[Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88]. / Ruolo della chirurgia mutilante nel trattamento dei sarcomi delle parti molli non chemiosensibili in età pediatrica. Esperienza degli studi Cooperativi Italiani RMS-79 ed RMS-88.

UNLABELLED: Aim of the study was to evaluate the role of mutilating surgery in the patients with non chemosensitive soft tissue sarcomas (STS) registered in the Italian Studies. Between 1979 and 1995, 114 patients (pts) with "non chemosensitive" localized STS were enrolled in the cooperative studies RMS-79 (33) and RMS-88 (81). Median age: 89 months (range 1-194). HISTOLOGY: fibrosarcoma 29, Malignant Perpheral Nerve Sheath Tumors (MPNST) 40, malignant fibrous histiocytoma 5, hemangiopericytoma 6, leiomyosarcoma 4, others 20, STS nos 10. The cases were classified according to the IRS staging system as Gr. I 46, Gr. II 24, Gr. III 44. Twelve out of 114 pts (7%), 5/33 (14%) in the first study and 7/81 (8%) in the second, underwent mutilating surgery: 8 pts (of whom 3 were < 2 y of age) had a fibrosarcoma and 4 a MPNST. The mutilating procedure was carried out at diagnosis in 6 cases (4 in RMS-79 and 2 in RMS-and 88) and achieved radicality in 5/6 cases. It was performed after ineffective chemotherapy (CT) in 5 pts (1 in RMS-79 and 4 in RMS-88). One pt (RMS-88) underwent mutilation after multiple local recurrences treated with CT, radiotherapy and conservative excisions (RMS-88) and died. The procedures consisted of 5 amputations, 4 demolitive excisions of extremities with functional impairment, 1 nephrectomy, 1 excision of deferents with spermatic vesicae and 1 mastectomy. OUTCOME: At present 6/12 pts, 5 with fibrosarcoma and 1 with MPNST, are alive with no evidence of disease (NED), 4 of the first and 2 of the second study. Of the 5 Gr. I patients, 4 are alive (NED) and 1 died of 2nd tumor; 1 Gr. II pt is alive NED; of the 5 Gr. III pts 1 is alive NED and 4 died (3 of metastatic spread and 1 of 2nd tumor); the pt amputated after repeated local relapses (Gr. II) is dead of pulmonary metastases. CONCLUSIONS: In the RMS-79 study the mutilations were frequent and were performed at diagnosis in several cases; this trend decreased in the 2nd study in which chemotherapy was attempted in most of the patients. Probably the timing of mutilating procedures was not always adequate (too early or too late). The outcome of pts undergone mutilations is similar to that of the whole group of 114 pts, whose overall survival at 10 years is 64% (C.I. 60-77). Only fibrosarcomas and MPNST probably requires a more aggressive surgical behaviour. At present we avoid initial demolitive surgery; it should be planned without delay after the first local relapse or after initial ineffective CT and RT.

Importance of local treatment in pediatric soft tissue sarcomas with microscopic residual after primary surgery: results of the Italian Cooperative Study RMS-88.

BACKGROUND: The goal of primary excision in soft tissue sarcomas is the complete removal of the tumor by a nonmutilating procedure. However, microscopic residuals may be left after a conservative procedure because of inadequate preoperative assessment or difficulties during the operation. The purpose of this report is to describe the treatment and the outcome in patients, enrolled in the Italian Cooperative Study RMS-88, with microscopic residuals after primary excision (IRS Group IIa). PROCEDURE: Microscopic residuals were evident at histology in 52 of 90 patients who had a macroscopic complete primary excision: 25 rhabdomyosarcomas (RMS) and 27 nonrhabdo-soft tissue sarcomas (NRSTS). Eighteen patients were treated with primary reexcision (PRE) and chemotherapy (CT) using VA or IVA regimens; 27 patients received radiation therapy (RT; 40 Gy) and IVA; 7 children in whom PRE was not feasible and RT could not be administered for age <3 years were treated with CT (IVA) alone. RESULTS: Of the 18 patients who underwent a successful PRE + CT, the local relapses were 3 (16.6%); of 27 cases who had RT + CT there were 4 local relapses (14.8%); 3 local relapses occurred in those 7 patients in whom CT alone was administered (43%). CONCLUSIONS: Microscopic residuals after primary surgery were difficult to manage because of the absence of a measurable target. PRE represented the treatment of choice for children <3 years of age who cannot receive RT and for paratesticular sites. PRE and RT showed similar results in achieving local control in extremity and trunk sites, but they could not always avoid local recurrence. In particular PRE was not effective in tumors larger than 5 cm. If microscopic residuals could not be avoided and PRE was not possible, adequate RT was effective both for RMS and for NRSTS.

