Congenital angiosarcoma with transient response to paclitaxel.

Angiosarcoma is a rare diagnosis with a poor prognosis. We report the first known case of congenital angiosarcoma. We also report a transient response with paclitaxel, an agent that may have a role in unresectable angiosarcoma.

Giant cell angioblastoma: three additional occurrences of a distinct pathologic entity.

Giant cell angioblastoma was described previously in a single case report as a congenital soft-tissue tumor with a unique morphology. In the current report, we describe three cases of giant cell angioblastoma found in three infants; one case was congenital and located in the hand, one appeared neonatally in the palate, and one on the scalp of an infant. Clinical findings and results of light microscopy, immunohistochemistry, and electron microscopy were evaluated. All tumors were ulcerated; the hand and palate tumors also infiltrated soft tissue and bone. They exhibited a solid, nodular, and plexiform proliferation of oval-to-spindle cells with a frequent striking, concentric aggregation around small vascular channels. These cells had characteristics of undifferentiated mesenchymal cells, fibroblasts, myofibroblasts, and pericytes. Co-mingled with these cells were large mononuclear and multinucleate giant cells with histiocytic features. The palatal giant cell angioblastoma, excised with positive margins, was managed with interferon-alpha and showed no progression after nearly 5 years. The hand tumor diminished in size after management with interferon-alpha, was subtotally excised, and did not progress after 27 months. Follow-up data are unavailable for the patient with the scalp lesion. Our findings validate the classification of giant cell angioblastoma as a distinct and rare entity that is locally infiltrative but slow growing. The morphology and diverse cellular differentiation are consistent with an unusual form of neoplastic angiogenesis.

Congenital primary cerebral angiosarcoma. Case report.

Reports of angiosarcoma arising in the central nervous system are rare. The authors present the case of a 30-day-old infant with clinical manifestations of projectile vomiting and tense anterior fontanelle resulting from a left frontotemporal tumor. Total excision of this highly vascular, well-circumscribed tumor was performed without incident, and histopathological examination revealed a malignant angiosarcoma. Immunohistochemical reaction of the neoplastic cells was diffusely positive for endothelium-specific antigens including factor VIII-related antigen, CD31, and CD34. The final diagnosis of congenital primary cerebral angiosarcoma was thus confirmed. The patient's postoperative course was uneventful, and he was discharged 2 weeks after the operation. He was in good condition with no sign of recurrence after 11 months; follow-up computerized tomography, magnetic resonance (MR) imaging, and abdominal ultrasonography studies demonstrated no tumor regrowth. The characteristic findings for this tumor on MR imaging, the immunohistochemical findings, and surgical outcome are discussed.

Congenital primary cerebral angiosarcoma: CT, US, and MR findings.

Congenital primary intracranial angiosarcoma (CAS) is an exceptionally rare tumor. To our knowledge the imaging features of intracranial angiosarcomas have only been briefly mentioned in the neuropathologic literature. To our knowledge, only one case of CAS has been reported. We present a case of a pathologically proven CAS found in a neonate.

Congenital, infiltrating giant-cell angioblastoma. A new entity?

This report describes histopathologic, immunohistologic, and ultrastructural features of a locally aggressive soft-tissue tumor present since birth in an upper extremity of an infant. Because of extensive infiltration of local structures, the lesion had to be treated by amputation. The outstanding histologic feature consisted of nodular cell clusters resembling inflammatory granulomas, often with giant, multinucleated cells. On the basis of our findings, these cellular aggregates were interpreted as distorted attempts at formation of vessels. This interpretation was strengthened by the more obviously vascular structure of the tumor in many areas. A high content of stromal cells positive for factor XIIIa and histocompatability antigen (HLA)-DR was a characteristic that the tumor shared in common with angiomatoid malignant fibrous histiocytoma. However, there were also important differences that singularize the tumor described in the present study. We could find no closely comparable precedent for a lesion with these characteristics in current treatises on infantile soft-tissue tumors.

Congenital spinal cord haemangioblastoma: another cause of spinal cord section syndrome in the newborn.

A newborn infant with negative perinatal history and characteristic clinical findings of upper cervical spinal cord section is described. Metrizamide myelography performed on the 7th and 22nd days of life was negative. Peroneal somatosensory evoked responses showed a conduction block at the cervical level. Necropsy revealed a haemangioblastoma extending from levels C1 to C5.

Congenital vascular tumours in the skin of horses.

Congenital vascular tumours in the skin of the legs of two horses showed histological features at biopsy which were consistent with capillary haemangioma but, at necropsy, one tumour had invaded the superficial digital flexor tendon and was therefore classified as a haemangiosarcoma.

[Congenital brain tumors in children under 1 year old]. / Vrozhdennye opukholi golovnogo mozga u detei pervogo goda zhizni.

Secondary, paraventricular and, less frequently, primary tumors of the lateral and third ventricles usually of supratentorial localization prevailed among congenital tumors of the brain. They impair the drainage of cerebrospinal fluid and cause internal hydrocephalus. The clinical picture of congenital cerebral tumors is characterized by a manifest hypertensive-hydrocephalic syndrome and a severe general condition. The neurological symptomatics, the EEG and the Echo-EG usually yielded exact information on internal hydrocephalus but rarely helped in determining the localization and character of the tumor. Pneumoventriculography, angiography, computer tomography, and radioisotope diagnosis were methods which provided the most information. Pneumoventriculography, however, causes many complications and the method of isotope diagnosis and computer tomography is therefore preferable because it is sparing and safe for the use in children of the first year of life.