Pretreatment diagnosis of suprasellar papillary craniopharyngioma and germ cell tumors of adult patients.

BACKGROUND AND PURPOSE: Suprasellar papillary craniopharyngiomas and germ cell tumors in adults share some clinical and imaging similarities but have different therapeutic strategies and outcomes. This study aimed to evaluate the pretreatment diagnosis of these 2 tumors to improve the therapeutic outcome. MATERIALS AND METHODS: We retrospectively enrolled 18 adults with papillary craniopharyngiomas and 17 with germ cell tumors. The MR imaging findings were evaluated, including signal change and anatomic extension. The medical records were reviewed to collect clinical findings, management, and outcomes. RESULTS: The clinical findings of papillary craniopharyngiomas versus germ cell tumors were as follows: age: 46 ± 13.9 years versus 23 ± 7.1 years (P < .0001); diabetes insipidus: 2/18 (11%) versus 11/17 (65%) (P = .001); recurrence 13/16 (81%) versus 4/17 (24%) (P = .0031). The MR imaging findings of papillary craniopharyngiomas versus germ cell tumors were as follows­pituitary stalk thickening: 1.6 ± 0.4 mm versus 5.4 ± 4.2 mm (P < .0001); vertical infundibular extension: 1/18 (6%) versus 16/17 (94%) (P < .0001); sagittal spheric shape: 17/18 (94%) versus 1/17 (6%) (P < .0001); diffusion restriction: 1/17 (6%) versus 8/12 (67%) (P = .0009). CONCLUSIONS: Younger age, diabetes insipidus, MR imaging characteristics of restricted diffusion, and vertical infundibular extension favor the diagnosis of germ cell tumors. Spheric shape without infundibular infiltration provides clues to papillary craniopharyngiomas, which originate from the pars tuberalis and are located outside the third ventricle. We suggest that suprasellar germ cell tumor is possibly an intraventricular lesion. Appropriate treatment planning can be initiated according to the diagnosis and anatomic location.

Evaluation of the outcomes of endovascular management for patients with head and neck cancers and associated carotid blowout syndrome of the external carotid artery.

AIM: To evaluate factors related to the technical and haemostatic outcomes of endovascular management in patients with head and neck cancers (HNC) associated with carotid blowout syndrome (CBS) of the external carotid artery (ECA). MATERIALS AND METHODS: Between 2002 and 2011, 34 patients with HNC with CBS involving branches of the ECA underwent endovascular therapy. Treatment included embolization with microparticles, microcoils, or acrylic adhesives. Fisher's exact test was used to examine demographic features, clinical and angiographic severities, and clinical and imaging findings as predictors of endovascular management outcomes. RESULTS: Technical success and immediate haemostasis were achieved in all patients. Technical complications were encountered in one patient (2.9%). Rebleeding occurred in nine patients (26.5%). Angiographic vascular disruption grading from slight (1) to severe (4) revealed that the 18 patients with acute CBS had scores of 2 (2/18, 11.1%), 3 (3/18, 16.7%), and 4 (13/18, 72.2%). The 16 patients with impending and threatened CBS had scores of 1 (1/16, 6.25%), 2 (5/16, 31.25%), and 3 (10/16, 62.5%; p = 0.0003). For the 25 patients who underwent preprocedural computed tomography (CT)/magnetic resonance imaging (MRI) examinations within 3 months of treatment, the agreement between clinical and imaging findings reached the sensitivity, specificity, and kappa values for recurrent tumours (1, 0.7143, 0.7826), soft-tissue defect (0.9091, 0.3333, 0.2424), and sinus tract/fistula (0.4737, 0, 0.4286). CONCLUSION: Endovascular management for patients with CBS of the ECA had high technical success and safety but was associated with high rebleeding rates. We suggest applying aggressive post-procedural follow-up and using preprocedural CT/MRI to enhance the periprocedural diagnosis.

Identification of two novel missense mutations in the KAL1 gene in Han Chinese subjects with Kallmann Syndrome.

