Rosai-Dorfman disease presenting as a meningioma

This report documents an unusal case of extranodal sinus histiocytosis (Rosai-Dorfman disease) which produced a solitary intracranial mass lesion that was diagnosed clinically and radiologically as a meningioma. Morphological features have been described and literature extensively reviewed.     

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): case report with nodal and diffuse muco-cutaneous involvement

PURPOSE: Based on a review of the background of MRI using inhaled hyperpolarized noble gases first experiences and perspectives for functional imaging in emphysema patients are presented. MATERIAL AND METHODS: In vonventional MRI, the spin density of protons, which is defined by the Boltzmann equilibrium, is the source of the imaging signal. Since proton density in the lungs is low and multiple air-tissue interfaces exist, MRI of the lung parenchyma is unsatisfactory. The possibility to artificially enrich the spin density (hyperpolarization) in noble gases (H3-3, Xe-129) by optical pumping results in a non-equilibrium polarization five orders of magnitude higher than the Boltzmann equilibrium. Hyperpolarized noble gases can then be applied as "inhaled contrast media" using a dedicated application device. At the MR unit several prerequisites must also be fulfilled: transmit-receive coil, boradband amplifier and fast sequences with low flip angles. These are essential for dynamic scans in breath-hold tecnique of the highly diffusible He-3 or the well soluble Xe-129. RESULTS: He-3 and Xe-129 have been successfully applied for imaging of the ventilated airspaces. Besides the well-known narcotic effects of Xenon no adverse effects were observed. A homogeneous distribution of signal intensity can be regarded as a normal findings in people without lung disease. Obstructive diseases and emphysematous changes lead to generalized or localized signal inhomogeneities. Most likely they are caused by disorders of the distribution of ventilation bases on a different functional vehavior of different alveolar regions. By making use of the paramagenetic properties of oxygen, He-3 can also be used for local measurements of oxygen partial pressure in the lung. Xe-129 exhibits a different chemical shift within alveoli, interstitial space and vessels which can be measured by MRI. CONCLUSIONS: MRI using inhaled hyperpolarized noble gases is a functional imaging modality with high spatial and/or temporal resolution. First studies for early detection of obstructive lung diseases and disorders of distribution of ventilation in emphysema are promising.     

Metastatic dermatofibrosarcoma protuberans mimicking meningioma

Relationships between body mass index (BMI) and weight gain with perinatal outcome and birthweight were examined. BMI was calculated on 582 consecutive pregnant women who delivered at or >37 weeks gestational age. Statistical analysis was done using Chi-square tests, analysis of variance, and multiple logistic regression. Of those studied, 13% were underweight, 39% normal, 13% overweight, and 35% obese. Obesity was associated with increasing age (P < .01), multiparity (P < .01), previous cesarean delivery (P < .01), previous macrosomia (P = .01), previous fetal death (P = .03), hypertensive disorders (P < .01), gestational diabetes (P = .02), cesarean delivery (P = .03), and neonatal intensive care unit admission (NICU) (P = .01). The underweight group had the most low birthweight (LBW) infants and the lowest mean birthweight. Ideal weight gain occurred in 31%, inadequate weight gain in 34%, and excessive weight gain in 35%. Inadequate weight gain had increased asthma (P < .05), and hyperemesis (P = .03). Women with ideal weight gain had less smokers (P < .01), fetal distress (P < .05), cesarean delivery (P = .02), and preeclampsia (P < .001). The mean birthweight was highest in the excessive weight gain (P < .01). With multivariate analysis, previous LBW, BMI, and tobacco use were significant predictors of LBW. Normal BMI and ideal weight gain in pregnancy is associated with decreased perinatal complications and an optimum birthweight.     

Intrasellar meningioma: case report and review of the literature

BACKGROUND: Intrasellar meningioma is a rare clinical entity, and surgical resection may be difficult when it is hypervascularized. METHODS: A case of subdiaphragmatic hypervascular intrasellar meningioma with attachment to the dura of the anterior wall of the sella turcica is described. Literature review of 18 cases with operatively confirmed intrasellar meningioma discloses unexpected intraoperative bleeding and relatively low resectability of the tumor are also described. RESULTS: We used preoperative endovascular embolization of feeding arteries, and resected a hypervascular intrasellar meningioma by a combined transsphenoidal-transcranial approach safely and without massive bleeding. CONCLUSIONS: Preoperative endovascular embolization of feeding arteries and combined transsphenoidal-transcranial approach are useful for the surgical resection of hypervascular intrasellar meningiomas.     

