*Department of Paediatric Surgery, Aristide Le Dantec Hospital, Dakar, Sénégal.
**Department of Orthopaedic and Trauma Surgery, Aristide Le Dantec Hospital,
Dakar, Sénégal.

Address for Correspondence
Dr Gabriel Ngom
BP: 6863 Dakar – Etoile
Phone: 552 – 00 – 80
Email: gngom2004@yahoo.fr

Introduction: The congenital fibrosarcoma is a malignant tumour of the soft tissue. We present a rare case of a congenital fibrosarcoma of the left hand in a newborn baby boy.
Observation: A two days old baby boy was brought to our paediatric surgery department for a hand swelling noticed since birth. The clinical examination noticed a hand tumour involving the palmar and dorsal side with associated homolateral cervical lymph nodes enlargement. The X ray of the hand showed a swelling of the soft tissue with associated cortical thickening of carpal, metacarpal and phalangeal bones. A biopsy was done which reveal a well differentiated congenital fibrosarcoma. A forearm amputation was done and no recurrence was noted after 4 years
Conclusion: The amputation is the only therapeutic means in diffuse forms of congenital fibrosarcoma.
Key words: congenital fibrosarcoma-hand-amputation.

J.Orthopaedics 2006;3(4)e19

Introduction:

The congenital fibrosarcoma is a rare malignant soft tissue tumour. It usually affects the extremities [1]. Few cases involving the hand were reported and a limited number were described during the neonatal period [2]. The diagnosis is histological and microscopically the
aspects are the same as that of and adult, however the prognosis is different. The adult forms give metastasis whiles the children develop locale metastasis. We report a case of congenital fibrosarcoma of the left hand in a 2 days old baby boy managed by forearm amputation and followed up for 4 years without recurrence nor metastasis.

Observation  

C.D. is a 2 days old full term baby boy, born through normal vaginal delivery. The mother attended full antenatal clinic. The birth weight was 3.5kg. He was admitted in the hospital on the 19th September 2001 for a left hand swelling noticed since birth. The clinical examination found a left hand swelling, firm in consistency involving the palmar and dorsal aspect. The skin was tense and shining. There were multiple homolateral axillary lymph nodes enlargement, the biggest of 1 .5cm in diameter. The rest of the physical examination was normal. Standard X ray of the left hand noted soft tissue swelling [Figure 1] and cortical thickening of carpal, metacarpal and phalangeal bones. The chest X ray and the abdominal ultrasonography were normal. The laboratory tests were normal except a prothrombine level of 29 percent managed with vitamine K. On September 29th   2001 a biopsy was done. The results are the following: “we observe a thin epithelium on proliferative tumoral fusiform cells. The cells are made of regular hyperchromic nuclei. Few mitotic activities were noticed. There was no myoblastic differentiation in the cytoplasm. Cells are grouped in homogenous tracts arranged in nodules. In conclusion, it is a mesenchymal fusiform cell tumour, a well differentiate fibrosarcoma”. The management was an amputation of the lower one third of the left forearm associated with axillary lymph nodes excision. The anatomopathology examination of the lymph nodes results was non specific inflammation .The child was followed up for 4 years and neither metastasis nor recurrences was noticed.

Discussion :

The congenital fibro sarcoma is a rare soft tissue malignant tumour. Its incidence is estimated at 9.5 percent of all the congenital tumours and 3 percent of all tumours of the childhood [3]. The tumour elective sites are the distal part of the arms, more on the lower than the upper [1].The tumour developed initially in the soft tissue. A limited number however developed primarily in the bones [4]. The tumour is particularly fast growing. The diagnosis is sometimes made base on a fast growing soft tissue swelling. At times it is discovered late before one year [2]. The standard X ray showed an important soft tissue swelling. It is also possible to find bone abnormalities such us thin cortex. At times bone lysis is noted in 4 percent of cases [5]. The diagnosis is done by the histology examination on view of fusiform cells, at times clustered or arranged in tracts. A large range exists from undifferentiated to well differentiate and slow growing. The differential diagnosis is made with the benign forms of infantile fibromatoses rich in cells and other infantile sarcomas particularly the congenital rhabdomyosarcoma more aggressive [2, 3]. On the management aspects radiotherapy is not advised because of the possible growth disturbances [3]. Chemotherapy may be given to children waiting for surgery. In this situation the aim is to reduce the volume of the tumour, however some authors got good results with chemotherapy alone and so considered as the best treatment for fibrosarcoma [6, 7, 8]. For these authors surgery is indicated only if the arm is saved. Some authors advocate large resection [2]. Amputation is indicated only in the cases of locale recurrence, giant tumour involving bone and neurovascular structures [2]. The prognosis of the congenital fibrosarcoma is at times good unlike that of the adult. The metastasis are rare and seen in the proximal lesions. Recurrences are frequent and affect equally the distal and roots of the arms [1]. In the case of our child the presence of diffuse soft tissue involvement of the hand, and the bony lesions incite us to do a biopsy which revealed a well differentiated congenital fibrosarcoma. The extent of the tumoral growth made us to perform an amputation. That gave us a good result because no metastasis or local recurrence were noted after 4 years however follow up should be longer to look for these evolutives aspects.

Reference :

1. Blocker S, Koenig J, Teraberg J. Congenital fibrosarcoma. J Pediatr Surg 1987;2: 65 – 70.
2. Sah SP, Agrawal CS, Rani S. Congenital infantile fibrosarcoma of the upper extremity.Indian J Pathol Microbiol 2000; 43: 347 – 349.
3. Vinnicombe SJ, Hall CM. Infantile fibrosarsoma: radiological and clinical features. Skeletal Radiol 1994; 23: 337 – 341.
4. Dahlin DC. Infantile fibrosarcoma. Skeletal Radiol 1982; 8: 77 – 78.Chung EB, Enzinger FM. Infantile fibrosarcoma. Cancer 1976; 38: 729 – 739.
5. Kynaston JA, Malcolm AJ, Craft AW, et al. Chemotherapy in the management of infantile fibrosarcoma. Med Pediatr Oncol 1993; 21: 488 – 493.
6. Grier HE, Perrez-Atayde AR, Weinstein HJ. Chemotherapy for inoperable infantile fibrosarcoma. Cancer 1985; 56: 1507 – 1510.
7. Delepine N, Cornille H, Desbois JC, et al. Letter to the editor: Complete response of congenital fibrosarcoma to chemotherapy. The Lancet 1986; 20: 1453 – 1454.
8. Surico G, Muggeo P, Diavicle RM, et al. Chemotherapy alone for the treatment of congenital fibrosarsoma: is surgery always needed? Medical and Paediatric oncology 2003;40: 268 – 270.

This is a peer reviewed paper 

Please cite as : Gabriel Ngom : Congenital hand fibrosarcoma in a child

J.Orthopaedics 2006;3(4)e19

URL: http://www.jortho.org/2006/3/4/e19

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