OBJECTIVES: To identify mutations in the KAL1, the KAL2, and PROKR2/PROK2 genes and to characterize phenotypic features in 5 Chinese subjects with Kallmann Syndrome (KS) and 6 subjects with normosmic hypogonadotrophic hypogonadism (NHH) in Taiwan. DESIGN AND PATIENTS: Five unrelated males (age range 22-52 yr) with clinical manifestations of KS and 6 unrelated males (age range 24-47 yr) with NHH were analyzed. In addition, 5 relatives of KS subjects were also evaluated. Genomic DNA extraction, PCR, and DNA sequence analyses were performed using standard procedures. RESULTS: The 1st patient had a single missense mutation in his copy of the KAL1 gene, a T→G transversion in codon 134 that results in replacement of cysteine by gly cine. The 2nd affected subject had a single missense mutation in the KAL1 gene, a T→C transition in codon 163 that results in replacement of cysteine by arginine. The 3rd case was hemizygous for a nonsense mutation in codon 424 of exon 9 (c.CGA→TGA) of the KAL1 gene. This mutation predicts a markedly truncated protein. Two of the mutations (p.C134G and p.C163R) we identified in the KAL1 gene are novel. CONCLUSIONS: We identified 3 mutations, including 2 novel mutations, in the KAL1 gene in patients with KS in Taiwan. These data extend the variety of KAL1 gene mutations in KS and further define the role of the KAL1 protein in olfactory bulb development.

Carotid blowout syndrome in patients with head-and-neck cancers: reconstructive management by self-expandable stent-grafts.

BACKGROUND AND PURPOSE: Some reports of reconstructive management of carotid blowout syndrome (CBS) with stent-grafts are promising, but some are unfavorable. This study sought to evaluate the hemostatic efficacy, safety, and outcome of reconstructive, endovascular stent-graft placement in patients with head-and-neck cancers in association with CBS. METHODS: Eight patients with head-and-neck cancers with CBS were treated with self-expandable stent-grafts. We evaluated the initial hemostatic results, complications, and outcomes by assessing the clinical and imaging findings. RESULTS: Immediate hemostasis was achieved in all patients. Initial complications included stroke in 1 patient and asymptomatic thrombosis of the carotid artery in 2 patients. Delayed complications included rebleeding, delayed carotid thrombosis, and brain abscess formation. Rebleeding was noted in 4 patients and was successfully managed with a second stent-graft and embolization in 2 of them. Delayed carotid thrombosis with follow-up after 3 months was found in 3 patients, 1 of whom had associated brain abscesses. CONCLUSION: Although stent-grafts achieved immediate and initial hemostasis in patients with head-and-neck cancers and CBS, long-term safety, stent patency, and permanency of hemostasis appeared unfavorable. This treatment may be for temporary or emergency purposes rather than serving as a permanent measure. We suggest its applications in patients with acute CBS that precludes performance of an occlusion test, as well as when carotid occlusion poses an unusually high risk of neurologic morbidity. We also propose prophylactic antibiotic treatment and combined embolization of pathologic vascular feeders to improve outcomes.

Brain abscess formation: a delayed complication of carotid blowout syndrome treated by self-expandable stent-graft.

A patient with hypopharyngeal cancer developed carotid blowout syndrome (CBS) treated by self-expandable stent-graft in the left carotid artery. CT scan for progressive right hemiparesis 4 months later showed multiple left cerebral abscesses and left carotid thrombosis. Although deployment of stent-grafts for CBS can achieve initial hemostasis in patients with head-and-neck cancer, the placement of a stent-graft in a field of necrosis and infection is associated with poor long-term outcome. We recommend the use of prophylactic antibiotics if endovascular foreign materials are placed in a contaminated field.

Is vasospasm requisite for posterior leukoencephalopathy in patients with primary thunderclap headaches?

