Neonatal soft tissue sarcomas: The influence of pathology on treatment and survival

Dillon, Peter W.; Whalen, Thomas V.; Azizkhan, Richard G.; Haase, Gerald M.; Coran, Arnold G.; King, Denis R.; Smith, Mark

doi:10.1016/0022-3468(95)90337-2

Cited by 45 publications

(31 citation statements)

References 22 publications

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“…Some authors consider as infantile ®brosarcomas those forms diagnosed before 4±5 years of age [5,10,11,13], while others accept only those cases within 2 years [3,22]. In a group of 12 neonatal ®brosarcomas, the outcome was favorable in all the patients: 10 had had a complete surgery and only 1 received postoperative CT [23].…”

Section: Discussionmentioning

confidence: 99%

Fibrosarcoma in pediatric patients: Results of the Italian Cooperative Group studies (1979–1995)

Cecchetto

Carli

Alaggio

et al. 2001

Journal of Surgical Oncology

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Background and ObjectivesFibrosarcoma is a rare soft tissue sarcoma (STS) that has two peaks of incidence in pediatric patients: one in infants and young childern (infantile fibrosarcoma), another in older children (“adult type” fibrosarcoma). The purpose of this study was to describe the clinical features and the treatment results in patients affected by fibrosarcoma enrolled in two consecutive studies run by the STS‐Italian Cooperative Group (ICG) between 1979 and 1995.Patients and MethodsTwenty‐five evaluable patients were grouped according the intergroup rhabdomyosarcoma staging (IRS) system: 12 Gr I, 5 Gr II, 8 Gr III. The cut‐off point between the two forms was considered the age of 2 years: 14 patients were affected by infantile and 11 by adult type of fibrosarcoma. The therapeutic guidelines were not strict and not different for the two forms: patients with initial macroscopic excision (Gr I–II) were given IVA or VAC; Gr III had VAC or VAIA and subsequent excision, if feasible. Radiation therapy (RT) was delivered in patients > 3 years with microscopic (42 Gy) and macroscopic (54 Gy) residuals.ResultsThirteen patients are alive without evidence of disease: 10 in first complete remission (CR), 7 Gr I, 1 Gr II, 2 Gr III, 3 in second CR after local relapse (LR) (2 Gr I, 1 Gr II). Two Gr I patients are alive with metastatic relapse. Nine patients died: six of progressive disease (PD) (three Gr II, three Gr III), two because of a second tumor (one Gr I, one Gr III) and one because of other causes (Gr III). One Gr III patient was lost at follow up, 2 years from diagnosis. The 10 years overall survival (OS) is 67.4% and the progression‐free survival (PFS) is 52.2%. The OS for patients < 2 years is 78.6% (11 of 14), versus 51% for patients > 2 years (5 of 11).ConclusionsThe complete excision at diagnosis was the treatment of choice and was related to the best outcome. Microscopical residuals were difficult to treat with chemo‐radiotherapy in both forms of fibrosarcoma. Neoadjuvant chemotherapy (CT) obtained a partial remission (PR) only in three of eight cases, while no conclusions concerning the efficacy of CT for infantile forms are possible. Patients < 2 years had a better outcome than the older ones: most of them had a tumor on extremities which was excised at diagnosis. J. Surg. Oncol. 2001;78:225–231. © 2001 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 99%

Fibrosarcoma in pediatric patients: Results of the Italian Cooperative Group studies (1979–1995)

Cecchetto

Carli

Alaggio

et al. 2001

Journal of Surgical Oncology

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“…Metastases occur in less than 10% of cases, but local tumor control can be difficult after initial therapy, with reported recurrence rates varying from 17 to 43%. Prognosis does not appear to be affected by tumor recurrence, and the 5-year survival rate is up to 84% [7,8]. In general, despite rapid growth and aggressive clinical behavior, most fibrosarcomas are cured by wide local excision and adjuvant chemotherapy, and have a better prognosis than adult-type fibrosarcoma.…”

