 4 patients and 2 patients developed new clinical symptoms from local tumor progression. Transient side effects such as headache were seen in 7 patients. Treatment-induced loss of vision was seen in 1 of 53 reirradiated patients with a Grade 3 meningioma 9 months after retreatment with IMRT. CONCLUSION: These data demonstrate that IMRT is an effective and safe treatment modality for long-term local control of complex-shaped and otherwise difficult to treat meningioma.     

Comparison between transversal SPECT, 3-dimensional rendering and 3-dimensional rendering plus clipping in the diagnosis of somatostatin receptor distribution in malignancies using 111In-DTPA-D-Phe1-octreotide scintigraphy

Two hundred and seven patients were investigated with [111In-DTPA-D-Phe1]-octreotide Single Photon Emission Computerized Tomography (SPECT) scintigraphy. A comparison was carried out of the diagnostic accuracy of the three display modalities, viz. transversal SPECT (trvSPECT), three dimensional volume rendering (3Dvr) and three dimensional volume rendering plus clipping (3Dvr + c) in the rendered volume. TrvSPECT could visualize a greater number of lesions in 85 (41%) cases when compared with 3Dvr. In 48 (23%) cases, trvSPECT could visualize a greater number of lesions when compared with 3Dvr + c. The differences were caused by radioactivity in intestines and the gallbladder and in lesions shaped like an hour-glass. In conclusion, 3Dvr + c imaging was regarded as the reference since it combines both transversal and volume information. An overall sensitivity of 0.998 was found for trvSPECT and the corresponding value for 3Dvr was 0.75. The specificity was 0.45 for trvSPECT and 0.80 for 3Dvr. The accuracy was 0.87 for trvSPECT and 0.76 for 3Dvr, when compared with 3Dvr + c. The value of 3Dvr is in high-contrast images, where it provides additional anatomical information, whereas in low-contrast images, 3Dvr was found to have low accuracy. In low-contrast images, trvSPECT was found to be almost equal in accuracy to 3Dvr + c imaging. However, in high-contrast images, trvSPECT was found to give more false positives than 3Dvr + c in the assessment of SPECT studies using [111In-DTPA-D-Phe1]-octreotide. The interpretation was that trvSPECT has to be used in conjunction with 3Dvr + c and, in high-contrast images, 3Dvr provides additional value.     

Comparative studies on the expression of somatostatin receptor subtypes, outcome of octreotide scintigraphy and response to octreotide treatment in patients with carcinoid tumours.

We have compared the expression of somatostatin receptor (sstr) subtypes with the outcome of somatostatin receptor scintigraphy and the effect of somatostatin receptor activation in patients with disseminated carcinoid tumours. Tumour tissues from nine patients with midgut carcinoids (ileal) and three patients with foregut carcinoids (gastric, thymic) were analysed using Northern blotting. Expression of somatostatin receptors was demonstrated in all tumours (12 out of 12), with all five receptor subtypes present in 9 out of 12 tumours. Somatostatin receptor scintigraphy using [111In]DTPA-D-Phe1-octreotide visualized tumours in all patients (12 out of 12). The 111In activity concentrations in tumour tissue (T) and blood (B) were determined in three tumours 1-7 days after injection of the radionuclide. The T/B 111In activity concentration ratios ranged between 32 and 651. Clinically, treatment with the long-acting somatostatin analogue octreotide resulted in marked symptom relief accompanied by a significant reduction in tumour markers, for example urinary-5-HIAA levels (28-71% reduction). Incubation of midgut carcinoid tumours in primary culture with octreotide (10 microM) resulted in a reduction in spontaneously secreted serotonin (45-71% reduction) and 5-HIAA (41-94% reduction). The results demonstrate that carcinoid tumours possess multiple somatostatin receptor subtypes and that somatostatin analogues such as octreotide, which preferentially bind to somatostatin receptor subtype 2 and 5, can be used in the diagnosis and medical treatment of these tumours. In the future, novel somatostatin analogues with subtype specific receptor profiles may prove to be of value for individualizing the treatment of disseminated carcinoid tumour disease.     

Proton therapy for base of skull chordoma: a report for the Royal College of Radiologists. The Proton Therapy Working Party

Chordomas of the base of the skull are rare. They are locally infiltrative and frequently arise close to radiosensitive structures, which limits the ability to deliver a high dose of radiotherapy. Complete surgical excision is not usually possible. Conventional postoperative radiotherapy can result in approximately 50% 5-year survival and effective palliation, but long-term local control and cure are rare. The well-defined Bragg peak of protons allows planning with a sharp cut-off outside the target volume. This permits a higher dose of radiotherapy to be delivered to the tumour while avoiding excessive irradiation to radiosensitive structures. Outcome after proton irradiation is superior to that reported for conventional photon irradiation. Radiotherapy schedules involving a mixed schedule of protons and photons have achieved an approximately 60% local control rate at 5 years. Some of this improvement may have resulted from better surgical techniques. Proton irradiation is also effective for base of skull chondrosarcomas. Protons of sufficient energy to treat base of skull tumours are not available in the UK. Patients have been referred to the proton facilities at the Harvard cyclotron, and at Orsay, France. They will continue to require referral abroad for proton therapy for base of skull tumours. Proton therapy has become standard treatment for ocular melanoma and this is available at the Douglas cyclotron at Clatterbridge Hospital. Proton therapy has the potential for improved dose distribution compared with conformal photon radiotherapy. This may be exploited effectively to irradiate target volumes close to radiosensitive structures such as the spinal cord. There is a need for further clinical research to evaluate proton therapy for tumours such as spinal and paraspinal sarcomas, and paediatric brain tumours.     

Chondromyxoid fibroma of the skull base: differential diagnosis and radiotherapy: two case reports and a review of the literature

Chondromyxoid fibromas are uncommon tumours mostly arising in long bones of young males. Involvement of the skull base is extremely rare. We describe two new cases of base of the skull chondromyxoid fibromas. The tumours were incompletely excised and irradiated with protons because of the high risk of complications of another surgical procedure. The rationale for proton therapy was based on the intimate relations between the tumour and the organs at risk. Skull base chondromyxoid fibroma is a very rare, slowly growing benign tumour that can cause severe disabilities due to tumour compression of critical structures. Only surgical resection has been shown to be relatively effective. We report two cases of incompletely excised lesions treated by postoperative high-dose radiation including proton therapy with no active disease and complication. Our review of the literature allows us to conclude that histological diagnosis of lesions in this site is a trap for pathologists and that radiotherapy is not contraindicated.