Primary thunderclap headache (TCH) is sometimes associated with cerebral vasospasm. However, the role of vasospasm in relation to the development of reversible or irreversible posterior leukoencephalopathy among patients with primary TCH has never been fully addressed. This paper includes a report on a 51-year-old woman with primary TCH complicated with posterior leukoencephalopathy and a literature review of 16 further patients with the same illness. Their magnetic resonance or conventional angiographic findings were clearly described. Our review found that all these 17 patients showed evidence of cerebral vasospasm. Eleven (65%) of them developed permanent ischaemic infarctions, almost exclusively located at the watershed zones. We suggest that the presence of vasospasm might be requisite for posterior leukoencephalopathy as well as for permanent infarctions in these patients. Therefore, searching for any clue of vasospasm is mandatory in treatment of patients with primary TCH. Absence of an accompanying vasospasm might predict a good outcome.

Evaluation of clinical and MR findings for the prognosis of spinal epidural haematomas.

AIM: The potential of MR and clinical findings of spinal epidural haematomas (SEHs), particularly the early MR findings, to help minimize delays in diagnosis, to aid prognosis and as a reference for conservative treatment, are evaluated. METHODS: Retrospectively 20 patients with SEHs (14 men and 6 women) were examined to record their neurological deficit, MR findings, management, clinical outcome, and interval between symptom onset and MRI or surgery. Two-tailed Fisher's exact test was used for these analyses. RESULTS: Of 8 patients with severe neurological deficit at the onset of symptoms, none had obvious clinical improvement after either surgical or conservative management. Of 12 patients with mild to moderate deficits, 11 (92%) showed improvement or recovery of clinical symptoms. T2-weighted images revealed myelopathy or infarction of the compressed spinal cord in 9 patients, 7 (78%) of whom had no improvement in neurological deficit with either conservative or surgical management. Images in 6 patients showed contrast enhancement in the haematomas. CONCLUSION: Poor clinical outcomes were observed mainly in those with severe neurological deficit and hyperintensity on T2-weighted images of the involved spinal cord. Surgery did not appear to improve outcome in many of these patients. In acute SEHs, MRI showed characteristic findings, such as contrast enhancement, which should not be confused with signs of inflammatory or neoplastic epidural disease.

Neuroradiological findings in atypical teratoid/rhabdoid tumor of the central nervous system.

PURPOSE: To evaluate the computed tomography (CT) and magnetic resonance imaging (MRI) findings of atypical teratoid tumor/rhabdoid tumor (AT/RT) of the central nervous system (CNS). MATERIAL AND METHODS: Twenty cases of CNS AT/RT have been found over the past 23 years in our hospital; these involving 11 boys and 9 girls whose mean age at diagnosis was 5.5 years. Their clinical data, the CT, and MRI findings were reviewed retrospectively. RESULTS: AT/RT was located in the cerebellum in 15 cases. Four cases arose from the supratentorial region, while only one occurred primarily in the lumbar spinal cord. Almost all cases revealed heterogeneous intensity and heterogeneous enhancement. Peripheral cystic components were common. Survival time ranged from 2 months to 3 years, with a mean survival of 11.6 months. CONCLUSION: Most cases of AT/RT are located in the cerebellum. The radiologic manifestations are non-specific. The diagnosis mainly depends on the pathologic findings. However, AT/RT should still remain in the differential diagnosis of brain tumors in young children, especially those located in the cerebellar hemisphere and with eccentric cysts.

Headache profiles in patients with a dilatated cyst of the cavum septi pellucidi.

The dilatated cyst of the cavum septi pellucidi (CSP) is rare and may be associated with headaches. We reviewed the computerized database of 54,000 patients' computed tomography or magnetic resonance images and found 22 cases (0.04%) involving a dilatated cyst of the CSP. Sixteen patients had a chief complaint of headache, which was classified as acute episodic headache (type I, n = 7, 43.7%), chronic daily headache (CDH) with acute onset (type II, n = 5, 31.3%), or CDH with insidious onset (type III, n = 4, 25%). Acute Valsalva-induced headaches were common with type I (85%) or II (100%); 70% of these responded to indomethacin. At follow-up, patients with type I headache had the highest remission rate (71%), and type III patients the lowest (0%). Dilatated cysts of the CSP should be considered a cause of acute Valsalva-induced headache or new daily persistent headache, and may respond to indomethacin. A protracted course (> or = 3 months) indicates a worse outcome.