Section: Discussionmentioning

confidence: 98%

“…As most infantile fibrosarcoma involves the extremities, it is easily detected and treated. Dillon et al [7] reported the outcome in a series of patients with fibrosarcoma in the abdomen. Compared with fibrosarcoma occurring in the extremities, tumors in an axial location have a similar local recurrence rate, but the metastasis and mortality rates indicate a more aggressive behavior for axial lesions [7].…”

Section: Discussionmentioning

confidence: 99%

“…Dillon et al [7] reported the outcome in a series of patients with fibrosarcoma in the abdomen. Compared with fibrosarcoma occurring in the extremities, tumors in an axial location have a similar local recurrence rate, but the metastasis and mortality rates indicate a more aggressive behavior for axial lesions [7]. The neonates who received either complete or incomplete intraabdominal tumor resection followed by adjuvant chemotherapy and were alive at a mean follow-up of 6.6 years.…”

Section: Discussionmentioning

confidence: 99%

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Combined Prenatal Ultrasound and Magnetic Resonance Imaging in an Extensive Congenital Fibrosarcoma: A Case Report and Review of the Literature

Huang¹,

Wang²,

Wang

et al. 2005

Fetal Diagn Ther

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Objective: Tumors of congenital origin differ in their symptoms and signs, predominant location and prognosis from those occurred later in childhood. To determine whether the prognosis of congenital (infantile) fibrosarcoma in the retroperitoneum, an extremely rare form of soft tissue malignant tumor, can be assessed prenatally. Methods: We describe a case of this condition diagnosed in utero, including the assessment of the tumor behavior by ultrasound and magnetic resonance imaging. We also review the English language literature in Medline over the past 10 years and describe the management options available in such cases. Results: Our case had precise prenatal findings revealed unfavorable conditions such as bulky tumor mass, spinal metastasis and pulmonary hypoplasia. Conclusion: Fetal imaging studies could accurately identify the invasion of tumor. The outcome is usually poor, especially for those fetuses affected by an axial location of the tumor.

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“…In the review by the multi-institutional Children's Cancer Group, only 2 of 11 neonates were long-term survivors and 1 was a bladder primary treated with cystectomy and chemotherapy [21]. This limited experience would suggest that more extensive resectional surgery in pelvic RMS in the neonate is warranted.…”

Section: Vulvar Rhabdomyosarcomamentioning

confidence: 97%

Pelvic neoplasms in children

Groff

2001

Journal of Surgical Oncology

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The pelvis of the infant and child has different anatomic relationships than the adolescent or adult pelvis, and the knowledge of congenital anomalies of the rectum and bladder can assist in treating the primary neoplasms of the pelvis. The most common neoplasms are rhabdomyosarcomas of the bladder, prostate, and vagina; sacrococcygeal teratoma; and the germ cell tumors, including teratomas, endodermal sinus tumors, and the choriocarcinomas. Rapidly improving chemotherapy for all of these lesions has resulted in a changed role for the surgeon. Less radical resection of these tumors is being performed whereas it is necessary to have more precise histologic and genetic identification of the tumor and the specific anatomic location and extent of the tumor in the pelvis. Survival for all types of childhood pelvic neoplasms has improved dramatically under the influence of the combined children's cancer study groups, which have been functioning for the last 20 years. Because of the complexities of diagnosis and treatment of these pelvic neoplasms in childhood and because the survival rates have dramatically improved with the most current therapy, these children should be cared for in a children's center that is part of the major children's cancer study groups.

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Neonatal soft tissue sarcomas: The influence of pathology on treatment and survival

Cited by 45 publications

References 22 publications

Fibrosarcoma in pediatric patients: Results of the Italian Cooperative Group studies (1979–1995)

Fibrosarcoma in pediatric patients: Results of the Italian Cooperative Group studies (1979–1995)

Combined Prenatal Ultrasound and Magnetic Resonance Imaging in an Extensive Congenital Fibrosarcoma: A Case Report and Review of the Literature

Pelvic neoplasms in children

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