成人复发性特巨大型脑膜瘤的临床分析(附1例报告)

The authors reported the case of a 35-year-old woman with an unusual huge recurrent meningioma in the left cerebral hemisphere confirmed by the pathological examination. This patient's clinical presentation was very unusual. Brain magnetic resonance imaging (MRI) demonstrated a huge tumor in the left frontal region. The extracranial surface dimensions of the mass were 12 cm craniocaudal × 11 cm transverse × 9 cm anteroposterior; and the intracranial dimensions were 9 cm craniocaudal × 7 cm transverse × 10 cm anteroposterior respectively. The patient underwent left frontal recurrent meningioma resection by microsurgery, with total tumor resection. Following which the patient was discharged in a stable condition. To the best of the authors' knowledge, such an unusual huge recurrent meningioma has not been reported. The authors reviewed the literatures with reference to recurrent meningioma, analyzed the unusual imaging appearances of this patient, and explored the mechanism underlying the huge volume formation. The fundamental principle to reducing or completely avoiding meningioma recurrence is to achieve total tumour resection. Recurrent meningiomas are also amenable to surgery.     

Trypanosoma cruzi meningoencephalitis in AIDS mimicking cerebral metastases: case report

BACKGROUND: It has been suggested that citrate salts might enhance aluminum (Al) absorption from a normal diet, posing a threat of Al toxicity even in subjects with normal renal function. We have recently reported that in normal subjects and patients with moderate renal failure, short-term treatment with tricalcium dicitrate (Ca3Cit2) does not significantly change urinary and serum Al levels. However, we have not assessed total body Al stores in patients on long-term citrate treatment. OBJECTIVE: The objective of this study was to ascertain body content of Al non-invasively using the increment in serum and urinary Al following the intravenous administration of deferoxamine (DFO) in patients with kidney stones and osteoporotic women undergoing long-term treatment with potassium citrate (K3Cit) or Ca3Cit2, respectively. METHODS: Ten patients with calcium nephrolithiasis and five with osteoporosis who were maintained on potassium citrate (40 mEq/day or more) or calcium citrate 800 mg calcium/day (40 mEq citrate) for 2 to 8 years, respectively, and 16 normal volunteers without a history of regular aluminum-containing antacid use participated in the study. All participants completed the 8 days of study, during which they were maintained on their regular home diet. Urinary Al excretion was measured during a two-day baseline before (Days 5, 6) and for 1 day (Day 7) immediately following a single intravenous dose of DFO (40 mg/kg). Blood for Al was obtained before DFO administration, and at 2, 5 and 24 hours following the start of the infusion. RESULTS: The median 24-hour urinary Al excretion (microgram/day) at baseline versus post-DFO value was 15.9 vs. 44.4 in the normal subjects and 13.3 vs. 35.7 in the patients. These values were all within normal limits and did not change significantly following DFO infusion (p = 0.003 and p = 0.0001, respectively). The median change of 17.1 micrograms/day in urinary Al in the normal subjects was not significantly different from the 18.7 micrograms/day change measured in the patient group (p = 0.30). Similarly, no change in the mean serum Al was detected at any time following the DFO infusion, either in the patient or control group (patients 4.1 to 4.3 ng/ml, controls 7.4 to 4.6 ng/ml). CONCLUSION: The results suggest that abnormal total body retention of Al does not occur during long-term citrate treatment in patients with functioning kidneys.     