Magnetic resonance imaging appearance of intradural spinal lipoma.

Intradural spinal lipoma not associated with spinal dysraphism is a rare tumor often presenting with nonspecific symptoms and indolent clinical course. Its intradural location and fat component is the key for proper preoperative diagnosis, which could hardly be made by traditional imaging studies including plain film and myelography. Both CT and MRI can reveal the fat component of the tumor, but MRI is superior to CT in demonstrating its relationship with adjacent normal nerve tissue. We report a 32-year-old man who had back pain for years and the symptom progressed rapidly in recent two months. MRI revealed an intradural tumor at T12 level with high signal intensity on both T1- and T2-weighted images. The signal intensity dropped dramatically with fat saturation technique, which confirmed fat as its main component. The patient received surgery and the tumor was proved to be an intraspinal lipoma.

Image evaluation of suprazygomatic masticator space lesions.

In order to understand lesions involving the suprazygomatic masticator space (SZMS) demonstrated on computered tomography (CT) or magnetic resonance (MR) images, we collected 45 cases of lesions in SZMS. There were hematomas in 26 cases, tumors in 16 cases, and abscesses in 3 cases. The location of these lesions in compartments of the SZMS and adjacent structures was analyzed. Most commonly hematoma was found in the deep loose connective tissue (80.8%), followed by the superficial dense connective tissue (61.5%). Only 2 SZMS hematoma involved the superficial fat pad. Five patients had air accumulation in the SZMS, and all were in the deep fat pad and with maxillary sinus fracture. Of the sixteen cases which were tumors, 12 were extended from adjacent structures, and four were primary tumor. In cases of tumor, compartments of SZMS involved most frequently were temporal muscles (15 of 16 cases), followed by deep fat pad (8 of 16 cases). The connective tissue layer of SZMS is a communication pathway for superficial spread of infection, hematoma, or tumor invasion to or from the scalp and face. Compartments of deep fat pad and temporal muscle are also pathways for the spread of disease to or from the face, intracranium and orbit. The primary tumors in infratemporal fossa and SZMS are rare. Malignant tumors in SZMS usually come from surrounding spaces.

Salvage surgery for recurrent nasopharyngeal carcinoma.

OBJECTIVE: To evaluate the efficacy of salvage surgery in the treatment of recurrent nasopharyngeal carcinoma (NPC) at the primary site. STUDY DESIGN: A retrospective investigation of the outcome of salvage surgery for 28 patients with recurrent NPC after definite radiation therapy. METHODS: The nasopharynx was approached anteroposteriorly by the transmaxillary approach (maxillary swing, maxillectomy) or inferior approach (midline mandibulotomy or median labiomandibular glossotomy), or laterally by modified facial translocation or transpterygoid approach; intentional ligation of the internal carotid artery was performed after establishment of extracranial-intracranial (EC-IC) bypass in one patient; postoperative irradiation was given to the patients with positive pathological margins. RESULTS: Nine patients lived without disease for 20 to 93 months (mean interval, 52 mo) after surgery; among them, eight patients had T1 tumors that were resected totally by surgery via anteroposterior approaches and the other patient had postoperative irradiation to control the disease. Seven patients had local recurrence 8 to 21 months after treatment. Four patients developed distant metastases, including one patient with a T2b tumor that was totally resected through modified facial translocation approach with ligation of internal carotid artery. Eight patients died of other causes; internal carotid artery blowout was the cause of death in four of these eight patients. CONCLUSIONS: In most cases of recurrence, T1 nasopharyngeal tumors can be resected totally by anteroposterior approaches; for T2 or larger tumors, postoperative irradiation is usually necessary. Otherwise, facial translocation offers a better chance to completely resect the tumors. Internal carotid artery is better ligated if patients have received greater than 70 Gy irradiation or if the artery must be exposed during the surgery. We suggest that EC-IC bypass be used to avoid the possible complications (or cerebral ischemic stroke) caused by ligation of internal carotid artery. The transmaxillary approach is favored in the management of nasopharyngeal tumor recurrence with nasal cavity extension, and midline mandibulotomy is more suitable for resection of posterior margin of nasopharyngeal tumor recurrence. Facial translocation offers the widest operative field and is the most versatile approach for radical resection of nasopharyngeal tumor recurrence, but the surgeon should be skilled in the management of the facial nerves to reduce morbidity.

