A Case of Dyschondroplasia Associated with Brain Stem Glioma: Diagnosis by Stereotactic Biopsy

Neurosurgery ◽  
1990 ◽  
Vol 27 (3) ◽  
pp. 487-491 ◽  
Author(s):  
Stephan Patt ◽  
Klaus Weigel ◽  
Heinz Michael Mayer
Keyword(s):  
Brain Stem ◽  
Malignant Degeneration ◽  
Low Grade ◽  
Brain Stem Glioma ◽  
Tumor Biopsy ◽  
Maffucci’S Syndrome ◽  
Right Lobe ◽  
Ollier’S Disease ◽  
The Right ◽  
Ollier's Disease

Abstract We present a 24-year-old patient with multiple chondromas of both hands, the pelvis, the left leg, and an associated brain stem glioma. There was no evidence of hemangioma or dyschromia. and the condition was diagnosed as Ollier's disease, a special type of dyschondroplasia like Maffucci's syndrome and Kast's disease. An increased overall risk for development of malignant skeletal and nonskeletal tumors is associated with Maffucci's syndrome. The risk of malignant degeneration is lower in Ollier's disease. A glioma in the pons and the right lobe of the cerebellum was found in this patient. The literature describes an association with gliomas in only 12 cases of dyschondroplasia and an infratentorial localization in just one case. Signs of malignancy were histologically confirmed in 7 cases without significant preponderance of any one type. Our patient had a low-grade brain stem astrocytoma with fibrillar and gemistocytic components. A stereotactic serial biopsy made it possible to rule out malignant degeneration. Stereotactic brain tumor biopsy as a routine neurosurgical procedure is particularly valuable for deep space-occupying processes and forms the basis for therapy. In the present case, irradiation was not recommended.

scholarly journals Immunohistochemical detection of procalcitonin in fibrolamellar hepatocellular carcinoma

2021 ◽  
Author(s):  
Kotaro Matsumoto ◽  
Kentaro Kikuchi ◽  
Ayako Hara ◽  
Hiromichi Tsunashima ◽  
Koichi Tsuneyama ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Histopathological Examination ◽  
Epigastric Pain ◽  
Positive Staining ◽  
C Reactive Protein ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Tumor Biopsy ◽  
Reactive Protein ◽  
Right Lobe ◽  
The Right

AbstractA 25-year-old woman with fever and epigastric pain was referred to our hospital. Blood examination showed significant liver dysfunction, markedly high C-reactive protein (CRP 19.1 mg/dL) and procalcitonin (48.3 ng/mL) levels. Dynamic computed tomography showed a tumor approximately 120 mm in size in the right lobe of the liver, but with no abscess formation. The patient was hospitalized and started on antibiotics; her CRP level improved, but the procalcitonin level did not decrease. Histopathological examination of the liver tumor biopsy revealed fibrolamellar hepatocellular carcinoma (FLC). Positive staining of the FLC with an anti-procalcitonin antibody suggested the production of procalcitonin.

Focal brain-stem astrocytomas causing symptoms of involvement of the facial nerve nucleus: long-term survival in six pediatric cases

1994 ◽  
Vol 80 (1) ◽  
pp. 20-25 ◽  
Author(s):  
Michael S. B. Edwards ◽  
William M. Wara ◽  
Samuel F. Ciricillo ◽  
A. James Barkovich
Keyword(s):  
Radiation Therapy ◽  
Facial Nerve ◽  
Brain Stem ◽  
Pilocytic Astrocytoma ◽  
Low Grade ◽  
Brain Stem Glioma ◽  
Term Survival ◽  
Juvenile Pilocytic Astrocytoma ◽  
Nerve Nucleus

✓ Six children with a history of isolated facial nerve dysfunction or dizziness and nausea were treated for brain-stem glioma between 1984 and 1992. Computerized tomography and/or magnetic resonance (MR) imaging showed a focal, uniformly enhancing mass involving the facial nerve nucleus of the pons. All patients underwent biopsy; the histological diagnosis was juvenile pilocytic astrocytoma in five cases. In the remaining case the biopsy was nondiagnostic, although the surgeon believed that the lesion was a glioma. Postoperatively, five patients underwent conventional focal megavoltage radiation therapy (180 to 200 cGy/day) over a period of 5½ weeks to a total dose of approximately 5400 cGy. One child's family refused radiation therapy; she remained well and stable for 4 years, despite persistent facial weakness, and was eventually lost to follow-up review. Four irradiation-treated patients had complete resolution of their tumors on MR images and have had no evidence of neuropsychological or neuroendocrinological deficits during 4½ to 8 years of follow-up evaluation. Patients whose neuroradiological studies show a lesion resembling those in this series should undergo biopsy and, if the histology of a low-grade tumor (in particular, a juvenile pilocytic astrocytoma) is confirmed, should then receive focal radiation therapy with conventional megavoltage dosages.

scholarly journals Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome Three Case Reports and Review of the Literature

2004 ◽  
Vol 43 (8) ◽  
pp. 705-710 ◽  
Author(s):  
Georges Noël ◽  
Loïc Feuvret ◽  
Valentin Calugaru ◽  
Khalid Hadadi ◽  
François Baillet ◽  
...  
Keyword(s):  
Case Reports ◽  
Review Of The Literature ◽  
Maffucci’S Syndrome ◽  
Ollier’S Disease ◽  
Base Of The Skull ◽  
Ollier's Disease

scholarly journals Knee chondrosarcoma secondary to Ollier’s disease: a report of one case with twelve-year follow-up

2012 ◽  
Vol 4 (2) ◽  
pp. 16
Author(s):  
Philippe Chiron ◽  
Jérôme Murgier
Keyword(s):  
Conservative Treatment ◽  
Knee Prosthesis ◽  
Low Grade ◽  
Total Knee Prosthesis ◽  
Total Knee ◽  
Ollier’S Disease ◽  
Ollier's Disease

Management of low-grade chondrosarcomas secondary to Ollier’s disease is generally radical. We report here a case of conservative treatment of a knee tumor with reconstruction of the lower femoral extremity with a hinged total knee prosthesis, cemented and coupled with a massive femoral allograft, with a twelve-year follow-up.

