Congenital Fibrosarcoma of The Chest Wall: Report of a Case

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Abstract

Congenital fibrosarcoma (CFS) is a rare soft tissue tumour that usually occurs before the age of 1, and involves the distal extremities. The literature regarding the precise diagnosis and treatment of these tumours is limited. We present and discuss a case of CFS which ended fatally due to lung metastasis after 2 years.

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Most cited references 26

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Infantile fibrosarcoma.

Katherine M Enzinger, Arlene Chung (1976)

The pathologic features and the behavior of 53 cases of infantile fibrosarcoma are presented. The tumor nearly always occurred during the first 2 years of life; 41 of the 53 cases occurred during the first, six during the second, and three each during the third and fourth year of life, respectively. Twenty of the 53 cases were present at birth. The tumor was more common in boys (60%) and affected chiefly the distal portions of the lower and upper extremities (72%). Most of the tumors grew rapidly and reached a large size within a few weeks or months. They were generally poorly circumscribed and infiltrated variously subcutaneous fat, muscle, fascia and tendons. Microscopically, they were composed of immature-appearing spindle-shaped cells and were marked by their high cellularity and prominent mitotic activity. Despite these features, follow-up information revealed a favorable clinical course, particularly as compared with the adult form of fibrosarcoma. Of the 48 patients with follow-up data, 31 were alive and well with no evidence of recurrence, eight were alive with recurrence, and one was alive following lobectomy for metastatic tumor. Of the living patients, 12 were treated with amputation, nine with radical or wide local excision, and 15 with simple excision. In two cases surgery was followed by chemotherapy, and in one, by radiotherapy. Eight of the 48 patients with follow-up had died, four of metastatic tumor (8.3%) and four of miscellaneous causes. Wide local excision appears to be the treatment of choice unless the size of the tumor and its anatomic location require amputation. Since late recurrent and metastatic lesions were encountered, long-term followup is necessary before one can safely assume that the patient has been cured.

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Fibrosarcoma in infants and children: a review of 110 cases.

John Pritchard, E Soule (1977)

This retrospective study summarizes the clinicopathologic findings in 110 children with fibrosarcoma. Seventy cases were selected from previous reports and 40 were from our files. The histologic criteria used for diagnosis are the same as those used for adults. Sixty-eight patients were in the first quinquennium of life, 13 in the second, and 29 in the third. Eleven patients died of their tumor, three in the first quinquennium and eight in ght third. Our findings indicate that children who are less than 5 years old have a 7.3% chance of developing metastatic spread even though the local recurrence rate is 43%. Children who are 10 years old or older had a metastatic rate of 50% at 5-year follow-up, a figure closely approximating that for adults. We believe that infants and children less than 5 years old may be treated initially by local excision of the lesion, without sacrificing significant function of the part. Recurrent tumor does not seem to predispose to metastatic disease.

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Congenital fibrosarcoma.

J Ternberg, Y Blocker, J. Koenig (1987)

Congenital fibrosarcoma is a rare fibrous tissue malignancy occurring most commonly in the extremities (71%), but presenting in axial locations as well (29%). Two of our five patients had lower extremity tumors and three had axial lesions located in the thoracolumbar region and anterior neck. Amputation was considered necessary to treat the extremity tumors. Two of the patients with axial lesions had incomplete resection, which raised the question of adjunctive therapy and prompted this review. Forty-seven additional cases of fibrosarcoma, present at birth or diagnosed within the first 3 months of life, were adequately described and documented in the literature giving a total of 52 cases. There was no sex predominance (male 26, female 20, unknown 6). Thirty-seven (71%) of the patients had extremity lesions. The local recurrence rate for extremity tumors was 32% with metastatic and mortality rates of 8% and 5%, respectively. Axial tumors (15 cases, 29%) had a local recurrence rate of 33%, which is similar to that for extremity lesions, but the metatstatic and mortality rates were 26% each indicating a more aggressive behavior for axial lesions. The overall mortality rate of 11.5% and metastatic rate of 13.5% for congenital fibrosarcoma, are not dissimilar to the figures quoted for older children. This pattern of tumor behavior, therefore, does not seem to warrant a different attitude because of the "congenital" occurrence of the tumor. Surgery must be considered the mainstay of therapy for fibrosarcoma, but there is a need for adjunctive therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

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Journal

Journal ID (nlm-ta): J Cutan Aesthet Surg

Journal ID (publisher-id): JCAS

Title: Journal of Cutaneous and Aesthetic Surgery

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 0974-2077

ISSN (Electronic): 0974-5157

Publication date (Print): Sep-Dec 2010

Volume: 3

Issue: 3

Pages: 177-180

Affiliations

[1]Department of Surgery, MGM Medical College and MY Hospital, Indore, Madhya Pradesh, India

[1 ]Division of Pediatric Surgery, MGM Medical College and MY Hospital, Indore, Madhya Pradesh, India

Author notes

Address for correspondence: Dr. Shilpi Singh Gupta, VPO Sangowal, Nakodar, Jalandhar - 144 041, Punjab, India. drguptashilpi@ 123456gmail.com

Article

Publisher ID: JCAS-3-177

DOI: 10.4103/0974-2077.74497

PMC ID: 3047738

PubMed ID: 21430833

SO-VID: 90955ea4-7167-488c-ae9b-1b959828ac54

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This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Congenital Fibrosarcoma of The Chest Wall: Report of a Case

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Most cited references 26

Infantile fibrosarcoma.

Fibrosarcoma in infants and children: a review of 110 cases.

Congenital fibrosarcoma.

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