Malignant hemangioendothelioma: a case of sudden unexpected death in infancy.

A case is reported in which an unsuspected multifocal malignant hemangioendothelioma indirectly caused the sudden, unexpected death of a 5-month-old girl because of its location. To our knowledge, this is the first reported case of sudden, unexpected death associated with this type of tumor. The findings of this unusual case are presented along with a review of relevant literature.

Neurofibromatosis cerebral vasculopathy in an infant: clinical, neuroradiographic, and neuropathologic studies.

An infant with neurofibromatosis developed recurrent cerebrovascular accidents beginning at 3 months of life. Cerebral arteriography demonstrated large-vessel occlusive disease and bilateral severe moyamoya. At autopsy these lesions were apparent on gross examination, and advanced intimal and medial dysplasia was seen on microscopic examination of both the anterior and posterior circulations. Although rare, neurofibromatosis cerebrovasculopathy may occur in young infants and may account for some strokes in this age group.

Liposarcoma arising within a cystosarcoma phyllodes.

A liposarcoma arising within a cystosarcoma phyllodes in a 17-year-old black female was treated by total mastectomy without the need of radiation and/or chemotherapy. The patient is alive and well with no evidence of recurrence or metastasis at 20 months recent follow-up. The clinicopathological features of malignant cystosarcoma phyllodes-liposarcoma are discussed.

Spontaneous expression of fra(11)(q23) in a patient with Ewing's sarcoma and t(11;22)(q23;q11).

Cytogenetic analysis of a Ewing's sarcoma revealed a 46,XX,t(8;18)(q11;q21.3), t(11;22)(q23-24;q11-12) chromosome pattern. Observation of t(11;22) is consistent with other reported cases of Ewing's sarcoma. One breakpoint in this translocation, 11q23, coincides with the location of a folate-sensitive fragile site. Examination of peripheral blood leukocyte chromosomes from the patient revealed a 46,XX chromosome pattern with spontaneous, fluorodeoxyuridine-, and Bactrim-induced expression of fra(11)(q23). This may be the first demonstration of constitutional fra(11)(q23) expression in a patient with a neoplasm that exhibits a chromosome rearrangement involving this breakpoint and the first observation of spontaneous expression of this fragile site. These results provide a basis for discussion of the relationship between fragile sites and chromosome rearrangements.

Bone marrow emboli versus fat emboli as the cause of unexpected death.

Thromboemboli were found diffusely throughout the pulmonary vasculature system of a 76-year-old white female who died unexpectedly 3 days following injury and repair of a left hip intracapsular fracture. A diffuse chronic nonspecific myocarditis with marked fat atrophy, an acute myocardial infarct of the posterior left ventricular papillary muscle, and an acute right lower lobe bronchopneumonia are believed to be the cause of death.

Benign mediastinal teratoma with immature elements exhibited clonal growth and motility in the human tumor clonogenic assay.

The human tumor clonogenic assay has been used to study the growth and drug sensitivity of a wide variety of malignant and a limited number of benign tumors. We conducted detailed studies on a multipotential benign teratoma from a young child that gave rise to colonies in soft agar culture. The colonies separated themselves from the surrounding agar and exhibited rapid rotary movement (15 r/min). Morphologic, ultrastructural, and immunologic studies showed the colonies to be comprised exclusively of ciliated respiratory epithelium. The uniform beating of the cilia resulted in a constant rolling motion of the colonies in a single direction.