Thomas T. Tang

Inflammatory myofibrohistiocytic proliferation simulating sarcoma in children

The term “inflammatory myofibrohistiocytic proliferation” (IMP) has been proposed to replace the conventional designations of plasma cell granuloma and inflammatory pseudotumor. Three cases of extrapulmonary IMP in children are reported, including an intracerebral lesion which has been formerly undescribed. In children, IMP may be associated with microcytic hypochromic anemia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. In this clinical setting, differentiation of a rapidly growing but benign IMP from a bona fide sarcoma is of paramount importance.

Chronic myopathy associated with coxsackievirus type A9. A combined electron microscopical and viral isolation study.

Virus-like crystalline structures in human skeletal muscles have been observed by many electron microspists, but no viruses have been isolated and identified in such cases. An 11-year-old girl who had had muscular weakness and physical retardation since early infancy died of pneumonia due to atrophy of diaphragmatic and intercostal muscles. Electron microscopy of these muscles revealed a heavy infiltration of picornavirus-like particles that measured 19 to 23 nm in diameter. Subsequent inoculation of primary human-amnion cells with a sonic-treated suspension of the patients diaphragmatic muscle induced an enterovirus-like cytopathic effect. The isolate was identified with use of Lim-Benyesh--Meinick enterovirus typing serum pools as coxsackievirus Type A-9. This viral isolation supports the belief that the organized electron-dense particles in human muscle are indeed virions.

Neonatal hypotonia and cardiomyopathy secondary to type IV glycogenosis

A neonate with deficiency of branching enzyme (glycogenosis type IV) presented symptoms of severe hypotonia pre- and postnatally, and dilated cardiomyopathy in early infancy. The classical clinical manifestation of liver cirrhosis was not present, although amylopectin-like inclusions were found in the hepatocytes. In contrast to a previous report, the neurons in the brain stem and spinal anterior horns contained PAS-positive, diastase-resistant deposits. The combined involvement of the muscles and motor neurones could account for the severity of hypotonia. The muscle biopsy, electromyogram and biochemical and enzyme assays were helpful in establishing the diagnosis.

Primary cerebral germinoma and ventriculoperitoneal shunt metastasis

A case of peritoneal metastasis via a ventriculoperitoneal shunt 14 months following biopsy and irradiation of an intracerebral germinoma is presented. Metastatic abdominal disease has been controlled at 32 months postmetastatic diagnosis by abdominal irradiation and systemic chemotherapy. The occurrence of ventriculoperitoneal shunt metastases and control of extracranial metastases from intracerebral germinomas are discussed.

Hepatic adenoma. Biochemical and electron microscopic study.

Five cases of benign tumors of the liver designated as hepatic adenomas were examined. In addition to light microscopy, tissues were available from 2 cases for electron and biochemical analyses. The gross appearance of these tumors was characterized by the central fibrous tissue core, with peripheral extensions producing nodulation. Light and electron microscopy revealed that these tumors were composed of normal‐appearing hepatic cells and bile ducts, lacking only the organization into hepatic lobules. Another characteristic feature was the proliferation of bile ducts in the centers of the tumor nodules. The most striking finding was the excessive accumulation of cytoplasmic glycogen in some of these tumors. However, biochemical analysis demonstrated normal values of the enzymes which are deficient in glycogen storage diseases. In addition, the activity of those enzymes decreased in experimental hepatomas was within normal limits in our material, thus indicating the benign nature of these lesions. Differentiation of regenerative hyperplasia, hamartoma, and adenoma of the liver is discussed.

Polydipsia, polyuria, and hypertension associated with renin-secreting Wilms tumor.

A 16-month-old black male infant had unusual thirst, polyuria, hyponatremia, and hypertension. His polyuria was unresponsive to vasopressin therapy, and his high blood pressure was not effectively controlled by antihypertensive drugs. Radiographic examinations revealed an occult Wilms tumor in the right kidney. After removal of the tumor, the signs and symptoms were relieved. The tumor had a renin activity about 280 times that of the adjacent renal cortex, and many intracytoplasmic secretory granules were found on electron microscopy. The pathogenesis of these clinical manifestations appears to be mediated through the physiologic pathways of renin-angiotensin II and renin-aldosterone.

Thyroid carcinoma following treatment for acute lymphoblastic leukemia.

A 21/2‐year‐old girl with acute lymphoblastic leukemia received chemotherapy and prophylactic cranial irradiation. After six years of remission, including three years off therapy, metastatic thyroid carcinoma appeared in the cervical lymph nodes. The predisposing factors for the development of thyroid carcinoma as a second malignancy in this case are discussed. It is suggested that thyroid carcinoma should be added to the growing list of second malignancies in acute lymphoblastic leukemia and that careful thyroid examination be included in the follow‐up of long‐term survivors.

Composite cerebral neuroblastoma and astrocytoma. A mixed central neuroepithelial tumor.

This report describes an exceptional example of cerebral neuroblastoma in association with neoplastic glial cells, which were frequently arranged in clusters around the supporting blood vessels. The neuroblastic nature of the tumor was confirmed by the presence of numerous dense‐core vesicles in the cell processes. The glial nature of the associated cells was confirmed by their positivity for glial fibrillary acid protein by immunoperoxidase staining and by the presence of abundant cytoplasmic filaments of intermediate size on electron microscopy. Of particular interest was the presence of dense‐core vesicles in some of the glial cells, and that of a basal lamina underlying the perivascular glial cells. The tumor was interpreted as a composite cerebral neuroblastoma and astrocytoma. No undifferentiated neuroepithelial stem cells could be identified. The cytogenesis of this mixed neuroepithelial neoplasm is uncertain.

Epithelioid Hemangioendothelioma of the Orbital Bones

PURPOSE The authors report a case of an epithelioid hemangioendothelioma arising in the orbital bones. A review of the literature related to this rare orbital neoplasm identified eight well-documented cases, one of which occurred in a patient younger than that reported here, but none of which originated in bone. METHODS A 3 1/2-month-old boy had a left inferior orbital mass that had grown rapidly over a 3-day period. An emergency computed tomography scan showed a large neoplasm with significant bone destruction of the zygoma and maxilla. Initial examination suggested a rhabdomyosarcoma, and a transconjunctival biopsy was performed, which was complicated by significant blood loss. The final pathologic diagnosis was an epithelioid hemangioendothelioma, or grade 2 hemangioendothelioma, of bone origin. No other sites of disease were found on metastatic survey. Subsequent treatment consisted of an en bloc tumor resection sparing the orbital soft tissues and globe. RESULTS The patient is free of disease and has normal visual fixation and ocular motility 20 months after surgery. CONCLUSION Epithelioid hemangioendothelioma, a vascular malignancy of endothelial cell origin, very rarely involves the orbit. This case is notable for its early development, rapid growth, bony origin, and epithelioid histology.

Anesthesia-induced rhabdomyolysis in infants with unsuspected Duchenne dystrophy

Anesthesia‐induced rhabdomyolysis in infancy may represent an unsuspected Duchenne dystrophy. In order to establish the diagnosis of this genetic disease more definitively, a dystrophin test is a requisite following the conventional creatine kinase test and light and electron microscopies of the muscle biopsy.