Aberrant cervical carotid artery.

Aberrant cervical carotid artery is an uncommon anomaly. Because this anomaly can lie in close proximity to the midline of the posterior part of the pharynx, it poses a significant risk during both major pharyngeal tumor resection and less extensive procedures such as tonsillectomy, adenoidectomy and palatopharyngoplasty. Five cases of aberrant cervical carotid artery were encountered and diagnosed using computerized tomography. In all five cases, the anomalous finding did not correlate with the presenting symptoms. Computerized tomographic images of these cases are provided. A review of the literature and the embryology of the aberrant carotid artery are presented. Awareness of the anomaly by radiologists and surgeons is essential to avoid accidental injury to the vessel during surgery.

Cavernous sinus gas.

Gas within the cavernous sinus is an unusual finding. We report three patients who demonstrated gas in the cavernous sinus on computerized tomography (CT). The clinical information of these patients was reviewed for the possible source of the gas and the symptoms induced by the gas. Cavernous sinus gas was seen in two patients with sphenoid sinus fracture and in one patient after intravenous fluid infusion. None of the patients had symptoms referable to the cavernous sinus gas, but one patient had a grave prognosis due to trauma. Identification of cavernous sinus gas on CT and correlation with the clinical information is mandatory for further management.

Endovascular embolization of intractable epistaxis.

BACKGROUND: We undertook this study to define the role of angiography and endovascular embolization in the treatment of patients with intractable epistaxis. METHODS: A series of 19 patients with massive intractable epistaxis, all treated with endovascular embolization were reviewed. There were 15 males and four females ranging in age from 18 to 70 years, with a mean age of 38 years. The predisposing factors of intractable epistaxis were head and neck tumors (n = 11), idiopathic symptoms (n = 3), surgical complications (n = 2), arteriovenous malformation of the face (n = 1), thrombocytopenia (n = 1) and trauma (n = 1). Polyvinyl alcohol (PVA) particles and gelfoam plugs were used as embolic agents in 11 patients for devascularization; detachable balloons with N-butyl-2-cyanoacrylate (NBCA) were used in four patients for vascular occlusion; NBCA and/or coils were used in four patients in the carotid and subclavian arteries for obliteration of pseudoaneurysms. RESULTS: Complete cessation of epistaxis was achieved in all 19 patients immediately after embolization. Seven patients had also undergone surgical tumor removal at two to five days after embolization. Two patients experienced mild to moderate facial pain on the first day after the procedure, but the symptoms later subsided. No significant complication or recurrence was observed in 18 patients. One patient with advanced hypopharyngeal carcinoma died two weeks after embolization due to another episode of massive epistaxis. Clinical follow-up for these patients was 15 days to eight years, with a mean of 41 months. CONCLUSIONS: Therapeutic endovascular embolization of intractable epistaxis is both efficient and safe. It should be considered as the primary treatment modality in intractable epistaxis.

Imaging of invasiveness of pituitary adenomas.