[Laparoscopic treatment of varicocele]. / Il trattamento laparoscopico del varicocele.

The Authors present their experience on the treatment of the varicocele; they performed laparoscopic clamping of the internal spermatic vein in 12 subfertile patients, utilizing only the bipolar coagulation without the use of clips. All patients were available for follow-up, which ranged from 6 to 12 months, including physical examination, Doppler C.W. and study of semen quality. Recurrence of varicocele was not observed, the semen quality demonstrated improvement in semen motility in 9 patients (75%) and normal on 3 patients (25%). The use of bipolar coagulation with smaller ports (phi 5 mm) resulted in less postoperative pain and shorter convalescence than when larger ports were used (phi 10 mm). The Authors conclude that the laparoscopic procedure, used as they do, is safe and costs less than classic ligation with clips as well as radiological occlusion procedures.

Inflammatory pseudotumors of the lung in children: conservative or aggressive approach?

BACKGROUND: Inflammatory pseudotumors (inflammatory myofibroblastic tumors) of the lung are myofibroblastic lesions of controversial nosology. The concept that these are benign lesions has been recently questioned given their capacity for local invasion and recurrence. PROCEDURE AND RESULTS: We observed five children with inflammatory pseudotumors of the lung in whom the tumor was resected using the most limited excision possible. Unusually, in all five cases the tumor was localized in the right upper lobe: Three were parenchymal, and the others involved the bronchus. The three parenchymal masses underwent wedge resection, whereas the two bronchial lesions required ex-cision followed by a bronchoplasty and an up-per lobectomy, respectively. In all, the pathology showed a complete resection, and the patients had uneventful recoveries. Follow-ups, including CT and bronchoscopies, ranged from 4 to 8 years (mean of 6.4 years) with no signs of recurrence. CONCLUSIONS: Our limited experience supports the idea that excisional surgery is the treatment of choice for inflammatory pseudotumors of the lung. Such excisions, both diagnostic and curative, carry minimal risks and avoid unnecessary mutilation. Closely monitored follow-up is mandatory, as the natural history of this lesion is not yet well understood.

The safety of home tracheostomy care for children.

Home tracheostomy care avoids prolonged hospitalization for tracheostomized children who must remain cannulated for a long time; however the safety aspects of this form of management are still under discussion. Since 1987 the authors have tutored parents in tracheostomy care. Once parental training was complete and the necessary equipment (aspirator, humidifier, etc) provided, children in stable condition were discharged and had period follow-up as outpatients. Thirty-four children had a total of 710 months in home care; 11 of them had decannulation. The only reported complications were two partial obstructions of the cannula and two accidental decannulations. The low number of documented accidents and the parents' profound appreciation in having their children at home indicate that patients with a tracheostomy in need of long-term care can be safely managed at home.

A simple exam to screen superficial masses: fine-needle aspiration cytology.

Fine-needle aspiration cytology (FNAC) is now considered a useful tool in the evaluation of adult patients with mass lesions. We reviewed the experience with FNAC in our Paediatric Surgical Department. One hundred and eleven FNACs were performed in children with a superficial mass and no definite diagnosis. All the exams were done by the same physician using a fine needle and no anesthesia. We routinely used May-Grüwald-Giemsa and Papanicolaou staining. Patients age ranged from 20 days to 17 years, with a mean age of 6.5 years. A clinically benign pathology was cytologically confirmed in 90 cases (81%). All children did well at follow-up. Malignancy was diagnosed in eight cases (7.2%) and in all was confirmed with a surgical biopsy. In nine children (8.1%) the specimen was considered insufficient for definite diagnosis. In four cases (3.6%) the pathologist diagnosed a possible malignancy that was excluded at surgical biopsy. The sensitivity was 100% and the specificity was 96%. Our experience confirms that FNAC is a fast, cheap, simple, and accurate diagnostic method and should be used for screening in all children with doubtful superficial masses.