Benign Brain Stem Lesions in Pediatric Patients with Neurofibromatosis: Case Reports

Neurosurgery ◽  
1989 ◽  
Vol 25 (6) ◽  
pp. 959-964 ◽  
Author(s):  
Corey Raffel ◽  
Gordon J. McComb ◽  
Sara Bodner ◽  
Floyd E. Gilles
Keyword(s):  
Brain Stem ◽  
Pediatric Patients ◽  
Tumor Imaging ◽  
Case Reports ◽  
Low Grade ◽  
Brain Stem Glioma ◽  
Computed Tomographic ◽  
Brain Stem Lesions ◽  
Stem Lesions ◽  
The Brain

Abstract The symptoms and clinical courses of 4 patients with neurofibromatosis and lesions of the brain stem identifiable on computed tomographic and/or magnetic resonance imaging scans are described. Two patients underwent biopsy and both had low-grade astrocytomas with no evidence of anaplasia. Both received radiation and chemotherapy. The other 2 patients have been monitored without biopsy or treatment. Three patients are alive and clinically stable, having been followed up for an average of 4 years; neuroimaging studies have shown no change in their tumors. The fourth patient died of a supratentorial primitive neuroectodermal tumor. Imaging studies had shown no change in his brain stem lesion, which at autopsy was found to be a focal collection of fibrillary astrocytes. These data suggest that some patients with brain stem lesions and neurofibromatosis may have a prognosis distinctly different from that of the typical patient with a brain stem glioma. We recommend caution against aggressive operative and adjuvant therapy for brain stem lesions in patients with neurofibromatosis, unless progression of the lesion is documented clinically and/or by imaging.

scholarly journals Lengthening the Lower Extremities of Children with Ollier’s and Maffucci’s Enchondromatosis Using Implantable Lengthening Nails

Children ◽  
2021 ◽  
Vol 8 (6) ◽  
pp. 502
Author(s):  
Aaron J. Huser ◽  
Jason Shih Hoellwarth ◽  
Valentino Coppa ◽  
David S. Feldman ◽  
Dror Paley
Keyword(s):  
Case Series ◽  
Limb Lengthening ◽  
Limb Length Discrepancy ◽  
Knee Extension ◽  
Limb Length ◽  
External Fixators ◽  
Manipulation Under Anesthesia ◽  
Maffucci’S Syndrome ◽  
Ollier’S Disease ◽  
Ollier's Disease

There are multiple forms of enchondromatosis with Ollier’s and Maffucci’s being the most prevalent types. Limb length discrepancy is a common problem in patients with Ollier’s and Maffucci’s enchondromatosis. There are multiple reports about lengthening bones in patients with enchondromatosis using external fixators. However, there are no case series regarding the use of implantable lengthening technology. The purpose of this paper is to describe our experience with implantable nail lengthening in patients with enchondromatosis. A retrospective chart and radiographic review of patients with enchondromatosis who underwent implantable nail limb lengthening was performed. Seven patients with 14 bony segments were reviewed. A total of 11/14 lengthenings were completed without difficulty. There were no issues in terms of fixation location in patients with Ollier’s disease. One patient with Maffucci’s syndrome experienced migration of the nail during two lengthenings due to a combination of intralesional fixation and preconsolidation. One patient with Ollier’s disease developed a knee extension contracture requiring manipulation under anesthesia. No other complications were recorded. The use of implantable nail lengthening to resolve limb length discrepancies in patients with Ollier’s disease appears to be safe and effective.

Diffuse brain stem tumor in an adolescent with multiple enchondromatosis (Ollier's disease)

1999 ◽  
Vol 15 (5) ◽  
pp. 222-225 ◽  
Author(s):  
D. Frappaz ◽  
A. C. Ricci ◽  
R. Kohler ◽  
P. Bret ◽  
C. Mottolese
Keyword(s):  
Brain Stem ◽  
Brain Stem Tumor ◽  
Multiple Enchondromatosis ◽  
Ollier’S Disease ◽  
Ollier's Disease ◽  
Diffuse Brain

Maffucci's Syndrome with Bilateral Cartilaginous Tumors of the Cerebellopontine Angle

Neurosurgery ◽  
1990 ◽  
Vol 27 (4) ◽  
pp. 625-628 ◽  
Author(s):  
Karl-August Bushe ◽  
Markus Naumann ◽  
Monika Warmuth-Metz ◽  
Jürgen Meixensberger ◽  
Justus Müller
Keyword(s):  
Cerebellopontine Angle ◽  
Subcutaneous Tissue ◽  
Radiological Diagnosis ◽  
Whole Body ◽  
Intracranial Involvement ◽  
Maffucci’S Syndrome ◽  
Successful Removal ◽  
Ollier’S Disease ◽  
Cartilaginous Tumors ◽  
Ollier's Disease

Abstract Maffucci's syndrome is characterized by the combination of multiple enchondromas (Ollier's disease) and hemangiomatosis. These hemangiomas develop in the subcutaneous tissue and form red-blue tumors dispersed over the whole body. Intracranial involvement is rare, making a preoperative radiological diagnosis and differentiation from other tumors rather difficult. The radiological characteristics and successful removal of the intracranial part of a chondrosarcoma of the cerebellopontine angle in a case of Maffucci's syndrome are reported in this paper.

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