Khang-Loon Ho

Primary meningioma of the nasal cavity and paranasal sinuses

A case of primary meningioma of the nasal cavity and ethmoidal sinus of a 65‐year‐old man is described. The clinical and pathologic characteristics of the 18 cases previously reported in the literature are reviewed. Although the histological features and biological behavior of the tumors were found to be similar to that of intracranial meningioma, primary meningiomas of the nasal cavity and paranasal sinuses were found more often to involve male patients and young individuals. Meningiomas should be considered in the differential diagnosis of any lesion in a young patient with progressive exophthalmos, nasal obstruction, or episodes of epistaxis. The pathogenesis of extracranial meningiomas is briefly discussed.

Concurrence of multiple sclerosis and primary intracranial neoplasms

The concurrence of multiple sclerosis and primary intracranial neoplasms is uncommon. The literature contains 20 cases of multiple sclerosis associated with gliomas and nine cases with non‐gliomatous neoplasms. This report describes a protoplasmic astrocytoma and arteriovenous malformation found incidentally at autopsy of a 63‐year‐old woman with a 25‐year history of multiple sclerosis. This is the first demonstrated association of multiple sclerosis with an arteriovenous malformation. Although neoplastic transformation of reactive glial cells in multiple sclerosis plaques has been proposed as the source of gliomas, the association of multiple sclerosis with astrocytoma and arteriovenous malformation in the present case is considered to be coincidental. Gliomas reported in association with multiple sclerosis are more frequently (30%) multicentric than are uncomplicated gliomas.

Endodermal sinus tumor of the pineal region. Case report and review of literature

This is a case report of an endodermal sinus tumor occurring in the pineal region of a 14‐year‐old boy who died 4 weeks after surgery and irradiation. Germ cell tumors of this type demonstrate a selective overgrowth of yolk sac endoderm associated with extraembryonic mesoblast and arise preferentially in the gonads of young children. This tumor in the pineal region is rare and carries a grave prognosis as in other extragonadal sites. A summary of previously reported cases is also presented.

Bilateral renal malakoplakia

A case of malakoplakia is presented which was confined to the renal parenchyma of both kidneys in a thirty-five-year-old woman in whom fatal renal failure developed in three weeks. Bilateral renal malakoplakia is rare, and unlike malakoplakia of the bladder, it behaves as a progressive, destructive, and fatal disease.

Sarcoma metastatic to the central nervous system: report of three cases and review of the literature.

Sarcoma metastatic to the central nervous system is generally considered to be rare. In this report three cases of sarcoma with metastasis to the central nervous system are presented. The case of rhabdomyosarcoma of the lung probably represents the first reported instance of such tumor metastatic to the central nervous system. The case of fibrosarcoma of the thigh is thought to be the first report of metastatic sarcoma with leptomeningeal dissemination. The metastatic brain lesions of the alveolar soft part sarcoma were incidental findings at autopsy. A summary of 22 cases previously reported and a brief discussion of the mode of metastasis of sarcoma, as compared to that of carcinoma, are also presented.

Tumors of the cerebral aqueduct

Two cases of tumor of the cerebral aqueduct are described: Case 1 is a pilocytic astrocytoma in a 16‐year‐old girl with a two‐year history of intermittent increase of intracranial pressure. The tumor was completely confined within the lumen of the aqueduct. Case 2 is a subependymoma of a 68‐year‐old man. The tumor extended beyond the aqueduct to the periaqueductal gray matter and produced signs and symptoms suggesting normal pressure hydrocephalus. The literature contains 18 other cases of tumor of the aqueduct: 13 gliomas and five vascular malformations. All, except one, produced clinical manifestations of generalized hydrocephalus lasting from 20 days to six years. The result generally did not correspond to the histologic type of the tumor. Like gliomas of the brainstem in general, those in the aqueduct tend to occur in childhood and adolescence and affect male more than female patients.

Fatal spinal cord infarction caused by fibrocartilaginous embolization of the anterior spinal artery

A fatal case of massive infarction of the cervical cord caused by fibrocartilaginous embolization of the anterior spinal artery in a 22 year old woman is reported. The source of such emboli is believed to be intervertebral disc material, but the pathogenesis of this entity is obscure. The previously reported 10 cases are reviewed and the clinical and pathological features summarized, showing that this unusual condition seems to affect females predominantly. The cervical cord is the most common site of involvement. The importance of multiple sections from various levels of the spinal cord and the application of special stains in order to determine the cause of spinal cord infarction are emphasized.

The role of alkaline protease in the development of cardiac lesions in myopathic hamsters: Effect of verapamil treatment☆

The role of alkaline protease in the development of myocardial lesions in myopathic hamsters was studied. There was abnormal elevation of alkaline protease in the myopathic heart at 1 month of age, preceding the development of cardiac lesions. In vivo treatments of verapamil were carried out in 1-month-old myopathic animals for 30 days. Results indicated that the drug treatment was effective in preventing the occurrence of the severe myocardial lesions found in the untreated animals at 2 months of age. Reduced lesion development was associated concomitantly with lowered levels of alkaline protease activity. Withdrawal of drug treatment caused the return of severe lesions and high levels of alkaline protease. The study of the alkaline protease activity in the skeletal muscle of the myopathic hamster also showed a parallel relationship between the enzyme levels and severity of lesions. The results are discussed in light of possible involvement of a serine protease and a Ca2+-activated protease in producing the cardiac lesions.

Sarcoidosis of urethra simulating carcinoma.

Sarcoidosis of the urethra was found during endometrial curettage in a thirty-nine-year-old woman with a history of menometrorrhagia and decreased urine stream. Sarcoidosis is known to occur in many parts of the body, but an extensive search of the literature has failed to reveal any previous report of urethral involvement.

Primary craniofacial chordoma: case report.

A 37-year-old man presented with right facial pain and a nonpalpable mass over the malar eminence. An incisional biopsy via the intraoral route was performed and interpreted as a vascular malformation with degenerative changes. His symptoms persisted, and a repeat biopsy was suggestive of an epithelioid nerve sheath tumor. Total resection of the tumor was planned to include the infraorbital and malar regions, the infratemporal fossa, and the pterygopalatine fossa. At surgery, the tumor was removed with tumor-free margins obtained along the course of the maxillary nerve just before its entrance into the cavernous sinus. The pathological findings and the immunohistochemistry demonstrated a typical chordoma with no chondroid or sarcomatous dedifferentiation. We think that with greater use of immunohistochemical markers and electron microscopy, patients with chordoma in this location may be diagnosed promptly and accurately.