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Biomedical subjects

G Perilongo

Publications and source records attributed to G Perilongo.

At least 55 records · Page 3Linked to original sources
PubMed1992

Choroid plexus carcinoma of childhood.

The presentation, growth patterns, and response to therapy of 11 consecutive children with choroid plexus carcinomas were analyzed, and the results were compared with the outcome reported in other series. Patients were a median of 26 months of age at diagnosis. Two patients had thalamic tumors, one had a posterior fossa primary, and the rest had ventricular lesions. Five of 11 (45%) children remain in continuous progression-free remission a median of 48 months from diagnosis. Four of the five in continuous remission had a "gross total" surgical resection, and only one received radiation therapy. Five of six patients with subtotal resections relapsed despite postoperative treatment with radiation therapy (three) and chemotherapy (one). The response to treatment with radiation therapy or chemotherapy at relapse was disappointing, with only one child (treated with etoposide) responding. In combination with other series, 11 of 14 children had prolonged progression-free survival after gross total resection (only two of whom received adjuvant therapy) compared with two of 20 after less than total resections, independent of the type of adjuvant therapy given. Adjuvant therapy for children with choroid plexus carcinomas is of unproven benefit, and this must be considered when analyzing innovative treatment trials for such children, especially for those with totally resected tumors. Patients with partially resected lesions fare poorly with present forms of treatment.

Child↗
PubMed1992

Malignant fibrous histiocytoma of the brain in a six-year-old girl.

We have prepared karyotypes from a malignant fibrous histiocytoma (MFH) of the brain of a 6-year-old girl. Sporadic cases of MFH in the central nervous system have been reported. However, to our knowledge, this is the first central nervous system tumor to be subjected to cytogenetic analysis. The tumor demonstrated a complex karyotype, with a variety of numerical and structural abnormalities. Although no specific cytogenetic abnormality was observed, the karyotype of this case was similar to those reported for adult MFH of soft tissues.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1992

Desmoplastic primitive neuroectodermal tumor with divergent differentiation. Broadening the spectrum of desmoplastic infantile neuroepithelial tumors.

We report an unusual large, multicystic, posterior fossa neuroepithelial neoplasm involving the cerebellum, brain-stem, and quadrigeminal cistern of a 9-month-old girl. The neoplasm consisted of variably sized, sharply demarcated nests of small cells with a high nuclear-cytoplasmic ratio and moderately basophilic nuclei, embedded in a desmoplastic, immature-appearing, mesenchymal stroma. The nests contained mitoses but none were seen in the stroma. Glial fibrillary acidic protein (GFAP), neurofilament protein, synaptophysin, and cytokeratin (AE-1) were expressed in the nests. Mesenchymal cells were negative for neural markers but positive for vimentin and desmin. The neoplasm was interpreted as a mixed mesenchymal and primitive neuroectodermal tumor (PNET) with histologic features reminiscent of a recently described intraabdominal desmoplastic small cell tumor. The tumor responded poorly to chemotherapy and a second operation was performed 1 year later. The second specimen bore no resemblance to the original and consisted of epithelial-like nests and clusters of neoplastic cells frequently interrupted by sinusoidal vessels. Tumor cells had medium-sized vesicular nuclei with small nucleoli, and a granular cytoplasm. Occasional less cellular islands of neuropil-like tissue contained larger cells having eccentric, vesicular nuclei with prominent nucleoli and abundant pink cytoplasm. Mitoses were not conspicuous. Many cells expressed synaptophysin, neurofilament protein, and GFAP. Neurofilament protein was strongly positive in the larger, neuron-like cells and synaptophysin stained the neuropil-like areas strongly but was less prominent in the neuronal perikarya. Unexpectedly, the neuropil-like areas expressed epithelial membrane antigen, whereas the neuronal cells were negative for chromogranin A. The peculiar histologic picture, combination of phenotypic markers, and remarkable biologic behavior of this unusual tumor defies classification according to existing nomenclature and exemplifies the broad range of phenotypes expressed by primitive neuro-epithelial neoplasms.

Brain Neoplasms↗
PubMed1992

Childhood meningiomas. Experience in the modern imaging era.