The purpose of our study was to examine the tumor size, imaging invasiveness of the pituitary macroadenomas (PMA) and to evaluate the directions of PMA spread. One hundred and thirty-five patients with PMA were examined with MRI and/or CT for pre-operative evaluation. We retrospectively reviewed the CT and MRI to identify tumor size, extension and to evaluate the directions of tumor spread. One hundred and seventeen patients (87%) had suprasellar extension with compression of optic apparatuses, twelve patients (9%) had extension of tumor upward to hypothalamus and third ventricle. Infrasellar extension via the floor of the sella and sphenoid sinus was found in thirty-eight patients (28%), and further downward extension to ethmoid sinus, nasopharynx and/or skull base was depicted in five patients (4%). Twenty-two patients (16%) had lateral invasion to the cavernous sinus and associated cranial nerves. Temporal and frontal extensions were depicted in seven patients (5%) and six patients (4%), respectively. Five patients (4%) had posterior subtentorial extension to posterior fossa. Histologically, only two patients showed microscopic invasive features. There was no correlation between histologic features and imaging invasiveness. The PMA had the potential of multi-directional extension. This experience indicated any type of pituitary adenoma could invade surrounding structures. Suprasellar invasion was the most common direction of pituitary adenoma spread, followed by infrasellar, lateral, anterior and posterior routes.

Fat in the cerebral falx.

BACKGROUND: Fat within the cerebral falx is commonly encountered in our daily practice but has never been characterized and reported. The purpose of this study was to determine the incidence of fat within the cerebral falx and to identify its clinical features. METHODS: The cerebral falx was prospectively imaged in 1,570 consecutive brain computed tomographic scans and its features characterized. Cerebral magnetic resonance imaging was done in some patients. RESULTS: Of the 1,570 cases studied, 115 revealed a small amount of fat in the cerebral falx. This fat had a purely negative Hounsfield value. It produced a high signal on both T1- and T2-weighted magnetic resonance images. There was no calcified component. The fat was seen most commonly in aged patients. No related symptoms were noted. CONCLUSIONS: Among the patients studied, fat in the cerebral falx had an incidence of 7.3%. It is an incidental finding in adults and requires no further evaluation or treatment.

Computed tomography and magnetic resonance imaging characteristics of giant pituitary adenomas.

PURPOSE: The purpose of this study was to characterize the computed tomography (CT) and magnetic resonance (MR) imaging features of giant pituitary adenoma (GPA) and to demonstrate the pathways of the spread of GPA. METHODS: CT and/or MR imaging scans of 356 patients evaluated preoperatively for single pituitary tumor were reviewed. Fourteen tumors (4%) fulfilled the radiologic criteria for GPA. There were 10 male and four female patients, ranging in age from 22 to 71 years (mean, 52 yr). We retrospectively reviewed the CT and MR imaging scans of these patients to characterize tumor appearances and identify the pathways of tumor extension. RESULTS: Thirteen tumors (93%) extended upward to the suprasellar cistern, and/or hypothalamus and third ventricle. Infrasellar extension through the sellar floor and sphenoid sinus to the skull base, or to the ethmoid sinus or the nasopharynx, was identified in seven patients (50%). Eight patients (57%) had lateral invasion to the cavernous sinus. Temporal and frontal extension was apparent in seven patients (50%) and six patients (43%), respectively. Five patients (36%) had posterior subtentorial extension to the posterior fossa. Histologically, only two GPAs showed invasive features. There was no correlation among histologic features, pituitary hormone concentrations, and evidence of tumor aggressiveness on CT and MRI scans. CONCLUSIONS: GPA has the potential for widespread, multi-directional extension. Our results indicate that any type of pituitary adenoma, regardless of its endocrinologic activity, may invade surrounding structures. Suprasellar invasion is the most common pathway of tumor spread, followed by infrasellar, lateral, anterior, and posterior routes.

Giant cell reparative granuloma: a case report.

Giant cell reparative granuloma (GCRG) is an infrequent benign lesion with undetermined etiopathogenesis affecting the maxillary and mandibular bone and, rarely, the skull. It is also extremely rare in the sphenoid bone. GCRG is usually diagnosed by histologic examination of bone lesions. We report a case of GCRG originating from the sphenoid bone. Computerized tomography revealed an expansile lesion with thinning or destruction of the cortical bone. The lesion itself was slightly hyperdense with good but inhomogeneous contrast enhancement. Reported magnetic resonance image findings showed hyperintensity on both T1-weighted and T2-weighted images and variable contrast enhancement. Plain skull radiographs usually reveal a lytic lesion within the bone.