Pelizaeus-Merzbacher-like disease: female case report

We experienced a 15-year-old female, whose healthy parents were second cousins, who was suspected of having dysmyelinating disease involving only the central nervous system (CNS). She was noticed to have congenital pendula nystagmus, and spastic gait disturbance developed at the age of 10 years. Mild athetosis of the upper limbs and ataxia were recognized at age 13 years, and dysarthria presented at age 15. MRI and electrophysiological findings showed the characteristics of Pelizaeus-Merzbacher disease (PMD), although the extensive nerve conduction slowing of the CNS was less severe than that in male patients with PMD. No promoter or exonic mutations of proteolipid protein (PLP) gene were detected. Although this patient might be heterozygous for a mutation of the extraexonic PLP gene sequences or of other unknown X-linked PLP associated genes, we speculate that this case had a dysmyelinating disease with an autosomal recessive trait characterized by the same phenotype as that of PMD.     

Traumatic intracranial aneurysms after removal of tuberculum sellae meningioma: case report]

Two cases of traumatic aneurysms (TA) of the internal carotid artery (IC) due to removal of tuberculum sellae meningioma (TSM) are presented, and ideal treatment of those aneurysms is discussed. The tumor, compressing the IC laterally, was removed out without arterial injury in case 1. Though the patient's postoperative course was uneventful, frontal and intraventricular hemorrhage developed 2 months after the operation, and an IC dorsal aneurysm was detected. Preoperative angiograms showed no aneurysm, so it was considered to be a traumatic aneurysm due to the surgical procedure. The aneurysm was clipped at once, but postoperative angiograms showed recurrence of the aneurysm. IC balloon occlusion was carried out as the Matas test was negative. In case 2, a small tear in the IC was inadvertently made during recurrent TSM removal, which was wrapped with muscle using fibrin glue. 2 weeks after the operation, frontal hemorrhage developed. Angiograms revealed a small aneurysm of the IC, which was considered to be a traumatic aneurysm. IC balloon occlusion was performed as the Matas test was negative. These 2 patients have had no episodes of rerupture after the IC balloon occlusion. Tumors and main arteries are frequently adhesive, so arteries are easily injured during removal of meningiomas. Muscle wrapping was not enough to prevent TA formation. Neck clipping was not appropriate for treatment of TA, but IC balloon occlusion was an effective and excellent therapy for TA of IC.     

An extensive cranial base meningioma extending bilaterally into Meckel's cave: case report

OBJECTIVE AND IMPORTANCE: A patient with an extensive cranial base meningioma that included bilateral invasion of Meckel's cave underwent surgical resection and had an unexpected rare complication, malocclusion from bilateral trigeminal dysfunction. CLINICAL PRESENTATION: A 19-year-old male patient was admitted to our hospital with alternating painful ophthalmoplegia. He had been blind since the age of 10 years. At the time of admission, neurological findings included bilateral visual loss and optic atrophy. Magnetic resonance images showed an extensive tumor located at the planum sphenoidale, tuberculum sellae, and bilaterally at Meckel's cave and the medial tentorial incisura. INTERVENTION: The patient underwent a two-stage operation. During the first procedure, the masses in the planum sphenoidale, tuberculum sellae, and the left side of Meckel's cave were excised intradurally. At the second operation, the mass in the right side of Meckel's cave was excised extradurally and the tentorial mass was removed intradurally. The patient's postoperative course was complicated by bilateral trigeminal nerve dysfunction, which caused malocclusion. CONCLUSION: Bilateral dysfunction of the trigeminal nerve may cause a number of problems. Thus, extreme caution must be taken to preserve the function of this nerve.     

Signet-ring cell melanoma mimicking adenocarcinoma. A case report

The fine needle aspiration findings in a lymph node involved by signet-ring cell melanoma, a very rare variant of malignant melanoma, are reported. The patient had a history of superficial spreading melanoma of the right foot, treated 10 years earlier by below-the-knee amputation. He presented with a right groin mass. Fine needle aspiration of the mass yielded poorly cohesive, large cells with eccentric nuclei and abundant, eosinophilic cytoplasm; many of them exhibited a signet-ring appearance. Melanin pigments were identified in a small proportion of tumor cells, and a diagnosis of metastatic melanoma was made. The subsequent lymph node excision revealed a metastatic tumor composed of polygonal and signet-ring cells that were positive for S-100 protein and HMB-45 but not cytokeratin. Nearly all reported cases of signet-ring cell melanoma occurred as metastatic or recurrent disease. It is important not to mistake signet-ring cell melanoma for adenocarcinoma in aspiration cytology, and a constellation of clinical features and of histochemical and immunohistochemical findings enables a correct diagnosis to be reached.     

