Sickler G K, Langford L A
Department of Pathology, University of Texas Health Science Center, Houston 77225.
Clin Neuropathol. 1990 May-Jun;9(3):125-8.
This is a report of extensive intracranial papillary endothelial hyperplasia in a 12-day-old twin. The infant presented with progressive macrocephaly, anemia and hydrocephalus. An MR image showed a large (6 x 5 cm) mass in the right middle cranial fossa. Histologic examination of the resected mass revealed multifocal hemorrhage, organizing in a floridly papillary pattern, in proximity to numerous fibrous bands containing reactive fibroblasts, scattered chronic inflammatory cells and foci of extramedullary hematopoiesis. Although the pattern was complex, neither the papillary regions nor the other areas in the lesion displayed solid proliferations of endothelial cells to support a diagnosis of angiosarcoma. Subsequent to the resection, the infant continued to have hemostatic abnormalities and increasing hydrocephalus. Repeat scans showed a recurrent mass (4 x 4 cm). The child was placed on a protocol for chemotherapy treatment. We hypothesize that the hemorrhage and subsequent organization could have been superimposed upon a preexistent vascular malformation or hemangioma even though Masson's trichrome stain does not unequivocally demonstrate this feature. Most importantly, we would like to emphasize the dilemmas involved in diagnosis and management of this benign disorder.
这是一篇关于一名12天大双胞胎颅内广泛乳头状内皮增生的报告。该婴儿表现为进行性巨头畸形、贫血和脑积水。磁共振成像显示右侧中颅窝有一个大肿块（6×5厘米）。对切除肿块的组织学检查显示多灶性出血，呈明显的乳头状模式，靠近许多含有反应性成纤维细胞、散在慢性炎症细胞和髓外造血灶的纤维带。尽管模式复杂，但病变中的乳头状区域和其他区域均未显示内皮细胞的实性增生，以支持血管肉瘤的诊断。切除术后，婴儿持续存在止血异常和脑积水加重。复查扫描显示复发肿块（4×4厘米）。该患儿开始接受化疗方案治疗。我们推测，出血及随后的机化可能叠加在先前存在的血管畸形或血管瘤之上，尽管马松三色染色并未明确显示这一特征。最重要的是，我们想强调这种良性疾病在诊断和治疗中所涉及的困境。
