Incidence of reactivation correlates with the stage of the disease at diagnosis. Incidence of sequelae correlates with the occurrence of reactivations.

The authors report their experience with 70 pediatric patients with spinal cord compression (SCC) due to malignancies identified among 898 patients with solid tumors. An extradural tumor was the most frequent cause of SCC (71%); 54% of these were soft tissue sarcomas and neuroblastoma. Most intradural tumors (70%) were outside the spinal cord, 9/12 being metastatic medulloblastomas. The SCC localized mainly to the dorsal and lumbosacral regions (42% each). Pain was the most common symptom (94%). MRI proved diagnostic in all cases in which it was used, while myelography was diagnostic in 85% of 26 patients. CT scan demonstrated the lesion in 83% of the patients. Laminectomy was provided for patients with paraplegia of less than 96 h evolution; isolated recurrence of the main tumor; a primary spinal cord tumor; progression of neurologic symptoms after chemotherapy/radiotherapy; chemotherapy and radiotherapy-resistant tumor (when known); resection of a paraspinal tumor. Surgery was avoided when prognosis of primary disease was poor or risks exceeded possible benefits. Twelve/twenty-one (57%) patients with paraplegia were able to walk after laminectomy only, while 14% (2/14) improved after chemotherapy and radiotherapy. Survival rates were 38% for the former and 36% for the latter. Overall survival was related to the original malignancy. All patients (12) admitted without paraplegia and submitted to laminectomy were able to walk, and of these, 6 presented a primary spinal cord tumor. The remaining had paraspinal tumors that extended to the spinal canal. Almost 87% (20/23) of the patients without paraplegia who submitted to medical treatment were able to walk, while only one progressed to paraplegia. Patients with SCC may entertain radio- and chemotherapy when harboring tumors responding to such therapies and present no evidence of neurologic damage progression. The latter manifestation is a strong indication for laminectomy without delay.

Cytologic diagnosis of peripheral neuroectodermal tumors (PNT) on fine‐needle aspiration (FNA) smears represents a challenge to the cytopathologist. Usually ancillary studies are used to achieve definitive diagnosis. We retrospectively examined FNA material from three cases of PNT with the aim of identifying their features. Positive and negative cytologic findings were recognized. Positive features for PNT included the presence of: rather uniform appearance of the cells, which display scant but almost always‐present perinuclear clear cytoplasm (suggesting a bland epithelial tumor); nuclei with distinctively smooth nuclear membrane contour, finely granular chromatin, and one or two small nucleoli (suggesting neuroendocrine anlage); and organization of the cells singly or in cohesive clusters. Negative findings included the absence of: frequent mitotic figures, large nucleoli, nuclear pleomorphism, cellular debris, histiocytes, and polymorphonuclear leucocytes. The smears appeared clean, with small, uniform cells having features suggesting a neuroendocrine epithelial tumor. These findings may prove useful for accurate cytologic diagnosis and differentiation of PNT from other small blue round cell tumors (SBRCT) of soft tissues without the use of ancillary studies since, when properly evaluated, cytomorphology of the latter group of tumors is more heterogeneous than generally believed. Diagn. Cytopathol. 16:513–517, 1997. © 1997 Wiley‐Liss, Inc.

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The authors retrospectively reviewed their experience with 14 cases of fetal rhabdomyomatous nephroblastoma (15.6% of all renal tumors seen in the 1984-1998 period), analyzing the diagnostic and treatment approach as well outcome. Mean age at presentation was 27 months; 10 (71%) patients were younger than 36 months (5 of these were less than 12 months) at diagnosis. Three patients had bilateral tumors. One case was extrarenal. Nine patients received preoperative chemotherapy after fine-needle aspiration biopsy. The response was null in five children and mild in three; in the remaining one the tumor increased in size. The only patient showing greater than 50% tumor shrinkage was the one treated with radiotherapy. Postoperative treatment according to staging followed the SIOP protocol. In the follow-up (mean 59.5 months) of 12 patients, 6 were in stages 1-2: 4 are alive and free of disease and 2 died (one due to intractable local recurrence and the other in complete clinical remission). All six patients presenting in advanced stages died (four due to disease progression and two due to complications while in complete clinical remission). Patients with fetal rhabdomyomatous nephroblastoma presenting with distant metastatic disease and high stage have unfavorable outcomes, confirming data from the literature indicating that chemotherapy is not as effective as in other variants of nephroblastoma. New protocols are needed to overcome this situation, for which surgery appears the only truly effective option.