Prevalencia de litiasis vesicular en una poblacion uruguaya / Prevalence of gallbladder lithiasis in a Uruguayan population

Con el objetivo de conocer la prevalencia de L.V. en Uruguay, la proporción de sintomáticos y asintomáticos y su asociación a determinados factores de riesgo, se realizaron ecografías a 693 funcionarios del Hospital de Clínicas de Montevideo que concurrieron voluntariamente, previo llenado de un formulario. La prevalencia encontrada fue de 10,4%, de acuerdo al tamaño de la nuestra, extrapolable a la población general con una confianza del 99%. 65% de los portadores de L.V. fueron asintomáticos. Se encontró una asociación estadísticamente significativa con los siguientes factores: grupo etáreo de 31 a 50 años, obesidad leve y AP de tener hijos en caso de mujeres portadoras de la enfermedad. No fue estadísticamente significativo, pero si se comprobó una clara tendencia con los siguientes factores: sexo femenino y AF de madre portadora de L.V. Al considerar todos estos factores en conjunto, la probabilidad de tener una L.V. llegó al 19%. Se discuten los resultados y se comparan con los de publicaciones extranjeras. Se concluye que podía esbozarse un perfil del uruguayo con mayores posibilidades de tener una L.V.: mujer, entre 31 y 50 años, obesa, con hijos y cuya madre tuvo o tiene la misma enfermedad

[Prevalence of gallbladder lithiasis in a Uruguayan population]. / Prevalencia de litiasis vesicular en una población uruguaya.

Ultrasound examinations were carried out in 693 volunteers from the health care personnel of the Hospital de Clinicas of Montevideo, with the aim of studying the prevalence of gallbladder gallstones in Uruguay, the proportion of symptomatic and asymptomatic people and its association to some definite risk factors. The prevalence found was 10.4%, which, according to the sample's size, is representative of the general population with a confidence of 99%. Sixty five per cent of gallstones carriers were asymptomatic. A statistically significant association with the following factors was found: people between 31 and 50 years old, slight obesity, and, for women, to have children. A marked tendency with the following factors was found, though it was not statistically significant: female sex, and a familiar history of mother carrying gallstones. Considering all these factors as a whole, the probability of having gallstones reached 19%. Results are discussed and compared with those of foreign publications. It is concluded that uruguayan people with more possibilities of having gallstones are: women between 31 and 50 years old, obese, with children, and whose mother has or had the same disease.

[Hepato-splenic hemangiosarcoma: presentation of a clinical case]. / Hemangiosarcoma hepato-esplénico: aportación de un caso clínico.

A 65-year-old farmer who had used arsenic as a plaguicide for many years developed a hepatosplenic hemangiosarcoma with metastasis in the colonic serosa, mesentery and omental. The tumor was complicated with intraabdominal hemorrhage originated by spontaneous intraperitoneal rupture. The echographic and post-mortem findings are presented. This is the first case of hepatic hemangiosarcoma reported in Uruguay.

La ecografía en el diagnóstico de la colestasis / The echography in the diagnosis of cholestasis

Para evaluar la utilidad de la ecografía en el diagnóstico de las colestasis, se analizan 32 casos de colestasis estudiados prospectivamente en los que se llegó a confirmar la etiología de la misma. Se comprobó una precisión del 90% en la diferenciación de las diferentes formas de colestasis, una sensibilidad, especificidad y valor predictivo positivo del 84%, 100% y 100% respectivamente en el diagnóstico de obstrucción subhepática. El rendimiento fue menor en el diagnóstico etiológico de las colestasis subhepáticas obteniéndose mejores resultados en obstrucciones neoplásicas que coledocolitiasis. Se comparan todos los resultados con la literatura internacional. Se concluye que la ecografía debe considerarse un procedimiento de primera elección en la etapa no invasiva del estudio de las colestasis, especialmente orientado en la selección de estudios invasivos a utilizarse posteriormente, permitiendo además "por si" el diagnóstico etiológico de un porcentaje aceptable de casos de colestasis subhepáticas