In A Child
Charles Diémé**, Mbaye Fall*,
Anne Aurore Sankalé*, Ibrahima Fall*,
Paediatric Surgery, Aristide Le Dantec Hospital, Dakar, Sénégal.
**Department of Orthopaedic and Trauma
Surgery, Aristide Le Dantec Hospital,
Address for Correspondence
Dr Gabriel Ngom
BP: 6863 Dakar – Etoile
Phone: 552 – 00 – 80
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.
The congenital fibrosarcoma is a rare
malignant soft tissue tumour. It usually affects the extremities
. Few cases involving the hand were reported and a limited
number were described during the neonatal period . 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.
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.
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 . The tumour elective sites are the
distal part of the arms, more on the lower than the upper
.The tumour developed initially in the soft tissue. A limited
number however developed primarily in the bones . 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 . 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 . 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 .
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 . Amputation is
indicated only in the cases of locale recurrence, giant tumour
involving bone and neurovascular structures . 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 . 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.
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This is a peer reviewed paper
Please cite as
Gabriel Ngom : Congenital
hand fibrosarcoma in a child