Twenty children with meningiomas (ages 18 months to 17 years) received initial therapy at the Children's Hospital of Philadelphia between January 1975 and June 1991, accounting for 2% of children with primary brain tumors seen during that time interval. All were verified histopathologically, and none had had prior irradiation. Fifteen were male and 5 female. Fifteen tumors were intracranial, all located supratentorially. Two of these also had a component within the optic canal. One tumor was entirely within the orbit. Four meningiomas arose within the spinal canal. Associated conditions were neurofibromatosis (NF) type I (1 patient), NF type II (2 patients), and a facial alveolar rhabdomyosarcoma (1 patient). A gross total resection as documented by postoperative scan and operative note was accomplished in 12 patients. Four of these relapsed, at a mean of 3.5 years from initial surgery. In 4 patients a near-total resection (> 90%) was performed. Of these, 2 progressed at 9 months and 1.5 years. One of these died of complications associated with reoperation. In 4 patients a partial resection (50-90%) was performed. Two of these progressed at 4 months and 1 year, and the other 2 have been followed for less than 2 years. Five patients received radiation therapy (RT). One patient received RT as adjunctive therapy after primary surgery because of papillary histology. The other 4 had RT following reoperation for recurrence at a mean of 1.5 years from diagnosis (range, 7 months to 2 years). These 4 patients remain alive and with stable disease at a mean of 6 years from diagnosis (range 2-8.8 years).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Whole-brain irradiation and decline in intelligence: the influence of dose and age on IQ score.

PURPOSE: Decline in intelligence can occur after whole-brain cranial irradiation for childhood malignancy. The purpose of this analysis was to estimate better the impact of dose and age at time of irradiation on IQ decline. PATIENTS AND METHODS: A total of 48 children were studied. We combined two previously reported studies that included 15 patients with pediatric acute lymphocytic leukemia (ALL) and 18 pediatric patients with medulloblastoma/posterior fossa primitive neural ectodermal tumors (PNETs) in whom serial IQ tests were administered. Another 15 patients (nine ALL and six PNET) were studied subsequent to these reports. This experience included ALL patients who were treated with whole-brain irradiation at doses of 18 Gy (n = 9) and 24 Gy (n = 15), and PNET patients who were treated with 18 Gy (n = 5), 22 to 24 Gy (n = 2), and 32 to 40 Gy (n = 17). Multiple regression models were constructed to estimate expected IQ score after treatment based on initial IQ score, age at treatment, and dose of whole-brain irradiation. RESULTS: Using a multiple linear regression model to correct for initial IQ and age at treatment, patients who received a dose of 36 Gy to the whole brain were estimated to score 8.2 points less on IQ testing than those with 24 Gy (95% confidence interval [CI], 1.8 to 14.6) and 12.3 points less than those who received 18 Gy (95% CI, 2.7 to 21.7). Older age at the time of irradiation resulted in less decline in subsequent IQ score. The predicted IQ decline is 11.9 points less in a 10-year-old patient than in a 3-year-old patient (95% CI, 4.2 to 19.6) for equivalent doses of irradiation. The model to predict IQ accounts for half the total variation in IQ score. There was no significant difference between the coefficients that reflected IQ decrease from radiation dose between subgroups who had ALL versus those with PNET. CONCLUSIONS: One can forecast final IQ score based on the initial IQ score, dose of irradiation, and age at time of irradiation. Our findings should aid in the selection of appropriate therapy when whole-brain irradiation is needed.

Adolescent↗
PubMed1992

Proton magnetic resonance spectroscopy of pediatric brain tumors.

1H magnetic resonance spectroscopy allows the regional quantitation of a number of metabolites from the brain in a noninvasive fashion. Spectra were obtained from 5 normal children and 25 children with brain tumors. Choline (Cho), N-acetylaspartate (NAA), creatine and phosphocreatine, and lactate were quantitated in the form of ratios. The brains of normal children showed relatively high concentrations of Cho and NAA and virtually no lactate, as has been shown in adults. Benign astrocytomas and ependymomas were characterized by an elevation of the Cho:NAA ratio and an abnormal accumulation of lactate. Intrinsic malignant tumors were remarkable for an even higher Cho:NAA ratio but had no more lactate than was found in the benign tumors. Proton magnetic resonance spectroscopy may prove useful in characterizing neoplastic tissue in conjunction with more conventional imaging modalities.

Adolescent↗
PubMed1991

Hepatocellular carcinoma following chronic delta virus hepatitis in a patient cured of leukemia.

A 20-year-old patient with chronic delta virus hepatitis (CDVH), cured of acute lymphoblastic leukemia (ALL), developed hepatocellular carcinoma (HCC) 14 years after hepatitis B virus (HBV) infection. The association between chronic HBV infection and HCC is well known, but CDVH patients affected by HCC are rarely reported in literature. To our knowledge, the case we describe is the first HCC case reported in literature occurring in a young boy with CDVH. We could expect further similar cases, considering 1) the high prevalence of HDV infection in children affected by ALL in our series, 2) the previous ALL treatment, and 3) a possible natural predisposition to cancer.

Adolescent↗
PubMed1991

Effectiveness and toxicity of cisplatin and doxorubicin (PLADO) in childhood hepatoblastoma and hepatocellular carcinoma: a SIOP pilot study.