Gallium imaging in pulmonary artery sarcoma mimicking pulmonary embolism: case report

Primary pulmonary artery sarcoma provides perfusion-ventilation images, as well as arteriographic studies, that can suggest pulmonary embolism. The awareness of atypical correlation among the studies for pulmonary embolism can lead to an early suspicion of pulmonary artery tumor. Imaging with 67Ga-citrate may facilitate earlier diagnosis.     

Pontine-crossed paralysis consequent to multiple trauma: case report

The case of a multiple trauma victim with a brain-stem injury, resulting in a syndrome of pontine-crossed paralysis, is presented. Demonstration of these disorders in the setting of multiple trauma is highly unusual, but the diagnosis is established quickly and accurately through magnetic resonance imaging. A possible mechanism of injury is presented for discussion.     

Cytology of primary pulmonary meningioma. Report of the first multiple case

BACKGROUND: Ectopic meningiomas arising in the lung are rare. We report here the first multiple primary case diagnosed by intraoperative imprint cytology. CASE: Asymptomatic pulmonary nodules, two in the left and three in the right lung, were found in a 61-year-old woman, and video-assisted thoracoscopic surgery was undertaken. Because the largest tumor was diagnosed as a meningioma by intraoperative imprint cytology using an excised biopsy specimen, further resection was not performed immediately. Histopathologically the tumor was characterized by whorled nests of cells accompanied by psammoma bodies intermingled with a fibrous pattern. The diagnosis was a transitional meningioma, positive for vimentin and epithelial membrane antigen and negative for keratin immunohistochemically. All the nodules were subsequently surgically resected and showed a similar cytohistologic appearance. Ultrastructurally the tumor cells demonstrated interdigitation of adjacent plasma membranes with numerous desmosomes and hemidesmosomes, typical of meningiomas. We failed to detect another primary tumor in the nervous system, and at this writing the patient was healthy three years after the operation. CONCLUSION: Because of the characteristic cytomorphologic features of primary pulmonary meningioma, the cytologic approach provides useful information for therapy.     

Primary meningioma intimately related to skull: case report and review of the literature

A report is made of a primary meningioma intimately associated with the left sphenoid ridge and temporal bone of a 41-year-old woman. The lesion produced hyperostosis of the involved bones, ipsilateral proptosis, and reduced vision for 4.5 years. The radiotherapy yielded little improvement of the exophthalmos and vision. Review of 125 reported meningiomas intimately related to the skull and presenting primarily with hyperostosis and minimal soft tissue component, including the current case, disclosed that all but one affected the cranium proper, particularly the middle ear, temporal bone, frontal bone, and parietal bone. The exceptional one was in the mandible. They tended to occur between the fifth and seventh decades of life with the peak occurrence between the fifth and sixth decades. The average age was 46 years for overall. The female-to-male ratio was 2:1. Six of 125 primary meningiomas closely associated with the skull were malignant and two of these spread to the lungs. The pulmonary metastasis was spontaneous in one case.     

Rosai-Dorfman disease involving the nasal septum

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a benign, self-limited pseudolymphomatous entity with characteristic histologic features. The disease may be atypically present in the absence of detectable nodal involvement. This is a case of nasal septum involvement of Rosai-Dorfman disease without clinically apparent lymphadenopathy.     

Benign retroperitoneal schwannoma mimicking a pancreatic cystic tumor: case report and literature review

A rare case of benign retroperitoneal schwannoma mimicking a pancreatic cystic tumor is reported herein. The tumor mass, based on a computed tomography scan and an abdominal echo examination, was initially suspected to be a mucinous cystadenoma or cystadenocarcinoma of the pancreas. However, the surgical and pathohistological findings made a final diagnosis of benign pancreatic schwannoma. The patient is doing well at a 2-year follow-up. The tumor, 18x17x15 cm in size, represented the largest among reported pancreatic schwannomas. Furthermore, a comprehensive review of reported cases of pancreatic schwannoma was carried out to summarize corresponding findings, including benign or malignant forms, adherence to other tissues, cystic change of the tumor, as well as relation to von Recklinghausen's neurofibromatosis.