Sixteen children, aged 16 days to 13 years with hepatoblastoma (HB) (13 patients) or hepatocellular carcinoma (HCC) (3 patients), were given a total of 89 courses of cisplatin and doxorubicin (PLADO) as IV continuous infusion. All tumors were confined to the liver except for 1 hepatoblastoma patient with pulmonary metastases at presentation. Tumor response to PLADO was evaluable in 10 children (8 HB, 2 HCC) treated with preoperative chemotherapy and in another 2 HB patients treated when they developed pulmonary metastases after initial treatment with surgery alone. There were 2 complete responses (2 HB with pulmonary recurrences), 7 very good partial responses (6 HB and 1 HCC), 2 partial responses (1 HB, 1 HCC), and 1 stable disease (HB). The last patient underwent orthotopic liver transplantation whereas all the other patients had their tumor completely excised at delayed surgery. Documented toxicity was BM depression (16 patients), infection (11), vomiting (11), mucositis (3), hearing loss (1), and cardiotoxicity (1). These data indicate that PLADO in continuous infusion is effective in the treatment of malignant epithelial liver tumors with acceptable toxicity.

Adolescent↗
PubMed1991

Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma.

Between 1975 and 1989, 108 children with newly diagnosed medulloblastoma/primitive neuroectodermal tumor (MB/PNET) of the posterior fossa were treated at the authors' institution. The patients were managed uniformly, and treatment included aggressive surgical resections, postoperative staging evaluations for extent of disease, and craniospinal radiation therapy with a local boost. Beginning in 1983, children with MB/PNET were prospectively assigned to risk groups; those with "standard-risk" MB/PNET were treated with radiation therapy alone, while those in the "poor-risk" group received similar radiation therapy plus adjuvant chemotherapy with 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), vincristine, and cisplatin. The 5-year actuarial disease-free survival rate for all patients treated between 1975 and 1982 was 68%, and 73% when patients who died within 2 weeks after operation were excluded. This survival rate was statistically better for patients treated after 1982 (82%) compared to those treated between 1975 and 1982 (49%) (p less than 0.004). There was no difference in disease-free survival rates over time for children with standard-risk factors; however, there was a significant difference in the 5-year survival rate for poor-risk patients treated prior to 1982 (35%) compared to those treated later (87%) (p less than 0.001). For the group as a whole, a younger age at diagnosis correlated with a poorer survival rate; however, this relationship between age and outcome was significant only for children treated before 1983 (p less than 0.001). These results demonstrated an encouraging survival rate for children with MB/PNET, especially those treated with aggressive surgical resection followed by both radiation therapy and chemotherapy. The results strongly suggest that chemotherapy has a role for some, and possibly all, children with MB/PNET.

Adolescent↗
PubMed1991

Does hepatitis B virus play a role in primary liver cancer in children of Western countries?

Hepatitis B virus (HBV) infection was evaluated in serum and liver specimens of eight Italian children with primary liver cancer. All children were negative for HBV markers in serum but four of them showed HBV-DNA sequences and/or HBs antigen expression in the liver. In one case, viral DNA was present in both neoplastic and non-neoplastic tissue, while in one case HBV-DNA was detectable only in nontumoral tissue and in one case only in the tumor area. In these cases, scattered HBsAg was expressed in the cytoplasm of cells in normal part of the liver and in another case neoplastic cells secreted HBsAg in culture. In two cases, the histologic diagnosis was HCC; one case had mixed HPB and one had macrotrabecular HPB. All children were more than 1 year of age. The remaining four children were histologically diagnosed as HPB and were less than 1 year of age. These findings suggest that HBV may be a cofactor for the development of liver cancer also in children of Western countries and that the risk of infection progressively increases with age.

Carcinoma, Hepatocellular↗
PubMed1989

Hepatoblastoma.

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Carcinoma, Hepatocellular↗
PubMed1989

Oncologic causes of precocious puberty.

Tumors are rare, but well-documented causes of precocious puberty in both sexes. The therapeutic and prognostic implications of a diagnosis of cancer require that the presence of a neoplastic process be ruled out in any case of precocious puberty. Granulosa-cell tumor of the ovary and Leydig-cell tumor of the testis are the most frequent gonadal tumors inducing precocious pseudopuberty in the two sexes. Adrenal tumors sustain a variety of endocrine syndromes, the most frequent one being virilization with or without hypercortisolism. Pure feminizing adrenal neoplasms have been described. For reasons not yet well understood, hypothalamochiasmatic glioma (beta-HCG) secreting tumors have almost never been described in association with female precocious puberty. Among these neoplasia, pineal germ-cell tumor inducing sexual maturation must be included. Hypothalamochiasmatic glioma and craniopharyngioma are the two cerebral tumors capable of inducing true precocious puberty. Even if equally distributed between both sexes, these tumors interfere with sexual maturation less frequently in girls than in boys. Hypothalamic hamartoma is considered a benign tumor, since it does represent a space-occupying mass. It more correctly could be called a malformation if its histologic characteristics are recalled. This cerebral lesion is now frequently described in children with true precocious puberty, probably because of improved diagnostic imaging methods.

Child↗